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Sample records for congenital unilateral hydrocephalus

  1. Prenatal diagnosis of congenital unilateral hydrocephalus

    International Nuclear Information System (INIS)

    Koga, Yasutsugn; Tahara, Yasuo; Kida, Takeshi; Matumoto, Yoshinori; Negishi, Hiroaki; Fujimoto, Seiichiro

    1997-01-01

    A case is presented in which fetal unilateral hydrocephalus that had not been definitively diagnosed by ultrasonography was confirmed by means of magnetic resonance imaging. Computed tomography performed in the neonatal period after intraventricular injection of contrast medium showed stenosis of the foramen of Monro. (orig.). With 1 fig

  2. Congenital Hydrocephalus.

    Science.gov (United States)

    Estey, Chelsie M

    2016-03-01

    There are several types of hydrocephalus, which are characterized based on the location of the cerebrospinal fluid (CSF) accumulation. Physical features of animals with congenital hydrocephalus may include a dome-shaped skull, persistent fontanelle, and bilateral ventrolateral strabismus. Medical therapy involves decreasing the production of CSF. The most common surgical treatment is placement of a ventriculoperitoneal shunt. Postoperative complications may include infection, blockage, drainage abnormalities, and mechanical failure. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. [Congenital hydrocephalus].

    Science.gov (United States)

    Malagón-Valdez, J

    2006-04-10

    Congenital hydrocephalus or ventriculomegaly is a disorder that now can be diagnosed in uterus with ultrasonography, this gives the chance of being able to give a treatment the earliest as possible. The clinical manifestations are reviewed, the diagnosis, the frequent treatment and causes of congenital hydrocephalus, being the first agenesis of the Sylvius' aqueduct, followed by Arnold-Chiari's malformations with mielomeningocele. In most of the cases the peritoneal-ventricle shunt is the best surgery treatment and now, the treatment with ventriculostomy of third ventricle by endoscopy has fewer complications apparently and in several cases it is the definitive treatment. The evolution of the diagnosis with the support of specific therapies is effective and the early treatment is good, of course taking into account the etiology.

  4. Congenital hydrocephalus in clinical practice : A genetic diagnostic approach

    NARCIS (Netherlands)

    Verhagen, J. M. A.; Schrander-Stumpel, C. T. R. M.; Krapels, P. C.; de Die-Smulders, C. E. M.; van Lint, F. H. M.; Willekes, C.; Weber, J. W.; Gavilanes, A. W. D.; Macville, M. V. E.; Stegmann, A. P. A.; Engelen, J. J. M.; Bakker, J.; Vos, Y. J.; Frints, S. G. M.

    2011-01-01

    Congenital hydrocephalus is a common and often disabling disorder. The etiology is very heterogeneous. Little is known about the genetic causes of congenital hydrocephalus. A retrospective survey was performed including patients with primary congenital hydrocephalus referred to the Department of

  5. The genetic landscape of familial congenital hydrocephalus.

    Science.gov (United States)

    Shaheen, Ranad; Sebai, Mohammed Adeeb; Patel, Nisha; Ewida, Nour; Kurdi, Wesam; Altweijri, Ikhlass; Sogaty, Sameera; Almardawi, Elham; Seidahmed, Mohammed Zain; Alnemri, Abdulrahman; Madirevula, Sateesh; Ibrahim, Niema; Abdulwahab, Firdous; Hashem, Mais; Al-Sheddi, Tarfa; Alomar, Rana; Alobeid, Eman; Sallout, Bahauddin; AlBaqawi, Badi; AlAali, Wajeih; Ajaji, Nouf; Lesmana, Harry; Hopkin, Robert J; Dupuis, Lucie; Mendoza-Londono, Roberto; Al Rukban, Hadeel; Yoon, Grace; Faqeih, Eissa; Alkuraya, Fowzan S

    2017-06-01

    Congenital hydrocephalus is an important birth defect, the genetics of which remains incompletely understood. To date, only 4 genes are known to cause Mendelian diseases in which congenital hydrocephalus is the main or sole clinical feature, 2 X-linked (L1CAM and AP1S2) and 2 autosomal recessive (CCDC88C and MPDZ). In this study, we aimed to determine the genetic etiology of familial congenital hydrocephalus with the assumption that these cases represent Mendelian forms of the disease. Exome sequencing combined, where applicable, with positional mapping. We identified a likely causal mutation in the majority of these families (21 of 27, 78%), spanning 16 genes, none of which is X-linked. Ciliopathies and dystroglycanopathies were the most common etiologies of congenital hydrocephalus in our cohort (19% and 26%, respectively). In 1 family with 4 affected members, we identified a homozygous truncating variant in EML1, which we propose as a novel cause of congenital hydrocephalus in addition to its suggested role in cortical malformation. Similarly, we show that recessive mutations in WDR81, previously linked to cerebellar ataxia, mental retardation, and disequilibrium syndrome 2, cause severe congenital hydrocephalus. Furthermore, we confirm the previously reported candidacy of MPDZ by presenting a phenotypic spectrum of congenital hydrocephalus associated with 5 recessive alleles. Our study highlights the importance of recessive mutations in familial congenital hydrocephalus and expands the locus heterogeneity of this condition. Ann Neurol 2017;81:890-897. © 2017 American Neurological Association.

  6. Risk factors for congenital hydrocephalus

    DEFF Research Database (Denmark)

    Munch, Tina Noergaard; Rasmussen, Marie-Louise Hee; Wohlfahrt, Jan

    2014-01-01

    OBJECTIVES: To investigate the associations between isolated congenital hydrocephalus (CHC) and maternal characteristics, maternal medical diseases, and medicine intake during pregnancy as well as birth characteristics of the child in a retrospective, register-based, nationwide cohort study...... of medicine during pregnancy from the National Prescription Drug Register, and birth characteristics of the child from the Danish National Birth Register. Rate ratios (RR) of isolated and syndromic CHC with 95% CI were estimated using log-linear Poisson regression. RESULTS: In a cohort of 1928666 live...

  7. Severe hydrocephalus associated with congenital varicella syndrome

    OpenAIRE

    Mazzella, Massimo; Arioni, Cesare; Bellini, Carlo; Allegri, Anna Elsa Maria; Savioli, Cesarina; Serra, Giovanni

    2003-01-01

    CONGENITAL VARICELLA SYNDROME refers to the spectrum of fetal anomalies associated with maternal varicella zoster virus (VZV) infection during the first trimester of pregnancy. The syndrome is rare and the risk to the fetus uncertain. We describe an unusual case of congenital varicella syndrome in which hydrocephalus was the main consequence and likely represented VZV reactivation in utero.

  8. Hydrocephalus

    Science.gov (United States)

    Hydrocephalus is the buildup of too much cerebrospinal fluid in the brain. Normally, this fluid cushions your ... though, it puts harmful pressure on your brain. Hydrocephalus can be congenital, or present at birth. Causes ...

  9. Familial aggregation of congenital hydrocephalus in a nationwide cohort

    DEFF Research Database (Denmark)

    Munch, Tina Nørgaard; Rostgaard, Klaus; Rasmussen, Marie-Louise Hee

    2012-01-01

    The objective of the study was to investigate familial aggregation of primary congenital hydrocephalus in an unselected, nationwide population. Based on the Danish Central Person Register, we identified all children born in Denmark between 1978 and 2008 and their family members (up to third......-degree relatives). Information on primary congenital hydrocephalus was obtained from the National Patient Discharge Register. Using binomial log-linear regression, we estimated recurrence risk ratios of congenital hydrocephalus. An alternative log-linear regression model was applied to quantify the genetic effect...... and the maternal effect. Of 1 928 683 live-born children, 2194 had a diagnosis of idiopathic congenital hydrocephalus (1.1/1000). Of those, 75 (3.4%) had at least one other family member with primary congenital hydrocephalus. Significantly increased recurrence risk ratios of primary congenital hydrocephalus were...

  10. Congenital intracerebral teratoma: a rare differential diagnosis in newborn hydrocephalus

    Energy Technology Data Exchange (ETDEWEB)

    Storr, U. [Landratsamt Neuburg-Schrobenhausen, Gesundheitsamt, Neuburg an der Donau (Germany)]|[Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany); Rupprecht, T. [Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany); Bornemann, A. [Inst. for General Pathology, Erlangen-Nuernberg Univ., Nuernberg (Germany); Ries, M. [Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany); Beinder, E. [Dept. of Obstetrics and Gynecology, Erlangen-Nuernberg Univ., Nuernberg (Germany); Boewing, B. [Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany); Harms, D. [Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany)

    1997-03-01

    Cogenital hydrocephalus is caused by a broad spectrum of underlying disorders. In the majority of cases it is due to aqueductal stenosis and other distinct congenital anomalies, like Arnold-Chiari malformation. Nevertheless, in the differential diagnosis rare conditions such as cerebral malignancies must also be considered. We present two cases of congenital intracerebral teratoma as a differential diagnosis in congenital obstructive hydrocephalus. A teratoma is suggested when a rapidly growing hydrocephalus with a central calcified and vascularized mass is found sonographically. Regular cerebral structures using cannot be detected. Early diagnosis in such cases is of clinical importance as the prognosis of congential intracerebral teratoma is generally very poor. (orig.)

  11. Hereditary congenital unilateral deafness : A new disorder?

    NARCIS (Netherlands)

    Dikkers, FG; Verheij, JBGM; van Mechelen, M

    Congenital unilateral deafness is a rare disorder. The prevalence rates are unknown. The prevalence of children with severe to profound hearing losses that are congenital (or acquired before the development of speech and language) is 0.5 to 3 per 1,000 live births. Evidently, congenital unilateral

  12. Cerebrospinal fluid biomarkers of infantile congenital hydrocephalus

    Science.gov (United States)

    Limbrick, David D.; Baksh, Brandon; Morgan, Clinton D.; Habiyaremye, Gakwaya; McAllister, James P.; Inder, Terrie E.; Mercer, Deanna; Holtzman, David M.; Strahle, Jennifer; Wallendorf, Michael J.; Morales, Diego M.

    2017-01-01

    Introduction Hydrocephalus is a complex neurological disorder with a pervasive impact on the central nervous system. Previous work has demonstrated derangements in the biochemical profile of cerebrospinal fluid (CSF) in hydrocephalus, particularly in infants and children, in whom neurodevelopment is progressing in parallel with concomitant neurological injury. The objective of this study was to examine the CSF of children with congenital hydrocephalus (CHC) to gain insight into the pathophysiology of hydrocephalus and identify candidate biomarkers of CHC with potential diagnostic and therapeutic value. Methods CSF levels of amyloid precursor protein (APP) and derivative isoforms (sAPPα, sAPPβ, Aβ42), tau, phosphorylated tau (pTau), L1CAM, NCAM-1, aquaporin 4 (AQP4), and total protein (TP) were measured by ELISA in 20 children with CHC. Two comparative groups were included: age-matched controls and children with other neurological diseases. Demographic parameters, ventricular frontal-occipital horn ratio, associated brain malformations, genetic alterations, and surgical treatments were recorded. Logistic regression analysis and receiver operating characteristic curves were used to examine the association of each CSF protein with CHC. Results CSF levels of APP, sAPPα, sAPPβ, Aβ42, tau, pTau, L1CAM, and NCAM-1 but not AQP4 or TP were increased in untreated CHC. CSF TP and normalized L1CAM levels were associated with FOR in CHC subjects, while normalized CSF tau levels were associated with FOR in control subjects. Predictive ability for CHC was strongest for sAPPα, especially in subjects ≤12 months of age (p<0.0001 and AUC = 0.99), followed by normalized sAPPβ (p = 0.0001, AUC = 0.95), tau, APP, and L1CAM. Among subjects ≤12 months, a normalized CSF sAPPα cut-point of 0.41 provided the best prediction of CHC (odds ratio = 528, sensitivity = 0.94, specificity = 0.97); these infants were 32 times more likely to have CHC. Conclusions CSF proteins such as s

  13. Eyelid Retraction in Isolated Unilateral Congenital Blepharoptosis

    OpenAIRE

    Michael S. Salman; Michael S. Salman; Ian H. Clark; Ian H. Clark

    2017-01-01

    Isolated unilateral congenital ptosis is encountered relatively infrequently in clinical practice. It typically consists of a unilateral droopy eyelid, weak levator palpebrae superioris muscle function, lid lag, and an absent upper lid crease with no other abnormalities on examination. We present a four-and-a-half-year-old girl with isolated and mild unilateral congenital ptosis who unexpectedly demonstrated a static upper eyelid on downgaze in conjunction with a well-formed upper lid skin cr...

  14. Hydrocephalus caused by unilateral foramen of Monro obstruction: A review on terminology

    Science.gov (United States)

    Nigri, Flavio; Gobbi, Gabriel Neffa; da Costa Ferreira Pinto, Pedro Henrique; Simões, Elington Lannes; Caparelli-Daquer, Egas Moniz

    2016-01-01

    Background: Hydrocephalus caused by unilateral foramen of Monro (FM) obstruction has been referred to in literature by many different terminologies. Precise terminology describing hydrocephalus confined to just one lateral ventricle has a very important prognostic value and determines whether or not the patient can be shunt free after an endoscopic procedure. Methods: Aiming to define the best term for unilateral FM obstruction, 19 terms were employed on PubMed database (http://www.ncbi.nlm.nih.gov/pubmed) as quoted phrases. Results: A total of 194 articles were found. Four patterns of hydrocephalus were discriminated as a result of our research term query and were divided by types for didactic purpose. Type A - partial dilation of the lateral ventricle; Type B - pure unilateral obstruction of the FM; Type C - previously shunted patients with secondary obstruction of the FM; and Type D - asymmetric lateral ventricles with patent FM. Conclusion: In unilateral FM obstruction hydrocephalus, an in-depth review on terminology application is critical to avoid mistakes that may compromise comparisons among different series. This terminology review suggests that Type B hydrocephalus, i.e., the hydrocephalus confined to just one lateral ventricle with no other sites of cerebrospinal fluid circulation blockage, are best described by the terms unilateral hydrocephalus (UH) and monoventricular hydrocephalus, the first being by far the most popular. Type A hydrocephalus is best represented in the literature by the terms uniloculated hydrocephalus and loculated ventricle; Type C hydrocephalus by the terms isolated lateral ventricle and isolated UH; and Type D hydrocephalus by the term asymmetric hydrocephalus. PMID:27274402

  15. Congenital Hydrocephalus in mono and dizygotic twins | Idowu ...

    African Journals Online (AJOL)

    Background: Information regarding the occurrence of hydrocephalus (HC) in twins is important in establishing the significance of environmental factors as well as a genetic basis in congenital HC aetiology. This was the basis for this study. Methods: A single institution retrospective study was conducted between August 1, ...

  16. Eyelid Retraction in Isolated Unilateral Congenital Blepharoptosis

    Directory of Open Access Journals (Sweden)

    Michael S. Salman

    2017-05-01

    Full Text Available Isolated unilateral congenital ptosis is encountered relatively infrequently in clinical practice. It typically consists of a unilateral droopy eyelid, weak levator palpebrae superioris muscle function, lid lag, and an absent upper lid crease with no other abnormalities on examination. We present a four-and-a-half-year-old girl with isolated and mild unilateral congenital ptosis who unexpectedly demonstrated a static upper eyelid on downgaze in conjunction with a well-formed upper lid skin crease. We attribute this uncommon sign in congenital ptosis to stiffness and presumed fibrosis of the levator muscle. Examining the function of the eyelids in all directions of gaze is important in patients with abnormalities of lid position, since additional useful information can be gleaned about the status of the levator muscle including, aberrant regeneration or fibrosis.

  17. Unilateral straight hair and congenital horner syndrome.

    Science.gov (United States)

    Wang, Frederick M; Wertenbaker, Christian; Cho, Hyung; Marmor, Maury A; Ahn-Lee, Sandra S; Bernard, Bruno A

    2012-06-01

    Congenital Horner syndrome is a rare disorder that accounts for less than 5% of all cases of Horner syndrome. Like Horner syndrome in general, it consists primarily of ptosis, miosis, and anhidrosis. Congenital Horner syndrome may manifest some special features such as iris heterochromia since the sympathetic nervous system is an essential component for the development and maintenance of eye color. We present 3 cases of unilateral straight hair in association with congenital Horner syndrome in which the patients had straight hair ipsilateral to the Horner syndrome, whereas on the contralateral side, it was curly, and we discuss possible mechanisms for this phenomenon.

  18. Giant unusual shaped chronic subdural hematoma in a patient with untreated congenital hydrocephalus

    OpenAIRE

    Mishra, Arvind; Ojha, Bal. K.; Chandra, Anil; Srivastava, Chhitij; Singh, Sunil Kumar

    2011-01-01

    Subdural hematoma is a well known complication of ventriculoperitoneal shunt insertion for hydrocephalus and usually spreads out over the cerebral convexity, and appears as a crescent shaped lesion on imaging. Chronic subdural hematoma in a case of untreated compensated congenital hydrocephalus has not been reported in English literature. We report the rare case of an adult with congenital hydrocephalus with a huge unusual shaped hemispheric subdural hematoma.

  19. A Case of Congenital Syndromic Hydrocephalus: A Subtype of ‘Game-Friedman-Paradice Syndrome'

    Directory of Open Access Journals (Sweden)

    Tapan Kumar Jana

    2013-01-01

    Full Text Available Human hydrocephalus is a disorder of abnormality in CSF flow or resorption, which has been classified in pertinent literature as congenital and acquired. Congenital hydrocephalus can present as an isolated phenomenon which is common; or with associated anomalies affecting other organs, disturbing physiology or presenting as a syndrome. This report describes a case with congenital foetal hydrocephalus, hypoplastic lungs with super-numery lobations and large left lobe of liver compared to right. Thus far, a review of the literature indicates that this case can be postulated as a subtype of Game-Friedman-Paradice syndrome.

  20. Congenital hydrocephalus - prevalence, prenatal diagnosis and outcome of pregnancy in four European regions

    DEFF Research Database (Denmark)

    Garne, Ester; Loane, Maria; Addor, Marie-Claude

    2009-01-01

    OBJECTIVE: To describe prevalence, prenatal diagnosis and outcome for fetuses and infants with congenital hydrocephalus. METHODS: Data were taken from four European registries of congenital malformations (EUROCAT). The registries included are based on multiple sources of information and include...... information about livebirths, fetal deaths with GA>/=20weeks and terminations of pregnancy for fetal anomaly (TOPFA). All cases from the four registries diagnosed with congenital hydrocephalus and born in the period 1996-2003 were included in the study. Cases with hydrocephalus associated with neural tube...... defects were not included in the study. RESULTS: Eighty-seven cases with congenital hydrocephalus were identified during the study period giving an overall prevalence of 4.65 per 10,000 births. There were 41 livebirths (47%), four fetal deaths (5%) and 42 TOPFA (48%). Nine percent of all cases were from...

  1. Congenital Unilateral Hypoplasia of Depressor Anguli Oris

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    Seckin O. Ulualp

    2012-01-01

    Full Text Available Objectives. Asymmetric facial appearance may originate from abnormalities of facial musculature or facial innervation. We describe clinical features of congenital hypoplasia of depressor anguli oris muscle in a child. Material and Methods. Chart of a 10-month-old female referred to a tertiary care pediatric hospital for assessment of facial paralysis was reviewed. Data included relevant history and physical examination, diagnostic work up, and management. Results. The child presented with asymmetric movement of lower lip since birth. Asymmetry of lower lip was more pronounced when she smiled and cried. Rest of the face movement was symmetric. On examination, the face appeared symmetric at rest. The child had inward deviation of right lower lip when she smiled. Facial nerve function, as determined by frowning/forehead, wrinkling, eye closure, nasolabial fold depth, and tearing, was symmetric. Magnetic resonance imaging of the temporal bones and internal auditory canals were within normal limits. Echocardiogram did not show cardiac abnormality. Auditory brainstem response showed no abnormality. Conclusions. Congenital hypoplasia of depressor anguli oris is a rare anomaly that causes asymmetric crying face. Pediatricians and otolaryngologists need to be cognizant of cardiac, head and neck, and central nervous system anomalies associated with congenital unilateral hypoplasia of depressor anguli oris.

  2. Hydrocephalus

    Science.gov (United States)

    ... hydrocephalus? The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning head. ... patient or is familiar with that patient's medical history. All NINDS-prepared information is in the public ...

  3. Congenital unilateral absence of the pulmonary artery in adults

    International Nuclear Information System (INIS)

    Gonzalez Garcia, Mauricio; Escalante Mora, Hector A; Lozano Castillo, Alfonso J

    2000-01-01

    Unilateral absence of a pulmonary artery is a rare anomaly. It occurs with pulmonary ipsilateral hypoplasia and it's frequently associated with other cardiovascular malformations. The majority of the cases are diagnosed in childhood. This is a case report of two adult patients of the Hospital Central de la Policia Nacional in Bogota, Colombia, with unilateral absence of the pulmonary artery one isolated and the other with patent ductus arteriosus. We describe the clinical and roentgenographic findings of this congenital anomaly

  4. Evidence that congenital hydrocephalus is a precursor to idiopathic normal pressure hydrocephalus in only a subset of patients

    Science.gov (United States)

    Wilson, Robin K; Williams, Michael A

    2007-01-01

    Objective To identify the proportion of patients in a large idiopathic normal pressure hydrocephalus (INPH) cohort with large head circumference (HC) who presumably have congenital hydrocephalus that has not become clinically apparent until late in life. Methods HC was measured in 158 patients diagnosed with communicating INPH and assigned to HC centile range adjusted for height and sex. The proportion of patients with INPH and HC above the 97th, 90th or 50th centiles was compared with the proportion expected in a normal population. Results The proportion of patients with HC >90th centile was significantly larger than would be expected in a normal distribution (19.6% vs 10%, p = 0.0001), as was the proportion of patients with HC >97th centile (8.9% vs 3%, p = 0.0001). The relative association between INPH and HC >97th centile was nearly tripled (relative association 2.95; CI 1.36 to 6.41), but the relative association between INPH and HC >50th centile was not significantly higher than predicted (relative association 1.13; CI 0.95 to 1.34). Conclusion A significantly larger proportion of elderly adults with INPH have a HC greater than the 90th or 97th centile than predicted by population norms, supporting the concept that compensated congenital hydrocephalus that does not become symptomatic until late in life is one aetiology of INPH, but is not responsible for all INPH. The mechanism that leads to the development of INPH in most patients remains elusive. PMID:17202229

  5. A 9 days old, female baby with Congenital Hydrocephalus | Mgelea ...

    African Journals Online (AJOL)

    A female infant, 3.5kg was born at Muhimbili National Hospital with a big head, Occipitofrontal Circumference (OFC) 49.5cm, and APGAR score 2 and 4 at first and fifth minutes respectively. She was admitted to ward 36 for the management of severe birth asphyxia. Cranial ultrasound revealed Hydrocephalus with bilateral ...

  6. A case of congenital unilateral absence of the vas deferens

    Directory of Open Access Journals (Sweden)

    Mo B

    2013-04-01

    Full Text Available Bi Mo,1 Vishnu Garla,2 Lawrence M Wyner1 1Department of Surgery, 2Department of Internal Medicine, Marshall University, Huntington, WV, USA Background: Congenital unilateral absence of the vas deferens occurs in 0.5%–1.0% of males. It has been associated with various genitourinary abnormalities, including renal agenesis. We report a case of congenital unilateral absence of the vas deferens found incidentally during vasectomy in a patient with known unilateral renal agenesis. Case presentation: A 24-year-old male presented to our urology clinic requesting vasectomy. His past history was significant for left renal agenesis. Following successful right vasectomy, several attempts to locate the left vas deferens were unsuccessful. We diagnosed congenital unilateral absence of the vas deferens. Follow-up semen analysis showed azoospermia. Conclusion: As vasectomies are increasingly performed in outpatient settings, it is imperative that physicians be aware of this condition, which can be recognized by a simple physical exam. Recognition could prevent unnecessary surgery and prompt providers to investigate for associated abnormalities. Keywords: vas deferens, embryology, abnormalities, surgery

  7. Congenital Unilateral Deafness Affects Cerebral Organization of Reading

    Directory of Open Access Journals (Sweden)

    Alice Mado Proverbio

    2013-06-01

    Full Text Available It is known that early sensory deprivation modifies brain functional structure and connectivity. The aim of the present study was to investigate the neuro-functional organization of reading in a patient with profound congenital unilateral deafness. Using event-related potentials (ERPs, we compared cortical networks supporting the processing of written words in patient RA (completely deaf in the right ear since birth and in a group of control volunteers. We found that congenital unilateral hearing deprivation modifies neural mechanisms of word reading. Indeed, while written word processing was left-lateralized in controls, we found a strong right lateralization of the fusiform and inferior occipital gyri activation in RA. This finding goes in the same direction of recent proposals that the ventral occipito-temporal activity in word reading seem to lateralize to the same hemisphere as the one involved in spoken language processing.

  8. Congenital hydrocephalus in an Egyptian baby with trisomy 18: a case report

    Directory of Open Access Journals (Sweden)

    Metwalley Kotb A

    2009-11-01

    Full Text Available Abstract Introduction Trisomy 18 is the second most common autosomal trisomy after Down syndrome (trisomy 21. A variety of anomalies of the central nervous system are observed in cases of trisomy 18. The association between trisomy 18 and congenital hydrocephalus is very rare. Case presentation A 4-month-old male Egyptian baby boy was referred to Assiut University hospital for evaluation of his large-sized head. The initial clinical examination revealed facial dysmorphism including a prominent wide forehead, wide anterior fontanel, bushy eyebrows, synophrosis, small palpebral fissures, ocular hypertelorism, high arched palate, depressed nasal bridge, low-set ears, micrognathia, bilateral clenched hands with over lapping fingers, rocker-bottom feet and penile hypospadius. A computed tomography scan of the patient's head showed a dilatation of all the ventricular systems of the brain that suggested hydrocephalus. A chromosome analysis of his peripheral blood confirmed a trisomy of chromosome 18 (47, XX+18. The hydrocephalus was treated with a ventriculoperitoneal shunt because of the abnormal increase in his head circumference. He was discharged home on nasogastric feeds at the age of 5 months. Despite the advice of the medical team, his parents did not bring him for further follow up. He died at the age of 7 months due to a sudden cardiorespiratory arrest at home. Conclusion Microcephaly is not mandatory for the diagnosis of trisomy 18 syndrome because some cases of trisomy 18 can be associated with other anomalies of the central nervous system, including hydrocephalus. There is no proven explanation for this association, and the management of hydrocephalus in such a situation is not different from the usual course of management.

  9. William Watson Cheyne (1852-1932): a life in medicine and his innovative surgical treatment of congenital hydrocephalus.

    Science.gov (United States)

    Watson, Caroline C; Griessenauer, Christoph J; Loukas, Marios; Blount, Jeffrey P; Tubbs, R Shane

    2013-11-01

    William Watson Cheyne lived and trained during a period of great advances in medical knowledge and surgical techniques. Despite his various contributions to the fields of bacteriology and surgery, little is known about his career or his life apart from his affiliations with Joseph Lister. This article aims to identify Cheyne as a pioneer in the treatment of congenital hydrocephalus and sheds light on the man who existed in Lister's shadow for most of his life. Cheyne's technique for surgical intervention of hydrocephalus was a great turning point and contributes to the current treatment strategy utilized today for hydrocephalus.

  10. Cortical thickness in a case of congenital unilateral perisylvian syndrome.

    Science.gov (United States)

    Kotini, A; Camposano, S; Hara, K; Salat, D; Cole, A; Stufflebeam, S; Halgren, E

    2004-11-30

    In congenital perisylvian syndrome, there is polymicrogyric cortex distributed in variable extensions around the sylvian fissure. Unilateral cases usually present with congenital hemiparesis, while bilateral cases have pseudobulbar paralysis of the oropharingoglossal region. Both unilateral and bilateral cases have a high rate of epilepsy. Polymicrogyric cortex is characterized by too many small convolutions. Often there are no intervening sulci, and almost no white matter can be seen under them. On MRI they appear to have increased thickness. Bilateral and symmetric polimycrogiria can be hard to recognize on standard MRIs. Accurate and automated methods for measuring the thickness of cerebral cortex are available. They have mainly been used to study a variety of disorders with diminished cortical thickness. We studied a case of right perisylvian polymicrogyria, who presented in adult life with epilepsy and had a normal neurological exam. Fischl and Dale's automated cortical thickness analysis rendered a very clear picture of increased cortical thickness with values up to 9 mm in the affected areas (normal cortical thickness varies between 1 and 4.5 mm). The thickest areas were seen over grossly abnormal gyri on the reconstructed cerebral cortex. On MEG he presented a prominent and monotonous 9 Hz activity that was located within the limits of a thick gyrus. There was a significant difference of thickness between homologous hemispheric areas. To our surprise some areas of the left hemisphere also appeared to have increased thickness, raising the question of a bilateral asymmetric case.

  11. Are Shunt Revisions Associated with IQ in Congenital Hydrocephalus? A Meta -Analysis.

    Science.gov (United States)

    Arrington, C Nikki; Ware, Ashley L; Ahmed, Yusra; Kulesz, Paulina A; Dennis, Maureen; Fletcher, Jack M

    2016-12-01

    Although it is generally acknowledged that shunt revisions are associated with reductions in cognitive functions in individuals with congenital hydrocephalus, the literature yields mixed results and is inconclusive. The current study used meta-analytic methods to empirically synthesize studies addressing the association of shunt revisions and IQ in individuals with congenital hydrocephalus. Six studies and three in-house datasets yielded 11 independent samples for meta-analysis. Groups representing lower and higher numbers of shunt revisions were coded to generate effect sizes for differences in IQ scores. Mean effect size across studies was statistically significant, but small (Hedges' g = 0.25, p IQ scores. Results show an association of lower IQ and more shunt revisions of about 3 IQ points, a small effect, but within the error of measurement associated with IQ tests. Although clinical significance of this effect is not clear, results suggest that repeated shunt revisions because of shunt failure is associated with a reduction in cognitive functions.

  12. Diffusion tensor imaging study of pediatric patients with congenital hydrocephalus: 1-year postsurgical outcomes.

    Science.gov (United States)

    Mangano, Francesco T; Altaye, Mekibib; McKinstry, Robert C; Shimony, Joshua S; Powell, Stephanie K; Phillips, Jannel M; Barnard, Holly; Limbrick, David D; Holland, Scott K; Jones, Blaise V; Dodd, Jonathan; Simpson, Sarah; Mercer, Deanna; Rajagopal, Akila; Bidwell, Sarah; Yuan, Weihong

    2016-09-01

    OBJECTIVE The purpose of this study was to investigate white matter (WM) structural abnormalities using diffusion tensor imaging (DTI) in children with hydrocephalus before CSF diversionary surgery (including ventriculoperitoneal shunt insertion and endoscopic third ventriculostomy) and during the course of recovery after surgery in association with neuropsychological and behavioral outcome. METHODS This prospective study included 54 pediatric patients with congenital hydrocephalus (21 female, 33 male; age range 0.03-194.5 months) who underwent surgery and 64 normal controls (30 female, 34 male; age range 0.30-197.75 months). DTI and neurodevelopmental outcome data were collected once in the control group and 3 times (preoperatively and at 3 and 12 months postoperatively) in the patients with hydrocephalus. DTI measures, including fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) values were extracted from the genu of the corpus callosum (gCC) and the posterior limb of internal capsule (PLIC). Group analysis was performed first cross-sectionally to quantify DTI abnormalities at 3 time points by comparing the data obtained in the hydrocephalus group for each of the 3 time points to data obtained in the controls. Longitudinal comparisons were conducted pairwise between different time points in patients whose data were acquired at multiple time points. Neurodevelopmental data were collected and analyzed using the Adaptive Behavior Assessment System, Second Edition, and the Bayley Scales of Infant Development, Third Edition. Correlation analyses were performed between DTI and behavioral measures. RESULTS Significant DTI abnormalities were found in the hydrocephalus patients in both the gCC (lower FA and higher MD, AD, and RD) and the PLIC (higher FA, lower AD and RD) before surgery. The DTI measures in the gCC remained mostly abnormal at 3 and 12 months after surgery. The DTI abnormalities in the PLIC were

  13. Congenital hydrocephalus and abnormal subcommissural organ development in Sox3 transgenic mice.

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    Kristie Lee

    Full Text Available Congenital hydrocephalus (CH is a life-threatening medical condition in which excessive accumulation of CSF leads to ventricular expansion and increased intracranial pressure. Stenosis (blockage of the Sylvian aqueduct (Aq; the narrow passageway that connects the third and fourth ventricles is a common form of CH in humans, although the genetic basis of this condition is unknown. Mouse models of CH indicate that Aq stenosis is associated with abnormal development of the subcommmissural organ (SCO a small secretory organ located at the dorsal midline of the caudal diencephalon. Glycoproteins secreted by the SCO generate Reissner's fibre (RF, a thread-like structure that descends into the Aq and is thought to maintain its patency. However, despite the importance of SCO function in CSF homeostasis, the genetic program that controls SCO development is poorly understood. Here, we show that the X-linked transcription factor SOX3 is expressed in the murine SCO throughout its development and in the mature organ. Importantly, overexpression of Sox3 in the dorsal diencephalic midline of transgenic mice induces CH via a dose-dependent mechanism. Histological, gene expression and cellular proliferation studies indicate that Sox3 overexpression disrupts the development of the SCO primordium through inhibition of diencephalic roof plate identity without inducing programmed cell death. This study provides further evidence that SCO function is essential for the prevention of hydrocephalus and indicates that overexpression of Sox3 in the dorsal midline alters progenitor cell differentiation in a dose-dependent manner.

  14. Subjective benefit after BAHA system application in patients with congenital unilateral conductive hearing impairment.

    NARCIS (Netherlands)

    Kunst, S.J.W.; Hol, M.K.S.; Mylanus, E.A.M.; Leijendeckers, J.M.; Snik, A.F.M.; Cremers, C.W.R.J.

    2008-01-01

    OBJECTIVE: To study whether unilateral Bone-anchored Hearing Aid (BAHA) fitting led to subjective hearing benefit in patients with congenital unilateral conductive hearing impairment. STUDY DESIGN: Prospective evaluation on 20 patients. SETTING: Tertiary referral center. PATIENTS: Ten adults and 10

  15. Residual symptoms after surgery for unilateral congenital superior oblique palsy.

    Science.gov (United States)

    Caca, Ihsan; Sahin, Alparslan; Cingu, Abdullah; Ari, Seyhmus; Akbas, Umut

    2012-01-01

    To establish the surgical results and residual symptoms in 48 cases with unilateral congenital superior oblique muscle palsy that had surgical intervention to the vertical muscles alone. Myectomy and concomitant disinsertion of the inferior oblique (IO) muscle was performed in 38 cases and myectomy and concomitant IO disinsertion and recession of the superior rectus muscle in the ipsilateral eye was performed in 10 cases. The preoperative and postoperative vertical deviation values and surgical results were compared. Of the patients who had myectomy and concomitant IO disinsertion, 74% achieved an "excellent" result, 21% a "good" result, and 5% a "poor" result postoperatively. The difference in deviation between preoperative and postoperative values was statistically significant (P < .001). Of the patients who had myectomy and concomitant inferior oblique disinsertion and ipsilateral superior rectus recession, 50% achieved an "excellent" result, 20% a "good" result, and 30% a "poor" result postoperatively. The difference in deviation between preoperative and postoperative values was statistically significant (P < .001). Both procedures are effective and successful in patients with superior oblique muscle palsy, but a secondary surgery may be required. Copyright 2012, SLACK Incorporated.

  16. Syngnathia-congenital unilateral bony fusion of the maxilla and mandible

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    G V Ramachandra Reddy

    2008-01-01

    Full Text Available Congenital disorders involving orofacial region represents approximately 20% of all birth defects. Out of these disorders, congenital bony fusion of the maxilla and mandible (syngnathia is rare. Usually syngnathia is associated with other anomalies and syndromes. This case report presents a unilateral fusion of maxilla and mandible with no other anomalies.

  17. [Congenital unilateral muscular hyperplasia of the hand - a rare malformation].

    Science.gov (United States)

    Pillukat, T; Lanz, U

    2004-01-01

    This is a report on eight cases of a rare congenital malformation in the upper extremity, consisting of a unilateral muscular hyperplasia. In addition to the hand, all segments of the upper extremity may be affected. The hyperplasia is always unilateral, preferably on the right hand side, in combination with accessory muscles. Hereditary dependence or association with other malformations has not been observed. Six of eight patients were male. Shoulder and arm function were normal in all cases. Ulnar drift of the fingers in the metacarpophalangeal joints (six of eight patients), flexion contractures of the metacarpophalangeal joints (six of eight patients) and extension contractures of the wrist (three of eight patients) to various degrees were seen. A prominence of the second and third metacarpal head with an enlarged space between them gave the affected hands a very typical appearance (six of eight patients). Deformities and functional limitations requiring surgical treatment were present in six patients. In all cases, accessory muscles were found intraoperatively and resected. The macroscopic and microscopic appearance of the muscle specimen did not differ from normal muscular tissue. In all cases, additional procedures were necessary to improve the overall function. Nevertheless, the reconstructive efforts did not lead to an entirely normal hand function or appearance. The malformation we describe can clearly be distinguished from other malformations such as arthrogryposis multiplex congenita, Freeman-Sheldon syndrome or macrodactyly. Up to now, only two other reports were found in the literature showing characteristics similar to those in our own cases. Four similar cases were observed by Benatar. From a pathomechanical point of view, a disturbance in the muscular balance seems to cause the deformities and functional limitations. This imbalance could be related to accessory muscles which are not opposed by defined antagonists or to an unbalanced hyperplasia of

  18. DTI study of Children with Congenital Hydrocephalus: 1 Year Post-Surgical Outcomes

    Science.gov (United States)

    Mangano, Francesco T.; Altaye, Mekibib; McKinstry, Robert C.; Shimony, Joshua S.; Powell, Stephanie K.; Phillips, Jannel M.; Barnard, Holly; Limbrick, David D.; Holland, Scott K.; Jones, Blaise V.; Dodd, Jonathon; Simpson, Sarah; Deanna, Mercer; Rajagopal, Akila; Bidwell, Sarah; Yuan, Weihong

    2016-01-01

    Object To investigate white matter structural abnormalities using diffusion tensor imaging (DTI) in children with hydrocephalus before CSF diversionary surgery (including ventriculoperitoneal shunting and endoscopic third ventriculoscopy) and the course of recovery post-surgery in association with neuropsychological and behavioral outcome. Methods This was a prospective study that included 54 children with congenital hydrocephalus (21F/33M; age range: 0.03–194.5 months) who underwent surgery and 64 normal controls (30F/34M, age range: 0.30–197.75 months). DTI and neurodevelopmental outcome data were collected once in the control group and at pre-surgery, 3-month, and 12-month post-surgery in the patients. DTI measures, including fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) values were extracted from the genu of corpus callosum (gCC) and the posterior limb of internal capsule (PLIC). Group analysis was performed first cross-sectionally to quantify DTI abnormalities at three time points by comparing the controls and the patients group at the three time points separately. Longitudinal comparisons were conducted pairwise between different time points in patients whose data were acquired at multiple time points. Neurodevelopmental data were collected and analyzed using the Adaptive Behavior Assessment System, Second Edition (ABAS-II) and the Bayley Scales of Infant Development, Third Edition (Bayley-III). Correlation analyses were performed between DTI and behavioral outcomes. Results Significant DTI abnormalities were found in both the gCC (lower FA and higher MD, AD, and RD) and the PLIC (higher FA, lower AD and RD) at pre-surgery. The DTI measures in the gCC remained mostly abnormal at 3-month and 12-month post-surgery. The DTI abnormalities in the PLIC were significant in FA and AD at 3-month post-surgery but did nor persist when tested at 12-month post-surgery. Significant longitudinal DTI changes in the

  19. Congenital torticollis caused by unilateral absence of the sternocleidomastoid muscle

    Energy Technology Data Exchange (ETDEWEB)

    Raman, Subha; Takhtani, Deepak; Wallace, E.C. [UMass Memorial Medical Center, Department of Radiology, Worcester, MA (United States)

    2009-01-15

    Congenital torticollis is most commonly caused by benign fibrosis of the sternocleidomastoid muscle. Absence of the sternocleidomastoid muscle is a rare cause of congenital torticollis. There have been fewer than a dozen reported cases of agenesis of the sternocleidomastoid muscle. We describe a case of congenital absence of the sternocleidomastoid diagnosed by US and confirmed on MRI. (orig.)

  20. Electron beam CT diagnosis of congenital unilateral absence of pulmonary artery

    International Nuclear Information System (INIS)

    Zhou Yuan; Dai Ruping; Cao Cheng; Zhang Gejun; Jing Baolian

    2003-01-01

    Objective: To evaluate the clinical value of electron beam CT (EBCT) in diagnosing congenital unilateral absence of pulmonary artery (UAPA). Methods: Patients with clinically suspected pulmonary artery disease or primary pulmonary hypertension underwent EBCT scanning. EBCT confirmed the diagnosis of UAPA in 11 patients, who were also evaluated with echocardiography and chest roentgenography. Cardioangiography and nuclear ventilation-perfusion scan were performed in some patients for a comparative study. Results: 4 female adults had UAPA with out associated congenital anomaly. 3 male children with coexisting complex congenital abnormality had unilateral absence of the left pulmonary artery and 4 patients coexisted other simple cardiovascular anomaly. EBCT scanning simultaneously displayed topographic pattern of both unilateral absence of pulmonary artery and coexisting congenital cardiovascular anomaly, as well as lung diseases. Conclusion: UAPA diagnosed in childhood usually has unilateral absence of the left pulmonary artery and associated congenital cardiovascular anomaly, while UAPA diagnosed in adult usually has UAPA on the right side without associated congenital anomaly. EBCT is one of the optimal imaging techniques in diagnosing UAPA and it greatly increases the diagnostic efficacy than echocardiography dose. Both EBCT and cardioangiography have their own advantages, however, EBCT, as a noninvasive method, should be complementary and not exclusive

  1. Clinical and laboratory diagnosis of congenital Zika virus syndrome and diaphragmatic unilateral palsy: case report

    Directory of Open Access Journals (Sweden)

    Alex Sandro Rolland Souza

    Full Text Available Abstract Introduction: several birth defects associated to congenital Zika virus infection have been reported, although the clinical features have not been fully characterized. Description: this is the first case report on unilateral diaphragmatic paralysis diagnosed on a neonate with congenital Zika confirmed by the examination of the amniotic fluid through polymerase chain reaction (ZIKV RT-PCR and the examination of cerebrospinal fluid by serological test (IgM ZIKV-ELISA after birth. The main manifestations detected by intrauterine ultrasound were: microcephaly, ventriculomegaly, intracranial calcifications, enlarged cisterna magna, increased amniotic fluid index and fetal akinesia syndrome. The newborn had acute respiratory failure in the first hours of life, requiring mechanical ventila-tion. The X- ray of the chest showed unilateral diaphragmatic paralysis and cardiomegaly. Discussion: diaphragmatic palsy in congenital Zika has not been previously reported, the etiopathogenic mechanisms of this event in congenital Zika virus needs to be elucidated.

  2. Congenital absence of the vas deferens and unilateral renal agenesis: implications for patient and family.

    Science.gov (United States)

    Lane, Victoria Alison; Scammell, Simon; West, Noreen; Murthi, Govind V

    2014-07-01

    At routine groin surgery in male paediatric patients occasionally the vas deferens may be absent. This finding usually leads to investigations to establish the status of the contralateral vas deferens and the status of the kidneys. It is not uncommon to find either an ipsilateral renal agenesis or congenital bilateral absence of the vas deferens. The latter finding prompts a test for cystic fibrosis. We report three patients who upon investigation were found to have the rare combination of congenital bilateral absence of the vas deferens and unilateral renal agenesis, and discuss the possible embryological basis, the clinical management and the long-term implications of these findings. We present three patients who were incidentally found to have absence of the vas deferens whilst undergoing elective groin surgery and following further tests were diagnosed with congenital bilateral absence of the vas deferens and unilateral renal agenesis. The case notes were reviewed, together with the results of radiological investigations, cystic fibrosis screening and the status of the contralateral vas deferens. All three patients were found to have congenital bilateral absence of the vas deferens, unilateral renal agenesis and were not found to have cystic fibrosis. The combination of congenital bilateral absence of the vas deferens and unilateral renal agenesis, without cystic fibrosis, is rare and not reported previously in the paediatric literature. These findings require appropriate counselling of the parents and child, with regards to the long-term implications of infertility and renal function.

  3. Dusp16 Deficiency Causes Congenital Obstructive Hydrocephalus and Brain Overgrowth by Expansion of the Neural Progenitor Pool.

    Science.gov (United States)

    Zega, Ksenija; Jovanovic, Vukasin M; Vitic, Zagorka; Niedzielska, Magdalena; Knaapi, Laura; Jukic, Marin M; Partanen, Juha; Friedel, Roland H; Lang, Roland; Brodski, Claude

    2017-01-01

    Hydrocephalus can occur in children alone or in combination with other neurodevelopmental disorders that are often associated with brain overgrowth. Despite the severity of these disorders, the molecular and cellular mechanisms underlying these pathologies and their comorbidity are poorly understood. Here, we studied the consequences of genetically inactivating in mice dual-specificity phosphatase 16 ( Dusp16 ), which is known to negatively regulate mitogen-activated protein kinases (MAPKs) and which has never previously been implicated in brain development and disorders. Mouse mutants lacking a functional Dusp16 gene ( Dusp16 -/- ) developed fully-penetrant congenital obstructive hydrocephalus together with brain overgrowth. The midbrain aqueduct in Dusp16 -/- mutants was obstructed during mid-gestation by an expansion of neural progenitors, and during later gestational stages by neurons resulting in a blockage of cerebrospinal fluid (CSF) outflow. In contrast, the roof plate and ependymal cells developed normally. We identified a delayed cell cycle exit of neural progenitors in Dusp16 -/- mutants as a cause of progenitor overproliferation during mid-gestation. At later gestational stages, this expanded neural progenitor pool generated an increased number of neurons associated with enlarged brain volume. Taken together, we found that Dusp16 plays a critical role in neurogenesis by balancing neural progenitor cell proliferation and neural differentiation. Moreover our results suggest that a lack of functional Dusp16 could play a central role in the molecular mechanisms linking brain overgrowth and hydrocephalus.

  4. Quality of Life in Individuals Surgically Treated for Congenital Hydrocephalus During Infancy: A Single-Institution Experience.

    Science.gov (United States)

    Khan, Saad Akhtar; Khan, Muhammad Faheem; Bakhshi, Saqib Kamran; Irfan, Omar; Khan, Hamza Abdur Rahim; Abbas, Asad; Awan, Safia; Bari, Muhammad Ehsan

    2017-05-01

    Congenital hydrocephalus (CH) is a frequently encountered birth anomaly that can hinder long-term neurologic maturity and social well-being of affected children. This study was undertaken to assess quality of life (QOL) 10-15 years after surgical treatment for primary CH during infancy at a tertiary care hospital in a developing country. This retrospective cohort study included individuals who presented to Aga Khan University Hospital, Karachi, Pakistan, between 1995 and 2005 at Hydrocephalus Outcome Questionnaire was used to assess outcomes with respect to QOL. Of 118 patients, 90 patients participated in the study. Mean age at first admission was 6.2 months. Mean length of follow-up was 5.4 years. Of these, 28 patients had died after surgery. Shunt infection (P = 0.012) and delayed milestones (P = 0.003) were found to be statistically significant factors affecting mortality in the patients who died. The mean overall health score was 0.67 ± 0.30. Age Hydrocephalus Outcome Questionnaire (P = 0.039). In our analysis, we assessed the QOL associated with CH. We hope that these results will provide insight for future prospective work with the ultimate goal of improving long-term QOL in children with CH. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. Fluid Distribution Pattern in Adult-Onset Congenital, Idiopathic, and Secondary Normal-Pressure Hydrocephalus: Implications for Clinical Care.

    Science.gov (United States)

    Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

    2017-01-01

    In spite of growing evidence of idiopathic normal-pressure hydrocephalus (NPH), a viewpoint about clinical care for idiopathic NPH is still controversial. A continuous divergence of viewpoints might be due to confusing classifications of idiopathic and adult-onset congenital NPH. To elucidate the classification of NPH, we propose that adult-onset congenital NPH should be explicitly distinguished from idiopathic and secondary NPH. On the basis of conventional CT scan or MRI, idiopathic NPH was defined as narrow sulci at the high convexity in concurrent with enlargement of the ventricles, basal cistern and Sylvian fissure, whereas adult-onset congenital NPH was defined as huge ventricles without high-convexity tightness. We compared clinical characteristics and cerebrospinal fluid distribution among 85 patients diagnosed with idiopathic NPH, 17 patients with secondary NPH, and 7 patients with adult-onset congenital NPH. All patients underwent 3-T MRI examinations and tap-tests. The volumes of ventricles and subarachnoid spaces were measured using a 3D workstation based on T2-weighted 3D sequences. The mean intracranial volume for the patients with adult-onset congenital NPH was almost 100 mL larger than the volumes for patients with idiopathic and secondary NPH. Compared with the patients with idiopathic or secondary NPH, patients with adult-onset congenital NPH exhibited larger ventricles but normal sized subarachnoid spaces. The mean volume ratio of the high-convexity subarachnoid space was significantly less in idiopathic NPH than in adult-onset congenital NPH, whereas the mean volume ratio of the basal cistern and Sylvian fissure in idiopathic NPH was >2 times larger than that in adult-onset congenital NPH. The symptoms of gait disturbance, cognitive impairment, and urinary incontinence in patients with adult-onset congenital NPH tended to progress more slowly compared to their progress in patients with idiopathic NPH. Cerebrospinal fluid distributions and

  6. A Severe and Rapidly Progressive Case of Proteus Syndrome in a Neonate Who Presented with Unilateral Hydrocephalus Apart from Other Typical Features of the Proteus Syndrome

    Science.gov (United States)

    Kumar, Rakesh; Bhagat, Puja

    2012-01-01

    Proteus syndrome is a rare hamartomatous disorder affecting multiple tissues and manifesting itself in a variety of ways. The understanding of the complete spectrum of clinical features, the natural clinical course of the disease and the proper management of such a rare but highly variable syndrome depend heavily on experiences gathered by previously reported cases. We present an unusually severely affected and rapidly progressive case of proteus syndrome in a neonate who presented with craniofacial hemihypertrophy, subcutaneous masses, capillary hemangioma, varicose veins, epidermal nevi and macrodactyly. The cranial ultrasonogram revealed unilateral hydrocephalus with partial obstruction of the foramen of monro. PMID:24027714

  7. Changes in auditory perceptions and cortex resulting from hearing recovery after extended congenital unilateral hearing loss

    Directory of Open Access Journals (Sweden)

    Jill B Firszt

    2013-12-01

    Full Text Available Monaural hearing induces auditory system reorganization. Imbalanced input also degrades time-intensity cues for sound localization and signal segregation for listening in noise. While there have been studies of bilateral auditory deprivation and later hearing restoration (e.g. cochlear implants, less is known about unilateral auditory deprivation and subsequent hearing improvement. We investigated effects of long-term congenital unilateral hearing loss on localization, speech understanding, and cortical organization following hearing recovery. Hearing in the congenitally affected ear of a 41 year old female improved significantly after stapedotomy and reconstruction. Pre-operative hearing threshold levels showed unilateral, mixed, moderately-severe to profound hearing loss. The contralateral ear had hearing threshold levels within normal limits. Testing was completed prior to, and three and nine months after surgery. Measurements were of sound localization with intensity-roved stimuli and speech recognition in various noise conditions. We also evoked magnetic resonance signals with monaural stimulation to the unaffected ear. Activation magnitudes were determined in core, belt, and parabelt auditory cortex regions via an interrupted single event design. Hearing improvement following 40 years of congenital unilateral hearing loss resulted in substantially improved sound localization and speech recognition in noise. Auditory cortex also reorganized. Contralateral auditory cortex responses were increased after hearing recovery and the extent of activated cortex was bilateral, including a greater portion of the posterior superior temporal plane. Thus, prolonged predominant monaural stimulation did not prevent auditory system changes consequent to restored binaural hearing. Results support future research of unilateral auditory deprivation effects and plasticity, with consideration for length of deprivation, age at hearing correction, degree and type

  8. A pilot study using residual newborn dried blood spots to assess the potential role of cytomegalovirus and Toxoplasma gondii in the etiology of congenital hydrocephalus.

    Science.gov (United States)

    Simeone, Regina M; Rasmussen, Sonja A; Mei, Joanne V; Dollard, Sheila C; Frias, Jaime L; Shaw, Gary M; Canfield, Mark A; Meyer, Robert E; Jones, Jeffrey L; Lorey, Fred; Honein, Margaret A

    2013-07-01

    Congenital hydrocephalus is a condition characterized by accumulation of cerebrospinal fluid in the ventricles of the brain. Prenatal infections are risk factors for some birth defects. This pilot study investigated whether residual dried blood spots (DBS) could be used to assess infections as risk factors for birth defects by examining the associations between prenatal infection with Toxoplasma gondii (T. gondii) or cytomegalovirus (CMV) with congenital hydrocephalus. Case-infants with hydrocephalus (N=410) were identified among live-born infants using birth defects surveillance systems in California, North Carolina, and Texas. Control-infants without birth defects were randomly selected from the same geographic areas and time periods as case-infants (N=448). We tested residual DBS from case- and control-infants for T. gondii immunoglobulin M and CMV DNA. When possible, we calculated crude odds ratios (cORs) and confidence intervals (CIs). Evidence for prenatal T. gondii infection was more common among case-infants (1.2%) than control-infants (0%; p=0.11), and evidence for prenatal CMV infection was higher among case-infants (1.5%) than control-infants (0.7%; cOR: 2.3; 95% CI: 0.48, 13.99). Prenatal infections with T. gondii and CMV occurred more often among infants with congenital hydrocephalus than control-infants, although differences were not statistically significant. This pilot study highlighted some challenges in using DBS to examine associations between certain infections and birth defects, particularly related to reduced sensitivity and specimen storage conditions. Further study with increased numbers of specimens and higher quality specimens should be considered to understand better the contribution of these infections to the occurrence of congenital hydrocephalus. Copyright © 2013 Wiley Periodicals, Inc.

  9. The Risk Factors of Symptomatic Communicating Hydrocephalus After Stereotactic Radiosurgery for Unilateral Vestibular Schwannoma: The Implication of Brain Atrophy

    International Nuclear Information System (INIS)

    Han, Jung Ho; Kim, Dong Gyu; Chung, Hyun-Tai; Paek, Sun Ha; Park, Chul-Kee; Kim, Chae-Yong; Hwang, Seung-Sik; Park, Jeong-Hoon; Kim, Young-Hoon; Kim, Jin Wook; Kim, Yong Hwy; Song, Sang Woo; Kim, In Kyung; Jung, Hee-Won

    2012-01-01

    Purpose: To identify the effect of brain atrophy on the development of symptomatic communicating hydrocephalus (SCHCP) after stereotactic radiosurgery (SRS) for sporadic unilateral vestibular schwannomas (VS). Methods and Materials: A total of 444 patients with VS were treated with SRS as a primary treatment. One hundred eighty-one patients (40.8%) were male, and the mean age of the patients was 53 ± 13 years (range, 11–81 years). The mean follow-up duration was 56.8 ± 35.8 months (range, 12–160 months). The mean tumor volume was 2.78 ± 3.33 cm 3 (range, 0.03–23.30 cm 3 ). The cross-sectional area of the lateral ventricles (CALV), defined as the combined area of the lateral ventricles at the level of the mammillary body, was measured on coronal T1-weighted magnetic resonance images as an indicator of brain atrophy. Results: At distant follow-up, a total of 25 (5.6%) patients had SCHCP. The median time to symptom development was 7 months (range, 1–48 months). The mean CALV was 334.0 ± 194.0 mm 2 (range, 44.70–1170 mm 2 ). The intraclass correlation coefficient was 0.988 (95% confidence interval [CI], 0.976–0.994; p < 0.001). In multivariate analysis, the CALV had a significant relationship with the development of SCHCP (p < 0.001; odds ration [OR] = 1.005; 95% CI, 1.002–1.007). Tumor volume and female sex also had a significant association (p < 0.001; OR = 1.246; 95% CI, 1.103–1.409; p < 0.009; OR = 7.256; 95% CI, 1.656–31.797, respectively). However, age failed to show any relationship with the development of SCHCP (p = 0.364). Conclusion: Brain atrophy may be related to de novo SCHCP after SRS, especially in female patients with a large VS. Follow-up surveillance should be individualized, considering the risk factors involved for each patient, for prompt diagnosis of SCHCP.

  10. The Risk Factors of Symptomatic Communicating Hydrocephalus After Stereotactic Radiosurgery for Unilateral Vestibular Schwannoma: The Implication of Brain Atrophy

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jung Ho [Department of Neurosurgery, Seoul National University Bundang Hospital, Gyeonggi-do (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Kim, Dong Gyu, E-mail: gknife@plaza.snu.ac.kr [Department of Neurosurgery, Seoul National University Hospital, Seoul (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Chung, Hyun-Tai; Paek, Sun Ha; Park, Chul-Kee [Department of Neurosurgery, Seoul National University Hospital, Seoul (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Kim, Chae-Yong [Department of Neurosurgery, Seoul National University Bundang Hospital, Gyeonggi-do (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Hwang, Seung-Sik [Department of Social and Preventive Medicine, Inha University School of Medicine, Incheon (Korea, Republic of); Park, Jeong-Hoon [Department of Neurosurgery, Seoul National University Bundang Hospital, Gyeonggi-do (Korea, Republic of); Kim, Young-Hoon [Department of Neurosurgery, Seoul National University Bundang Hospital, Gyeonggi-do (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Kim, Jin Wook; Kim, Yong Hwy; Song, Sang Woo; Kim, In Kyung; Jung, Hee-Won [Department of Neurosurgery, Seoul National University Hospital, Seoul (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2012-11-15

    Purpose: To identify the effect of brain atrophy on the development of symptomatic communicating hydrocephalus (SCHCP) after stereotactic radiosurgery (SRS) for sporadic unilateral vestibular schwannomas (VS). Methods and Materials: A total of 444 patients with VS were treated with SRS as a primary treatment. One hundred eighty-one patients (40.8%) were male, and the mean age of the patients was 53 {+-} 13 years (range, 11-81 years). The mean follow-up duration was 56.8 {+-} 35.8 months (range, 12-160 months). The mean tumor volume was 2.78 {+-} 3.33 cm{sup 3} (range, 0.03-23.30 cm{sup 3}). The cross-sectional area of the lateral ventricles (CALV), defined as the combined area of the lateral ventricles at the level of the mammillary body, was measured on coronal T1-weighted magnetic resonance images as an indicator of brain atrophy. Results: At distant follow-up, a total of 25 (5.6%) patients had SCHCP. The median time to symptom development was 7 months (range, 1-48 months). The mean CALV was 334.0 {+-} 194.0 mm{sup 2} (range, 44.70-1170 mm{sup 2}). The intraclass correlation coefficient was 0.988 (95% confidence interval [CI], 0.976-0.994; p < 0.001). In multivariate analysis, the CALV had a significant relationship with the development of SCHCP (p < 0.001; odds ration [OR] = 1.005; 95% CI, 1.002-1.007). Tumor volume and female sex also had a significant association (p < 0.001; OR = 1.246; 95% CI, 1.103-1.409; p < 0.009; OR = 7.256; 95% CI, 1.656-31.797, respectively). However, age failed to show any relationship with the development of SCHCP (p = 0.364). Conclusion: Brain atrophy may be related to de novo SCHCP after SRS, especially in female patients with a large VS. Follow-up surveillance should be individualized, considering the risk factors involved for each patient, for prompt diagnosis of SCHCP.

  11. Neurolues congênita associada a hidrocefalia Congenital neurosyphilis associated with hydrocephalus

    Directory of Open Access Journals (Sweden)

    Julinho Aisen

    1970-09-01

    Full Text Available É apresentado um caso de neurolues congênita (meningovascular e parenquimatosa associada a hidrocefalia do tipo bloqueado. A natureza luética da afecção foi confirmada por exames paraclínicos e histopatológico. O registro do caso se justifica pelo fato da não existência na literatura compulsada de outro semelhante comprovado histopatològicamente. A hidrocefalia ocorreu em conseqüência do bloqueio do sistema ventricular pelo processo inflamatório de natureza crônica.A case of meningovascular and parenchymatous neurosyphilis associated with a non-communicating hydrocephalus is reported. The syphilitic nature of the disease was confirmed by laboratory and histopathological findings. The authors were unable to find in the medical literature other reports of histologically documented cases of hydrocephalus secondary to a block of the ventricular system determined by neurosyphilis.

  12. Fibrosis unilateral congénita con enoftalmo y ptosis Congenital unilateral fibrosis with enophthalmos and ptosis

    Directory of Open Access Journals (Sweden)

    Yaimir Estévez Miranda

    2011-06-01

    Full Text Available El síndrome de fibrosis congénita es definido como un grupo de desórdenes congénitos raros caracterizado por la restricción de los músculos extraoculares y el reemplazo de los músculos por el tejido fibroso. Muchos autores utilizan la clasificación en la que se manifiesta en cinco tipos diferentes. El caso que se presenta corresponde a un niño quien desde el nacimiento está imposibilitado de elevar su ojo izquierdo. Al examen oftalmológico resultó positivo una ptosis palpebral en su ojo izquierdo, enoftalmo, limitación de la abducción, elevación en supraabducción y depresión. La agudeza visual en ambos ojos era de la unidad. El diagnóstico confirmado por genética fue de una fibrosis unilateral congénita con enoftalmo y ptosis. A pesar de tratarse de un desorden genético poco común, tiene formas esporádicas más raras aún. De ahí la importancia de presentar este caso, poco frecuente en nuestra práctica médica diaria.The syndrome of congenital fibrosis is defined like a group of rare congenital disorders characterized by restriction of extraocular muscles and replacement of fibrous tissue muscles. Many authors use the classification in which it is manifested in five different types. Present case is a child who from its birth can not to raise its left eye. The ophthalmic examination was positive to palpebral ptosis in this eye, enophthalmos, limitation of abduction, raise in supra-abduction and depression. The visual acuity in both eyes was of the unit. The diagnosis confirmed by genetics was that of a congenital unilateral fibrosis with enophthalmos and ptosis. Despite it is uncommon genetic disorder, it has more rare sporadic ways yet. Presentation of this uncommon case in our daily medical practice is very significant.

  13. Anoftalmia clínica bilateral associada à hidrocefalia congênita em cão Bilateral clinical anophthalmia associated with congenital hydrocephalus in the dog

    Directory of Open Access Journals (Sweden)

    Mariana Isa Poci Palumbo

    2011-07-01

    Full Text Available A ausência completa do bulbo ocular é muito rara em cães e gatos, enquanto a hidrocefalia é comumente observada como distúrbio congênito em cães de raças miniatura ou braquicefálicas, com menos de um ano de idade. O presente trabalho relata a ocorrência de anoftalmia clínica bilateral associada à hidrocefalia congênita em um cão da raça poodle, sendo este o primeiro relato de caso da associação dessas alterações no Brasil.The complete absence of the eyeball is rare in dogs and cats, and hydrocephalus is commonly seen as a congenital disorder in toy or brachycephalic dogs before one year old. This paper describes for the first time in Brazil the occurrence of bilateral clinical anophthalmia associated to congenital hydrocephalus in a dog.

  14. Genetics of human hydrocephalus

    Science.gov (United States)

    Williams, Michael A.; Rigamonti, Daniele

    2006-01-01

    Human hydrocephalus is a common medical condition that is characterized by abnormalities in the flow or resorption of cerebrospinal fluid (CSF), resulting in ventricular dilatation. Human hydrocephalus can be classified into two clinical forms, congenital and acquired. Hydrocephalus is one of the complex and multifactorial neurological disorders. A growing body of evidence indicates that genetic factors play a major role in the pathogenesis of hydrocephalus. An understanding of the genetic components and mechanism of this complex disorder may offer us significant insights into the molecular etiology of impaired brain development and an accumulation of the cerebrospinal fluid in cerebral compartments during the pathogenesis of hydrocephalus. Genetic studies in animal models have started to open the way for understanding the underlying pathology of hydrocephalus. At least 43 mutants/loci linked to hereditary hydrocephalus have been identified in animal models and humans. Up to date, 9 genes associated with hydrocephalus have been identified in animal models. In contrast, only one such gene has been identified in humans. Most of known hydrocephalus gene products are the important cytokines, growth factors or related molecules in the cellular signal pathways during early brain development. The current molecular genetic evidence from animal models indicate that in the early development stage, impaired and abnormal brain development caused by abnormal cellular signaling and functioning, all these cellular and developmental events would eventually lead to the congenital hydrocephalus. Owing to our very primitive knowledge of the genetics and molecular pathogenesis of human hydrocephalus, it is difficult to evaluate whether data gained from animal models can be extrapolated to humans. Initiation of a large population genetics study in humans will certainly provide invaluable information about the molecular and cellular etiology and the developmental mechanisms of human

  15. The role of bone conduction hearing aids in congenital unilateral hearing loss: A systematic review.

    Science.gov (United States)

    Liu, C Carrie; Livingstone, Devon; Yunker, Warren K

    2017-03-01

    To systematically review the literature on the audiological and/or quality of life benefits of a bone conduction hearing aid (BCHA) in children with congenital unilateral conductive or sensorineural deafness. A systematic search was performed according to the PRISMA guidelines using the PubMed, Medline, and Embase databases. Data were collected on the following outcomes of interest: speech reception threshold, speech discrimination, sound localization, and quality of life measures. Given the heterogeneity of the data for quantitative analysis, the results are qualitatively summarized. Eight studies were included in the review. Four studies examined the audiological outcomes associated with bone conduction hearing aid implantation. There was a consistent gain in speech reception thresholds and speech discrimination, especially in noisy environments. Results pertaining to sound localization was inconsistent. The studies that examined quality of life measures reported a high usage rate of BCHAs among children. Quality of life improvements are reported with suggested benefit in the subdomain of learning. Given the potential benefits of a BCHA, along with the fact that it can be safely trialed using a headband, it is reasonable to trial a BCHA in children with congenital unilateral deafness. Should the trial offer audiological and/or quality of life benefits for the individual child, then BCHA implantation can be considered. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Inner ear anomalies in children with isolated unilateral congenital aural atresia.

    Science.gov (United States)

    Halle, Tyler R; Soares, Bruno P; Todd, N Wendell

    2017-04-01

    We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia. Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Scans were reviewed according to the Jahrsdoerfer criteria and further evaluated for anomalies of the vestibule, semi-circular canals, cochlea, internal auditory canal and vestibulocochlear nerve. Inner ear dysplasia was seen in two of 70 atretic ears: one with a dysmorphic lateral semicircular canal and another with a large vestibule assimilating the lateral semicircular canal. Abnormalities of the oval window and round window ipsilateral to the atresia were identified in 21% (15) and 7% (5), respectively, of the atretic ears. Oval window and round window abnormalities were associated with disproportionately lower Jahrsdoerfer scores compared to aural atresia patients without these abnormalities (P atresia cases, we found inner ear and oval window abnormalities less common in children with isolated non-syndromic unilateral aural atresia. However, round window anomalies seem to occur at about the same frequency. Copyright © 2017 Elsevier B.V. All rights reserved.

  17. Effect of unilateral congenital ptosis on ocular higher order aberrations in children.

    Science.gov (United States)

    Ashok Kumar, Dhivya; Agarwal Ms, Amar; Prakash, Gaurav; Vajpayee Boptm, Neha; Packiyalakshmi, Sathiya; Agarwal, Athiya

    2013-01-01

    To analyse the effect of congenital unilateral ptosis on the ocular higher order aberrations (HOA) and to compare these eyes with normal fellow eyes this study has been performed. In this observational comparative case series, 16 eyes of 16 patients less than 15 years old with congenital unilateral upper eyelid ptosis were included. Corrected distance visual acuity (CDVA), corneal topography, ocular HOA's with Zywave workstation was recorded. The amount of ptosis was measured from marginal reflex distance (MRD1). The ocular HOA's were compared between the ptosis and the normal fellow eyes after making necessary corrections to avoid errors due to enantiomeric midline symmetry. The mean age was 12.5±2.7years (range7-15years). The mean MRD1 was -0.9±1.8mm in the ptosis eyes. There was significant difference noted in the mean 6mm Zernicke coefficients Z3 (-3) (p=0.002), Z4 (-2) (p=0.034), Z4 (2) (p=0.008), Z5 (-5) (p=0.044), Z5 (1) (p=0.039), Z5 (3) (p=0.036), Z5 (5) (p=0.044) between the ptosis and the fellow eyes. The mean Z3-3 was -0.17±0.15 and 0.07±0.12 in the ptosis and the normal eyes respectively. There was a significant difference (p=0.023) in total RMS (root mean square) between the ptosis and the normal eyes. Total coma aberration correlated with CDVA (p=0.004) and MRD (p=0.030) in the ptosis eyes. There was no correlation (p=0.815) between the age (duration of ptosis) and total RMS. In conclusion, Eyes with congenital ptosis differed from their normal fellow eyes in the higher order aberrations. None of the HOA's which differed between the two groups affected the visual acuity in the ptosis eyes.

  18. Congenital spinal tumor in a patient with encephalocele and hydrocephalus: a case report

    OpenAIRE

    Radmanesh, Farid; Nejat, Farideh; Mahjoub, Fatemeh; El Khashab, Mostafa

    2011-01-01

    Abstract Introduction Encephalocele is a rare congenital abnormality of the central nervous system, where brain tissue protrudes from a defect in the skull. Some anomalies are associated with encephalocele. However, the association of spinal teratoma and encephalocele has not been reported in the English literature. Case presentation We report the case of an Iranian girl with a history of encephalocele surgery, who, at the age of four years, developed an intramedullary spinal teratoma, and di...

  19. Sound Localization in Patients With Congenital Unilateral Conductive Hearing Loss With a Transcutaneous Bone Conduction Implant.

    Science.gov (United States)

    Vyskocil, Erich; Liepins, Rudolfs; Kaider, Alexandra; Blineder, Michaela; Hamzavi, Sasan

    2017-03-01

    There is no consensus regarding the benefit of implantable hearing aids in congenital unilateral conductive hearing loss (UCHL). This study aimed to measure sound source localization performance in patients with congenital UCHL and contralateral normal hearing who received a new bone conduction implant. Evaluation of within-subject performance differences for sound source localization in a horizontal plane. Tertiary referral center. Five patients with atresia of the external auditory canal and contralateral normal hearing implanted with transcutaneous bone conduction implant at the Medical University of Vienna were tested. Activated/deactivated implant. Sound source localization test; localization performance quantified using the root mean square (RMS) error. Sound source localization ability was highly variable among individual subjects, with RMS errors ranging from 21 to 40 degrees. Horizontal plane localization performance in aided conditions showed statistically significant improvement compared with the unaided conditions, with RMS errors ranging from 17 to 27 degrees. The mean RMS error decreased by a factor of 0.71 (p conduction implant. Some patients with congenital UCHL might be capable of developing improved horizontal plane localization abilities with the binaural cues provided by this device.

  20. Supramaximal levator resection for unilateral congenital ptosis: cosmetic and functional results.

    Science.gov (United States)

    Cruz, Antonio A V; Akaishi, Patricia M S; Mendonça, Ana K T S; Bernadini, Francesco; Devoto, Martin; Garcia, Denny M

    2014-01-01

    To analyze the cosmetic and functional results of a large series of patients with unilateral congenital ptosis without spontaneous compensatory ipsilateral frontalis hyperaction who underwent supramaximal levator resection (SMLPSr). A multicenter retrospective review of 35 children (14 girls and 21 boys) of mean age 5.5±3.6 SD years with unilateral congenital ptosis who underwent surgery in 3 different countries: Italy, n=8; Argentina, n=11; and Brazil, n=16. Preoperative evaluation included measurements of upper eyelid margin reflex distance (MRD1) and levator palpebrae superioris muscle excursion, assessment of frontalis hyperaction, and ocular motility examination. At least 6 months postoperatively, photographs were used to measure the upper eyelid contour of OU. The spontaneous blinking amplitude and downward eyelid saccades of OU were quantified in a subset of 14 patients. Quantitative comparison among the 3 centers was performed with nonparametric 1-way analysis of variance (Kruskal-Wallis). Paired t tests were used to compare the pre- and postoperative measurements, and p valueMRD1 of the operated eyes increased from 0.5±1.1 SD mm to 3.4±0.84 SD mm (t=15.9; p<0.000001), consequently the eyelid positional asymmetry decreased from 3.1±1.21 mm to 0.1±0.86 SD mm (t=16.5; p≤0.000001). Twenty-nine percent of eyelids had mild contour abnormalities and 31.4% showed some degree of lash ptosis. Spontaneous blinks were abnormal in 93% of the cases (eyelids). The amplitude of the abnormal blinks ranged from 12.9% to 65.4% (mean=37.1%) of the contralateral eyelids. Downward eyelid saccades were reduced in 79% of the eyelids. The amplitudes the saccades ranged from 2.2% to 84.6% (mean=54.8%). In unilateral congenital ptosis, SMLPSr effectively reduces the positional asymmetry between eyelids. Mild contour abnormalities and lash ptosis are the main complications of the surgery. Postoperatively, spontaneous blinks and downward saccades were reduced in most eyelids

  1. Congenital spinal tumor in a patient with encephalocele and hydrocephalus: a case report

    Directory of Open Access Journals (Sweden)

    Mahjoub Fatemeh

    2011-01-01

    Full Text Available Abstract Introduction Encephalocele is a rare congenital abnormality of the central nervous system, where brain tissue protrudes from a defect in the skull. Some anomalies are associated with encephalocele. However, the association of spinal teratoma and encephalocele has not been reported in the English literature. Case presentation We report the case of an Iranian girl with a history of encephalocele surgery, who, at the age of four years, developed an intramedullary spinal teratoma, and discuss the pathogenesis of this association. Conclusion To the best of our knowledge, this is the first report of an association between encephalocele and spinal teratoma.

  2. Mixed Feelings of Children and Adolescents with Unilateral Congenital Below Elbow Deficiency : An Online Focus Group Study

    NARCIS (Netherlands)

    de Jong, I.G.M.; Reinders-Messelink, H.A.; Janssen, W.G.M.; Poelma, M.J.; van Wijk, I.; van der Sluis, C.K.

    2012-01-01

    The existing literature is inconsistent about the psychosocial functioning of children and adolescents with Unilateral Congenital Below Elbow Deficiency (UCBED). The objective of this qualitative study was to explore the psychosocial functioning of children and adolescents with UCBED in terms of

  3. Long-term Compliance and Satisfaction With Percutaneous Bone Conduction Devices in Patients With Congenital Unilateral Conductive Hearing Loss

    NARCIS (Netherlands)

    Nelissen, R.C.; Mylanus, E.A.M.; Cremers, C.W.R.J.; Hol, M.K.S.; Snik, A.F.M.

    2015-01-01

    OBJECTIVE: Patients with congenital unilateral conductive hearing loss (UCHL) can either be watchful monitored or treated surgically through the fitting of a percutaneous bone conduction device (BCD) or, in some cases, atresia repair. The current study evaluated the long-term compliance and

  4. Botulinum toxin for treating unilateral apraxia of eyelid opening in a patient with congenital myotonia

    Directory of Open Access Journals (Sweden)

    Estrella Fernández

    Full Text Available ABSTRACT A 37-year-old female presented with severe apraxia of lid opening (ALO affecting the right upper lid associated with Becker congenital myotonia (MC. The patient had a history of right upper lid ptosis for 25 years that was exacerbated over the previous month with severe incapacity to open her right eye. No other associated neurological or ophthalmic symptoms were observed. The patient was treated with botulinum toxin (BoNT-A injection into the pretarsal and lateral canthus region of the orbicularis oculi of the affected eyelid. Treatment with BoNT-A is an effective method of managing ALO in Becker MC. This is the first case of unilateral ALO in the course of Becker MC that was successfully treated with injections of botulinum toxin.

  5. Botulinum toxin for treating unilateral apraxia of eyelid opening in a patient with congenital myotonia.

    Science.gov (United States)

    Fernández, Estrella; Latasiewicz, Marta; Pelegrin, Laura; Romera, Manuel; Schellini, Silvana; Galindo-Ferreiro, Alicia

    2017-01-01

    A 37-year-old female presented with severe apraxia of lid opening (ALO) affecting the right upper lid associated with Becker congenital myotonia (MC). The patient had a history of right upper lid ptosis for 25 years that was exacerbated over the previous month with severe incapacity to open her right eye. No other associated neurological or ophthalmic symptoms were observed. The patient was treated with botulinum toxin (BoNT-A) injection into the pretarsal and lateral canthus region of the orbicularis oculi of the affected eyelid. Treatment with BoNT-A is an effective method of managing ALO in Becker MC. This is the first case of unilateral ALO in the course of Becker MC that was successfully treated with injections of botulinum toxin.

  6. Down syndrome with congenital hydrocephalus: case report Síndrome de Down associada a hidrocefalia congênita: relato de caso

    Directory of Open Access Journals (Sweden)

    Cassiano Mateus Forcelini

    2006-09-01

    Full Text Available Down syndrome is the most frequent genetic cause of mental retardation. Although usually presenting dysmorphic features and organ malformations, it is rarely associated with congenital hydrocephalus. The case of male neonate whose hydrocephalus was detected since the pregnancy and was discovered to have the syndrome at birth is reported. Chromosomal analysis confirmed the genetic disorder, and hydrocephalus was treated with ventriculoperitoneal shunt because of abnormal increase of head circumference. The patient has been accompanied and his development is considered normal when compared to the expected for those affected by the syndrome.A síndrome de Down é a causa genética mais freqüente de retardo mental. Embora comumente apresentando dismorfias e malformações de órgãos, raramente está associada à hidrocefalia congênita. O caso de um recém-nascido masculino cuja hidrocefalia foi detectada desde a gravidez e que se descobriu ser portadora da síndrome ao nascimento é relatado. O cariótipo confirmou a anormalidade genética, e a hidrocefalia foi tratada com derivação ventriculoperitoneal devido ao aumento anormal do perímetro cefálico. O paciente está sendo acompanhado e seu desenvolvimento é considerado normal quando comparado ao esperado para os portadores da síndrome.

  7. Comparison between eyelid indices of ptotic eye and normal fellow eye in patients with unilateral congenital ptosis.

    Science.gov (United States)

    Bagheri, Abbas; Tavakoli, Mehdi; Najmi, Hadi; Erfanian Salim, Reza; Yazdani, Shahin

    2016-01-01

    To evaluate the relationship between levator muscle function (LMF) and other eyelid parameters in the normal and affected eyes of patients with unilateral congenital ptosis. This study includes subjects with unilateral congenital upper lid ptosis who were referred for operation over a 2-year period. Patients with other eyelid abnormalities and previous eyelid surgery were excluded. Eyelid parameters including LMF, lid fissure height (LFH) and margin reflex distance (MRD) were measured in both eyes and analyzed. A total of 77 patients with mean age of 26.4 ± 16.4 years were enrolled in the study. Mean LMF was 8.3 ± 4.6 mm in the ptotic and 13.1 ± 3.6 mm in the normal fellow eyes. Each millimeter of difference in LMF was associated with 0.30 mm of difference in LFH (95% CI: 0.25-0.35, P muscle dysfunction in the development of congenital ptosis and its severity. Furthermore, a direct correlation was also present between LMF of ptotic and non-ptotic eyes suggesting possible bilateral involvement in apparently unilateral congenital ptosis. Copyright © 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  8. Congenital ganglioglioma.

    Science.gov (United States)

    Karthikeyan, Gengaimuthu; Subburam, Paiyanan; Ravishankar, Soundian Soundian

    2002-03-01

    Congenital neoplasms of brain presenting at birth are extremely uncommon. We report a case of congenital ganglioglioma presenting at birth with hydrocephalus. Ventriculoperitoneal shunt and surgical debulking of the tumour along with histopathological confirmation were done at 6 months of age. On follow-up at 18 months, the child's hydrocephalus is static and his assessed developmental age is 10-12 months.

  9. Diagnostic value of echocardiography in isolated congenital unilateral lung agenesis combined with different anomalies: Two rare cases in children.

    Science.gov (United States)

    He, Huan; Sun, Feifei; Ren, Weidong; Wang, Yonghuai; Xu, Shu

    2016-10-01

    Unilateral lung agenesis, a rare congenital deformity, is secondary to abnormal embryonic development, leading to a shift of the mediastinum and remaining lung. In our case reports, we describe the diagnostic courses of two pediatric patients with unilateral lung agenesis admitted to our hospital in 2014. We present and compare the findings in transthoracic echocardiography with that in CT. Echocardiography played a pivotal role in revealing pulmonary deformities and leading to the diagnosis. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:514-517, 2016. © 2016 Wiley Periodicals, Inc.

  10. Spontaneous Rupture Of A Massive Hydrocephalus Resulting In ...

    African Journals Online (AJOL)

    Objective: To highlight the possible complication of hydrocephalus, when neurosurgical attention is not easily accessible and affordable. Materials and Methods: A three year old Nigerian female child with congenital hydrocephalus. Results: The head of a three year old Nigerian female child with congenital hydrocephalus ...

  11. Congenital anomalies in children with cerebral palsy: a population-based record linkage study

    DEFF Research Database (Denmark)

    Rankin, Judith; Cans, Christine; Garne, Ester

    2009-01-01

    with ataxic CP (41.7%) and lowest in those with dyskinetic CP (2.1%). Cerebral anomalies were found in 8.4% and 7% of children with bilateral and unilateral spastic CP respectively. The most frequent cerebral anomalies were primary microcephaly (26.5%) and congenital hydrocephalus (17.3%). The most common non...

  12. Normal pressure hydrocephalus

    Science.gov (United States)

    Hydrocephalus - occult; Hydrocephalus - idiopathic; Hydrocephalus - adult; Hydrocephalus - communicating; Dementia - hydrocephalus; NPH ... Ferri FF. Normal pressure hydrocephalus. In: Ferri FF, ed. ... Elsevier; 2016:chap 648. Rosenberg GA. Brain edema and disorders ...

  13. Tratamiento simultáneo de anquilosis temporomandibular unilateral congénita y del microlaterognatismo asociado Simultaneously treament of congenital unilateral temporomandibular ankylosis and consecutive microlatherognathism

    Directory of Open Access Journals (Sweden)

    José Felipe Basulto Varela

    2013-03-01

    Full Text Available Este artículo tiene como objetivo reportar los resultados obtenidos a corto y largo plazo, del tratamiento con enfoque multidisciplinario de una anquilosis unilateral congénita de la articulación temporomandibular asociada a un síndrome de malformación embrionaria, en un niño de 12 años de edad, en el que se utilizó un distractor externo bidimensional con un doble propósito: como fijador para mantener el espacio logrado con la artroplastia y como distractor para elongar la rama mandibular hipotrófica, activado 5 días después de la osteotomía, con el objetivo de eliminar la anquilosis y el microlaterognatismo mandíbular consecutivo de ella, simultaneamente de manera funcional y dinámica.The paper reports the short- and long-term results obtained from the multidisciplinary treatment of a congenital unilateral ankylosis of the temporomandibular joint associated to an embryonic malformation in a 12-year-old boy, using an external bidimensional distraction device with a two-fold purpose: as fixator to maintain the space achieved by arthroplasty, and as distractor to elongate the hypotrophic mandibular branch, activated 5 days after osteotomy, with the purpose of eliminating ankylosis and consecutive mandibular microlaterognatism, both functionally and dynamically.

  14. Age at first prosthetic fitting and later functional outcome in children and young adults with unilateral congenital below-elbow deficiency : A cross-sectional study

    NARCIS (Netherlands)

    Huizing, Karin; Reinders-Messelink, Heleen; Maathuis, Carel; Hadders-Algra, Mijna; van der Sluis, Corry K.

    The objective of this study was to evaluate whether prosthetic fitting before the age of one year is associated with better outcomes in children with unilateral congenital below-elbow deficiency compared to children fitted after the age of one. Twenty subjects aged 6-21 years were recruited (five

  15. Use and functioning of the affected limb in children with unilateral congenital below-elbow deficiency during infancy and preschool age : A longitudinal observational multiple case study

    NARCIS (Netherlands)

    Hadders-Algra, Mijna; Reinders-Messelink, Heleen A.; Huizing, Karin; van den Berg, Rik; van der Sluis, Corry K.; Maathuis, Carel G. B.

    Background: Children with unilateral congenital below elbow deficiency (UCBED) lack a part of an arm, thereby lacking a part of the typical hand function. Little is known on the performance of daily activities in very young children with UCBED, usage of their affected arm with or without prosthesis

  16. Normal Pressure Hydrocephalus (NPH)

    Science.gov (United States)

    ... local chapter Join our online community Normal Pressure Hydrocephalus (NPH) Normal pressure hydrocephalus is a brain disorder ... Symptoms Diagnosis Causes & risks Treatments About Normal Pressure Hydrocephalus Normal pressure hydrocephalus occurs when excess cerebrospinal fluid ...

  17. Timing of surgical correction for the treatment of unilateral congenital ptosis: Effects on cosmetic and functional results.

    Science.gov (United States)

    Quaranta-Leoni, Francesco M; Sposato, Sabrina; Leonardi, Antonella; Iacoviello, Licia; Costanzo, Simona

    2017-12-01

    The authors analyzed the cosmetic and functional results of a series of patients with unilateral congenital ptosis who underwent levator resection, to compare the outcome of surgery according to the age of intervention, and to evaluate the chance of ptosis recurrence in different age groups. Analysis of the clinical charts of 44 patients who underwent a unilateral levator muscle resection under the care of one surgeon from February 2000 to March 2012 was performed. Age at the time of surgery ranged from 2.1 to 12 years. The study population was divided into different groups according to the age of surgery. Preoperative evaluation included measurements of upper eyelid margin reflex distance (MRD1), levator function, frontalis function, and complete extraocular motility examination. This study adheres to the principles outlined in the Declaration of Helsinki. The patients' follow-up ranged between 2 and 12 years. The outcome of surgery was more satisfactory (MRD1 increase: p < 0.002) and the increase of levator function was better (p < 0.0001) when surgery was performed in children aged 2 to 4 years. No ptosis recurrence was observed in children aged 2 to 4 years, as opposed to 6 (22%) children of other groups (p = 0.067). Unilateral levator resection effectively reduces the asymmetry between eyelids. The age of the operation appears to influence the outcome of surgery, as in this series cosmetic and functional results are better and the rate of ptosis recurrence is lower if the child is operated on before the age of 4 years.

  18. Unilateral Congenital Knee and Hip Dislocation with Bilateral Clubfoot – A rare Packaging disorder

    Directory of Open Access Journals (Sweden)

    Mukesh Tiwari

    2013-04-01

    Full Text Available ntroduction: Reduced intrauterine space gives rise to ‘packaging disorder’ which may involve joint dislocations or contractures. We present an unique case where mutiple joints were dislocated involving left congenital knee dislocation (CDK, bilateral congenital hip dislocation (CDH and congenital talipes equino varus (CTEVdeformities. Case Report: A preterm baby boy born to mother with diagnosed oligohydramios presented with left CDK bilateral DDH and CTEV. The knee dislocation was treated first with gradual streaching and weekly above knee cast. At 7th week good flexion was achieved at both knees and abduction splint for DDH (using double diaper with ponseti cast for CTEV was done. At one year follow up all joints were reduced and maintained well with baby able to stand with support. Conclusion: Packaging disorders may present with multiple dislocations and deformities. Early intervention with serial casting and manipulation minimises disability and prevents ambulatory problems. In our case there was a good response to manipulation and serial casting. This differs from cases with inherent pathology like arthrogryposis where response to treatment is not so good. Keywords: Congenital genu recurvatum, Develpmental dysplasia hip, CTEV, Clubfoot, serial manipulation, packaging disorders

  19. Polypropylene mesh repair of a unilateral, congenital hernia in the inguinal region in a Thoroughbred filly

    Science.gov (United States)

    Moorman, Valerie J.; Jann, Henry W.

    2009-01-01

    A 2-day-old Thoroughbred intact female was presented for a large subcutaneous swelling in the right inguinal region. Surgical repair was performed using a double layer polypropylene mesh. To the authors’ knowledge, there have been no previous reports of surgical repair of congenital body wall hernias with polypropylene mesh in foals. PMID:19721780

  20. Unilateral Congenital Knee and Hip Dislocation with Bilateral Clubfoot - A rare Packaging disorder.

    Science.gov (United States)

    Tiwari, Mukesh; Sharma, Nishith

    2013-01-01

    Reduced intrauterine space gives rise to 'packaging disorder' which may involve joint dislocations or contractures. We present an unique case where mutiple joints were dislocated involving left congenital knee dislocation (CDK), bilateral congenital hip dislocation (CDH) and congenital talipes equino varus (CTEV)deformities. A preterm baby boy born to mother with diagnosed oligohydramios presented with left CDK bilateral DDH and CTEV. The knee dislocation was treated first with gradual streaching and weekly above knee cast. At 7th week good flexion was achieved at both knees and abduction splint for DDH (using double diaper) with ponseti cast for CTEV was done. At one year follow up all joints were reduced and maintained well with baby able to stand with support. Packaging disorders may present with multiple dislocations and deformities. Early intervention with serial casting and manipulation minimises disability and prevents ambulatory problems. In our case there was a good response to manipulation and serial casting. This differs from cases with inherent pathology like arthrogryposis where response to treatment is not so good.

  1. The influence of ocular sighting dominance on Fundus torsion in patients with unilateral congenital superior oblique palsy.

    Science.gov (United States)

    Kim, Dae Hee; Kim, Hyuna; Lim, Hyun Taek

    2017-12-01

    The purpose of our study was to determine whether ocular sighting dominance may influence the ocular torsion in patients with unilateral congenital superior oblique palsy (UCSOP). This retrospective study included 22 UCSOP patients with radiologic evidence of unilateral superior oblique muscle hypoplasia on orbital magnetic resonance imaging and 66 healthy individuals with normal ocular motility as controls. Ocular torsion was assessed both quantitatively and qualitatively using digital fundus photography. The disc-fovea angle (DFA) was measured quantitatively using image software on a computer screen. All fundus photographs were qualitatively graded as normal torsion, extorsion, or intorsion in all subjects, based on the location of the optic disc relative to the fovea, according to the Bixenman and von Noorden's criteria. Ocular sighting dominance was assessed by the hole-in-the-card test and the pointing test. The Mann-Whitney U test and Fisher's exact test were used to determine the association between the ocular sighting dominance and the ocular torsion. The median DFA was significantly larger in the eyes of patients with UCSOP (9.1° in the paretic eyes and 9.3° in the non-paretic eyes) than the eyes of the control group (4.3°, p extorsion, none had intorsion, and all the eyes of remaining 12 patients had no abnormal torsion, qualitatively. All the eyes showing extorsion in fundus photography were non-dominant eyes, regardless of whether the eyes were paretic or non-paretic. Our findings illuminate the importance of considering ocular sighting dominance for properly assessing ocular torsion in patients with UCSOP. Ocular sighting dominance may have an influence on objective ocular torsion in a way that decreases the torsion in the dominant eye, thereby hindering the abnormal ocular torsion from appearing in that eye.

  2. Long-term Compliance and Satisfaction With Percutaneous Bone Conduction Devices in Patients With Congenital Unilateral Conductive Hearing Loss.

    Science.gov (United States)

    Nelissen, Rik C; Mylanus, Emmanuel A M; Cremers, Cor W R J; Hol, Myrthe K S; Snik, Ad F M

    2015-06-01

    Patients with congenital unilateral conductive hearing loss (UCHL) can either be watchful monitored or treated surgically through the fitting of a percutaneous bone conduction device (BCD) or, in some cases, atresia repair. The current study evaluated the long-term compliance and satisfaction with a percutaneous BCD in this specific population. Fifty-three consecutive patients with congenital UCHL treated with a percutaneous BCD in our tertiary referral center between 1998 and 2011 were identified. Clinical and audiological data were retrospectively gathered from the patients' files. The patients were interviewed by telephone about their current device usage status and were asked to complete the Speech, Spatial and Qualities of Hearing Scale (SSQ). Compliance with the BCD was 56.6% after a mean follow-up of 7 years. The mean age at implantation of the users (22 years) was significantly higher than that of the nonusers (10 years). The mean time of device usage before the patients stopped using the BCD was 5 years. The primary reasons mentioned for quitting the BCD were experiencing excess background noise and/or subjectively not receiving enough benefit. Objectively measured features of binaural processing affected by the BCD were found to correlate with long-term BCD usage. The SSQ revealed significant improvement in the aided condition compared with the nonaided condition in the users, in contrast to the nonusers. The current disappointing long-term compliance figures indicate the need for an even more careful and individualized approach with life-long follow-up when fitting BCDs in this specific population, especially in children.

  3. Formation of a unilateral form deprivation myopia (FDM) due to a congenital eyelid entropium with a consecutive corneal ulcer: an illustrated case report.

    Science.gov (United States)

    Schellenbeck, M; Hartmann, Chr; Schwarz, E C

    2002-06-01

    Eye growth is regulated by a precise, sharp image of a distant object on the photoreceptor layer. A variety of laboratory investigations report that translucent occluders may induce axial eye elongation in animals. This axial growth is also called form deprivation myopia (FDM). In our pediatric ophthalmology clinic we observed the clinical correlate of FDM in a male infant with unilateral corneal ulcer due to a congenital tarsal malformation. Examination in the postnatal period under anesthesia revealed identical axial length in both eyes. Refraction of the affected eye could not be measured due to severe corneal ulcer. During an observation period of two years, the deep corneal opacification of the affected eye induced unilateral disproportionate axial elongation with formation of myopia. Formation of FDM may complicate treatment of amblyopia following unilateral media opacification. Periodic measurements of refraction should be performed to allow appropriate correction.

  4. Unilateral congenital hypoplasia of the internal carotid artery in a newborn: a rare case report

    Directory of Open Access Journals (Sweden)

    Liliana Valentino

    2015-04-01

    Full Text Available Hypoplasia of one or both internal carotid artery (ICA is a rare congenital developmental abnormality. The early neurological presentation of this disorder is rare because many of these cases remain asymptomatic and go undetected due to the presence of collateral vessels. We describe a newborn that presented with seizures at 27 hours after birth. Extended ischemia of the right hemisfere was observed on computed tomography (CT, while the 3D MIP reconstruction showed hypoplasia of right internal carotid artery. After about 3 weeks, the rapid improvement of the newborn’s cerebral ultrasound and EEG allowed to discontinue corticosteroid and sedative therapy. The infant was discharged after 40 days of life in good clinical condition.

  5. Hydrocephalus and Shunts

    Science.gov (United States)

    SBA National Resource Center: 800-621-3141 Hydrocephalus and Shunts Approximately 80% of people with SB have Hydrocephalus Hydrocephalus means there is a build-up of cerebrospinal also called CSF, around ...

  6. Facts and Stats - Hydrocephalus Association

    Science.gov (United States)

    Knowledge Hydrocephalus La Hidrocefalia Normal Pressure Hydrocephalus Physician Directory Publications Resource Library Community Community Network HydrocephalusCONNECT Peer Support Contact Us Parents Teens and ...

  7. Congenital unilateral hydrocele

    African Journals Online (AJOL)

    ABEOLUGBENGAS

    We describe a case of ultrasound diagnosis of hydrocele in a woman at 35 weeks of gestation. Diagnosis was confirmed at birth, distinguishing this from the transient cases usually encountered. This finding during a routine obstetrics scan further highlights the relevance of ultrasound to obstetricians and neonatologists.

  8. Congenital unilateral hydrocele

    African Journals Online (AJOL)

    ABEOLUGBENGAS

    Abstract. Foetal hydrocoele is a condition diagnosed in utero in male foetus. It is characterised by a half-moon fluid image around the testis. We describe a case of ultrasound diagnosis of hydrocele in a woman at 35 weeks of gestation. Diagnosis was confirmed at birth, distinguishing this from the transient cases usually.

  9. Late diagnosis and surgical treatment of patients diagnosed with unilateral congenital cataract at Fundación Visión, in Asuncion, Paraguay

    Directory of Open Access Journals (Sweden)

    Manuel Zegarra

    2014-10-01

    Full Text Available Purpose: Providing data on the late diagnosis and surgical treatment of patients who underwent surgery for total unilateral congenital cataract. Methods: Systematic retrospective review of the medical record of all patients between 0 and 16 years old with total unilateral congenital cataract who underwent surgery at Fundación Vision between January 2010 and July 2012. Results: Medical records of 37 patients (51 % females were studied, age was 7.4 (± 4.9 years (average ± SD and 62% lived on Departamento Central (the most populated region from Paraguay. A total of 97.3% patients underwent late surgical treatment and 86.5% received a late diagnosis. The average time elapsed between the diagnosis and the surgical treatment was one month, and 62.2% of the patients underwent surgery within six months from the diagnosis. Conclusion: This study evidences that most of the patients in our series had a late treatment as a result of a late diagnosis. Based on these results we recommend establishing strategies to improve the early detection and surgical treatment of the newborns.

  10. Late diagnosis and surgical treatment of patients diagnosed with unilateral congenital cataract at Fundación Visión, in Asuncion, Paraguay.

    Science.gov (United States)

    Zegarra, Manuel; Burga, Gisella Harumi; Lansingh, Van; Samudio, Margarita; Duarte, Edgar; Ferreira, Rocio; Dorantes, Yesenia; Ginés, Juan Carlos; Zepeda, Luz

    2014-10-01

    Purpose: Providing data on the late diagnosis and surgical treatment of patients who underwent surgery for total unilateral congenital cataract. Methods: Systematic retrospective review of the medical record of all patients between 0 and 16 years old with total unilateral congenital cataract who underwent surgery at Fundación Vision between January 2010 and July 2012. Results: Medical records of 37 patients (51 % females) were studied, age was 7.4 (± 4.9) years (average ± SD) and 62% lived on Departamento Central (the most populated region from Paraguay). A total of 97.3% patients underwent late surgical treatment and 86.5% received a late diagnosis. The average time elapsed between the diagnosis and the surgical treatment was one month, and 62.2% of the patients underwent surgery within six months from the diagnosis. Conclusion: This study evidences that most of the patients in our series had a late treatment as a result of a late diagnosis. Based on these results we recommend establishing strategies to improve the early detection and surgical treatment of the newborns.

  11. Unilateral testicular tumour associated to congenital adrenal hyperplasia: Failure of specific tumoral molecular markers to discriminate between adrenal rest and leydigioma.

    Science.gov (United States)

    Fenichel, P; Bstandig, B; Roger, C; Chevallier, D; Michels, J-F; Sadoul, J-L; Hieronimus, S; Brucker-Davis, F

    2008-11-01

    Testicular adrenal rest tumours are frequently associated with congenital adrenal hyperplasia (CAH). These ACTH-dependent tumours cannot be easily distinguished histologically from Leydig-cell tumours. We report the case of a 30-year-old man who was explored for infertility, azoospermia and unilateral testicular tumour. High levels of 17-OH progesterone and ACTH, low cortisol and undetectable gonadotropins levels, associated to bilateral adrenal hyperplasia, led to the diagnosis of CAH by 21-OH deficiency with a composite heterozygoty. The testicular tumour was first considered as adrenal rest. However, histological analysis of this unilateral painful tumour showed a steroid-hormone-secreting cell proliferation with atypical and frequent mitosis. To discriminate between a benign adrenal rest tumour and a possible malignant leydigioma, tumoral expression of specific gene products was analyzed by RT-PCR. No 11-beta-hydroxylase nor ACTH receptor mRNAs could be found in the tumour, which did not behave like usual adrenal rest cells. For this unilateral testicular tumour, the lack of adrenal-specific markers associated with a high rate of mitosis and pleiomorphism supported a leydigian origin with malignant potential. However, lack of tumoral LH-R mRNA expression and a tumour-free 3-year follow-up led us to retain the diagnosis of adrenal rest tumour with loss of adrenal gene expression and progressive autonomous behaviour.

  12. Three-year experience with the Sophono in children with congenital conductive unilateral hearing loss: tolerability, audiometry, and sound localization compared to a bone-anchored hearing aid.

    Science.gov (United States)

    Nelissen, Rik C; Agterberg, Martijn J H; Hol, Myrthe K S; Snik, Ad F M

    2016-10-01

    Bone conduction devices (BCDs) are advocated as an amplification option for patients with congenital conductive unilateral hearing loss (UHL), while other treatment options could also be considered. The current study compared a transcutaneous BCD (Sophono) with a percutaneous BCD (bone-anchored hearing aid, BAHA) in 12 children with congenital conductive UHL. Tolerability, audiometry, and sound localization abilities with both types of BCD were studied retrospectively. The mean follow-up was 3.6 years for the Sophono users (n = 6) and 4.7 years for the BAHA users (n = 6). In each group, two patients had stopped using their BCD. Tolerability was favorable for the Sophono. Aided thresholds with the Sophono were unsatisfactory, as they did not reach under a mean pure tone average of 30 dB HL. Sound localization generally improved with both the Sophono and the BAHA, although localization abilities did not reach the level of normal hearing children. These findings, together with previously reported outcomes, are important to take into account when counseling patients and their caretakers. The selection of a suitable amplification option should always be made deliberately and on individual basis for each patient in this diverse group of children with congenital conductive UHL.

  13. Revisiting hydrocephalus as a model to study brain resilience.

    Directory of Open Access Journals (Sweden)

    Matheus Fernandes De Oliveira

    2012-01-01

    Full Text Available Hydrocephalus is an entity which embraces a variety of diseases whose final result is the enlarged size of cerebral ventricular system, partially or completely. The physiopathology of hydrocephalus lies in the dynamics of circulation of cerebrospinal fluid (CSF. The consequent CSF stasis in hydrocephalus interferes with cerebral and ventricular system development. Children and adults who sustain congenital or acquired brain injury typically experience a diffuse insult that impacts many areas of the brain. Development and recovery after such injuries reflects both restoration and reorganization of cognitive functions. Classic examples were already reported in literature. This suggests the presence of biological mechanisms associated with resilient adaptation of brain networks. We will settle a link between the notable modifications to neurophysiology secondary to hydrocephalus and the ability of neuronal tissue to reassume and reorganize its functions.Key words: hydrocephalus; resilience; brain; neural networks; plasticity.

  14. Medfødt hydrocephalus--forekomst og prognose. Mortalitet og morbiditet i en populationsbaseret opgørelse

    DEFF Research Database (Denmark)

    Christensen, Jacob Haaber; Hansen, Lars Kjaersgård; Garne, Ester

    2003-01-01

    The aim was to describe the prevalence and to estimate the prognosis of congenital hydrocephalus (HC) in fetuses and children.......The aim was to describe the prevalence and to estimate the prognosis of congenital hydrocephalus (HC) in fetuses and children....

  15. Congenital Malrotation of the Atlas with Unilateral Hypertrophy of the Atlanto-occipital Joint-A Rare Anomaly of the Craniovertebral Junction and Its Management.

    Science.gov (United States)

    Nath, Pratap Chandra; Mishra, Sudhansu Sekhar; Deo, Rama Chandra; Mahanta, Itibrata

    2016-04-01

    Congenital anomalies are commonly encountered in the craniovertebral junction because of its unique embryologic development. The craniovertebral junction usually comprises the occiput, atlas, and axis. However, malrotation of the atlas (C1) in between the occiput (C0) and axis (C2) with Chiari I malformation as well as unilateral hypertrophy of the atlanto-occipital joint and a lateral mass manifesting features of high cervical myelopathy is a rarely reported anomaly. A 22-year-old woman presented to us with high cervical compressive myelopathy. Imaging revealed rotation of the C1 vertebra approximately 20° toward the left side with right atlanto-occipital joint dislocation and hypertrophy. Imaging also revealed hypertrophy of a right lateral mass of C1, Chiari I malformation, and right atlantoaxial dislocation exposing the right C2 superior facet. She underwent a right extreme far lateral approach comprising a right C1 posterior arch excision and medialization of the right vertebral artery with excision of the hypertrophied atlanto-occipital joint extradurally and posteromedial excision of the superior articular facet of C1. The patient's compressive myelopathy features improved postoperatively. We report the successful management of this rare congenital anomaly and review the literature. Copyright © 2016 Elsevier Inc. All rights reserved.

  16. Gevolgen van hydrocephalus

    NARCIS (Netherlands)

    Tromp, Cornelis Nicolaas

    1984-01-01

    Hydrocephalus is een aandoening van het centrale zenuwstelsel die de clinicus vrij lang voor grote problemen ten aanzien van de behandeling heeft qesteld. Door de toepassing van de ventriculo-atriale shunttechniek zijn de behandelingsmogelijkheden van hydrocephalus sterk verbeterd. Deze studie

  17. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

    Directory of Open Access Journals (Sweden)

    Jane Jackie David

    2016-01-01

    Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

  18. Cerebrospinal fluid circulation and hydrocephalus.

    Science.gov (United States)

    Leinonen, Ville; Vanninen, Ritva; Rauramaa, Tuomas

    2017-01-01

    Hydrocephalus (HC) is classically defined as dynamic imbalance between the production and absorption of cerebrospinal fluid (CSF) leading to enlarged ventricles. Potential causative factors include various brain disorders like tumors causing obstruction of CSF flow within the ventricular system or the subarachnoid space. Classification of HC is based on the site of CSF flow obstruction guiding optimal treatment, with endoscopic third ventriculostomy in intraventricular obstruction and CSF shunt in communicating HC. Another clinically relevant classification is acute and chronic; the most frequent chronic form is idiopathic normal-pressure hydrocephalus (iNPH). The reported incidence of HC varies according to the study population and classification used. The incidence of congenital HC is approximately 0.4-0.6/1,000 newborns and the annual incidence of iNPH varies from 0.5/100,000 to 5.5/100,000. Radiologically, ventricular dilatation may be nonspecific, and differentiation of iNPH from other neurodegenerative diseases may be ambiguous. There are no known specific microscopic findings of HC but a systematic neuropathologic examination is needed to detect comorbid diseases and possible etiologic factors of HC. Depending on the etiology of HC, there are several nonspecific signs potentially to be seen. Copyright © 2017 Elsevier B.V. All rights reserved.

  19. Papilledema in children with hydrocephalus: incidence and associated factors.

    Science.gov (United States)

    Lee, Haeng Jin; Phi, Ji Hoon; Kim, Seung-Ki; Wang, Kyu-Chang; Kim, Seong-Joon

    2017-06-01

    OBJECTIVE The aim of this study was to report the incidence of and the factors associated with papilledema in children with hydrocephalus. METHODS Patients younger than 15 years of age who had been diagnosed with hydrocephalus and treated by extra-ventricular drainage or ventriculoperitoneal shunt surgery between 2005 and 2015 were retrospectively reviewed. Factors including patient age and sex, etiology of hydrocephalus, duration of signs or symptoms, intracranial pressure (ICP), and presence of papilledema were evaluated. RESULTS Forty-six patients, whose mean age was 6.3 ± 4.7 years, were included in the study. The 19 patients without papilledema had a mean age of 2.7 ± 2.7 years, and the 27 patients with papilledema had a mean age of 8.8 ± 4.2 years (p hydrocephalus were tumor (59%), congenital anomaly (19%), hemorrhage (13%), and infection (9%). CONCLUSIONS Papilledema was more common in patients who were older, who had higher ICP, and whose hydrocephalus had been induced by brain tumor. However, since papilledema was absent in 41% of the children with hydrocephalus, papilledema's absence does not ensure the absence of hydrocephalus, especially in younger patients.

  20. Characteristics of individuals with hydrocephalus and their dental care needs.

    Science.gov (United States)

    de Morais Gallarreta, Fernanda Weber; Bernardotti, Fernanda Pierobon Lopes; de Freitas, Aldevina Campos; de Queiroz, Alexandra Mussolino; Faria, Gisele

    2010-01-01

    Hydrocephalus is considered a congenital disease affecting the central nervous system, and is characterized by accumulation of cerebrospinal fluid in the lateral ventricles of the brain causing progressive ventricular dilatation. This review paper reports on the oral characteristics of persons with hydrocephalus and discusses their dental care needs. It has been reported that patients with hydrocephalus can present chronological changes in the eruption of their teeth, changes in the occlusion, greater accumulation of plaque, and have a higher caries prevalence. Regular dental visits are needed to control caries as well as to educate the caretakers about oral hygiene for children with hydrocephalus. In addition, the dental practitioner should be careful not to put pressure on the ventricular-peritoneal drainage tube during dental treatment. Special devices are available to support the patient's head if they have macrocephally.

  1. Unilateral CHARGE association

    NARCIS (Netherlands)

    Trip, J; van Stuijvenberg, M; Dikkers, FG; Pijnenburg, MWH

    A case with a predominantly unilateral CHARGE association is reported. The CHARGE association refers to a combination of congenital malformations. This boy had left-sided anomalies consisting of choanal atresia. coloboma and peripheral facial palsy. The infant had a frontal encephalocele. an anomaly

  2. Normal Pressure Hydrocephalus

    Science.gov (United States)

    ... improves the chance of a good recovery. Without treatment, symptoms may worsen and cause death. What research is being done? The NINDS conducts and supports research on neurological disorders, including normal pressure hydrocephalus. Research on disorders such ...

  3. Hydrocephalus in children.

    Science.gov (United States)

    Rekate, Harold L; Blitz, Ari M

    2016-01-01

    Imaging of hydrocephalus in utero, in infants and children is critically dependent on an understanding of the pathophysiology and treatment options for this condition in this age spectrum. For this reason, this chapter deals not only with the imaging modalities used to study hydrocephalus and how they are applied but also reviews key aspects of the pathophysiology and treatment of hydrocephalus in children. Imaging techniques to establish the diagnosis of chronic hydrocephalus fall into two categories: (1) tracer-type techniques that require an injection and observation of the transit of an injected substance through the ventricular system or subarachnoid space and (2) cross-sectional imaging, which allows for direct visualization of a point of obstruction within the ventricular system or subarachnoid space. For cross-sectional imaging, both magnetic resonance imaging (MRI) and computed tomography can be used, but MRI is usually preferred. Nomenclature has obscured the description of imaging findings in hydrocephalus. We suggest that most hydrocephalus is obstructive and propose to designate ventriculomegaly, the condition in which the ventricles are large on imaging, but there is no true obstruction to the outflow of cerebrospinal fluid. © 2016 Elsevier B.V. All rights reserved.

  4. Hydrocephalus in cerebral venous thrombosis

    NARCIS (Netherlands)

    Zuurbier, Susanna M.; van den Berg, René; Troost, Dirk; Majoie, Charles B.; Stam, Jan; Coutinho, Jonathan M.

    2015-01-01

    Increased intracranial pressure is common in cerebral venous thrombosis (CVT), but hydrocephalus is rarely reported in these patients. We examined the frequency, pathophysiology and associated clinical manifestations of hydrocephalus in patients with CVT admitted to our hospital between 2000 and

  5. Sonographic pattern of hydrocephalus among the under five children in Sokoto North Western Nigeria

    Directory of Open Access Journals (Sweden)

    Sule Ahmed Saidu

    2015-01-01

    Full Text Available Background: Hydrocephalus among children is an important medical problem in view of its neurological sequelae in the growing child. This situation is compounded by the acute shortage of neurosurgeons in third world countries like Nigeria; hence, the need for its early detection and proper management. Objective: Evaluation of the ultrasound (US appearances in children under 5 years of age presenting with clinical signs of raised intracranial pressure suggestive of hydrocephalus. Patients and Methods: A retrospective review of transfontanelle US scans done in 64 children (39 boys and 25 girls attending the Department of Radiology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria over a period of 2 years was carried out. The patients had a mean age of 5.0 ± 4.6 months (range: 1–60 months at the time of their US examination. Some of the clinical indications for US scan included: Congenital hydrocephalus, encephalocele, meningomyelocele, and meningitis. All scans were performed through the anterior fontanelle using SIUI Apogee 800 PLUS scanner with a curvilinear probe using multi-frequency transducer of 2–5 MHz. Results: Fifty-two patients (81.3% had hydrocephalus of congenital origin. Eleven cases (17.2% had postmeningitic hydrocephalus while only 1 case (1.6% was posthemorrhagic. Twenty-five patients (48.0% of the congenital cases were due to cerebral aqueduct stenosis. Eleven (21.2% of the congenital cases were from obstruction at the exit foramina of Luschka and Magendie resulting in the communicating type of hydrocephalus. Conclusion: Hydrocephalus is a known cause of neurological morbidity among infants in developing countries. Majority of the cases are congenital in origin and most commonly due to cerebral aqueduct stenosis. Transfontanelle US is cheap, affordable, nonhazardous, and more accessible than other imaging modalities. It should serve as the first-line investigation of infants with suspected hydrocephalus for early

  6. Clinical analysis of aqueductal stenosis in patients with hydrocephalus in a Kenyan setting.

    Science.gov (United States)

    Kaur, Loyal Poonamjeet; Munyiri, Nderitu Joseph; Dismus, Wekesa Vincent

    2017-01-01

    Aqueductal stenosis is the commonest cause of congenital hydrocephalus. The scope of this paper is to highlight the disease burden of hydrocephalus attributed to aqueductal stenosis which still remains unknown in our setting. In a descriptive cross-sectional study, 258 records of patients diagnosed with hydrocephalus were analyzed after ethical approval from Kenyatta National Hospital- University of Nairobi (KNH-UON) ethics and research committee from January 2010 to May 2016. Patients with a diagnosis of hydrocephalus due to aqueductal stenosis were included in this study. Patients age, sex, mode of delivery, associated comorbidities, presenting complaints, neurosurgical intervention performed, Kafarnosky score were recorded. Data were divided into 2 sets based on the patient's age i.e. whether 12 years. Data were recorded on google data collection form and analyzed using Google spreadsheets. Out of 258 cases of hydrocephalus, 52 had aqueductal stenosis. Male to female sex ratio for this condition was 3:2. There were 25 cases 12 years old who were diagnosed with hydrocephalus due to aqueductal stenosis. Associated conditions were bilateral congenital talipes equinovarus, spina bifida, Arnold Chairi malformations, meningitis and HIV. The presenting complaints differed according to the age groups. Neurosurgical interventions included Endoscopic Third Ventriculostomy (ETV) in 21 cases, insertion of Ventriculoperitoneal (VP) shunt and ETV were done in 3 cases while the rest had only insertion of VP shunt. The Kafanosky score improve from hydrocephalus. Clinical presentation differs according to patients age. Accurate diagnosis and treatment remain a cardinal to improving patient outcome.

  7. Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Basant R. Nassar BS

    2016-04-01

    Full Text Available Idiopathic normal pressure hydrocephalus (iNPH is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” “gait disturbances,” “cognitive function,” “neuropsychology,” “imaging,” and “pathogenesis,” articles were obtained for this review. The majority of the articles were retrieved from the past 10 years. The purpose of this review article is to aid general practitioners in further understanding current findings on the pathogenesis, diagnosis, and treatment of iNPH.

  8. MR elastography of hydrocephalus

    Science.gov (United States)

    Pattison, Adam J.; Lollis, S. Scott; Perrinez, Phillip R.; Weaver, John B.; Paulsen, Keith D.

    2009-02-01

    Hydrocephalus occurs due to a blockage in the transmission of cerebrospinal fluid (CSF) in either the ventricles or subarachnoid space. Characteristics of this condition include increased intracranial pressure, which can result in neurologic deterioration [1]. Magnetic resonance elastography (MRE) is an imaging technique that estimates the mechanical properties of tissue in vivo. While some investigations of brain tissue have been performed using MRE [2,3,4,5], the effects due to changes in interstitial pressure and fluid content on the mechanical properties of the brain remain unknown. The purpose of this work is to assess the potential of MRE to differentiate between the reconstructed properties of normal and hydrocephalic brains. MRE data was acquired in 18 female feline subjects, 12 of which received kaolin injections resulting in an acute form of hydrocephalus. In each animal, four MRE scans were performed during the process including one pre-injection and three post-injection scans. The elastic parameters were obtained using a subzone-based reconstruction algorithm that solves Navier's equations for linearly elastic materials [6]. The remaining cats were used as controls, injected with saline instead of kaolin. To determine the state of hydrocephalus, ventricular volume was estimated from segmenting anatomical images. The mean ventricular volume of hydrocephalic cats significantly increased (P ~ 0.5) for the control cats. Also, there was an observable increase in the recorded elastic shear modulus of brain tissue in the normal and hydrocephalic acquisitions. Results suggest that MRE is able to detect changes in the mechanical properties of brain tissue resulting from kaolin-induced hydrocephalus, indicating the need for further study.

  9. An unusual cause of hydrocephalus: aqueductal developmental venous anomaly

    Energy Technology Data Exchange (ETDEWEB)

    Yagmurlu, Banu; Fitoz, Suat; Atasoy, Cetin; Erden, ilhan [Ankara University School of Medicine, Department of Radiology, Ankara (Turkey); Deda, Gulhis; Unal, Ozlem [Ankara University School of Medicine, Division of Pediatric Neurology, Ankara (Turkey)

    2005-06-01

    Vascular malformations are infrequent causes of aqueductal stenoses, developmental venous anomaly (DVA) being the rarest among them. DVAs, also known as venous angiomas, are congenital in origin and characterized by dilatation of vessels in the superficial and deep venous system. Although they are usually clinically silent, they can be complicated by hemorrhage, seizures and neurologic deficits. Herein, we report MR imaging findings of a 7-year-old girl whose hydrocephalus was due to an abnormal vein coursing through the aqueduct. (orig.)

  10. Cognitive disorders in children's hydrocephalus.

    Science.gov (United States)

    Zielińska, Dorota; Rajtar-Zembaty, Anna; Starowicz-Filip, Anna

    Hydrocephalus is defined as an increase of volume of cerebrospinal fluid in the ventricular system of the brain. It develops as a result of cerebrospinal fluid flow disorder due to dysfunctions of absorption or, less frequently, as a result of the increase of its production. Hydrocephalus may lead to various cognitive dysfunctions in children. In order to determine cognitive functioning in children with hydrocephalus, the authors reviewed available literature while investigating this subject. The profile of cognitive disorders in children with hydrocephalus may include a wide spectrum of dysfunctions and the process of neuropsychological assessment may be very demanding. The most frequently described cognitive disorders within children's hydrocephalus include attention, executive, memory, visual, spatial or linguistic dysfunctions, as well as behavioral problems. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  11. Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children?

    Energy Technology Data Exchange (ETDEWEB)

    Aziz, Azian Abd.; Coleman, Lee [Royal Children' s Hospital Melbourne, Department of Medical Imaging, Parkville, Victoria (Australia); Morokoff, Andrew; Maixner, Wirginia [Royal Children' s Hospital Melbourne, Department of Neurosurgery, Parkville (Australia)

    2005-08-01

    Hydrocephalus is a common neurological disorder in children and the result of a variety of causes. However, with the advancement of imaging modalities, particularly MRI, previously reported rarer causes of hydrocephalus in children are now being more readily appreciated. We report an 11-year-old boy with diffuse villous hyperplasia of the choroid plexus. He had a ventriculo-peritoneal (VP) shunt in-situ and a prior diagnosis from infancy of congenital aqueduct stenosis as the cause of his hydrocephalus. His current presentation was with further shunt dysfunction. CT and MRI demonstrated enlarged choroid plexuses but did not confirm aqueduct stenosis. CSF overproduction was demonstrated from the externalized ventricular drain. The enlarged choroid plexuses were surgically resected and histology confirmed choroid plexus hyperplasia. Identification of choroid plexus hyperplasia is important since the neurosurgical management of hydrocephalus is not VP shunt insertion, but resection of the hyperplastic choroid plexus. (orig.)

  12. Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children?

    International Nuclear Information System (INIS)

    Aziz, Azian Abd.; Coleman, Lee; Morokoff, Andrew; Maixner, Wirginia

    2005-01-01

    Hydrocephalus is a common neurological disorder in children and the result of a variety of causes. However, with the advancement of imaging modalities, particularly MRI, previously reported rarer causes of hydrocephalus in children are now being more readily appreciated. We report an 11-year-old boy with diffuse villous hyperplasia of the choroid plexus. He had a ventriculo-peritoneal (VP) shunt in-situ and a prior diagnosis from infancy of congenital aqueduct stenosis as the cause of his hydrocephalus. His current presentation was with further shunt dysfunction. CT and MRI demonstrated enlarged choroid plexuses but did not confirm aqueduct stenosis. CSF overproduction was demonstrated from the externalized ventricular drain. The enlarged choroid plexuses were surgically resected and histology confirmed choroid plexus hyperplasia. Identification of choroid plexus hyperplasia is important since the neurosurgical management of hydrocephalus is not VP shunt insertion, but resection of the hyperplastic choroid plexus. (orig.)

  13. Unilateral pulmonary artery agenesis: An unusual cause of unilateral ARDS

    Directory of Open Access Journals (Sweden)

    Lakshmi Saladi, MD

    Full Text Available Unilateral pulmonary artery agenesis (UPAA is a rare malformation that can present as an isolated anomaly or may be associated with certain congenital cardiac anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Clinical presentation is non-specific which makes the diagnosis elusive; chronic dyspnea, hemoptysis or recurrent infections are the most common manifestations. Patients may remain asymptomatic until adulthood. There is no definitive treatment for patients with UPAA. Acute respiratory distress syndrome (ARDS is usually a bilateral disease, unilateral ARDS has been described after lung resection or trauma. We present a case of a 39 year-old woman who developed unilateral ARDS and was later diagnosed with isolated UPAA. Keywords: Unilateral pulmonary artery agenesis, Acute respiratory distress syndrome, Pulmonary hypertension, Pulmonary edema, Hemoptysis

  14. Ventriculoperitoneal shunt for treatment of hydrocephalus in a French bulldog puppy

    Science.gov (United States)

    Giacinti, Jolene A.

    2016-01-01

    A 6.5-week-old bulldog was presented with lethargy, anorexia, and stunted growth. A domed skull, ventrolateral strabismus, hypermetria, and delayed hopping were observed. Congenital hydrocephalus was diagnosed and a ventriculoperitoneal shunt was placed. After surgery, a shunt obstruction occurred but resolved with treatment. The puppy responded well and neurological deficits continued to improve after surgery. PMID:26933271

  15. Hydrocephalus and intestinal aganglionosis : Is L1CAM a modifier gene in Hirschsprung disease?

    NARCIS (Netherlands)

    Parisi, MA; Kapur, RP; Neilson, [No Value; Hofstra, RMW; Holloway, LW; Michaelis, RC; Leppig, KA

    2002-01-01

    Congenital hydrocephalus associated with aqueductal stenosis and/or agenesis of the corpus callosum has been described in newborn males with mutations in L1CAM, a gene that encodes a neural cell adhesion molecule. These males usually have severe mental retardation and may have spastic paraplegia and

  16. SNX27 Deletion Causes Hydrocephalus by Impairing Ependymal Cell Differentiation and Ciliogenesis.

    Science.gov (United States)

    Wang, Xin; Zhou, Ying; Wang, Jian; Tseng, I-Chu; Huang, Timothy; Zhao, Yingjun; Zheng, Qiuyang; Gao, Yue; Luo, Hong; Zhang, Xian; Bu, Guojun; Hong, Wanjin; Xu, Huaxi

    2016-12-14

    therapeutic interest to congenital hydrocephalus. Copyright © 2016 the authors 0270-6474/16/3612586-12$15.00/0.

  17. HYDROCEPHALUS IN THREE JUVENILE NORTH AMERICAN BLACK BEARS (URSUS AMERICANUS).

    Science.gov (United States)

    Ferguson, Sylvia H; Novak, Janelle; Hecht, Silke; Craig, Linden E

    2016-06-01

    Hydrocephalus has been reported in a variety of species, including the North American black bear ( Ursus americanus ). This report describes three cases of hydrocephalus in this species from wild bears aged 3-4 mo considered retrospectively from necropsy records of one institution. Clinical signs included cortical blindness and ataxia. Primary gross findings were doming of the skull, gyri compression and flattening, and lateral ventricle dilation. Two cases had severe bilateral ventricular dilation with loss of the septum pellucidum; atrophy of the surrounding corpus callosum; and bilateral periventricular tears involving the caudate nuclei, internal capsule, and adjacent cerebrum. Histologically, the cases with periventricular tearing had severe axonal loss and degeneration, malacia, hemorrhage, and variable periventricular astrocytosis. All cases were likely congenital, given the bears' age and lack of an apparent acquired obstruction.

  18. Management and outcome of infantile hydrocephalus in a tertiary health institution in Nigeria

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    Ayodeji Salman Yusuf

    2017-01-01

    Full Text Available Background: Hydrocephalus is a leading cause of disability among children worldwide. The outcome depends on morphology and whether insult is pre- or post-natal. There has been improvement in morbidity in developed countries due to improved surgical care. A paucity of trained personnel impacts negatively on care and outcome of infants with hydrocephalus in many low-income countries resulting poorer outcome. We conducted an audit of patients with hydrocephalus managed in our institution to determine common etiology and outcome. Object: The objective of this retrospective review was to conduct an audit of hydrocephalus care in our institution. Materials and Methods: Information was retrieved from case notes, ward records, imaging results, operation notes, and follow-up clinic charts. Type of hydrocephalus, onset, treatment offered, outcome, complications, and follow-up duration were documented. Results: Management of 58 infants with complete data was analyzed. Most hydrocephalus 40 (69% were congenital with 14 (35% occurring in association with myelomeningocele and 8 patients confirmed with aqueductal stenosis. Ventriculoperitoneal shunts insertion 53 (91% was the most common treatment modality. Conclusions: There is need to improve surgical intervention in the form of endoscopic third ventriculostomy in suitable patients. Subsiding cost of care may be considered for indigent patients.

  19. Management and Outcome of Infantile Hydrocephalus in a Tertiary Health Institution in Nigeria.

    Science.gov (United States)

    Yusuf, Ayodeji Salman; Omokanye, Habeeb Kayodele; Adeleke, Nurudeen Abiola; Akanbi, Rukeme Oluaseun; Ajiboye, Sikiru Olalekan; Ibrahim, Hakeem Gbadebo

    2017-01-01

    Hydrocephalus is a leading cause of disability among children worldwide. The outcome depends on morphology and whether insult is pre- or post-natal. There has been improvement in morbidity in developed countries due to improved surgical care. A paucity of trained personnel impacts negatively on care and outcome of infants with hydrocephalus in many low-income countries resulting poorer outcome. We conducted an audit of patients with hydrocephalus managed in our institution to determine common etiology and outcome. The objective of this retrospective review was to conduct an audit of hydrocephalus care in our institution. Information was retrieved from case notes, ward records, imaging results, operation notes, and follow-up clinic charts. Type of hydrocephalus, onset, treatment offered, outcome, complications, and follow-up duration were documented. Management of 58 infants with complete data was analyzed. Most hydrocephalus 40 (69%) were congenital with 14 (35%) occurring in association with myelomeningocele and 8 patients confirmed with aqueductal stenosis. Ventriculoperitoneal shunts insertion 53 (91%) was the most common treatment modality. There is need to improve surgical intervention in the form of endoscopic third ventriculostomy in suitable patients. Subsiding cost of care may be considered for indigent patients.

  20. Unilateral Dislocation of the Posterior Tibialis Tendon (PTT) and Flexor Digitorum Longus Tendon With Contralateral PTT Subluxation in a Patient With Congenitally Shallow Flexor Groove.

    Science.gov (United States)

    Waldron, Jacob E; Bernhardson, Andrew S; Fellars, Todd A

    2017-10-01

    Flexor tendon dislocation from the flexor tendon groove posterior of the medial malleolus has been previously described, and may be difficult to diagnose initially, but is amendable to surgical treatment with good outcomes. We present a unique case of unilateral dislocation of the posterior tibialis and flexor digitorum longus tendons with contralateral flexor digitorum longus subluxation that was treated surgically with a good outcome. A 37-year-old active duty male sustained a dislocation and subluxation of the flexor tendons bilaterally after a forced dorsiflexion injury. Bilateral ankle magnetic resonance imaging revealed the injuries that this patient sustained and aided in surgical planning. Surgical Treatment. Bilateral flexor tendon groove deepening with periosteal flap elevation and retinacular repair. This injury has not been previously described in the literature after a forced dorsiflexion mechanism. Advanced imaging is helpful as this injury may be initially misdiagnosed. This case shows that delayed bilateral reconstruction of the flexor tendon grooves and retinacula are reliable methods for pain relief to allow a patient to return to a physically demanding level of function. Level V.

  1. Evolution of surgical interventions for hydrocephalus: patient ...

    African Journals Online (AJOL)

    The treatment of hydrocephalus has undergone remarkable transformation since it was first documented over two thousand years ago. Currently, the focus of hydrocephalus research is on minimally invasive techniques of treatment. This article reviews the evolution of hydrocephalus therapy, and examines current attitudes ...

  2. Sustained Attention in Children With Two Etiologies of Early Hydrocephalus

    Science.gov (United States)

    Swartwout, Maegan D.; Cirino, Paul T.; Hampson, Amy W.; Fletcher, Jack M.; Brandt, Michael E.; Dennis, Maureen

    2008-01-01

    Several studies have shown that children with spina bifida meningomyelocele (SBM) and hydrocephalus have attention problems on parent ratings and difficulties in stimulus orienting associated with a posterior brain attention system. Less is known about response control and inhibition associated with an anterior brain attention system. Using the Gordon Vigilance Task (Gordon, 1983), we studied error rate, reaction time, and performance over time for sustained attention, a key anterior attention function, in 101 children with SBM, 17 with aqueductal stenosis (AS; another condition involving congenital hydrocephalus), and 40 typically developing controls (NC). In SBM, we investigated the relation between cognitive attention and parent ratings of inattention and hyperactivity and explored the impact of medical variables. Children with SBM did not differ from AS or NC groups on measures of sustained attention, but they committed more errors and responded more slowly. Approximately one-third of the SBM group had attention symptoms, although parent attention ratings were not associated with task performance. Hydrocephalus does not account for the attention profile of children with SBM, which also reflects the distinctive brain dysmorphologies associated with this condition. PMID:18999350

  3. Postoperative epidural haematomas associated with hydrocephalus caused by intraoperative overdrainage of cerebrospinal fluid: two case reports with a literature review of 19 cases

    Science.gov (United States)

    Niimura, Manabu; Takai, Keisuke; Taniguchi, Makoto

    2015-01-01

    We report two cases with postoperative epidural haematomas (EDHs) associated with hydrocephalus and discuss the cause of haematoma development on the basis of a literature review. A 13-year-old boy presented with obstructive hydrocephalus caused by a sellar mass lesion. Multifocal EDHs occurred after partial resection of the lesion via a transcallosal approach following ventricular drainage. In the second case, a 26-year-old man who had a history of ventriculoperitoneal shunting for congenital hydrocephalus presented with hydrocephalus caused by ventricular catheter obstruction. An EDH occurred after replacement of the ventricular catheter with a new burr hole opening. On the basis of a review of 19 cases including our two cases, the authors concluded that postoperative EDH development associated with hydrocephalus was mostly caused by intraoperative overdrainage of cerebrospinal fluid, resulting in rapid shrinkage of the brain with dilation of the epidural space, a situation that may have caused dural venous bleeding. PMID:25666241

  4. Cirurgia da catarata infantil unilateral Unilateral pediatric cataract surgery

    Directory of Open Access Journals (Sweden)

    Adriana Maria Drummond Brandão

    2008-04-01

    Full Text Available OBJETIVO: Analisar os resultados visuais de uma série de crianças operadas de catarata unilateral. MÉTODOS: Um estudo retrospectivo foi realizado através da análise de 35 prontuários médicos do Serviço de Catarata Congênita da UNIFESP/EPM. RESULTADOS: Quanto à etiologia, a primeira causa de catarata foi idiopática, a segunda causa foi o trauma e a terceira foi a rubéola congênita. Em 51,4% dos olhos tinham acuidade visual pré-operatória de ausência de fixação. E em 42,8% dos casos operados a acuidade visual final foi igual ou melhor que 20/200. DISCUSSÃO: Embora a cirurgia em catarata unilateral seja motivo de controvérsias entre os oftalmologistas, obteve-se melhora de acuidade visual em número significativo de casos.PURPOSE: To analyze the results in a series of children submitted to unilateral cataract surgery. METHODS: A retrospective study was conducted through the analysis of 35 patient files from the Congenital Cataract Service of UNIFESP/EPM. RESULTS: The main cause of unilateral cataract was idiopathic, the second cause was ocular trauma and the third cause was congenital rubella. Initial visual acuity was very poor in 51.4% of the cases (did not fix or follow, and the best corrected final visual acuity was better than 20/200 in 42.8% of the eyes. DISCUSSION: Although controversial, the surgical treatment of unilateral cataract, in this study, showed improvement in many cases.

  5. Pediatric hydrocephalus outcomes: a review

    Directory of Open Access Journals (Sweden)

    Vinchon Matthieu

    2012-08-01

    Full Text Available Abstract The outcome of pediatric hydrocephalus, including surgical complications, neurological sequelae and academic achievement, has been the matter of many studies. However, much uncertainty remains, regarding the very long-term and social outcome, and the determinants of complications and clinical outcome. In this paper, we review the different facets of outcome, including surgical outcome (shunt failure, infection and independence, and complications of endoscopy, clinical outcome (neurological, sensory, cognitive sequels, epilepsy, schooling and social integration. We then provide a brief review of the English-language literature and highlighting selected studies that provide information on the outcome and sequelae of pediatric hydrocephalus, and the impact of predictive variables on outcome. Mortality caused by hydrocephalus and its treatments is between 0 and 3%, depending on the duration of follow-up. Shunt event-free survival (EFS is about 70% at one year and 40% at ten years. The EFS after endoscopic third ventriculostomy (ETV appears better but likely benefits from selection bias and long-term figures are not available. Shunt infection affects between 5 and 8% of surgeries, and 15 to 30% of patients according to the duration of follow-up. Shunt independence can be achieved in 3 to 9% of patients, but the definition of this varies. Broad variations in the prevalence of cognitive sequelae, affecting 12 to 50% of children, and difficulties at school, affecting between 20 and 60%, attest of disparities among studies in their clinical evaluation. Epilepsy, affecting 6 to 30% of patients, has a serious impact on outcome. In adulthood, social integration is poor in a substantial number of patients but data are sparse. Few controlled prospective studies exist regarding hydrocephalus outcomes; in their absence, largely retrospective studies must be used to evaluate the long-term consequences of hydrocephalus and its treatments. This review

  6. Pediatric hydrocephalus outcomes: a review

    Science.gov (United States)

    2012-01-01

    The outcome of pediatric hydrocephalus, including surgical complications, neurological sequelae and academic achievement, has been the matter of many studies. However, much uncertainty remains, regarding the very long-term and social outcome, and the determinants of complications and clinical outcome. In this paper, we review the different facets of outcome, including surgical outcome (shunt failure, infection and independence, and complications of endoscopy), clinical outcome (neurological, sensory, cognitive sequels, epilepsy), schooling and social integration. We then provide a brief review of the English-language literature and highlighting selected studies that provide information on the outcome and sequelae of pediatric hydrocephalus, and the impact of predictive variables on outcome. Mortality caused by hydrocephalus and its treatments is between 0 and 3%, depending on the duration of follow-up. Shunt event-free survival (EFS) is about 70% at one year and 40% at ten years. The EFS after endoscopic third ventriculostomy (ETV) appears better but likely benefits from selection bias and long-term figures are not available. Shunt infection affects between 5 and 8% of surgeries, and 15 to 30% of patients according to the duration of follow-up. Shunt independence can be achieved in 3 to 9% of patients, but the definition of this varies. Broad variations in the prevalence of cognitive sequelae, affecting 12 to 50% of children, and difficulties at school, affecting between 20 and 60%, attest of disparities among studies in their clinical evaluation. Epilepsy, affecting 6 to 30% of patients, has a serious impact on outcome. In adulthood, social integration is poor in a substantial number of patients but data are sparse. Few controlled prospective studies exist regarding hydrocephalus outcomes; in their absence, largely retrospective studies must be used to evaluate the long-term consequences of hydrocephalus and its treatments. This review aims to help to establish

  7. Our initial experience with ventriculo-epiplooic shunt in treatment of hydrocephalus in two centers.

    Science.gov (United States)

    Grigorean, Valentin Titus; Sandu, Aurelia Mihaela; Popescu, Mihai; Florian, Ioan Stefan; Lupascu, Cristian Dumitru; Ursulescu, Corina Lupascu

    Hydrocephalus represents impairment in cerebrospinal fluid (CSF) dynamics. If the treatment of hydrocephalus is considered difficult, the repeated revisions of ventriculo-peritoneal (VP) shunts are even more challenging. The aim of this article is to evaluate the efficiency of ventriculo-epiplooic (VEp) shunt as a feasible alternative in hydrocephalic patients. A technical modification regarding the insertion of peritoneal catheter was imagined: midline laparotomy 8-10cm long was performed in order to open the peritoneal cavity; the great omentum was dissected between its two layers; we placed the distal end of the catheter between the two epiplooic layers; a fenestration of 4cm in diameter into the visceral layer was also performed. A retrospective study of medical records of 15 consecutive patients with hydrocephalus treated with VEp shunt is also presented. Between 2008 and 2014 we performed VEp shunt in 15 patients: 5 with congenital hydrocephalus, 8 with secondary hydrocephalus and 2 with normal pressure hydrocephalus. There were 7 men and 8 women. VEp shunt was performed in 13 patients with multiple distal shunt failures and in 2 patients, with history of abdominal surgery, as de novo extracranial drainage procedure. The outcome was favorable in all cases, with no significant postoperative complications. VEp shunt is a new, safe and efficient surgical technique for the treatment of hydrocephalus. VEp shunt is indicated in patients with history of recurrent distal shunt failures, and in patients with history of open abdominal surgery and high risk for developing abdominal complications. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  8. Hydrocephalus due to multiple ependymal malformations is caused by mutations in the MPDZ gene.

    Science.gov (United States)

    Saugier-Veber, Pascale; Marguet, Florent; Lecoquierre, François; Adle-Biassette, Homa; Guimiot, Fabien; Cipriani, Sara; Patrier, Sophie; Brasseur-Daudruy, Marie; Goldenberg, Alice; Layet, Valérie; Capri, Yline; Gérard, Marion; Frébourg, Thierry; Laquerrière, Annie

    2017-05-01

    Congenital hydrocephalus is considered as either acquired due to haemorrhage, infection or neoplasia or as of developmental nature and is divided into two subgroups, communicating and obstructive. Congenital hydrocephalus is either syndromic or non-syndromic, and in the latter no cause is found in more than half of the patients. In patients with isolated hydrocephalus, L1CAM mutations represent the most common aetiology. More recently, a founder mutation has also been reported in the MPDZ gene in foetuses presenting massive hydrocephalus, but the neuropathology remains unknown. We describe here three novel homozygous null mutations in the MPDZ gene in foetuses whose post-mortem examination has revealed a homogeneous phenotype characterized by multiple ependymal malformations along the aqueduct of Sylvius, the third and fourth ventricles as well as the central canal of the medulla, consisting in multifocal rosettes with immature cell accumulation in the vicinity of ependymal lining early detached from the ventricular zone. MPDZ also named MUPP1 is an essential component of tight junctions which are expressed from early brain development in the choroid plexuses and ependyma. Alterations in the formation of tight junctions within the ependyma very likely account for the lesions observed and highlight for the first time that primary multifocal ependymal malformations of the ventricular system is genetically determined in humans. Therefore, MPDZ sequencing should be performed when neuropathological examination reveals multifocal ependymal rosette formation within the aqueduct of Sylvius, of the third and fourth ventricles and of the central canal of the medulla.

  9. Neuroradiological findings in multiloculated hydrocephalus

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    Albanese, V.; Tomasello, F.; Sampaolo, S.; Picozzi, P. (Naples Univ. (Italy). Inst. of Neurosurgery)

    1982-01-01

    The characteristic neuroradiological features of multiloculated hydrocephalus following neonatal meningitis and ventriculitis in five infants are presented. The role of ventriculography and computerized tomography in detecting multiloculated and enlarged ventricles and paraventricular cavities, as well as complete and incomplete septa, is emphasized for early diagnosis and surgical management. CT scan also provides clues indicating the presence of thin but not visualized septa. It is suggested that post-meningitic hydrocephalus should be repeatedly followed-up for detection of multiloculated transformation. Probably this entity occurs less uncommonly than was thought in the past. The CT scan appears the most helpful and safe procedure for the serial evaluation, but ventriculography may be usefully combined with it in order to plan the most suitable operative approach for fenestration of membranes.

  10. Neuroradiological findings in multiloculated hydrocephalus

    International Nuclear Information System (INIS)

    Albanese, V.; Tomasello, F.; Sampaolo, S.; Picozzi, P.

    1982-01-01

    The characteristic neuroradiological features of multiloculated hydrocephalus following neonatal meningitis and ventriculitis in five infants are presented. The role of ventriculography and computerized tomography in detecting multiloculated and enlarged ventricles and paraventricular cavities, as well as complete and incomplete septa, is emphasized for early diagnosis and surgical management. CT scan also provides clues indicating the presence of thin but not visualized septa. It is suggested that post-meningitic hydrocephalus should be repeatedly followed-up for detection of multiloculated transformation. Probably this entity occurs less uncommonly than was thought in the past. The CT scan appears the most helpful and safe procedure for the serial evaluation, but ventriculography may be usefully combined with it in order to plan the most suitable operative approach for fenestration of membranes. (Author)

  11. Congenital anomalies of the neonatal head

    International Nuclear Information System (INIS)

    Benson, C.B.; Teele, R.L.; Dobkin, G.R.; Fine, C.; Bundy, A.L.; Doubilet, P.M.

    1987-01-01

    US is a noninvasive modality that has proved highly valuable in the assessment of congenital intracranial anomalies in the neonate. The patterns of malformation must be familiar to those who obtain and interpret neonatal cranial sonograms. The authors present a variety of cases of congenital anomalies studied with US, including agenesis of the corpus callosum, Dandy-Walker malformation, occipital enecphalocele, congenital hydrocephalus, vein of Galen aneurysm, hydranencephaly, holoprosencephaly, absence of the septum pellucidum, schizencephaly, and hypoplastic cerebellum. Correlation with other radiographic modalities and pathologic follow-up, available in a majority of cases, is included

  12. Gait apraxia in communicating hydrocephalus.

    Science.gov (United States)

    Estañol, B V

    1981-01-01

    Apraxia of gait in patients with communicating hydrocephalus appears in the context of a generalised motor disorder that includes defective righting reflexes, generalised increased tone to passive movements, grasp reflexes, difficulty with serial movements of the hands and defective smooth pursuit eye movements. The inability to walk does not appear to be due to a motor disorder but to release of proprioceptive supporting reactions. This mechanism is triggered by proprioceptive stimuli. PMID:7241157

  13. Pediatric hydrocephalus outcomes: a review

    OpenAIRE

    Vinchon, Matthieu; Rekate, Harold; Kulkarni, Abhaya V

    2012-01-01

    Abstract The outcome of pediatric hydrocephalus, including surgical complications, neurological sequelae and academic achievement, has been the matter of many studies. However, much uncertainty remains, regarding the very long-term and social outcome, and the determinants of complications and clinical outcome. In this paper, we review the different facets of outcome, including surgical outcome (shunt failure, infection and independence, and complications of endoscopy), clinical outcome (neuro...

  14. Unilateral pulmonary agenesis associated with tracheal stenosis : a case report

    International Nuclear Information System (INIS)

    Lee, Yong Suk; Yoon, Chong Hyun; Kim, Kyung Sook; Kim, Ki Soo; Pi, Soo Young

    1998-01-01

    Unilateral pulmonary agenesis is a rare congenital anomaly and is frequently associated with other congenital anomalies. We report a case of left pulmonary agenesis associated with congenital tracheal stenosis in a newborn infant. Simple chest radiographs showed an overinflate right lung and mediastinal shifting to the left side. Chest ST and reconstructed three-dimensional images showed left pulmonary agenesis and tracheal stenosis. These anomalies of the tracheobronchial system were confirmed by bronchography. (author). 10 refs., 3 figs

  15. Congenital unilateral hydrocele: a sonographic finding | Ukwenya ...

    African Journals Online (AJOL)

    Foetal hydrocoele is a condition diagnosed in utero in male foetus. It is characterised by a half-moon fluid image around the testis. We describe a case of ultrasound diagnosis of hydrocele in a woman at 35 weeks of gestation. Diagnosis was confirmed at birth, distinguishing this from the transient cases usually encountered.

  16. Hydrocephalus in Africa: A surgical perspective

    African Journals Online (AJOL)

    Hydrocephalus is the excessive accumulation of cerebrospinal fluid (CSF) within the cranial vault due to excessive production or inadequate absorption. The management of hydrocephalus in developing nations is hindered by significant economic constraints and delays in treatment. - most patients in fact do not present for ...

  17. Bilateral population receptive fields in congenital hemihydranencephaly

    NARCIS (Netherlands)

    Fracasso, Alessio|info:eu-repo/dai/nl/372646905; Koenraads, Yvonne; Porro, Giorgio L; Dumoulin, Serge O|info:eu-repo/dai/nl/314406514

    PURPOSE: Congenital hemihydranencephaly (HH) is a very rare disorder characterised by prenatal near-complete unilateral loss of the cerebral cortex. We investigated a patient affected by congenital right HH whose visual field extended significantly into the both visual hemifields, suggesting a

  18. Multiple congenital defects in a newborn foal

    Directory of Open Access Journals (Sweden)

    J.F. Silva

    2014-12-01

    Full Text Available A case of multiple congenital defects in a newborn foal is reported. The animal showed hypoplasia of the left pelvic limb bones, uterus unicornis, congenital diaphragmatic hernia, and unilateral renal and ureteral agenesis. This report includes the macroscopic and microscopic lesions observed in the case.

  19. Bilateral population receptive fields in congenital hemihydranencephaly

    NARCIS (Netherlands)

    Fracasso, Alessio; Koenraads, Yvonne; Porro, Giorgio L.; Dumoulin, Serge O.

    2016-01-01

    Purpose: Congenital hemihydranencephaly (HH) is a very rare disorder characterised by prenatal near-complete unilateral loss of the cerebral cortex. We investigated a patient affected by congenital right HH whose visual field extended significantly into the both visual hemifields, suggesting a

  20. Disruption of the mouse Jhy gene causes abnormal ciliary microtubule patterning and juvenile hydrocephalus

    Science.gov (United States)

    Appelbe, Oliver K.; Bollman, Bryan; Attarwala, Ali; Triebes, Lindy A.; Muniz-Talavera, Hilmarie; Curry, Daniel J.; Schmidt, Jennifer V.

    2013-01-01

    SUMMARY Congenital hydrocephalus, the accumulation of excess cerebrospinal fluid (CSF) in the ventricles of the brain, affects one of every 1,000 children born today, making it one of the most common human developmental disorders. Genetic causes of hydrocephalus are poorly understood in humans, but animal models suggest a broad genetic program underlying the regulation of CSF balance. In this study, the random integration of a transgene into the mouse genome led to the development of an early onset and rapidly progressive hydrocephalus. Juvenile hydrocephalus transgenic mice (JhylacZ) inherit communicating hydrocephalus in an autosomal recessive fashion with dilation of the lateral ventricles observed as early as postnatal day 1.5. Ventricular dilation increases in severity over time, becoming fatal at 4-8 weeks of age. The ependymal cilia lining the lateral ventricles are morphologically abnormal and reduced in number in JhylacZ/lacZ brains, and ultrastructural analysis revealed disorganization of the expected 9+2 microtubule pattern. Rather, the majority of JhylacZ/lacZ cilia develop axonemes with 9+0 or 8+2 microtubule structures. Disruption of an unstudied gene, 4931429I11Rik (now named Jhy) appears to underlie the hydrocephalus of JhylacZ/lacZ mice, and the Jhy transcript and protein are decreased in JhylacZ/lacZ mice. Partial phenotypic rescue was achieved in JhylacZ/lacZ mice by the introduction of a bacterial artificial chromosome (BAC) carrying 60-70% of the JHY protein coding sequence. Jhy is evolutionarily conserved from humans to basal vertebrates, but the predicted JHY protein lacks identifiable functional domains. Ongoing studies are directed at uncovering the physiological function of JHY and its role in CSF homeostasis. PMID:23906841

  1. Cerebrospinal fluid dynamics study in communicating hydrocephalus.

    Science.gov (United States)

    Ramesh, Vengalathur Ganesan; Narasimhan, Vidhya; Balasubramanian, Chandramouli

    2017-01-01

    Communicating hydrocephalus often poses a challenge in diagnosis and management decisions. The objective of this study is to measure the opening pressure (P o ), pressure volume index (PVI), and cerebrospinal fluid outflow resistance (R out ), in patients with communicating hydrocephalus using bolus lumbar injection method and to evaluate its diagnostic and prognostic value. The study was conducted in 50 patients with communicating hydrocephalus, including normal pressure hydrocephalus (NPH) (19), post-meningitic hydrocephalus (23) and post-traumatic hydrocephalus (8). An improvised bolus lumbar injection method [the Madras Institute of Neurology (MIN) method] was used. In the NPH Group, the CSF dynamics studies correlated well with the clinico-radiological classification. The prediction of shunt responsiveness by CSF dynamics studies correlated with good outcome in 87.5%. In the post-meningitic hydrocephalus group, the value of CSF dynamics studies in predicting patients needing shunt was 89.5%. The CSF dynamics studies detected patients who needed shunt earlier than clinical or radiological indications. In the post-traumatic hydrocephalus group, 62.5% of patients improved with the treatment based on CSF dynamics studies. The improvised bolus lumbar injection method (MIN method) is a very simple test with fairly reliable and reproducible results. Study of CSF dynamics is a valuable tool in communicating hydrocephalus for confirmation of diagnosis and predicting shunt responsiveness. This is the first time that the value of CSF dynamics has been studied in patients with post-meningitic hydrocephalus. It was also useful for early selection of cases for shunting and for identifying patients with atrophic ventriculomegaly, thereby avoiding unnecessary shunt.

  2. Biomarkers in Neonatal Posthemorrhagic Hydrocephalus

    Science.gov (United States)

    Merhar, Stephanie

    2011-01-01

    Posthemorrhagic hydrocephalus (PHH) is a rare but serious outcome among premature babies in the NICU, with consequences including mortality and severe neurodevelopmental disabilities. The causes of PHH are still not entirely understood, and its prevention and treatment are controversial. Various cerebrospinal fluid biomarkers have been studied in infants with PHH in order to recognize the causes, diagnose brain injury, and predict neurodevelopmental outcomes. This systematic review summarizes studies on biomarkers of extracellular matrix activity, fibrinolysis/coagulation, hypoxia/cell death, and inflammation in the cerebrospinal fluid of infants with PHH. PMID:21791933

  3. Clinical and morphological characteristics of malformations in infants with congenital cytomegalovirus infection and congenital toxoplasmosis

    Directory of Open Access Journals (Sweden)

    L. Yu. Barycheva

    2015-01-01

    Full Text Available The results of following up infants with intrauterine infections and malformations were retrospectively analyzed. Infants with malformations were diagnosed as having congenital cytomegalovirus infection and congenital toxoplasmosis in 127 and 69 cases, respectively. The aim of the study was to characterize malformations in infants with congenital cytomegalovirus and congenital Toxoplasma infections. The infants with malformations in congenital cytomegalovirus infection were found to have higher mortality rates (61,4% than those with congenital toxoplasmosis (34,8%. Postmortem analysis indicated that there was a predominance of embryopathies in infants with congenital cytomegalovirus infection and that of fetopathies in those with congenital toxoplasmosis. The dead infants with congenital cytomegalovirus infection had more commonly developed visceral defects, including heart diseases, pneumopathies, gastrointestinal and genitourinary abnormalities; fetopathies of the central nervous system and eye were prevalent in congenital toxoplasmosis. The surviving children with congenital toxoplasmosis were more frequently observed to have disabling CNS and ocular sequels as obstructive hydrocephalus, infantile cerebral palsy, complete or partial blindness, and cerebrasthenic disorders than those with congenital cytomegalovirus infection. 

  4. Imaging of the ventriculoperitoneal shunt-related complications in infants and children with hydrocephalus

    Energy Technology Data Exchange (ETDEWEB)

    Jeon, Se Ok; Kim, Ji Hye; Oh, Eun Young; Hwang, Hee Young; Lee, Seon Kyu; Lee, Eun Joo; Kwak, Min Sook; Kim, Hyung Sik [Gachon Medical College Gil Medical Center, Incheon (Korea, Republic of)

    1999-01-01

    To evaluate the frequency and imaging findings of various ventriculo-peritoneal shunt-related complications in pediatric patients with hydrocephalus. We retrospectively reviewed 246 plain radiographs, three shuntograms, 53 ultrasounds, 133 CT scans, and 24 MR images obtained before and after the ventriculo-peritoneal shunt procedure in 33 pediatric patients with hydrocephalus. Using preoperative images, the etiology of the hydrocephalus was assessed. Changes in the size and shape of the ventricles, the location and continuity of shunt apparatus, and the presence of any abnormal enhancement, hemorrhage, edema or tissue loss, or other findings of complications, were analyzed on postoperative images ; the frequency and imaging findings of shunt-related complications such as shunt malfunction, infection, homorrhage or isolated ventricle, and complications caused by overdrainage, were thus evaluated. The frequency of such complications was analyzed according to the etiology of the hydrocephalus, and in addition, medical records were reviewed and correlated with imaging findings. In 18 of the 33 patients(54%), a total of 31 complications was detected. These were present in four of five cases (80%) of hydrocephalus caused by meningitis and ventriculitis, seven of twelve (58%) intraventricular hemorrhage, two of four (50%) unknown cases, three of nine (33%) congenital malformations, one of two (50%) tumors, and one (100%) congenital infection. Shunt malfunction was most common (n=15), and was accompanied by findings of enlarged ventricles, periventricular and peritubal edema, and abnormal location of the shunt tube. Symptoms and signs of increased intracranial pressure were also noted. Subdural hemorrhage and infection were present in four cases each ; findings of infection were enhancement of the ventricular wall, meninges, and parenchyma, as well as sonographically noted intraventricular septation and increased ventricular wall echo. Isolated lateral ventricle (n=4) or 4

  5. Control volume based hydrocephalus research

    Science.gov (United States)

    Cohen, Benjamin; Voorhees, Abram; Wei, Timothy

    2008-11-01

    Hydrocephalus is a disease involving excess amounts of cerebral spinal fluid (CSF) in the brain. Recent research has shown correlations to pulsatility of blood flow through the brain. However, the problem to date has presented as too complex for much more than statistical analysis and understanding. This talk will highlight progress on developing a fundamental control volume approach to studying hydrocephalus. The specific goals are to select physiologically control volume(s), develop conservation equations along with the experimental capabilities to accurately quantify terms in those equations. To this end, an in vitro phantom is used as a simplified model of the human brain. The phantom's design consists of a rigid container filled with a compressible gel. The gel has a hollow spherical cavity representing a ventricle and a cylindrical passage representing the aquaducts. A computer controlled piston pump supplies pulsatile volume fluctuations into and out of the flow phantom. MRI is used to measure fluid velocity, and volume change as functions of time. Independent pressure measurements and flow rate measurements are used to calibrate the MRI data. These data are used as a framework for future work with live patients.

  6. Cerebrospinal fluid hypersecretion in pediatric hydrocephalus.

    Science.gov (United States)

    Karimy, Jason K; Duran, Daniel; Hu, Jamie K; Gavankar, Charuta; Gaillard, Jonathan R; Bayri, Yasar; Rice, Hunter; DiLuna, Michael L; Gerzanich, Volodymyr; Marc Simard, J; Kahle, Kristopher T

    2016-11-01

    Hydrocephalus, despite its heterogeneous causes, is ultimately a disease of disordered CSF homeostasis that results in pathological expansion of the cerebral ventricles. Our current understanding of the pathophysiology of hydrocephalus is inadequate but evolving. Over this past century, the majority of hydrocephalus cases has been explained by functional or anatomical obstructions to bulk CSF flow. More recently, hydrodynamic models of hydrocephalus have emphasized the role of abnormal intracranial pulsations in disease pathogenesis. Here, the authors review the molecular mechanisms of CSF secretion by the choroid plexus epithelium, the most efficient and actively secreting epithelium in the human body, and provide experimental and clinical evidence for the role of increased CSF production in hydrocephalus. Although the choroid plexus epithelium might have only an indirect influence on the pathogenesis of many types of pediatric hydrocephalus, the ability to modify CSF secretion with drugs newer than acetazolamide or furosemide would be an invaluable component of future therapies to alleviate permanent shunt dependence. Investigation into the human genetics of developmental hydrocephalus and choroid plexus hyperplasia, and the molecular physiology of the ion channels and transporters responsible for CSF secretion, might yield novel targets that could be exploited for pharmacotherapeutic intervention.

  7. Frontal and occipital horn ratio is associated with multifocal intraparenchymal hemorrhages in neonatal shunted hydrocephalus.

    Science.gov (United States)

    Oushy, Soliman; Parker, Jonathon J; Campbell, Kristen; Palmer, Claire; Wilkinson, Corbett; Stence, Nicholas V; Handler, Michael H; Mirsky, David M

    2017-11-01

    OBJECTIVE Placement of a cerebrospinal fluid diversion device (i.e., shunt) is a routine pediatric neurosurgical procedure, often performed in the first weeks of life for treatment of congenital hydrocephalus. In the postoperative period, shunt placement may be complicated by subdural, catheter tract, parenchymal, and intraventricular hemorrhages. The authors observed a subset of infants and neonates who developed multifocal intraparenchymal hemorrhages (MIPH) following shunt placement and sought to determine any predisposing perioperative variables. METHODS A retrospective review of the electronic medical record at a tertiary-care children's hospital was performed for the period 1998-2015. Inclusion criteria consisted of shunt placement, age hydrocephalus. Markers of severity of ventriculomegaly (FOR) and ventricular response to CSF diversion (∆FOR) were significantly associated with occurrence of MIPH. Choice of shunt and etiology of hydrocephalus were also significantly associated with MIPH. After adjusting for corrected age, etiology of hydrocephalus, and shunt setting, the authors found that ∆FOR after shunting was still associated with MIPH. A prospective study of MIPH prevention strategies and assessment of possible implications for patient outcomes is needed.

  8. Periventricular hyperintensity in children with hydrocephalus

    Energy Technology Data Exchange (ETDEWEB)

    Akbari, S.H.A.; Ragan, Dustin K. [St. Louis Children' s Hospital, Washington University School of Medicine, Department of Neurological Surgery, St. Louis, MO (United States); Limbrick, David D. [St. Louis Children' s Hospital, Washington University School of Medicine, Department of Neurological Surgery, St. Louis, MO (United States); McKinstry, Robert C.; Shimony, Joshua S. [St. Louis Children' s Hospital, Washington University School of Medicine, Mallinckrodt Institute of Radiology, St. Louis, MO (United States); Altaye, Mekibib [Cincinnati Children' s Hospital, Division of Biostatistics and Epidemiology, Cincinnati, OH (United States); Yuan, Weihong; Holland, Scott K. [Cincinnati Children' s Hospital, Department of Pediatric Radiology, Cincinnati, OH (United States); Mangano, Francesco T. [Cincinnati Children' s Hospital, Department of Pediatric Neurological Surgery, Cincinnati, OH (United States)

    2015-08-15

    Magnetic resonance images of children with hydrocephalus often include a rim of hyperintensity in the periventricular white matter (halo). The purpose of this study was to decide between the hypothesis that the halo is caused by cerebrospinal fluid (CSF) flow during the cardiac cycle, and the alternate hypothesis that the halo is caused by anatomical changes (stretching and compression of white matter). Participants were selected from a multicenter imaging study of pediatric hydrocephalus. We compared 19 children with hydrocephalus to a group of 52 controls. We quantified ventricle enlargement using the frontal-occipital horn ratio. We conducted qualitative and quantitative analysis of diffusion tensor imaging in the corpus callosum and posterior limb of the internal capsule. Parameters included the fractional anisotropy (FA), mean diffusivity, axial diffusivity and radial diffusivity. The halo was seen in 16 of the 19 children with hydrocephalus but not in the controls. The corpus callosum of the hydrocephalus group demonstrated FA values that were significantly decreased from those in the control group (P = 4 . 10{sup -6}), and highly significant increases were seen in the mean diffusivity and radial diffusivity in the hydrocephalus group. In the posterior limb of the internal capsule the FA values of the hydrocephalus group were higher than those for the control group (P = 0.002), and higher values in the hydrocephalus group were also noted in the axial diffusivity. We noted correlations between the diffusion parameters and the frontal-occipital horn ratio. Our results strongly support the hypothesis that the halo finding in hydrocephalus is caused by structural changes rather than pulsatile CSF flow. (orig.)

  9. Isolated neurosarcoidosis presenting with recurrent hydrocephalus

    Directory of Open Access Journals (Sweden)

    Frederick L Hitti

    2015-01-01

    Full Text Available Sarcoidosis is an inflammatory process that is characterized by the formation of noncaseating granulomas. This protean disease may afflict nearly any organ system, including the central nervous system. Here, we present a case of isolated neurosarcoidosis that initially presented with hydrocephalus requiring ventriculoperitoneal shunt placement. The patient's hydrocephalus recurred multiple times and required two additional shunt placements over the 3-year course of her illness. Due to the lack of systemic involvement, sarcoidosis was only diagnosed after a tissue biopsy of a Cauda equine lesion. This case highlights the importance of tissue diagnosis and the diagnostic workup for sarcoidosis in cases of cryptogenic hydrocephalus.

  10. Aqueductal stenosis and development of hydrocephalus in prenatal methylmercury poisoning in mice

    Energy Technology Data Exchange (ETDEWEB)

    Choi, B.H.; Caple, M.; Espinosa, T.; Kim, D.; Song, D.

    1986-03-01

    Methylmercury (MeHg) is a ubiquitous environmental pollutant that can cause irreparable CNS damage, particularly upon the developing fetal CNS. As an ongoing study of the effects of MeHg upon the developing CNS, timed-pregnant C57BL/6J mice were poisoned by intraperitoneal injections of methylmercuric chloride (MMC), 12 mg/kg, in divided doses on E-14, 15 and 16. In another experiment, the pregnant animals were fed MMC (2 mg/kg/day) in their drinking water, starting on E-2 and continuing throughout gestation. The animals were allowed to come to term and the offspring sacrificed on postnatal days 5, 10, and 20. In both of these experiments, up to 9% of the offspring affected by prenatal MeHg poisoning developed marked hydrocephalus. Serial on ..mu..m sections of the aqueductus revealed severe stenosis associated with marked edema and spongy changes of ependymal cells and surrounding neuropil. No inflammatory or gliotic reactions of the periaqueductal tissue was seen. These changes are very similar to those observed in congenital hydrocephalus in humans and suggest that toxic damage to developing ependymal cells due to prenatal exposures of agents such as MeHg may have etiological role in some of the human congenital hydrocephalus. The details of the scanning and transmission electron microscopy along with demonstration of mercury grains in tissues will be presented.

  11. Congenital TORCH infections of the brain--CT manifestation (with analysis of 7 cases)

    International Nuclear Information System (INIS)

    Li Xin; Li Minglin; Yang Zhiyong

    1997-01-01

    To study the neuropathologic changes and CT manifestations in congenital TORCH infection of the brain. Analysis of 7 cases of congenital TORCH infection of the neonates and infants demonstrated by serum examination was performed. There were congenital toxoplasmosis 3 cases, congenital syncytial virus infection 1 case, congenital rubella virus infection 1 case, congenital cytomegalovirus infection 2 cases, and congenital herpes simplex virus infection 1 case. Cerebral hypoplasia, ventricular dilatation or hydrocephalus, subependymal and parenchymal calcifications, microcephalic focal cortical migration anomalies, schizencephaly polymicrogyria, et al, were demonstrated by CT with congenital TORCH infection. The earlier the infection, the more severe the brain developmental anomalies. The extent and appearance of calcification in brain were related to the degree, extent and course of TORCH infection. Basal ganglia calcification of unknown cause in infant was suggestive of congenital TORCH infection. Typical CT manifestations together with clinical picture may suggest congenital TORCH infection, while serological test can be diagnostic

  12. Trends in hospitalization of preterm infants with intraventricular hemorrhage and hydrocephalus in the United States, 2000-2010.

    Science.gov (United States)

    Christian, Eisha A; Jin, Diana L; Attenello, Frank; Wen, Timothy; Cen, Steven; Mack, William J; Krieger, Mark D; McComb, J Gordon

    2016-03-01

    OBJECT Even with improved prenatal and neonatal care, intraventricular hemorrhage (IVH) occurs in approximately 25%-30% of preterm infants, with a subset of these patients developing hydrocephalus. This study was undertaken to describe current trends in hospitalization of preterm infants with posthemorrhagic hydrocephalus (PHH) using the Nationwide Inpatient Sample (NIS) and the Kids' Inpatient Database (KID). METHODS The KID and NIS were combined to generate data for the years 2000-2010. All neonatal discharges with ICD-9-CM codes for preterm birth with IVH alone or with IVH and hydrocephalus were included. RESULTS There were 147,823 preterm neonates with IVH, and 9% of this group developed hydrocephalus during the same admission. Of patients with Grade 3 and 4 IVH, 25% and 28%, respectively, developed hydrocephalus in comparison with 1% and 4% of patients with Grade 1 and 2 IVH, respectively. Thirty-eight percent of patients with PHH had permanent ventricular shunts inserted. Mortality rates were 4%, 10%, 18%, and 40%, respectively, for Grade 1, 2, 3, and 4 IVH during initial hospitalization. Length of stay has been trending upward for both groups of IVH (49 days in 2000, 56 days in 2010) and PHH (59 days in 2000, 70 days in 2010). The average hospital cost per patient (adjusted for inflation) has also increased, from $201,578 to $353,554 (for IVH) and $260,077 to $495,697 (for PHH) over 11 years. CONCLUSIONS The number of neonates admitted with IVH has increased despite a decrease in the number of preterm births. Rates of hydrocephalus and mortality correlated closely with IVH grade. The incidence of hydrocephalus in preterm infants with IVH remained stable between 8% and 10%. Over an 11-year period, there was a progressive increase in hospital cost and length of stay for preterm neonates with IVH and PHH that may be explained by a concurrent increase in the proportion of patients with congenital cardiac anomalies.

  13. Biodriven microsystem for treatment of hydrocephalus

    Science.gov (United States)

    Joswig, Jurgen; Oswald, Jens; Seifert, Steffen

    1995-09-01

    A microvalve system made of silicon for use in hydrocephalus therapy is presented, which will provide an excellent intracranial pressure stabilization. Design and processing are described. Testing results (in vitro) are presented.

  14. [Neurocysticercosis with hydrocephalus and secondary bilateral hemianopia].

    Science.gov (United States)

    Salcedo-Villanueva, G; Rueda-Villa, A; Hernández-Ábrego, M P

    2014-01-01

    A 45-year-old woman with a history of seizures, headaches, nausea, vomiting, and decreased visual acuity of 5 years. Visual field detected a bitemporal heteronymous hemianopia. Magnetic resonance imaging revealed basal cistern arachnoiditis and supratentorial hydrocephalus. Cranial computed tomography revealed supratentorial calcifications, scolex in the left occipital region, and hydrocephalus secondary to entrapment of the fourth ventricle. Neurocysticercosis can cause bitemporal hemianopsia due to chiasmatic compression secondary to obstructive hydrocephalus. The positivity of anti-cysticercus antibodies determined by ELISA evidence active disease. However patients with hydrocephalus and negative antigen may have sequelae of infection with non-living parasites. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

  15. A hydroelastic model of hydrocephalus

    Science.gov (United States)

    Smillie, Alan; Sobey, Ian; Molnar, Zoltan

    2005-09-01

    We combine elements of poroelasticity and of fluid mechanics to construct a mathematical model of the human brain and ventricular system. The model is used to study hydrocephalus, a pathological condition in which the normal flow of the cerebrospinal fluid is disturbed, causing the brain to become deformed. Our model extends recent work in this area by including flow through the aqueduct, by incorporating boundary conditions that we believe accurately represent the anatomy of the brain and by including time dependence. This enables us to construct a quantitative model of the onset, development and treatment of this condition. We formulate and solve the governing equations and boundary conditions for this model and give results that are relevant to clinical observations.

  16. Hydrocephalus

    Science.gov (United States)

    ... JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice . 7th ed. Philadelphia, PA: Elsevier; 2016:chap 88. Review Date 10/18/2017 Updated by: Neil K. Kaneshiro, MD, MHA, Clinical Professor of Pediatrics, University of Washington School of Medicine, ...

  17. Hydrocephalus

    Science.gov (United States)

    ... prevent injury Removing waste products of the brain's metabolism Flowing back and forth between the brain cavity ... spinal cord Central nervous system infections, such as bacterial meningitis or mumps Bleeding in the brain from ...

  18. CT in normal pressure hydrocephalus

    International Nuclear Information System (INIS)

    Fujita, Katsuzo; Nogaki, Hidekazu; Noda, Masaya; Kusunoki, Tadaki; Tamaki, Norihiko

    1981-01-01

    CT scans were obtained on 33 patients (age 73y. to 31y.) with the diagnosis of normal pressure hydrocephalus. In each case, the diagnosis was made on the basis of the symptoms, CT and cisternographic findings. Underlying diseases of normal pressure hydrocephalus are ruptured aneurysms (21 cases), arteriovenous malformations (2 cases), head trauma (1 case), cerebrovascular accidents (1 case) and idiopathie (8 cases). Sixteen of 33 patients showed marked improvement, five, moderate or minimal improvement, and twelve, no change. The results were compared with CT findings and clinical response to shunting. CT findings were classified into five types, bases on the degree of periventricular hypodensity (P.V.H.), the extent of brain damage by underlying diseases, and the degree of cortical atrophy. In 17 cases of type (I), CT shows the presence of P.V.H. with or without minimal frontal lobe damage and no cortical atrophy. The good surgical improvements were achieved in all cases of type (I) by shunting. In 4 cases of type (II), CT shows the presence of P.V.H. and severe brain damage without cortical atrophy. The fair clinical improvements were achieved in 2 cases (50%) by shunting. In one case of type (III), CT shows the absence of P.V.H. without brain damage nor cortical atrophy. No clinical improvement was obtained by shunting in this type. In 9 cases of type (IV) with mild cortical atrophy, the fair clinical improvement was achieved in two cases (22%) and no improvement in 7 cases. In 2 cases of type (V) with moderate or marked cortical atrophy, no clinical improvement was obtained by shunting. In conclusion, it appeared from the present study that there was a good correlation between the result of shunting and the type of CT, and clinical response to shunting operation might be predicted by classification of CT findings. (author)

  19. Infantile hydrocephalus: a review of epidemiology, classification and causes

    Science.gov (United States)

    Tully, Hannah M; Dobyns, William B

    2015-01-01

    Hydrocephalus is a common but complex condition caused by physical or functional obstruction of CSF flow that leads to progressive ventricular dilatation. Though hydrocephalus was recently estimated to affect 1.1 in 1,000 infants, there have been few systematic assessments of the causes of hydrocephalus in this age group, which makes it a challenging condition to approach as a scientist or as a clinician. Here, we review contemporary literature on the epidemiology, classification and pathogenesis of infantile hydrocephalus. We describe the major environmental and genetic causes of hydrocephalus, with the goal of providing a framework to assess infants with hydrocephalus and guide future research. PMID:24932902

  20. Complete dorsal pancreatic agenesis and unilateral renal agenesis.

    Science.gov (United States)

    Moreira, Adriana; Carvalho, André; Portugal, Inês; Jesus, José Miguel

    2018-02-01

    Dorsal pancreatic agenesis is a very rare congenital anomaly. Unilateral renal agenesis, on the other hand, is a relatively common congenital anomaly, although its etiology is not fully understood. Renal and pancreatic embryologic development appears to be nonrelated. We report a case of a 34-year-old man who was referred to our hospital for evaluation of cholestasis and microalbuminuria. Ultrasound and magnetic resonance imaging examinations showed empty right renal fossa and absence of the pancreatic neck, body, and tail. Our case report is the second case of a dorsal pancreatic agenesis and unilateral renal agenesis in a young male patient.

  1. CT angiography of mildly symptomatic, isolated, unilateral right pulmonary vein atresia

    Energy Technology Data Exchange (ETDEWEB)

    Mataciunas, Mindaugas; Tamosiunas, Algirdas E. [Vilnius University, Faculty of Medicine, Clinic of Thoracic Diseases, Allergology and Radiology, Vilnius (Lithuania); Gumbiene, Lina; Cibiras, Sigitas; Tarutis, Virgilijus [Vilnius University, Faculty of Medicine, Clinic of Cardiac and Vascular Diseases, Vilnius (Lithuania)

    2009-10-15

    We report a mildly symptomatic 12-year-old boy with a very rare congenital anomaly - isolated unilateral pulmonary vein atresia. Diagnosis was made using CT angiography and its role in diagnosis is discussed. (orig.)

  2. Complete dorsal pancreatic agenesis and unilateral renal agenesis

    OpenAIRE

    Moreira, Adriana; Carvalho, André; Portugal, Inês; Jesus, José Miguel

    2017-01-01

    Dorsal pancreatic agenesis is a very rare congenital anomaly. Unilateral renal agenesis, on the other hand, is a relatively common congenital anomaly, although its etiology is not fully understood. Renal and pancreatic embryologic development appears to be nonrelated. We report a case of a 34-year-old man who was referred to our hospital for evaluation of cholestasis and microalbuminuria. Ultrasound and magnetic resonance imaging examinations showed empty right renal fossa and absence of the ...

  3. Complete dorsal pancreatic agenesis and unilateral renal agenesis

    OpenAIRE

    Adriana Moreira, MD; André Carvalho, MD; Inês Portugal, MD; José Miguel Jesus, MD

    2018-01-01

    Dorsal pancreatic agenesis is a very rare congenital anomaly. Unilateral renal agenesis, on the other hand, is a relatively common congenital anomaly, although its etiology is not fully understood. Renal and pancreatic embryologic development appears to be nonrelated. We report a case of a 34-year-old man who was referred to our hospital for evaluation of cholestasis and microalbuminuria. Ultrasound and magnetic resonance imaging examinations showed empty right renal fossa and absence of the ...

  4. Congenital ankyloblepharon in a leopard gecko (Eublepharis macularius).

    Science.gov (United States)

    Rival, Franck

    2015-01-01

    A 6-month-old leopard gecko with unilateral partially fused eyelids since birth was presented for examination. A diagnosis of congenital ankyloblepharon was made and surgical correction was performed successfully. © 2014 American College of Veterinary Ophthalmologists.

  5. [2 cases of congenital humero-radial synostosis].

    Science.gov (United States)

    Sólyom, L

    1978-01-01

    Two cases of a rare congenital deformity, humeroradial aplasia are reported in the article. In the first case the deformity was unilateral, in the second case a bilateral aplasia was associated to hip dislocation and aplasia of the os ischii.

  6. Childhood hydrocephalus – is radiological morphology associated with etiology

    DEFF Research Database (Denmark)

    Foss-Skiftesvik, Jon; Andresen, Morten; Juhler, Marianne

    2013-01-01

    Clinicians use a non-standardized, intuitive approach when correlating radiological morphology and etiology of hydrocephalus.......Clinicians use a non-standardized, intuitive approach when correlating radiological morphology and etiology of hydrocephalus....

  7. Cerebral hemodynamics and functional prognosis in hydrocephalus

    International Nuclear Information System (INIS)

    Hirai, Osamu; Nishikawa, Michio; Watanabe, Shu; Yamakawa, Hiroyasu; Kinoshita, Yoshimasa; Uno, Akira; Handa, Hajime

    1989-01-01

    The functional outcome of cerebral hemodynamics in the chronic stage of juvenile hydrocephalus was determined using single photon emission computed tomography (SPECT). Five patients including three with aqueductal stenosis, one with post-meningitic hydrocephalus, and one case with hydrocephalus having developed after repair of a huge occipital encephalocele. Early images of cerebral blood flow (CBF) were obtained 25 minutes after intravenous injection of 123-I-iodoamphetamine (IMP), and late images were scanned 3 hours later. Cerebral blood volume (CBV) was also measured using 99m Tc in three patients. Twenty cases with adult communicating hydrocephalus were also investigated from the point of view of shunt effectiveness. Although there was no remarkable change in the cerebrovascular bed in the juvenile cases, CBF of the remnant brain parenchyma was good irrespective of the degree of ventricular dilatation. There was a periventricular-related IMP uptake in each case; however, it somehow matched the ventricular span. Functional outcome one to 23 years after the initial shunt operation was good in every case, despite multiple shunt revisions. Redistribution on late images had no bearing on clinical states. In adult cases, 8 patients with effective shunting demonstrated a relatively localized periventricular low perfusion, with preoperative increased cerebrospinal fluid (CSF) pressure. On the contrary, 12 patients with no improvement with or without ventricular-reduced IMP uptake, despite low CSF pressure. The present study indicates that periventricular hemodynamics may play an important role in cerebral function compromised by hydrocephalus. (J.P.N.)

  8. Cerebral hemodynamics and functional prognosis in hydrocephalus

    Energy Technology Data Exchange (ETDEWEB)

    Hirai, Osamu; Nishikawa, Michio; Watanabe, Shu; Yamakawa, Hiroyasu; Kinoshita, Yoshimasa; Uno, Akira; Handa, Hajime (Hamamatsu Rosai Hospital, Shizuoka (Japan))

    1989-11-01

    The functional outcome of cerebral hemodynamics in the chronic stage of juvenile hydrocephalus was determined using single photon emission computed tomography (SPECT). Five patients including three with aqueductal stenosis, one with post-meningitic hydrocephalus, and one case with hydrocephalus having developed after repair of a huge occipital encephalocele. Early images of cerebral blood flow (CBF) were obtained 25 minutes after intravenous injection of 123-I-iodoamphetamine (IMP), and late images were scanned 3 hours later. Cerebral blood volume (CBV) was also measured using {sup 99m}Tc in three patients. Twenty cases with adult communicating hydrocephalus were also investigated from the point of view of shunt effectiveness. Although there was no remarkable change in the cerebrovascular bed in the juvenile cases, CBF of the remnant brain parenchyma was good irrespective of the degree of ventricular dilatation. There was a periventricular-related IMP uptake in each case; however, it somehow matched the ventricular span. Functional outcome one to 23 years after the initial shunt operation was good in every case, despite multiple shunt revisions. Redistribution on late images had no bearing on clinical states. In adult cases, 8 patients with effective shunting demonstrated a relatively localized periventricular low perfusion, with preoperative increased cerebrospinal fluid (CSF) pressure. On the contrary, 12 patients with no improvement with or without ventricular-reduced IMP uptake, despite low CSF pressure. The present study indicates that periventricular hemodynamics may play an important role in cerebral function compromised by hydrocephalus. (J.P.N.).

  9. Congenital hemifacial hyperplasia

    Directory of Open Access Journals (Sweden)

    S A Deshingkar

    2011-01-01

    Full Text Available Congenital hemifacial hyperplasia (CHH is a rare congenital malformation characterized by marked unilateral overdevelopment of hard and soft tissues of the face. Asymmetry in CHH is usually evident at birth and accentuated with age, especially at puberty. The affected side grows at a rate proportional to the nonaffected side so that the disproportion is maintained thr oughout the life. Multisystem involvement has resulted in etiological heterogeneity including heredity, chromosomal abnormalities, atypical forms of twinning, altered intrauterine environment, and endocrine dysfunctions; however, no single theory explains the etiology adequately. Deformities of all tissues of face, including teeth and their related tissues in the jaw, are key findings for correct diagnosis of CHH. Here an attempt has been made to present a case of CHH with its archetypal features and to supplement existing clinical knowledge.

  10. Communicating hydrocephalus. Cisternographic and neuropathologic studies

    International Nuclear Information System (INIS)

    Price, D.L.; James, E.; Sperber, E.; Strecker, E.P.

    1976-01-01

    Chronic communicating hydrocephalus was produced in adult dogs by injection of silastic into the subarachnoid space. Electron microscopy was used to verify the sequence of pathologic changes in the ventricular wall. The pathologic findings were correlated with cisternographic images and measurements of cerebrospinal fluid (CSF) pressure. Early in hydrocephalus, the CSF pressure was increased and cisternograms showed ventricular entry and clearing; the ependyma was stretched and fluid accumulated in subependymal regions. In animals with chronic hydrocephalus, the CSF pressure was normal and cisternograms disclosed radioactivity persisting in the ventricles. At this time the ependyma was severely damaged, the subependymal white matter showed enlargement of the extracellular space, and degenerative changes were present in axons and myelin sheaths

  11. Simulation of hydrocephalus condition in infant head

    Science.gov (United States)

    Wijayanti, Erna; Arif, Idam

    2014-03-01

    Hydrocephalus is a condition of an excessive of cerebrospinal fluid in brain. In this paper, we try to simulate the behavior of hydrocephalus conditions in infant head by using a hydro-elastic model which is combined with orthotropic elastic skull and with the addition of suture that divide the skull into two lobes. The model then gives predictions for the case of stenosis aqueduct by varying the cerebral aqueduct diameter, time constant and brain elastic modulus. The hydrocephalus condition which is shown by the significant value of ventricle displacement, as the result shows, is occurred when the aqueduct is as resistant as brain parenchyma for the flow of cerebrospinal fluid. The decrement of brain elastic modulus causes brain parenchyma displacement value approach ventricle displacement value. The smaller of time constant value causes the smaller value of ventricle displacement.

  12. Ruptured Massa Intermedia Secondary to Hydrocephalus.

    Science.gov (United States)

    El Damaty, Ahmed; Langner, Soenke; Schroeder, Henry W S

    2017-01-01

    We report a case of ruptured massa intermedia (MI) as a sequela of hydrocephalus. A single case report is presented describing the sequelae of tumor bed hematoma after a posterior fossa hemangioblastoma resection in which the patient bled 3 days after surgery, resulting in secondary hydrocephalus and subsequently dilatation of the third ventricle, which resulted in rupture of the MI. The patient was managed on emergency basis with an external ventricular drain then endoscopically with a third ventriculostomy and clot extraction. Absent MI is not uncommon in hydrocephalic patients, and it is assumed to be the result of rupture from acute dilatation of the third ventricle. Our case report proves this assumption and documents the presence and absence of the MI before and after developing hydrocephalus. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Unilateral Pulmonary Agenesis and Gastric Duplication Cyst: A Rare Association

    OpenAIRE

    Halilbasic, Amir; Skokic, Fahrija; Hotic, Nesad; Husaric, Edin; Radoja, Gordana; Muratovic, Selma; Dedic, Nermina; Halilbasic, Meliha

    2013-01-01

    Lung agenesis and gastric duplication cysts are both rare congenital anomalies. Gastric duplication cysts can present with nausea, vomiting, hematemesis, or vague abdominal pain. Unilateral pulmonary agenesis can present with respiratory distress which usually occurs due to retention of bronchial secretions and inflammations. We report the unique case of right pulmonary agenesis associated with gastric duplication cyst.

  14. Posttraumatic hydrocephalus associated with decompressive cranial defect in severe brain-injured patients

    Directory of Open Access Journals (Sweden)

    SHI Song-sheng

    2012-02-01

    Full Text Available 【Abstract】Objective: To investigate the occurrence of posttraumatic hydrocephalus (PTH in severe brain- injured patients who underwent decompressive craniectomy (DC and to discuss the management. Methods: A total of 389 patients suffering from severe head trauma between January 2004 and May 2010 were enrolled in this study. Clinical data were analyzed retrospectively. Of them, 149 patients who underwent DC were divided into two groups according to the presence of PTH: hydrocephalus group and nonhydrocephalus group. Clinical factors including preoperative Glasgow Coma Score (GCS, bilateral or unilateral decompression, and duraplasty in DC were assessed by single factor analysis to determine its relationship with the occurrence of PTH. Results: Of the 149 patients undergoing DC, 25 (16.8% developed PTH; while 23 developed PTH (9.6% among the rest 240 patients without DC. Preoperative GCS, bilateral or unilateral decompression, duraplasty in DC were significantly associated with the development of PTH. Ventriculoperitoneal shunt was performed on 23 of 25 patients with PTH after DC. Frontal horn was preferred for the placement of the catheter. Sixteen of them were operated upon via frontal approach and 7 via occipital approach. After shunt surgery, both radiological and clinical improvements were confirmed in 19 patients. Radiological improvement was found in 2 patients. One patient died eventually of severe pneumonia. Shunt-related infection occurred in 1 patient, which led to the removal of the catheter. Conclusions: It is demonstrated that the occurrence of PTH is high in patients with large decompressive skull defect. Patients with low GCS and bilateral decompression tend to develop PTH after DC. Duraplasty in DC might facilitate reducing the occurrence of PTH. Patients with PTH concomitant skull defect should be managed deliberately to restore the anatomical and physiological integrity so as to facilitate the neurological resuscitation. Key

  15. A mutation inCcdc39causes neonatal hydrocephalus with abnormal motile cilia development in mice.

    Science.gov (United States)

    Abdelhamed, Zakia; Vuong, Shawn M; Hill, Lauren; Shula, Crystal; Timms, Andrew; Beier, David; Campbell, Kenneth; Mangano, Francesco T; Stottmann, Rolf W; Goto, June

    2018-01-09

    Pediatric hydrocephalus is characterized by an abnormal accumulation of cerebrospinal fluid (CSF) and is one of the most common congenital brain abnormalities. However, little is known about the molecular and cellular mechanisms regulating CSF flow in the developing brain. Through whole-genome sequencing analysis, we report that a homozygous splice site mutation in coiled-coil domain containing 39 ( Ccdc39 ) is responsible for early postnatal hydrocephalus in the progressive hydrocephal us ( prh ) mouse mutant. Ccdc39 is selectively expressed in embryonic choroid plexus and ependymal cells on the medial wall of the forebrain ventricle, and the protein is localized to the axoneme of motile cilia. The Ccdc39 prh/prh ependymal cells develop shorter cilia with disorganized microtubules lacking the axonemal inner arm dynein. Using high-speed video microscopy, we show that an orchestrated ependymal ciliary beating pattern controls unidirectional CSF flow on the ventricular surface, which generates bulk CSF flow in the developing brain. Collectively, our data provide the first evidence for involvement of Ccdc39 in hydrocephalus and suggest that the proper development of medial wall ependymal cilia is crucial for normal mouse brain development. © 2018. Published by The Company of Biologists Ltd.

  16. Unilateral pulmonary veins atresia: evaluation by computed tomography; Atresia unilateral das veias pulmonares: avaliacao por tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Eifer, Diego Andre; Arsego, Felipe Veras, E-mail: felipesoarestorres@gmail.com [Hospital de Clinicas de Porto Alegre (HCPA), RS (Brazil). Serv. de Radiologia; Torres, Felipe Soares [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Escola de Medicina

    2013-11-15

    Unilateral pulmonary vein atresia is a rare congenital condition. In addition to cardiac malformations or pulmonary hypertension, patients may present with recurrent pulmonary infections or hemoptysis in childhood or adolescence. The authors report a case where the typical findings of such condition were observed at computed tomography in an adult patient. (author)

  17. Congenital toxoplasmosis

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/001360.htm Congenital toxoplasmosis To use the sharing features on this page, please enable JavaScript. Congenital toxoplasmosis is a group of symptoms that occur when ...

  18. Congenital Hypothyroidism

    Science.gov (United States)

    ... Disease Featured Resource Find an Endocrinologist Search Congenital Hypothyroidism March 2012 Download PDFs English Espanol Editors Rosalind S. ... Resources MedlinePlus (NIH) Mayo Clinic What is congenital hypothyroidism? Newborn babies who are unable to make enough ...

  19. Agenesis of internal carotid artery associated with congenital anterior hypopituitarism

    Energy Technology Data Exchange (ETDEWEB)

    Moon, W.-J. [Department of Diagnostic Radiology, Samsung Medical Center, Seoul (Korea); Institute of Neuroradiology, University of Frankfurt (Germany); Porto, L.; Lanfermann, H.; Zanella, F.E. [Institute of Neuroradiology, University of Frankfurt (Germany); Weis, R. [Department of Pediatric Neurology, University of Frankfurt (Germany)

    2002-02-01

    We report a rare case of unilateral agenesis of the internal carotid artery in association with congenital anterior hypopituitarism. The collateral circulation is supplied by a transsellar intercavernous anastomotic vessel connecting the internal carotid arteries. These abnormalities are well depicted on MRI and MRA. The agenesis of the internal carotid artery may explain the pathogenesis of some of congenital anterior hypopituitarism. (orig.)

  20. Agenesis of internal carotid artery associated with congenital anterior hypopituitarism

    International Nuclear Information System (INIS)

    Moon, W.-J.; Porto, L.; Lanfermann, H.; Zanella, F.E.; Weis, R.

    2002-01-01

    We report a rare case of unilateral agenesis of the internal carotid artery in association with congenital anterior hypopituitarism. The collateral circulation is supplied by a transsellar intercavernous anastomotic vessel connecting the internal carotid arteries. These abnormalities are well depicted on MRI and MRA. The agenesis of the internal carotid artery may explain the pathogenesis of some of congenital anterior hypopituitarism. (orig.)

  1. Uptake of [14C]deoxyglucose into brain of young rats with inherited hydrocephalus

    International Nuclear Information System (INIS)

    Richards, H.K.; Bucknall, R.M.; Jones, H.C.; Pickard, J.D.

    1989-01-01

    The effect of hydrocephalus on cerebral glucose utilization as reflected by deoxyglucose uptake has been examined in rats with inherited hydrocephalus at 10, 20, and 28 days after birth using a semiquantitative method. Injection of [14C]deoxyglucose intraperitoneally was followed by freezing the brain, sectioning, and quantitative autoradiography of 10 brain regions. Brain [14C] concentration, cortical thickness, and plasma glucose concentrations were measured. Maximal thinning of the cerebral cortex had already occurred by 10 days after birth, although obvious symptoms such as gait disturbance developed after 20 days. In control rats, the cerebral isotope concentration was lower and more homogeneous at 10 days than at 20 or 28 days, which may be a reflection of the use of metabolic substrates other than glucose in younger animals. In order to make comparisons between control and hydrocephalic groups, tissue isotope concentrations were normalized to cerebellar cortex which was not affected by the hydrocephalus at any age. In hydrocephalic rats at 10 and 20 days, the concentration of [14C] was lower in all areas except the inferior colliculi and pons but the reduction was only significant in the sensory-motor cortex at 10 days and in the caudate nuclei at 20 days. By 28 days after birth, all areas except the cerebellum (six cortical regions, inferior colliculi, pons, and caudate) had significantly lower isotope concentrations in the hydrocephalic group. It is concluded that cerebral glucose metabolism is significantly reduced by 28 days after birth in H-Tx rats with congenital hydrocephalus and that less marked reductions occur prior to 28 days

  2. Neuropathology and Structural Changes in Hydrocephalus

    Science.gov (United States)

    Del Bigio, Marc R.

    2010-01-01

    In the context of spina bifida, hydrocephalus is usually caused by crowding of the posterior fossa with obstruction to cerebrospinal fluid flow from the forth ventricle, and less often by malformation of the cerebral aqueduct. Enlargement of the cerebral ventricles causes gradual destruction of periventricular white matter axons. Motor, sensory,…

  3. Unilateral Nevoid Telangiectasis: Report of Five Cases

    Directory of Open Access Journals (Sweden)

    Zehra Aşiran Serdar

    2010-12-01

    Full Text Available Unilateral nevoid telangiectasia (UNT is a rare cutaneous disorder characterized by superficial telangiectasias distributed unilaterally. UNT can be congenital or acquired. Acquired UNT may be seen during pregnancy, puberty, and in association with cirrhosis and hepatitis C. Besides, the disease has been reported in healthy individuals. In the etiology, elevated blood estrogen levels and/or increased estrogen and progesterone receptors in the involved skin are suggested to cause this disorder. Here, we present four male patients aged 26-33 years, without an identified underlying disease and an 18-year-old female hepatitis B carrier with diagnosis of acquired UNT. Since UNT is seen rarely, it is discussed in the light of the literature data.

  4. Encephalocele development from a congenital meningocele: case report.

    Science.gov (United States)

    Gandhoke, Gurpreet S; Goldschmidt, Ezequiel; Kellogg, Robert; Greene, Stephanie

    2017-11-01

    A fetal MRI study obtained at 21 weeks' gestation revealed a suboccipital meningocele without hydrocephalus. One day after term birth, MRI demonstrated an acquired cerebellar encephalocele, and MRI obtained 5 months later showed progressive enlargement of the encephalocele, still without obvious hydrocephalus. The patient underwent an operation in which an external ventricular drain was placed, the grossly normal cerebellum was reduced into the posterior fossa without resection, and the dural defect was closed. The drain was weaned out over 5 days, and no ventriculoperitoneal shunt was placed. Postoperative MR images revealed normal cerebellum and no hydrocephalus. The patient is developmentally normal. Meningocele and encephalocele are embryologically distinct. An acquired encephalocele could develop from hydrocephalus (which was not present in this case), or secondary to the lower resistance to expansion into the dural defect of the meningocele relative to the resistance to expansion of the fetal skull. The cerebellar tissue was normal in this case, and was thus preserved. The developmental prognosis is excellent. To the authors' knowledge, this is the first reported case of this occurrence. It is important to differentiate between congenital and acquired encephalocele etiologies, because resection of the cerebellar tissue in an acquired encephalocele (as is routinely done in cases of congenital encephalocele) would be expected to result in neurological deficits.

  5. Unilateral pulmonary vein atresia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, So Hwa; Kim, Ki Jun [Dept. of Radiology, Incheon St. Mary' s Hospital, The Catholic University of Korea College of Medicine, Incheon (Korea, Republic of)

    2014-01-15

    Unilateral pulmonary vein atresia is a rare congenital anomaly. Its symptoms begin to manifest in childhood and a broad spectrum of clinical severity has been described, ranging from asymptomatic, recurrent pulmonary infection, severe hemoptysis, to death. Only a few adult cases with this condition, with no or mild symptoms, have been reported. Pulmonary angiography has been typically used for definite diagnosis. However, pulmonary angiography may be replaced with the current developing multidetector CT. This report presents an adult case with mild symptoms, diagnosed by multidetector CT.

  6. A family study of hydrocephalus resulting from aqueduct stenosis.

    Science.gov (United States)

    Howard, F M; Till, K; Carter, C O

    1981-01-01

    Stenosis of the aqueduct of Sylvius accounts for about one third of cases of congenital hydrocephalus. At least 32 families have been reported in which the aqueduct stenosis is inherited in an X linked fashion. In half of these families, flexed adducted thumbs were noted in some affected family members. Occasionally other male members were mentally retarded, suggesting limited expression of the gene. The problem of giving genetic advice to an isolated, clinically unremarkable, case of aqueduct stenosis remains, so a family study was undertaken based on 24 such cases seen at The Hospital for Sick Children over a 19-year period. There were 15 male and nine female index patients. The diagnosis was confirmed in all cases by air encephalogram. One boy had a radial club hand and another developed clasped thumbs secondary to spasticity. No cases had hyaloidoretinal dysplasia. The 15 boys had 18 brothers and 19 sisters, of whom one sister was similarly affected. The nine girls had 12 sibs, none of whom was affected. This study, combined with a similar study in the USA, suggests that the empirical risk of recurrence of a sporadic case of aqueduct stenosis is about 4.5%. PMID:7277416

  7. Congenital Pseudoarthrosis of the clavicle - Case report

    International Nuclear Information System (INIS)

    Holguin Maldonado, Esteban; Rossellli Cock, Pablo

    2006-01-01

    Congenital pseudoarthrosis of the clavicle (CPC) is a tare malformation of the scapular waist of unknown etiology. The lesion is usually unilateral and right sided. It is seldom symptomatic and X rays confirm the diagnosis. Treatment is usually non surgical, unless functional or cosmetic compromise of the shoulder is present

  8. Subclinical Congenital Cytomegalovirus Infection and Hearing Impairment

    Science.gov (United States)

    Dahle, Arthur J.; And Others

    1974-01-01

    When the hearing sensitivity of children with subclinical congenital cytomegalovirus infection was evaluated and compared with that of a group of matched control subjects, nine of the 18 infected subjects were found to have some hearing loss, ranging from slight high-frequency impairments to a severe-to-profound unilateral loss. (MYS)

  9. Glymphatic MRI in idiopathic normal pressure hydrocephalus.

    Science.gov (United States)

    Ringstad, Geir; Vatnehol, Svein Are Sirirud; Eide, Per Kristian

    2017-10-01

    The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer's disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic function. In

  10. 236 children with developmental hydrocephalus: causes and clinical consequences

    Science.gov (United States)

    Tully, Hannah M; Ishak, Gisele E; Rue, Tessa C; Dempsey, Jennifer C; Browd, Samuel R; Millen, Kathleen J; Doherty, Dan; Dobyns, William B

    2016-01-01

    Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed MRIs and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to one of five subtypes and compared on the basis of clinical characteristics, developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth. 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. 53% of surgically-treated patients experienced at least one shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms. PMID:26184484

  11. Mechanisms of Hydrocephalus after Neonatal and Adult Intraventricular Hemorrhage

    OpenAIRE

    Strahle, Jennifer; Garton, Hugh J.L.; Maher, Cormac O.; Muraszko, Karin M.; Keep, Richard F.; Xi, Guohua

    2012-01-01

    Intraventricular hemorrhage (IVH) is a cause of significant morbidity and mortality and is an independent predictor of a worse outcome in intracerebral hemorrhage (ICH) and germinal matrix hemorrhage (GMH). IVH may result in both injuries to the brain as well as hydrocephalus. This paper reviews evidence on the mechanisms and potential treatments for IVH-induced hydrocephalus. One frequently cited theory to explain hydrocephalus after IVH involves obliteration of the arachnoid villi by microt...

  12. Prevalence of hydrocephalus in 157 patients with vestibular schwannoma

    Energy Technology Data Exchange (ETDEWEB)

    Rogg, Jeffrey M.; Ahn, S.H.; Tung, G.A. [Rhode Island Hospital, Department of Diagnostic Imaging, Providence, Rhode Island (United States); Reinert, S.E. [Rhode Island Hospital, Lifespan Medical Computing, Providence, Rhode Island (United States); Noren, G. [Rhode Island Hospital, Department of Neurosurgery, Providence, Rhode Island (United States)

    2005-05-01

    The purpose of this study was to determine the prevalence of hydrocephalus in patients with vestibular schwannoma. A second objective was to investigate possible etiologies for hydrocephalus in this population by attempting to correlate the incidence and severity of hydrocephalus with tumor volume and extent of fourth ventricular compression. The MRI examinations of 157 adult patients with vestibular schwannoma were retrospectively reviewed. Tumor size was quantified, and the presence of accompanying hydrocephalus was assessed, categorized as communicating type or non-communicating type and then rated as mild, moderate or severe (grades 1-3). Next, the degree of fourth ventricular distortion caused by tumor mass effect was evaluated and categorized as mild, moderate or severe (grades 1-3). Spearman's rank correlation coefficient was used to test the relationships between tumor volume and (1) the extent of fourth ventricular effacement and (2) severity of hydrocephalus. Hydrocephalus was present in 28/157 (18%) cases and was categorized as mild in 11/28 (39%), moderate in 15/28 (54%) and severe in 2/28 (7%). Communicating-type hydrocephalus was present in 17/28 (61%) and non-communicating type in 11/28 (39%). There was a positive correlation between the grade of non-communicating hydrocephalus and tumor volume (r=0.38; P<0.001) and between the severity of fourth ventricular compression and extent of hydrocephalus in this group(r=0.43; P<0.001). (orig.)

  13. Mechanisms of hydrocephalus in cerebral cysticercosis: implications for therapy

    Energy Technology Data Exchange (ETDEWEB)

    Estanol, B.; Kleriga, E.; Loyo, M.; Mateos, H.; Lombardo, L.; Gordon, F.; Saguchi, A.F.

    1983-08-01

    Patients with hydrocephalus secondary to cerebral cysticercosis are a highly heterogeneous group. The mechanisms of hydrocephalus in these patients are multiple. Intraventricular cysts may be found in the 3rd and 4th ventricles, the sylvian aqueduct, and the foramen of Monro. Intraventricular cysts can be suspected when the 3rd and 4th ventricles or aqueduct remain enlarged despite shunting. Intraventricular contrast medium demonstrates the presence of the parasites. The intraventricular cysts should be removed surgically. Hydrocephalus due to cisternal cysticercosis can be diagnosed by isotope cisternography. These patients should receive shunts, but the long term prognosis is probably poor. Guidelines for the management of hydrocephalus due to cysticercosis are suggested.

  14. Stereotactic CO2 laser therapy for hydrocephalus

    Science.gov (United States)

    Kozodoy-Pins, Rebecca L.; Harrington, James A.; Zazanis, George A.; Nosko, Michael G.; Lehman, Richard M.

    1994-05-01

    A new fiber-optic delivery system for CO2 radiation has been used to successfully treat non-communicating hydrocephalus. This system consists of a hollow sapphire waveguide employed in the lumen of a stereotactically-guided neuroendoscope. CO2 gas flows through the bore of the hollow waveguide, creating a path for the laser beam through the cerebrospinal fluid (CSF). This delivery system has the advantages of both visualization and guided CO2 laser radiation without the same 4.3 mm diameter scope. Several patients with hydrocephalus were treated with this new system. The laser was used to create a passage in the floor of the ventricle to allow the flow of CSF from the ventricles to the sub-arachnoid space. Initial postoperative results demonstrated a relief of the clinical symptoms. Long-term results will indicate if this type of therapy will be superior to the use of implanted silicone shunts. Since CO2 laser radiation at 10.6 micrometers is strongly absorbed by the water in tissue and CSF, damage to tissue surrounding the lesion with each laser pulse is limited. The accuracy and safety of this technique may prove it to be an advantageous therapy for obstructive hydrocephalus.

  15. Congenital tuberculosis

    African Journals Online (AJOL)

    Prof Ezechukwu

    2012-06-20

    Jun 20, 2012 ... Key words: Congenital tuberculo- sis, case report, miliary tuberculosis. Introduction. Congenital tuberculosis defines tuberculosis in infants of .... tary TB and otitis media, resulting in seizures, deafness, and death. It is therefore not surprising that the index case who presented at twelve weeks of age, had ...

  16. Congenital Myopathy

    Science.gov (United States)

    ... results in weakness. Congenital myopathy refers to a group of muscle disorders that appear at birth or in infancy. Typically, an infant with a congenital myopathy will be "floppy," have difficulty breathing or feeding, and will lag behind other babies in meeting ...

  17. Congenital Myasthenia

    Science.gov (United States)

    ... Symptoms of congenital myasthenia usually appear in the first few years of childhood, but may not be noticeable until much later, ... Symptoms of congenital myasthenia usually appear in the first few years of childhood, but may not be noticeable until much later, ...

  18. [Unilateral forearm agenesis and prosthesis].

    Science.gov (United States)

    Choumon, B; Ritz, A; Corbet, E; Gréco, J; Bérard, C

    2002-04-01

    Antebrachial agenesis is a congenital deformity which is not invalidating for the children who have a level of independence comparable to that of other children of the same age. Although the appropriateness of a prosthesis is rarely questioned by healthcarers, it is clear that it is the healthcarers' own representation of this condition (their knowledge of the deformity and of the prosthesis proposed) that leads to the indication. The purpose of this study was to better understand the respective representations of unilateral antebrachial agenesis to help determine appropriate health care proposals. An intensive survey using semi-directive interviews was conducted in 16 families. Group interviews with three healthcare teams were then conducted. Three leading topics appeared: worry about the social integration of the child, a paradoxical representation of the child perceived as independent but handicapped, and a largely negative image of the prosthesis. There was a rather important difference in the representations formulated by the parents and by the healthcarers. The discussion focused on awareness of the narcissistic content of the expectations and the plastic and functional implications of prosthesis fitting, perceived differently by parents and healthcarers. The nature of the expected result involves a change in the representation of the child more than a change in the child's body, a concept which in itself is not a true objective of healthcare. Taken the understandably difficult position of healthcarers, it might be useful to propose a different scheme for the first consultation.

  19. Abnormalities of the oculomotor nerve in congenital fibrosis of the extraocular muscles and congenital oculomotor palsy.

    Science.gov (United States)

    Lim, Key Hwan; Engle, Elizabeth C; Demer, Joseph L

    2007-04-01

    High-resolution magnetic resonance imaging (MRI) can now directly demonstrate innervation to extraocular muscles and quantify optic nerve size. A quantitative MRI technique was developed to study the oculomotor nerve (CN3) and applied to congenital fibrosis of extraocular muscles (CFEOM) and congenital oculomotor palsy. The subarachnoid portions of the CN3s were imaged with a 1.5-T MRI scanner and conventional head coils, acquiring heavily T(2)-weighted oblique axial planes 1-mm thick and parallel to the optic chiasm. Thirteen normal subjects, 14 with CFEOM, and 3 with congenital CN3 palsy were included. Digital image analysis was used to measure CN3 diameter, which was correlated with motility findings. In CFEOM, CN3 diameter was bilaterally subnormal in eight subjects, unilaterally subnormal in three subjects, and normal in three subjects. Mean +/- SD CN3 diameter in CFEOM was 1.14 +/- 0.61 mm, significantly smaller than the diameter in normal subjects, which measured 2.01 +/- 0.36 mm (P congenital CN3 palsy showed bilateral CN3 hypoplasia, but CN3 diameter was normal in two other subjects with congenital CN3 palsy. Unilateral or bilateral hypoplasia of CN3 is quantitatively demonstrable using MRI in many cases of CFEOM and occasionally in congenital CN3 palsy. Variations in CN3 diameter in CFEOM and congenital CN3 palsy suggest mechanistic heterogeneity of these disorders that may be clarified by further imaging and genetic studies.

  20. Phenotypic characteristics of hydrocephalus in stillborn Friesian foals.

    Science.gov (United States)

    Sipma, K D; Cornillie, P; Saulez, M N; Stout, T A E; Voorhout, G; Back, W

    2013-11-01

    Hydrocephalus is uncommon in horses. However, in recent years, it has become clear that the prevalence of hydrocephalus is greater in Friesian horses than in other breeds probably due to their limited gene pool. Before identification of candidate genes that predispose to the development of hydrocephalus in Friesian horses can be pursued, an in-depth, phenotypic, pathological description of the condition in Friesians would be of great benefit. Our study aimed to characterize the morphology of hydrocephalus in Friesian horses, to support further investigation of the genetic background of this condition. Four stillborn Friesian foals with hydrocephalus were examined macroscopically and microscopically and compared with 2 normal stillborn Friesian foals without hydrocephalus. In all clinical cases, tetraventricular and venous dilatations were observed, together with malformation of the petrosal bone and, as a result, narrowing of the jugular foramen. These observations suggest a communicative hydrocephalus with a diminished absorption of cerebrospinal fluid into the systemic circulation at the venous sinuses due to a distorted, nonfunctional jugular foramen. This type of hydrocephalus is also recognized in humans and dogs and has been linked genetically to chondrodysplasia; this has already been recognized in dwarfism, which is another monogenetic defect in Friesian horses.

  1. Ocular features seen in children with hydrocephalus at University of ...

    African Journals Online (AJOL)

    The data was analysed with a scientific calculator Casio FX-82 Lb and Epi info version 6 software. Results: Fifteen children with un-operated hydrocephalus confirmed by transfontanelle ultrasonography were examined at presentation for ocular complications of hydrocephalus. The mean age at presentation was 29.8 ...

  2. Hydrocephalus in adults with community-acquired bacterial meningitis

    NARCIS (Netherlands)

    Soemirien Kasanmoentalib, E.; Brouwer, Matthijs C.; van der Ende, Arie; van de Beek, Diederik

    2010-01-01

    Objective: To evaluate the occurrence, treatment, and outcome of hydrocephalus complicating community-acquired bacterial meningitis in adults. Methods: Case series from a prospective nationwide cohort study from Dutch hospitals from 2006 to 2009. Results: Hydrocephalus was diagnosed in 26 of 577

  3. Meaning Construction and Integration in Children with Hydrocephalus

    Science.gov (United States)

    Barnes, Marcia A.; Faulkner, Heather; Wilkinson, Margaret; Dennis, Maureen

    2004-01-01

    Text comprehension processes were investigated in children with hydrocephalus, a neurodevelopmental disorder associated with good word decoding, but deficient reading comprehension. In Experiment 1, hydrocephalus and control groups were similar in processes related to activating word meanings and using context to enhance meaning. The hydrocephalus…

  4. Our experience in the management of infantile hydrocephalus: A ...

    African Journals Online (AJOL)

    Background: Hydrocephalus is a frequent central nervous system disorder in children, and despite its importance, it has not been suffi ciently studied in developing countries. Patients and Methods: A prospective and descriptive study on 35 cases of infantile hydrocephalus was carried out in the neurosurgery unit of the ...

  5. Unilateralism in International Law

    DEFF Research Database (Denmark)

    Hartmann, Jacques

    2015-01-01

    international law. This note considers when and how a State or a regional organisation may legitimately take unilateral measures to protect the environment. The note will does not consider the legality of including foreign aircraft within the ETS, which has been dealt with elsewhere. Instead, it will focus...... on the legality and importance of unilateral acts for the development of international law.......The 1997 Kyoto Protocol deferred negotiations on emissions from aviation to the International Civil Aviation Organization (ICAO). Also within this specialised body, agreement on how to deal with emissions from aviation has been difficult to reach.Frustrated by the lack of progress, the European...

  6. Congenital Abnormalities

    Science.gov (United States)

    ... Stages Ages and Stages Prenatal Baby (0-12 mos.) Toddler 1-3yrs. Preschool 3-5yrs Grade School ... Categories of Congenital Abnormalities Chromosome Abnormalities Chromosomes are structures that carry genetic material inherited from one generation ...

  7. Tracheomalacia - congenital

    Science.gov (United States)

    ... other congenital abnormalities, such as heart defects, developmental delay, or gastroesophageal reflux. Aspiration pneumonia can occur from ... urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows ...

  8. Extensive spinal epidural abscess complicated with hydrocephalus

    Directory of Open Access Journals (Sweden)

    Balan Corneliu

    2015-12-01

    Full Text Available Spinal epidural abscess is a rare but severe infection requiring prompt recognition in order to have a favorable outcome and appropriate treatment, mainly surgical. We present one of the largest extensions of such abscess in literature, involving the whole spine. No surgical treatment was tempted due to the involvement of 19 levels but antibiotics. The evolution of the lesion was complicated with hydrocephalus, by mechanism of cervical block of CSF flow, and needed first external derivation and later ventriculo-peritoneal drainage.

  9. Normal pressure hydrocephalus: did publications alter management?

    Science.gov (United States)

    Vanneste, J; van Acker, R

    1990-01-01

    Forty six Dutch neurologists and neurosurgeons were interviewed to evaluate the clinical value of research articles on normal pressure hydrocephalus (NPH). From this survey it appears that most clinicians still limit investigations to psychometry, CSF-tap test(s), and cisternography. The main reasons for not using other techniques were: their invasiveness, technical complexity, poor availability, lack of time and doubt on their additional predictive value. There is an obvious discrepancy between the quantity of publications on NPH and their impact and their ability to assist clinicians in selecting potential NPH patients for a shunt. PMID:2391518

  10. Our experience in the management of infantile hydrocephalus: A study on thirty-five regrouped cases in Yaounde, Cameroon

    Directory of Open Access Journals (Sweden)

    F F Mouafo Tambo

    2011-01-01

    Full Text Available Background: Hydrocephalus is a frequent central nervous system disorder in children, and despite its importance, it has not been sufficiently studied in developing countries. Patients and Methods: A prospective and descriptive study on 35 cases of infantile hydrocephalus was carried out in the neurosurgery unit of the Yaounde Central Hospital, from March 2008 to January 2010. Results: The mean age of the patients was 6.69΁1.58 months, and the majority of them were in the 0-6 months age group (71.43%. The most frequent causes were congenital malformations, with stenosis of the aqueduct of Sylvius being the most represented (31.43%. As radiological workup, a CT scan was done in more than half of the cases (57.15%, and ventriculo-peritoneal shunting was the main surgical method of management used (94.29%. Infectious complications were observed in 22.86% of our cases. Conclusion: Hydrocephalus is a frequent disorder in this Cameroonian setting affecting mostly the 0-6months age group. For early diagnosis to be made, the head circumference of neonates should be routinely measured in the labour room and followed-up in all medical visits. To avert complications following surgery, rigorous surgical procedures with effective asepsis and appropriate methods and materials for shunting should be used.

  11. Congenital asymmetric crying face: a case report

    Directory of Open Access Journals (Sweden)

    Semra Kara

    2011-12-01

    Full Text Available Congenital asymmetric crying face is an anomalia caused by unilateral absence or weakness of depressor anguli oris muscle The major finding of the disease is the absence or weakness in the outer and lower movement of the commissure during crying. The other expression muscles are normal and the face is symmetric at rest. The asymmetry in congenital asymmetric crying face is most evident during infancy but decreases by age. Congenital asymmetric crying face can be associated with cervicofacial, musclebone, respiratory, genitourinary and central nervous system anomalia. It is diagnosed by physical examination. This paper presents a six days old infant with Congenital asymmetric crying face and discusses the case in terms of diagnosis and disease features.

  12. Obstructed labour caused by rare congenital anomaly of foetus: managed by foetal abdominal tapping leading to successful vaginal delivery

    OpenAIRE

    Neelam Swaroop; Priya Sharma; Kalpana Kumari; Shikha Seth

    2015-01-01

    Obstructed labour with high maternal morbidity and mortality is still prevalent in the developing world. The common causes of obstructed labour include contracted pelvis, uterine abnormalities, maternal pelvic tumours, foetal malpresentation, and congenital foetal abnormalities like hydrocephalus, polycystic kidneys/hydronephrosis or locked twins. Here we present a case of rare congenital anomaly of foetus, Prune belly syndrome causing obstructed labour in its course and managed by foetal abd...

  13. Congenital tumors of the central nervous system

    International Nuclear Information System (INIS)

    Severino, Mariasavina; Schwartz, Erin S.; Thurnher, Majda M.; Rydland, Jana; Nikas, Ioannis; Rossi, Andrea

    2010-01-01

    Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into ''definitely congenital'' (present or producing symptoms at birth), ''probably congenital'' (present or producing symptoms within the first week of life), and ''possibly congenital'' (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors, where aggressive surgical treatment leads to disease

  14. Congenital tumors of the central nervous system

    Energy Technology Data Exchange (ETDEWEB)

    Severino, Mariasavina [G. Gaslini Children' s Hospital, Department of Neuroradiology, Genoa (Italy); Schwartz, Erin S. [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Thurnher, Majda M. [Medical University of Vienna, Department of Radiology, Vienna (Austria); Rydland, Jana [MR Center, St. Olav' s Hospital HF, Trondheim (Norway); Nikas, Ioannis [Agia Sophia Children' s Hospital, Imaging Department, Athens (Greece); Rossi, Andrea [G. Gaslini Children' s Hospital, Department of Neuroradiology, Genoa (Italy); G. Gaslini Children' s Hospital, Department of Pediatric Neuroradiology, Genoa (Italy)

    2010-06-15

    Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into ''definitely congenital'' (present or producing symptoms at birth), ''probably congenital'' (present or producing symptoms within the first week of life), and ''possibly congenital'' (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors

  15. Spontaneous canine hydrocephalus: cerebrospinal fluid dynamics1

    Science.gov (United States)

    Sahar, A.; Hochwald, G. M.; Kay, W. J.; Ransohoff, J.

    1971-01-01

    Cerebrospinal fluid dynamics were studied in 14 dogs with spontaneous hydrocephalus. In nine of the dogs aqueductal obstruction was observed and the remainder had a `communicating type' hydrocephalus. The major histological findings consisted of severe ependymal destruction, spongy changes in the periventricular white matter, increased density of capillaries in this area, and varying degrees of thickening, fibrosis, and fusion of the choroid villi. The formation and absorption of CSF were studied by perfusion of the cerebral ventricles. The rate of formation of CSF was found to decrease with perfusion pressure by Vf = 0·02595−0·00022 P ml./min (P = pressure in cm H2O). The absorption of spinal fluid was found to increase linearly with pressure by Va = 0·0165 + 0·00050 P. The various factors influencing the formation and absorption of the spinal fluid are discussed. The meaning and attainment of `arrest' of the hydrocephalic process in terms of the measured rates of CSF formation and absorption in these animals are considered. Images PMID:5571319

  16. Hearing loss in hydrocephalus: a review, with focus on mechanisms.

    Science.gov (United States)

    Satzer, David; Guillaume, Daniel J

    2016-01-01

    While neither hydrocephalus nor cerebrospinal fluid (CSF) shunt placement is traditionally considered in the differential diagnosis of hearing loss, there is substantial evidence that CSF circulation and pressure abnormalities can produce auditory dysfunction. Several indirect mechanisms may explain association between hydrocephalus and hearing loss, including mass effect, compromise of the auditory pathway, complications of prematurity, and genetically mediated hydrocephalus and hearing loss. Nevertheless, researchers have proposed a direct mechanism, which we term the hydrodynamic theory. In this hypothesis, the intimate relationship between CSF and inner ear fluids permits relative endolymphatic or perilymphatic hydrops in the setting of CSF pressure disturbances. CSF is continuous with perilymph, and CSF pressure changes are known to produce parallel perilymphatic pressure changes. In support of the hydrodynamic theory, some studies have found an independent association between hydrocephalus and hearing loss. Moreover, surgical shunting of CSF has been linked to both resolution and development of auditory dysfunction. The disease burden of hydrocephalus-associated hearing loss may be large, and because hydrocephalus and over-shunting are reversible, this relationship merits broader recognition. Hydrocephalic patients should be monitored for hearing loss, and hearing loss in a patient with shunted hydrocephalus should prompt further evaluation and possibly adjustment of shunt settings.

  17. Diagnosis and Differential Diagnosis of Hydrocephalus in Adults.

    Science.gov (United States)

    Langner, Sönke; Fleck, Steffen; Baldauf, Jörg; Mensel, Birger; Kühn, Jens Peter; Kirsch, Michael

    2017-08-01

    Purpose  Hydrocephalus is caused by an imbalance of production and absorption of cerebrospinal fluid (CSF) or obstruction of its pathways, resulting in ventricular dilatation and increased intracranial pressure. Imaging plays a crucial role in the diagnosis, differential diagnosis and planning of treatment. Methods  This review article presents the different types of hydrocephalus und their typical imaging appearance, describes imaging techniques, and discusses differential diagnoses of the different forms of hydrocephalus. Results and Conclusion  Imaging plays a central role in the diagnosis of hydrocephalus. While magnetic resonance (MR) imaging is the first-line imaging modality, computed tomography (CT) is often the first-line imaging test in emergency patients. Key points   · Occlusive hydrocephalus is caused by obstruction of CSF pathways.. · Malabsorptive hydrocephalus is caused by impaired CSF absorption.. · The MR imaging protocol should always include sagittal high-resolution T2-weighted images.. · When an inflammatory etiology is suspected, imaging with contrast agent administration is necessary.. Citation Format · Langner S, Fleck S, Baldauf J et al. Diagnosis and Differential Diagnosis of Hydrocephalus in Adults. Fortschr Röntgenstr 2017; 189: 728 - 739. © Georg Thieme Verlag KG Stuttgart · New York.

  18. Diagnosis, treatment and long-term outcome in fetal hydrocephalus

    International Nuclear Information System (INIS)

    Yamasaki, Mami; Nonaka, Masahiro; Bamba, Yohei; Teramoto, Chika; Ban, Chiaki; Pooh, Ritsuko

    2011-01-01

    The objective of this study was to evaluate the method of prenatally estimating an appropriate clinical outcome in fetal hydrocephalus. Retrospective study, single institute (Osaka National Hospital). Hundred and seventeen cases with fetal hydrocephalus treated at Osaka National Hospital from 1992 to 2010 were analysed. Of the 117 cases analysed, 38% are diagnosed as isolated ventriculomegaly (IVM), 51% as other types of malformation (30 cases of myelomeningocele, 4 cases of holoprosencephaly, 4 of Dandy Walker syndrome, 10 of arachnoid cyst and 6 of encephalocele etc.) and 11% as secondary hydrocephalus. They are diagnosed between 17 and 40 weeks of gestation (average 27 weeks), 17% diagnosed between 17 and 21 weeks, 30% between 22 and 27 weeks and 53% after 28 weeks. With the exception of 9 aborted cases and 30 unknown cases too young to be evaluated or lost due to lack of follow-up, final outcome was analyzed in 78 cases. Of these 78 cases, 15% died in utero or after birth, 23% showed severe retardation, 17% moderate retardation, 26% mild retardation, and 19% showed good outcome. Long term consequences were mostly influenced by basic disease and accompanied anomalies. Hydrocephalus associated with arachnoid cyst, atresia of Monro, corpus callosum agenesis and hydrocephalus due to fetal intracranial hemorrhage are categorized in the good outcome group. On the other hand, holoprosencephaly, hydrocephalus associated with encephalocele, syndromic hydrocephalus and hydrocephalus due to fetal virus infection are categorized in the poor outcome group. In order to accurate diagnosis and proper counseling, establishment of diagnosis protocol and treatment policy for fetal hydrocephalus including not only fetal sonography, fetal MRI, toxoplasma, rubella, cytomegalovirus, herpes simplex (TORCH) screening test but also chromosomal and gene testing is required. (author)

  19. Vertebrobasilar dolichoectasia induced hydrocephalus: the water-hammer effect

    Directory of Open Access Journals (Sweden)

    Vaso Zisimopoulou

    2015-05-01

    Full Text Available Vertebrobasilar dolichoectasia is a clinical entity associated rarely with obstructive hydrocephalus. We present a 48-year old male with a profound dilatation of the ventricular system due to a dolichoectatic basilar artery, as appeared in imaging studies. The patient suffered from longstanding hydrocephalus and presenile dementia. The underlying mechanism for obstructive hydrocephalus due to vertebrobasilar dolichoectasia is considered to be both a water-hammer effect and a direct compression of adjacent structures. We suggest prompt surgical intervention upon diagnosis as a first choice treatment in order to avoid further complications.

  20. Chronic hydrocephalus and alcohol abuse in a young male suicide.

    Science.gov (United States)

    Montisci, Massimo; Terranova, Claudio; Snenghi, Rossella; Ferrara, Santo Davide

    2006-12-01

    The paper describes a case of suicide in a young man affected by compensated chronic hydrocephalus who was subject to alcohol abuse. The case was studied by means of a complex set of analyses, including circumstantial and clinical data, anatomohistopathological findings, and chemicotoxicologic tests. What clearly emerges in the case is the importance of a continuing neuropsychological follow-up in patients with shunted hydrocephalus. The forensic interest in the case is due to the peculiar autopsy findings discussed in relation to the possible causes of sudden death in subjects with hydrocephalus.

  1. [Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery].

    Science.gov (United States)

    Furuya, Tomonori; Iida, Ryoji; Konishi, Jyumpei; Kato, Jitsu; Suzuki, Takahiro

    Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although there are several reports regarding pregnancy in patients with unilateral absence of a pulmonary artery, there are no case reports describing anesthesia for Cesarean section in a patient with unilateral absence of a pulmonary artery. We present a patient with unilateral absence of a pulmonary artery who underwent Cesarean sections twice at the ages of 24 and 26 years under spinal anesthesia for surgery and epidural analgesia for postoperative pain relief. Both times, spinal anesthesia and epidural analgesia enabled successful anesthesia management without the development of either pulmonary hypertension or right heart failure. Spinal anesthesia combined with epidural analgesia is a useful anesthetic method for a Cesarean section in patients with unilateral absence of a pulmonary artery. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  2. Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery.

    Science.gov (United States)

    Furuya, Tomonori; Iida, Ryoji; Konishi, Jyumpei; Kato, Jitsu; Suzuki, Takahiro

    Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although there are several reports regarding pregnancy in patients with unilateral absence of a pulmonary artery, there are no case reports describing anesthesia for Cesarean section in a patient with unilateral absence of a pulmonary artery. We present a patient with unilateral absence of a pulmonary artery who underwent Cesarean sections twice at the ages of 24 and 26 years under spinal anesthesia for surgery and epidural analgesia for postoperative pain relief. Both times, spinal anesthesia and epidural analgesia enabled successful anesthesia management without the development of either pulmonary hypertension or right heart failure. Spinal anesthesia combined with epidural analgesia is a useful anesthetic method for a Cesarean section in patients with unilateral absence of a pulmonary artery. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

  3. Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong [Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang (Korea, Republic of); Kim, Seon Jeong [Dept. of Radiology, Myongji Hospital, Goyang (Korea, Republic of)

    2017-05-15

    Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia.

  4. Congenital club foot in a teaching hospital in Lagos, Nigeria.

    Science.gov (United States)

    Adewole, O A; Giwa, S O; Kayode, M O; Shoga, M O; Balogun, R A

    2009-06-01

    Congenital club foot has been sparsely reported in literature in Nigeria, although it has been reported as the commonest congenital musculoskeletal abnormality. This study enumerates the point prevalence of this disease in a university teaching hospital in Lagos. Better understanding of the epidemiology in our community should improve awareness, and influence management. Between June 2005 and July 2006, 72 consecutive patients with congenital club feet were seen in the orthopaedic clinic of our Hospital. Demographic data, birth weight, family history, birth facility, maternal age and associated congenital anomalies were recorded and analysed using Statistical Programme for Social Sciences (SPSS) version 15. A total of 72 patients were seen, 28 of whom had bilateral club feet resulting in a total of 100 feet. There were 38 males and 34 females. Only 29% presented in the first month of life and 28% in the second month. Maternal ages ranged between 19 and 38 years and no family history of congenital club foot was given,. Babies delivered outside the orthodox medical system (churches, traditional healers, home etc) constituted 28%. The commonest associated congenital anomalies were tibia hemimelia, hydrocephalus, inguinal hernia and umbilical hernia. A default rate of 28% was observed during treatment. Congenital club foot may not be uncommon in Nigeria. Late presentation and high default rate before correction of the deformity were observed. Establishment of special club foot clinics should reduce the default rate. Training of healthcare workers in maternity units as well as Public awareness should encourage early referral to specialists.

  5. Subdural fluid collection and hydrocephalus following cervical schwannoma resection: hydrocephalus resolution after spinal pseudomeningocele repair: case report.

    Science.gov (United States)

    Benedetto, Nicola; Cagnazzo, Federico; Gambacciani, Carlo; Perrini, Paolo

    2016-12-01

    The authors report the case of a 31-year-old man who developed neck pain and headache 2 months after the uncomplicated resection of a cervical schwannoma. MR imaging revealed infratentorial subdural fluid collections and obstructive hydrocephalus associated with cervical pseudomeningocele. The clinical symptoms, subdural fluid collections, and ventricular dilation resolved after surgical correction of the pseudomeningocele. This report emphasizes that hydrocephalus may be related to disorders of cerebrospinal fluid flow dynamics induced by cervical pseudomeningocele. In these rare cases, both the hydrocephalus and the symptoms are resolved by the simple correction of the pseudomeningocele.

  6. Low-Field Magnetic Resonance Imaging of Canine Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Z. Adamiak* and M. Jaskólska and A. Pomianowski1

    2012-01-01

    Full Text Available The aim of presented study was to evaluate selected surface spine coil, and low-field magnetic resonance (MR selected sequences in diagnosing hydrocephalus in dogs. This paper discusses 19 dogs (14 canine patients with hydrocephalus and 5 healthy dogs, of five breeds, subjected to low-field magnetic resonance imaging (MRI of hydrocephalus. Area of the lateral ventricles and brain were examined in dogs with hydrocephalus using low-field MRI (at 0.25 Tesla. The MRI of FSE REL, SE, FLAIR, STIR, 3D HYCE, T3DT1, GE STIR 3D and 3D SHARC sequences with an indication of the most effective sequences are described. Additionally, coils for MR were compared, and models for infusion anesthesia were described. As a result of performed study all estimated sequences were diagnostically useful. However, spinal coil No. 2 (ESAOTE was the most optimal for examining and positioning the cranium.

  7. Post-hemorrhagic hydrocephalus and diabetes insipidus in preterm infants.

    Science.gov (United States)

    Borenstein-Levin, Liron; Koren, Ilana; Kugelman, Amir; Bader, David; Toropine, Arina; Riskin, Arieh

    2014-11-01

    We present two cases of transient central diabetes insipidus in preterm neonates with post-hemorrhagic hydrocephalus. Although the association between intraventricular hemorrhage and diabetes insipidus has been described in preterm infants, the association between diabetes insipidus and hydrocephalus, and the fact that such central diabetes insipidus could be reversible with the reduction of ventricular size, either because of spontaneous resolution or the placement of ventriculo-peritoneal shunt is first described here in neonates.

  8. Increased risk of hydrocephalus in long-term dialysis patients.

    Science.gov (United States)

    Wang, I-Kuan; Lin, Cheng-Li; Cheng, Yu-Kai; Chou, Che-Yi; Liang, Chih-Chia; Yen, Tzung-Hai; Sung, Fung-Chang

    2016-05-01

    The risk of hydrocephalus in end-stage renal disease (ESRD) patients on dialysis has not been studied in depth. Using Taiwan National Health Insurance claims data, we identified 29 684 incident ESRD patients from 2000 to 2010, including 10 030 peritoneal dialysis (PD) patients and 19 654 hemodialysis (HD) patients. The control cohort consisted of 118 736 people randomly selected from those without kidney disease, frequency matched with ESRD patients by age, sex and index year. We also established propensity score-matched cohorts with 10 014 PD and 10 014 HD patients. The incidence rates and hazard ratios (HRs) of hydrocephalus were calculated until the end of 2011. Incidence rates of hydrocephalus were greater in HD and PD patients than in controls (8.44 and 11.0 versus 4.11 per 10 000 person-years, respectively), with an adjusted HR of 1.86 [95% confidence interval (CI) 1.43-2.41] for all ESRD patients compared with controls. A higher proportion of hydrocephalus patients underwent surgical bypass to relieve hydrocephalus in ESRD patients than controls, 40.7% (46/113) versus 24.5% (67/273), with an adjusted odds ratio of 2.11 (95% CI 1.33-3.36). Compared with controls, the adjusted HRs of communicating hydrocephalus for HD and PD patients were 1.77 (95% CI 1.22-2.55) and 2.51 (95% CI 1.61-3.89), respectively. The propensity score-matched analysis showed an HR of 0.72 (95% CI 0.42-1.23) for hydrocephalus in HD patients compared with PD patients. Patients with ESRD are at an increased risk of hydrocephalus. The risk difference between HD and PD patients is not significant. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  9. Congenital amusias.

    Science.gov (United States)

    Tillmann, B; Albouy, P; Caclin, A

    2015-01-01

    In contrast to the sophisticated music processing reported in the general population, individuals with congenital amusia show deficits in music perception and production. Congenital amusia occurs without brain damage, sensory or cognitive deficits, and has been suggested as a lifelong deficit with genetic origin. Even though recognized for a long time, this disorder has been systematically studied only relatively recently for its behavioral and neural correlates. The currently most investigated hypothesis about the underlying deficits concerns the pitch dimension, notably with impaired pitch discrimination and memory. Anatomic and functional investigations of pitch processing revealed that the amusic brain presents abnormalities in the auditory and inferior frontal cortices, associated with decreased connectivity between these structures. The deficit also impairs processing of pitch in speech material and processing of the time dimension in music for some of the amusic individuals, but does not seem to affect spatial processing. Some studies suggest at least partial dissociation in the disorder between perception and production. Recent studies revealed spared implicit pitch perception in congenital amusia, supporting the power of implicit cognition in the music domain. Current challenges consist in defining different subtypes of congenital amusia as well as developing rehabilitation programs for this "musical handicap." © 2015 Elsevier B.V. All rights reserved.

  10. Congenital Constrictions

    OpenAIRE

    K Pavithran; Ramachandran Nair

    1984-01-01

    A young man having congenital annular constrictions of the legs with lymph-oedema of the left foot below the constriction, webbing of the fingers of both hands, anonychia of two fingers and intrauterine amputation of terminal phalanges of two finger and one big toe is reported.

  11. Congenital Constrictions

    Directory of Open Access Journals (Sweden)

    K Pavithran

    1984-01-01

    Full Text Available A young man having congenital annular constrictions of the legs with lymph-oedema of the left foot below the constriction, webbing of the fingers of both hands, anonychia of two fingers and intrauterine amputation of terminal phalanges of two finger and one big toe is reported.

  12. Impact of operation details on hydrocephalus after decompressive craniectomy

    Science.gov (United States)

    Wang, Qiang-Ping; Ma, Jun-Peng; Zhou, Zhang-Ming; You, Chao

    2016-01-01

    Objective: To evaluate the correlation between the distance of craniectomy from the midline and hydrocephalus after DC. Methods: The following electronic databases were searched from their inception to June 2015: Cochrane Library, MEDLINE, Science Direct, EMBASE, Scopus, Google Scholar, the Chinese Biomedical Database (CBM), and the Chinese National Knowledge Infrastructure (CNKI). All randomized clinical trials, prospective cohort, retrospective observational cohort, and case-control studies investigating the relationship between distance of craniectomy from the midline and hydrocephalus after DC were enrolled. The Cochrane Collaboration’s software RevMan 5.3 was used for meta-analysis. Results: Six retrospective cohort studies involving 462 participants were included. Pooled analysis of 4 studies suggested that craniectomy close to the midline (hydrocephalus (odds ratio [OR] = 3.61, 95% confidence interval [CI]: 1.3 - 9.97, p=0.01). However, meta-analysis of 4 studies did not find statistical differences when comparing the distance of craniectomy from the midline in the hydrocephalus group and that in the non-hydrocephalus group (OR = −0.14, 95% CI: −0.44 - 0.15, p=0.34). Conclusions: Available evidence was insufficient to support the theory that craniectomy close to the midline increases the risk of developing hydrocephalus after DC. Well-conducted randomized clinical trials are required to verify this issue. PMID:26818161

  13. Hydrocephalus after decompressive craniectomy for malignant hemispheric cerebral infarction.

    Science.gov (United States)

    Wang, Qiang-Ping; Ma, Jun-Peng; Zhou, Zhang-Ming; Yang, Min; You, Chao

    2016-08-01

    Several studies have investigated the incidence and risk factors of hydrocephalus after decompressive craniectomy (DC) for malignant hemispheric cerebral infarction. However, the results are controversial. Therefore, the following is a retrospective cohort study to determine the incidence and risk factors of hydrocephalus after DC for malignant hemispheric cerebral infarction. From January 2004 to June 2014, patients at two medical centres in south-west China, who underwent DC for malignant hemispheric cerebral infarction, were included. The patients' clinical and radiologic findings were retrospectively reviewed. A chi-square test, Mann-Whitney U-test and logistic regression model were used to identify the risk factors. A total of 128 patients were included in the study. The incidence of ventriculomegaly and shunt-dependent hydrocephalus were 42.2% (54/128) and 14.8% (19/128), respectively. Lower preoperative Glasgow Coma Scale (GCS) score and presence of subarachnoid haemorrhage (SAH) were factors significantly associated with the development of post-operative hydrocephalus after DC. Cerebral infarction patients receiving DC have a moderate tendency to suffer from post-operative hydrocephalus. A poor GCS score and the presence of SAH were significantly associated with the development of hydrocephalus after DC.

  14. Unilateral hyperhidrosis and hypothermia

    DEFF Research Database (Denmark)

    Dyring-Andersen, B; Kamp, S; Madelung, A

    2016-01-01

    A 34-year old man presented at the Department of Dermato-allergology, Herlev and Gentofte Hospital with unilateral hyperhidrosis localized to the right axilla through approximately 10 years without progression of symptoms. The patient often experienced that his right hand was colder than the left......, especially during bouts of hyperhidrosis. The symptoms usually arose in stressful situations. The patient had no sensory symptoms (pain and light touch of upper extremitieswere assessed) and no medical history of other diseases including lung disease or neurological disease. This article is protected...

  15. Congenital monomelic muscular hypertrophy of the upper extremity.

    NARCIS (Netherlands)

    Gilhuis, H.J.; Zophel, O.T.; Lammens, M.M.Y.; Zwarts, M.J.

    2009-01-01

    Pathological muscular hypertrophy results from either muscular or neurogenic damage. Rarely, it is caused by a congenital malformation consisting of a unilateral muscular hyperplasia of the upper extremity. We report on a young woman with an enlargement of the right upper extremity. Electromyography

  16. Congenital hearing loss. Is CT enough? | Agha | Alexandria Journal ...

    African Journals Online (AJOL)

    Patients and methods: This is a prospective study including 60 patients, 24 males and 36 females aged from 1 to 7 years, who were presented by unilateral or bilateral congenital conductive (CHL) or/and sensorineural hearing loss (SNHL). All patients were evaluated by HRCT scan with post-processing multiplanar ...

  17. Unilateral removable partial dentures.

    Science.gov (United States)

    Goodall, W A; Greer, A C; Martin, N

    2017-01-27

    Removable partial dentures (RPDs) are widely used to replace missing teeth in order to restore both function and aesthetics for the partially dentate patient. Conventional RPD design is frequently bilateral and consists of a major connector that bridges both sides of the arch. Some patients cannot and will not tolerate such an extensive appliance. For these patients, bridgework may not be a predictable option and it is not always possible to provide implant-retained restorations. This article presents unilateral RPDs as a potential treatment modality for such patients and explores indications and contraindications for their use, including factors relating to patient history, clinical presentation and patient wishes. Through case examples, design, material and fabrication considerations will be discussed. While their use is not widespread, there are a number of patients who benefit from the provision of unilateral RPDs. They are a useful treatment to have in the clinician's armamentarium, but a highly-skilled dental team and a specific patient presentation is required in order for them to be a reasonable and predictable prosthetic option.

  18. Unilateral hydronephrosis with congenital absence of contralateral kidney in children

    International Nuclear Information System (INIS)

    Muren, C.; Wikstad, I.

    1988-01-01

    The case histories of six children with absence of functioning renal parenchyma on one side and dilatation of the contralateral pelvis are reviewed. The hydronephrosis was obstructive in two cases. In the others no cause could be found. The development of this combination of renal malformations is discussed against an embryologic background. (orig.)

  19. Helical CT of congenital ossicular anomalies

    International Nuclear Information System (INIS)

    Osada, Hisato; Machida, Kikuo; Honda, Norinari

    2001-01-01

    Since January 1996 to December 2000, 26 cases of congenital ossicular anomaly could be diagnosed with helical CT. All cases were unilateral. In 8 patients with malformation of the external ear, CT showed malleoincudal fixation (n=5), malleoincudal fixation and deformed incuts long process (n=1), deformed incus long process (n=1), and partial fusion of malleus neck and incus long process (n=1). In 18 patients with normal external ear, CT showed defect of the incus long process (n=5), defect of both the incus long process and stapes superstructure (n=8, 2 patients with congenital cholesteatoma, 1 with hypoplastic oval window), defect of the stapes superstructure (n=2, 1 patient with oval window absence), defect of the malleus manubrium (n=1), ossification of the stampede's tendon (n=1), and monopod stapes (n=1). Helical CT can evaluate the auditory ossicular chain in detail and is useful for diagnosing congenital ossicular anomaly. (author)

  20. An update on research priorities in hydrocephalus: overview of the third National Institutes of Health-sponsored symposium "Opportunities for Hydrocephalus Research: Pathways to Better Outcomes".

    Science.gov (United States)

    McAllister, James P; Williams, Michael A; Walker, Marion L; Kestle, John R W; Relkin, Norman R; Anderson, Amy M; Gross, Paul H; Browd, Samuel R

    2015-12-01

    Building on previous National Institutes of Health-sponsored symposia on hydrocephalus research, "Opportunities for Hydrocephalus Research: Pathways to Better Outcomes" was held in Seattle, Washington, July 9-11, 2012. Plenary sessions were organized into four major themes, each with two subtopics: Causes of Hydrocephalus (Genetics and Pathophysiological Modifications); Diagnosis of Hydrocephalus (Biomarkers and Neuroimaging); Treatment of Hydrocephalus (Bioengineering Advances and Surgical Treatments); and Outcome in Hydrocephalus (Neuropsychological and Neurological). International experts gave plenary talks, and extensive group discussions were held for each of the major themes. The conference emphasized patient-centered care and translational research, with the main objective to arrive at a consensus on priorities in hydrocephalus that have the potential to impact patient care in the next 5 years. The current state of hydrocephalus research and treatment was presented, and the following priorities for research were recommended for each theme. 1) Causes of Hydrocephalus-CSF absorption, production, and related drug therapies; pathogenesis of human hydrocephalus; improved animal and in vitro models of hydrocephalus; developmental and macromolecular transport mechanisms; biomechanical changes in hydrocephalus; and age-dependent mechanisms in the development of hydrocephalus. 2) Diagnosis of Hydrocephalus-implementation of a standardized set of protocols and a shared repository of technical information; prospective studies of multimodal techniques including MRI and CSF biomarkers to test potential pharmacological treatments; and quantitative and cost-effective CSF assessment techniques. 3) Treatment of Hydrocephalus-improved bioengineering efforts to reduce proximal catheter and overall shunt failure; external or implantable diagnostics and support for the biological infrastructure research that informs these efforts; and evidence-based surgical standardization with

  1. [Congenital aniridia].

    Science.gov (United States)

    Chiruţa, Daria; Stan, Cristina

    2014-01-01

    Aniridia is a rare congenital, hereditary, bilateral disease which is associated with various systemic and ocular defects. We present the case of a 61 year old patient who was admitted in the hospital of ophthalmology Cluj Napoca, for the symptoms caused by the ocular defects associated with aniridia. In this case, aniridia is autosomal dominant transmitted with incomplete penetrance and it is not accompanied by any systemic defects. The disease also affects three of her sons and two nephews of the patient.

  2. Congenital diplopodia

    International Nuclear Information System (INIS)

    Brower, Jason S.; Wootton-Gorges, Sandra L.; Costouros, John G.; Boakes, Jennette; Greenspan, Adam

    2003-01-01

    Diplopodia, or duplicated foot, is a rare congenital anomaly. It differs from polydactyly in that supernumerary metatarsal and tarsal bones are present as well as extra digits. Only a few cases of this anomaly have been reported in the literature to date. We present a newborn male without intrauterine teratogen exposure who was born with a duplicate foot of the left lower extremity and imperforate anus. (orig.)

  3. Hydrocephalus-history of surgical treatment over the centuries

    Directory of Open Access Journals (Sweden)

    Milojević Aleksandar J.

    2012-01-01

    Full Text Available To trace the history of the treatment of hydrocephalus is to document the parallel development of medicine as a whole; when one reviews the treatment of hydrocephalus, the integral relationship between basic science and therapy is reaffirmed. The treatment of hydrocephalus, over the centuries, underwent three stages of evolution. Prior to the late 19 th century, treatment for 'water on the brain' involved more observation than intervention. During antiquity, middle ages and renaissance, hydrocephalus was not understood. Medical treatment was useless; surgery was hopeless. The second stage extends from the 19th century to the end of the first half of the 20 th century. Cerebrospinal fluid circulation was now understood; surgery however, remained inefficient, but some patients survived with arrested hydrocephalus. The third stage begins in the nineteen fifties with the development of silicone shunts with a valve. Surgery transforms the prognosis of hydrocephalus, but the number of post-operative complications creates new problems. The different attempts that have been made during these past two decades to solve these problems are reviewed. They have resulted in a reduction of the mechanical and infectious complications. CSF over drainage has been minimized. Percutaneous ventriculo-cisternostomies have in some cases replaced shunts. In the future, to improve outcome in these hydrocephalics, surgery, when indicated, should be performed as early as possible. Knowledge and prevention of the causes of hydrocephalus should be developed. As we progress further in this new millennium, it is appropriate to reflect on the past understanding and treatment of this disorder, review strategies to curb this disease process, and consider therapies and possibly cures that will be available in the future.

  4. Congenital toxoplasmosis.

    Science.gov (United States)

    Kieffer, François; Wallon, Martine

    2013-01-01

    Congenital toxoplasmosis results from the transplacental transmission of the parasite Toxoplasma gondii after a maternal infection acquired in pregnancy. Prevalence of congenital infection ranges from 0.1 to 0.3 per 1000 live births. The maternal-fetal transmission rate increases with gestational age at maternal seroconversion, from less than 15% at 13 weeks of gestation to over 70% at 36 weeks. Conversely, the later the maternal infection, the lower the risk of symptomatic congenital infection (infections acquired during the third trimester are most often asymptomatic at birth). Prenatal diagnosis is currently performed by PCR analysis in amniotic fluid. Antenatal management and treatment vary considerably among countries. In some European countries, maternal infections are detected through serological screening allowing a prompt treatment with spiramycin, which is expected to reduce the risk of vertical transmission. If PCR analysis in amniotic fluid is positive or if maternal infection was acquired in the third trimester of pregnancy, a combination with pyrimethamine and sulphonamide is given until delivery. Benefits of antenatal treatments remain controversial. Infected newborns are prescribed pyrimethamine and sulphonamide for 12 months. Despite antenatal and postnatal treatment, chorioretinitis can occur at any age (prevalence>20% at 10 years of age): long-term ophthalmological follow-up remains necessary. Copyright © 2013 Elsevier B.V. All rights reserved.

  5. A case of unilateral dysmenorrhea

    Directory of Open Access Journals (Sweden)

    Tulon Borah

    2010-01-01

    Full Text Available Unilateral dysmenorrhea in an adolescent may be associated with uterine malformation. Relevant investigations in suspected cases and timely intervention can prevent future complications in such cases. Here, we present a case of unicornuate uterus with rudimentary horn in an adolescent complaining of unilateral dysmenorrhea.

  6. Isolated unilateral pulmonary agenesis and other fetal thoracic anomalies.

    Science.gov (United States)

    Russell, Bronwyn C; Whitecar, Paul; Nitsche, Joshua F

    2014-06-01

    Although the diagnostic workup and management regimens for many of the common fetal thoracic lesions have been well described, the understanding of pulmonary agenesis is more limited. A better understanding of the published reports of this condition is essential to provide proper care for these complicated pregnancies. The aims of this study were to provide a better understanding of the difficulties in the diagnosis and management of fetal thoracic lesions and to consolidate what is known about unilateral pulmonary agenesis. We performed a review of the English medical literature covering the last 20 years (1993-2013) in PubMed, MEDLINE, and MD Consult using search terms pulmonary agenesis, pulmonary aplasia, unilateral pulmonary agenesis, unilateral pulmonary aplasia, lung agenesis, lung aplasia, unilateral lung agenesis, and unilateral lung aplasia. Prenatal diagnosis of pulmonary agenesis and other fetal thoracic lesions can be particularly challenging given that many anomalies have similar appearance on ultrasound. Fetal magnetic resonance imaging has been used in several of the reported cases to clarify the diagnosis. Once confirmed, there are several important prognostic factors to consider in the management of unilateral pulmonary agenesis. Poor prognostic factors include right-sided agenesis, the presence of genetic abnormalities, and other associated congenital anomalies. Fetal magnetic resonance imaging can be a useful imaging modality when the diagnosis is unclear after ultrasound imaging. The management of cases with a poor prognosis should be guided by the nature of the associated anomalies. Cases of isolated pulmonary agenesis have an overall good prognosis and can be managed conservatively during pregnancy, with consideration of delivery at a tertiary care facility in case postnatal respiratory issues arise.

  7. Congenital uronephropathy pattern in children

    Directory of Open Access Journals (Sweden)

    Husein Alatas

    2001-10-01

    Full Text Available To obtain the basic data of congenital uronephropathy pattern and the affecting factors in children, we conducted a cross-sectional study at the Department of Child Health Cipto Mangunkusumo (CM Hospital Jakarta from 1995 to 1999 and 9 teaching hospitals throughout Indonesia. During the study period 134 patients were obtained, 116 patients from the CM Hospital and 18 patients from other teaching hospitals. Most patients (48.8% were below 1 year of age; male were affected more than female (2.4:1. The disorder was classified into two groups, i.e., congenital nephropathy and uropathy. There were 10 children with nephropathy, i.e., 4 with unilateral renal hypoplasia, 3 with polycystic kidney, and 3 with renal agenesis. In the uropathy group, 43 were with hypospadia, 22 with primary reflux vesicoureter, 18 with neurogenic bladder, and 17 with ureteropelvic junction obstruction. The complications found were urinary tract infection (71.2%, chronic renal failure (15.7%, hypertension (3.7%, and acute renal failure (1.5%. Consanguinity, familial disorders, maternal diseases, x-ray exposure and abortion efforts were found in a small proportion of patients. History of drug or herbs use in the first trimester of pregnancy was found in a large proportion of patients, mostly took analgesics (especially acetaminophen. In conclusion, uropathy disorders were much more common than congenital nephropathy. The most common complication was urinary tract infection, followed by chronic renal failure, hypertension, and acute renal failure.

  8. Acute hydrocephalus in nonaneurysmal perimesencephalic hemorrhage: evidence of CSF block at the tentorial hiatus

    NARCIS (Netherlands)

    Rinkel, G. J.; Wijdicks, E. F.; Vermeulen, M.; Tans, J. T.; Hasan, D.; van Gijn, J.

    1992-01-01

    The acute hydrocephalus in patients with nonaneurysmal perimesencephalic hemorrhage suggests an extraventricular obstruction of CSF flow. We studied the occurrence of acute hydrocephalus and the site of cisternal blood in 40 consecutive patients with perimesencephalic hemorrhage. In all 11 patients

  9. Features of Somatic and Reproductive Status of Women, who Gave Birth to a Child with Congenital Malformations, and Their Identifiсation Using «Case — Control» Method According to Data of Lviv Regional Clinical Perinatal Center in 2007–2009

    Directory of Open Access Journals (Sweden)

    Zborovska N.V. Zborovska N.V.

    2012-04-01

    While comparing a range of reproductive anamnesis indices there was noticed a higher percentage of dead children, stillbirths, anemia in pregnancy, gestosis in the first half of pregnancy, gestosis in the second half of pregnancy, abnormal labors in mothers who gave birth to a child with congenital malformations than in women of control group. According to ultrasound diagnosis congenital abnormalities in pregnancy were detected in 31.6 % of cases. There were diagnosed 4 cases of Arnold — Chiari syndrome (spina bifida and internal hydrocephalus; and 3 cases of each: hydrocephalus, gastroschisis, small intestinal atresia, and 2 cases of each: congenital malformations, hypoplastic left heart syndrome, Down’s syndrome, congenital diaphragmatic hernia, omphalocele, congenital rectal atresia, and 1 case of each: spina bifida, congenital absence of forearm and hand, left ventricular rabdomioma, encephalocele.

  10. Unilateral sixth nerve palsy.

    Science.gov (United States)

    Sotoodehnia, Mehran; Safaei, Arash; Rasooli, Fatemeh; Bahreini, Maryam

    2017-06-01

    The diagnosis of cerebral venous sinus thrombosis still remains a real challenge. Seizure, unusual headache with sudden onset, unexplained persistently unilateral vascular headache and neurologic deficit-which is difficult to be attributed to a vascular territory are some of the suggestive symptoms. An isolated sixth nerve palsy is discussed as a rare presentation for cerebral venous thrombosis. Following the extensive investigation to rule out other possible diagnoses, magnetic resonance venogram revealed the final etiology of sixth nerve palsy that was ipsilateral left transverse sinus thrombosis; therefore, anticoagulant treatment with low molecular weight heparin was administered. Rapid and accurate diagnosis and treatment cause to achieve excellent outcomes for most patients. Considering different clinical features, risk factors and high index of suspicion are helpful to reach the diagnosis. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Telangiectasia nevoide unilateral adquirida em homem hígido Acquired unilateral nevoid telangiectasia in a healthy men

    Directory of Open Access Journals (Sweden)

    Juliana Merheb Jordão

    2010-12-01

    Full Text Available A Telangiectasia Nevoide Unilateral é uma dermatose vascular rara, caracterizada por áreas de telangiectasia superficial, em uma distribuição linear unilateral, descrita, em 1899, por Zeisler e Blaschko. Diversas teorias foram desenvolvidas para explicar sua etiopatogenia, sendo a provável (e a mais fundamentada a relação com elevação dos níveis de estrogênio. Há duas formas: a congênita e a adquirida. Esta, geralmente, se relaciona a hepatopatias, em pacientes do sexo masculino. Em homens hígidos, a ocorrência da forma adquirida é rara, com poucos relatos na literatura, sendo sua causa desconhecida. Este trabalho tem como objetivo relatar um caso de telangiectasia nevoide unilateral adquirida em homem jovem, sem comorbidades, alterações clínicas e/ou laboratoriais sugestivas de hiperestrogenismo.Unilateral nevoid telangiectasia is a rare vascular dermatosis, characterized by areas of superficial telangiectases distributed in a linear unilateral pattern. It was described in 1899 by Zeisler and Blascko. Several theories where developed in order to explain its etiopathogenesis. The most widely accepted is the one which establishes its probable association with an increase in the estrogen levels. There are two types: congenital and acquired. The latter is associated with hepatopathies in male patients. The acquired form is rarely observed in healthy men, with a few cases reported in the medical literature, and its etiology is unknown. This study reports the case of a healthy young man with acquired unilateral nevoid telangiectasia, without any comorbidities, clinical and /or laboratory findings indicative of hyperestrogenism.

  12. Congenital cataracts and other abnormalities in a female with 46.X, del(X)(q26q28)mat: A new locus for X-linked congenital cataract?

    Energy Technology Data Exchange (ETDEWEB)

    Babul, R.; Chitayat, D.; Teshima, I. [Univ. of Toronto, Ontario (Canada)] [and others

    1994-09-01

    Three forms of X-linked congenital cataracts have been delineated: congenital cataract with posterior Y-sutural opacities in heterozygotes, congenital cataract and microcornea or microphthalmia and congenital cataract-dental syndrome (Nance-Horan syndrome). Of these, only the Nance-Horan syndrome has been mapped to Xp22.3-p21.1. However, Warburg has suggested that these different forms of X-linked congenital cataracts are due to deletions of varying sizes, placing them in the vicinity of the Nance-Horan syndrome region. We report on a female patient born to a 29-year-old primigravida woman who at birth was found to have hypotonia, dysmorphic facial features, hydrocephalus and dense white congenital bilateral cataracts. Other ophthalmological findings included bilateral nystagmus and shallow orbits. Chromosome analysis revealed 46,X,del(X)(q26q28)mat. The mother, however, is phenotypically normal. Brain CT scan on the female infant revealed communicating hydrocephalus and a muscle biopsy showed congenital muscle fiber disproportion. An EMG and NCV were normal. At 4 years of age, her height and weight were below -3SD and her OFC was +2SD. Molecular studies using DNA markers located in Xq26-qter have revealed that the proximal breakpoint in the patient and her mother is defined by the HPRT locus while the distal breakpoint is defined by the locus DXS1108. This indicates that the deletion is not terminal but rather interstitial, retaining sequences proximal to the telomeric region. Other molecular studies are in progress to determine the X-inactivation status of the deleted chromosome in our patient and her mother as a possible explanation for the variation in the phenotype. These clinical and molecular findings suggest that another locus for X-linked congenital cataract exists at Xq26-28.

  13. Normal pressure hydrocephalus: Diagnostic and predictive evaluation

    Directory of Open Access Journals (Sweden)

    Benito Pereira Damasceno

    Full Text Available Abstract In typical cases, normal pressure hydrocephalus (NPH manifests itself with the triad of gait disturbance, which begins first, followed by mental deterioration and urinary incontinence associated with ventriculomegaly (on CT or MRI and normal cerebrospinal fluid (CSF pressure. These cases present minor diagnostic difficulties and are the most likely to improve after shunting. Problems arise when NPH shows atypical or incomplete clinical manifestations (25-50% of cases or is mimicked by other diseases. In this scenario, other complementary tests have to be used, preferentially those that can best predict surgical outcome. Radionuclide cisternography, intracranial pressure monitoring (ICP and lumbar infusion tests can show CSF dynamics malfunction, but none are able to confirm whether the patient will benefit from surgery. The CSF tap test (CSF-TT is the only procedure that can temporarily simulate the effect of definitive shunt. Since the one tap CSF-TT has low sensitivity, it cannot be used to exclude patients from surgery. In such cases, we have to resort to a repeated CSF-TT (RTT or continuous lumbar external drainage (LED. The most reliable prediction would be achieved if RTT or LED proved positive, in addition to the occurrence of B-waves during more than 50% of ICP recording time. This review was based on a PubMed literature search from 1966 to date. It focuses on clinical presentation, neuroimaging, complementary prognostic tests, and differential diagnosis of NPH, particularly on the problem of selecting appropriate candidates for shunt.

  14. Cognitive Profile of Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Makoto Saito

    2011-07-01

    Full Text Available Background/Aims: Frontal lobe dysfunction is believed to be a primary cognitive symptom in idiopathic normal pressure hydrocephalus (iNPH; however, the neuropsychology of this disorder remains to be fully investigated. The objective of this study was to delineate a comprehensive profile of cognitive dysfunction in iNPH and evaluate the effects of cerebrospinal fluid (CSF shunt surgery on cognitive dysfunction. Methods: A total of 32 iNPH patients underwent neuropsychological testing of memory, attention, language, executive function, and visuoperceptual and visuospatial abilities. Of these 32 patients, 26 were reevaluated approximately 1 year following CSF shunt surgery. The same battery of tests was performed on 32 patients with Alzheimer’s disease (AD and 30 healthy elderly controls. Results: The iNPH patients displayed baseline deficits in attention, executive function, memory, and visuoperceptual and visuospatial functions. Impairments of attention, executive function, and visuoperceptual and visuospatial abilities in iNPH patients were more severe than in those with AD, whereas the degree of memory impairment was comparable to that in AD patients. A significant improvement in executive function was observed following shunt surgery. Conclusion: Patients with iNPH are impaired in various aspects of cognition involving both ‘frontal’ executive functions and ‘posterior cortical’ functions. Shunt treatment can ameliorate executive dysfunction.

  15. Pulsatile flow in ventricular catheters for hydrocephalus

    Science.gov (United States)

    Giménez, Á.; Galarza, M.; Thomale, U.; Schuhmann, M. U.; Valero, J.; Amigó, J. M.

    2017-05-01

    The obstruction of ventricular catheters (VCs) is a major problem in the standard treatment of hydrocephalus, the flow pattern of the cerebrospinal fluid (CSF) being one important factor thereof. As a first approach to this problem, some of the authors studied previously the CSF flow through VCs under time-independent boundary conditions by means of computational fluid dynamics in three-dimensional models. This allowed us to derive a few basic principles which led to designs with improved flow patterns regarding the obstruction problem. However, the flow of the CSF has actually a pulsatile nature because of the heart beating and blood flow. To address this fact, here we extend our previous computational study to models with oscillatory boundary conditions. The new results will be compared with the results for constant flows and discussed. It turns out that the corrections due to the pulsatility of the CSF are quantitatively small, which reinforces our previous findings and conclusions. This article is part of the themed issue `Mathematical methods in medicine: neuroscience, cardiology and pathology'.

  16. Preventricular lucency in computerized tomography of various forms of hydrocephalus

    International Nuclear Information System (INIS)

    Sprung, C.

    1981-01-01

    602 patients with 8 different types of hydrocephalus have been examined for PVL on the basis of 18,413 CT pictures. The localisation of PVL, in the periventricular white matter of the auterior horns, and its configuration are independent of the type of hydrocephalus and of the intraventricular pressure. In contrast, the intensity and incidence of PVL are a function of the type of hydrocephalus. Factors influencing these two parameters are in order of importance: The intraventricular pressure, the acuteness of the process leading to stenosis (i.e. the rate of development of hydrocephalus), the extent of ventricular enlargement, the compliance of ventricular wall and osseous part of the skull, the volume of the ventricles, and the localisation of the obstructing and displacing process. Independent of the type of hydrocephalus, disappearance of PVL is always well correlated with improved clinical symptoms and, in most cases, also with a reduction of the ventricular volume. As far as the pathomechanism is concerned, the studies suggest passive inflow of liquor through the damaged ependyma rather than an active absorption process. (orig./MG) [de

  17. New concept of the pathogenesis and therapeutic orientation of acquired communicating hydrocephalus.

    Science.gov (United States)

    Xu, Hao

    2016-09-01

    Hydrocephalus is a common medical condition characterized by abnormalities in the secretion, circulation and absorption of cerebrospinal fluid (CSF), resulting in ventricle dilatation. For the communicating hydrocephalus, without etiological treatment, its pathogenesis has been considered as a research emphasis. Many factors can damage the CSF system and trigger communicating hydrocephalus, including tumor surgery and hydrocephalus neurological diseases, such as brain trauma, infection, ICH and SAH. But according to our clinical experience, a big proportion of patients do not develop hydrocephalus. That is because the absorbing ability of CSF can compensate within a certain range. If the damage exceeds that range, hydrocephalus will occur. Once it occurs, it is not likely to be reversed, so a shunt surgery is always needed. Therefore, we believe that our orientation could transform the treatment of patient who has already showed hydrocephalus symptoms to the prevention of the occurrence in the patient with high risk of hydrocephalus. Based on the hypothesis above, we first divide the process of hydrocephalus into three stages and we believe that hydrocephalus are possible be reversed or halted in stage 1 and 2. The new concept of the pathogenesis in hydrocephalus will enrich our understanding and provide new insights to the therapeutic orientation. In conclusion, the future research direction should be the prevention of hydrocephalus, which should take a long period from the immediate occurrence of brain injury to several months or even years after the injury.

  18. At what age is hydrocephalus detected, and what is the role of head circumference measurements?

    NARCIS (Netherlands)

    Breuning-Broers, J.M.; Deurloo, J.A.; Gooskens, R.H.; Verkerk, P.H.

    2014-01-01

    To investigate at what age hydrocephalus is detected and to assess the role of head circumference measurements in detecting hydrocephalus, we performed a retrospective chart review in children with hydrocephalus treated in a tertiary paediatric hospital in the Netherlands. The study group contained

  19. Secondary pediatric encephalocele after ventriculosubgaleal shunting for posthemorrhagic hydrocephalus.

    Science.gov (United States)

    Seeburg, Daniel; Ahn, Edward; Huisman, Thierry

    2014-08-01

    Intraventricular hemorrhage and posthemorrhagic hydrocephalus continue to be common complications in very low-birth-weight premature infants, often requiring ventricular shunting for cerebrospinal fluid diversion. We report on two infants with posthemorrhagic hydrocephalus that developed a secondary encephalocele after ventriculosubgaleal shunting. Encephaloceles can act as a source of seizure activity and can result in various additional complications including meningitis, abscess formation, and infarction of herniated brain parenchyma. With continued improvements in neonatal intensive care, the survival of infants with significant medical comorbidities-including those that develop posthemorrhagic hydrocephalus requiring ventricular shunting-continues to increase. It is thus important for the radiologist and treating physician to be aware of this rare, potential complication. Georg Thieme Verlag KG Stuttgart · New York.

  20. Alexander Monro Tertius and his works on hydrocephalus.

    Science.gov (United States)

    Tubbs, R Shane; Tubbs, Isaiah; Loukas, Marios; Oakes, W Jerry

    2015-03-01

    The Monros of the University of Edinburgh reigned over anatomy instruction for over a century. The last of these men, Monro Tertius, was the weaker teacher of the family but still contributed to the anatomical and surgical literature. Herein, we describe the life of Alexander Monro Tertius and his writings, particularly on childhood hydrocephalus. Monro Tertius will not be remembered as a great anatomist or teacher. However, he collected and published important books on his observations and those of others on hydrocephalus. These texts contained the knowledge of his era on this topic.

  1. Vascular factors in suspected normal pressure hydrocephalus

    Science.gov (United States)

    Agerskov, Simon; Rabiei, Katrin; Marlow, Thomas; Jensen, Christer; Guo, Xinxin; Kern, Silke; Wikkelsø, Carsten; Skoog, Ingmar

    2016-01-01

    Objective: We examined clinical and imaging findings of suspected idiopathic normal pressure hydrocephalus (iNPH) in relation to vascular risk factors and white matter lesions (WMLs), using a nested case-control design in a representative, population-based sample. Methods: From a population-based sample, 1,235 persons aged 70 years or older were examined with CT of the brain between 1986 and 2000. We identified 55 persons with hydrocephalic ventricular enlargement, i.e., radiologic findings consistent with iNPH. Among these, 26 had clinical signs that fulfilled international guideline criteria for probable iNPH. These cases were labeled suspected iNPH. Each case was matched to 5 controls from the same sample, based on age, sex, and study cohort. Data on risk factors were obtained from clinical examinations and the Swedish Hospital Discharge Register. History of hypertension, diabetes mellitus (DM), smoking, overweight, history of coronary artery disease, stroke/TIA, and WMLs on CT were examined. Risk factors associated with iNPH with a p value <0.1 in χ2 tests were included in conditional logistic regression models. Results: In the regression analyses, suspected iNPH was related to moderate to severe WMLs (odds ratio [OR] 5.2; 95% confidence interval [CI]: 1.5–17.6), while hydrocephalic ventricular enlargement was related to hypertension (OR 2.7; 95% CI: 1.1–6.8), moderate to severe WMLs (OR 6.5; 95% CI: 2.1–20.3), and DM (OR 4.3; 95% CI: 1.1–16.3). Conclusions: Hypertension, WMLs, and DM were related to clinical and imaging features of iNPH, suggesting that vascular mechanisms are involved in the pathophysiology. These findings might have implications for understanding disease mechanisms in iNPH and possibly prevention. PMID:26773072

  2. Prevalence of idiopathic normal-pressure hydrocephalus

    Science.gov (United States)

    Rabiei, Katrin; Marlow, Thomas; Jensen, Christer; Skoog, Ingmar; Wikkelsø, Carsten

    2014-01-01

    Objectives: The aim of this study was to determine the prevalence of idiopathic normal-pressure hydrocephalus (iNPH) in elderly persons in a large population-based sample using radiologic and clinical examinations. Methods: We examined representative elderly populations aged 70 years and older that had undergone neuropsychiatric evaluations and CT of the brain between 1986 and 2000 (n = 1,238). Gait was evaluated by clinical examination and history of walking difficulty. Cognitive function was evaluated with the Mini-Mental State Examination and urinary incontinence by self-report. iNPH was diagnosed in concordance with the American-European iNPH guidelines. Exclusion criteria were history of meningitis, severe head trauma, and subarachnoid hemorrhage. Results: The prevalence of probable iNPH was 0.2% in those aged 70–79 years (n = 2) and 5.9% (n = 24) in those aged 80 years and older, with no difference between men and women. Only 2 of these persons had been treated for iNPH. Hydrocephalic ventricular enlargement, i.e., a CT image consistent with NPH, was found in 56 persons (4.5%). An Evans Index >0.3 was found in 256 (20.7%) and occluded sulci at the high convexity in 67 persons (5.4%). All of these findings were more common in the older age groups. Conclusions: Many elderly possess clinical and imaging features of iNPH, especially those older than 80 years. The number of persons with iNPH is probably much higher than the number of persons currently treated. PMID:24682964

  3. The Unilateral Urogenital Anomalies (UUA) Rat: A New Mutant Strain Associated with Unilateral Renal Agenesis, Cryptorchidism, and Malformations of Reproductive Organs Restricted to the Left Side

    Science.gov (United States)

    Amakasu, Kohei; Suzuki, Katsushi; Suzuki, Hiroetsu

    2009-01-01

    We established an inbred rat strain with unilateral urogenital anomalies from an incidentally identified male rat with unilateral renal agenesis and an undescended left testis. These rats were characterized by unilateral renal agenesis in both sexes, undescended testes with agenesis and hypoplasia of the accessory sex organs in male rats, and complete and partial agenesis of the uterine horn in female rats. All of these urogenital anomalies were unilateral and restricted to the left side; we named this phenotype unilateral urogenital anomalies (UUA). Breeding tests showed that these abnormalities were inherited as polygenic traits. The weight of right kidneys of affected rats was 1.7-fold higher than that of normal rats; histologically, glomerulosclerosis, tubular dilations, and tubular casts were detected at 30 wk of age. These alterations may have resulted from compensatory renal adaptation to the lack of 1 kidney. The cryptorchid left testes of affected male rats showed atrophy of seminiferous tubules and degeneration of spermatocytes and spermatids. These results indicate that the UUA rat may be a good model to study the etiology of unilateral renal agenesis accompanied by agenesis of the reproductive tract and to study compensatory alterations resulting from the congenital loss of 1 kidney. PMID:19619415

  4. Factors Associated with Acute and Chronic Hydrocephalus in Nonaneurysmal Subarachnoid Hemorrhage.

    Science.gov (United States)

    Kang, Peter; Raya, Amanda; Zipfel, Gregory J; Dhar, Rajat

    2016-02-01

    Hydrocephalus requiring external ventricular drain (EVD) or shunt placement commonly complicates aneurysmal subarachnoid hemorrhage (SAH), but its frequency is not as well known for nonaneurysmal SAH (NA-SAH). Those with diffuse bleeding may have greater risk of hydrocephalus compared to those with a perimesencephalic pattern. We evaluated the frequency of hydrocephalus in NA-SAH and whether imaging factors could predict the need for EVD and shunting. We collected admission clinical and imaging variables for 105 NA-SAH patients, including bicaudate index (BI), Hijdra sum score (HSS), intraventricular hemorrhage (IVH) score, modified Fisher scale (mFS), and bleeding pattern. Hydrocephalus was categorized as acute (need for EVD) or chronic (shunt). We applied logistic regression to determine whether hydrocephalus risk was independently related to bleeding pattern or mediated through blood volume or ventriculomegaly. Acute hydrocephalus was seen in 26 (25%) patients but was more common with diffuse (15/28, 54%) versus perimesencephalic (10/59, 17%, p hydrocephalus had worse clinical grade and higher BI, HSS, and IVH scores. Adjusting the relationship between hydrocephalus and diffuse bleeding for HSS (but not BI) nullified this association. Nine (35%) patients requiring EVD eventually required shunting for chronic hydrocephalus, which was associated with greater blood burden but not poor clinical grade. Acute hydrocephalus occurs in one-quarter of NA-SAH patients. The greater risk in diffuse bleeding appears to be mediated by greater cisternal blood volume but not by greater ventriculomegaly. Imaging characteristics may aid in anticipatory management of hydrocephalus in NA-SAH.

  5. Communicating hydrocephalus due to cerebral venous sinus thrombosis treated with ventriculoperitoneal shunt

    Science.gov (United States)

    Chakor, Rahul T.; Jakhere, Sandeep; Gavai, Bhakti Yeragi; Santhosh, N. S.

    2012-01-01

    Cerebral venous sinus thrombosis (CVT) is a rare cerebrovascular disease with variable presentation. CVT rarely causes hydrocephalus. Communicating hydrocephalus due to CVT is extremely rare. We describe a patient of CVT presenting with chronic headache and communicating hydrocephalus. The patient was successfully treated with ventriculoperitoneal (VP) shunt. A 40 year old man presented with moderate to severe headache since six months and progressive visual loss since two months. Head Computed tomogram showed mild hydrocephalus without obstruction. Lumbar puncture (LP) demonstrated elevated pressure but was otherwise normal. Magnetic resonance venogram showed extensive CVT. Repeated CSF drainage and thecoperitoneal shunt did not relieve the severe headache hence a VP shunt was placed. Post shunt headache subsided with resolution of hydrocephalus. CVT can present as communicating hydrocephalus. Gradual reduction of intra-ventricular pressure by repeated LPs followed by VP shunt can safely treat hydrocephalus due to CVT. PMID:23349607

  6. Communicating hydrocephalus due to cerebral venous sinus thrombosis treated with ventriculoperitoneal shunt.

    Science.gov (United States)

    Chakor, Rahul T; Jakhere, Sandeep; Gavai, Bhakti Yeragi; Santhosh, N S

    2012-10-01

    Cerebral venous sinus thrombosis (CVT) is a rare cerebrovascular disease with variable presentation. CVT rarely causes hydrocephalus. Communicating hydrocephalus due to CVT is extremely rare. We describe a patient of CVT presenting with chronic headache and communicating hydrocephalus. The patient was successfully treated with ventriculoperitoneal (VP) shunt. A 40 year old man presented with moderate to severe headache since six months and progressive visual loss since two months. Head Computed tomogram showed mild hydrocephalus without obstruction. Lumbar puncture (LP) demonstrated elevated pressure but was otherwise normal. Magnetic resonance venogram showed extensive CVT. Repeated CSF drainage and thecoperitoneal shunt did not relieve the severe headache hence a VP shunt was placed. Post shunt headache subsided with resolution of hydrocephalus. CVT can present as communicating hydrocephalus. Gradual reduction of intra-ventricular pressure by repeated LPs followed by VP shunt can safely treat hydrocephalus due to CVT.

  7. Communicating hydrocephalus due to cerebral venous sinus thrombosis treated with ventriculoperitoneal shunt

    Directory of Open Access Journals (Sweden)

    Rahul T Chakor

    2012-01-01

    Full Text Available Cerebral venous sinus thrombosis (CVT is a rare cerebrovascular disease with variable presentation. CVT rarely causes hydrocephalus. Communicating hydrocephalus due to CVT is extremely rare. We describe a patient of CVT presenting with chronic headache and communicating hydrocephalus. The patient was successfully treated with ventriculoperitoneal (VP shunt. A 40 year old man presented with moderate to severe headache since six months and progressive visual loss since two months. Head Computed tomogram showed mild hydrocephalus without obstruction. Lumbar puncture (LP demonstrated elevated pressure but was otherwise normal. Magnetic resonance venogram showed extensive CVT. Repeated CSF drainage and thecoperitoneal shunt did not relieve the severe headache hence a VP shunt was placed. Post shunt headache subsided with resolution of hydrocephalus. CVT can present as communicating hydrocephalus. Gradual reduction of intra-ventricular pressure by repeated LPs followed by VP shunt can safely treat hydrocephalus due to CVT.

  8. Congenital amusia.

    Science.gov (United States)

    Williamson, Victoria J; Stewart, Lauren

    2013-01-01

    For most people, music, like language, is acquired effortlessly in early life. But a few percent of the population have lifelong difficulties in the perception and production of music. In this chapter we discuss psycho-acoustic and behavioral studies that have attempted to delineate the nature of the auditory perceptual deficits in this group and consider whether these difficulties extend outside the musical domain. Finally, we review structural imaging studies in this group which point to subtle anomalies in temporal and frontal areas. We suggest that amusia can be considered a disorder of neural development, which has relatively specific consequences at the behavioral level. Studies of congenital amusia provide a unique window on the neurocognitive architecture of music processing. Copyright © 2013 Elsevier B.V. All rights reserved.

  9. Congenital hypothyroidism

    Directory of Open Access Journals (Sweden)

    Pankaj Agrawal

    2015-01-01

    Full Text Available Congenital hypothyroidism (CH is the one of the most common preventable cause of mental retardation. In the majority of patients, CH is caused by an abnormal development of the thyroid gland (thyroid dysgenesis that is a sporadic disorder and accounts for 85% of cases and the remaining 15% of cases are caused by dyshormonogenesis. The clinical features of congenital hypothyroidism are so subtle that many newborn infants remain undiagnosed at birth and delayed diagnosis leads to the most severe outcome of CH, mental retardation, emphasizing the importance of neonatal screening. Dried capillary blood is used for screening and it is taken from heel prick optimally between 2 and 5 days of age. Blood spot TSH or thyroxine (T4 or both are being used for CH screening in different programs around the world. Neonates with abnormal thyroid screening tests should be recalled immediately for examination and a venipuncture blood sample should be drawn for confirmatory serum testing. Confirmatory serum should be tested for TSH and free T4, or total T4. Serum TSH and T4 undergo dynamic changes in the first weeks of life; it is important to compare serum results with age-normal reference ranges. Treatment should be started promptly and infant should be rendered euthyroid as early as possible, as there is an inverse relationship between intelligence quotient (IQ and the age at diagnosis. Levothyroxine (l-thyroxine is the treatment of choice and American academy of pediatrics and European society of pediatric endocrinology recommend 10-15μgm/kg/day as initial dose. The immediate goal of therapy is to normalize T4 within 2 weeks and TSH within one month. The overall goal of treatment is to ensure growth and neurodevelopmental outcomes as close as possible to their genetic potential.

  10. Latex allergy in an infant with acquired hydrocephalus | Ehiozw ...

    African Journals Online (AJOL)

    We report the case of a 3 month old male infant with acquired hydrocephalus undergoing ventriculo-peritoneal shunt insertion who developed wheals and suffered a respiratory arrest following contact with latex gloves. The need for anaesthetists to effectively diagnose and properly manage this rare clinical entity is ...

  11. The triad of idiopathic normal-pressure hydrocephalus: a clinical ...

    African Journals Online (AJOL)

    ... incrementing doses of both donepezil and carbidopa-levodopa. Urinary urgency was believed to have been due to prostate hypertrophy. A head CT with contrast revealed moderate ventriculomegaly in the setting of mild diffuse cortical atrophy. A diagnosis of idiopathic normal-pressure hydrocephalus (INPH) was made.

  12. Management and functional outcome of childhood hydrocephalus at ...

    African Journals Online (AJOL)

    Methods: A retrospective analysis was conducted on 200 childrenwho were admitted to the neurosurgical unit at the Kenyatta National Hospital and diagnosed with hydrocephalus between January 2000 and April 2013. The records were analyzed for demographic characteristics, clinical presentation and management ...

  13. Prevalence of neural tube defect and hydrocephalus in Northern ...

    African Journals Online (AJOL)

    All the cases reported in this study were open neural tube defect (NTD). The most common defect was hydrocephalus occurring in 33 patients representing 57.9%, with spinal bifida occurring in 21 patients representing 38.6%. Encephalocele or cranium bifida occurred in only 5.3% (3 patients). Among the spinal bifida cases ...

  14. Hydrocephalus in spina bida | Morgado | South African Medical ...

    African Journals Online (AJOL)

    Hydrocephalus is one of the most common complications of spinal dysraphism. Although few patients require cerebrospinal fluid diversion immediately at birth or within the first few days of life, most patients with myelomeningocele, which comprises the most prevalent, clinically significant form of spina bifida, will eventually ...

  15. Everolimus Alleviates Obstructive Hydrocephalus due to Subependymal Giant Cell Astrocytomas.

    Science.gov (United States)

    Moavero, Romina; Carai, Andrea; Mastronuzzi, Angela; Marciano, Sara; Graziola, Federica; Vigevano, Federico; Curatolo, Paolo

    2017-03-01

    Subependymal giant cell astrocytomas (SEGAs) are low-grade tumors affecting up to 20% of patients with tuberous sclerosis complex (TSC). Early neurosurgical resection has been the only standard treatment until few years ago when a better understanding of the molecular pathogenesis of TSC led to the use of mammalian target of rapamycin (mTOR) inhibitors. Surgical resection of SEGAs is still considered as the first line treatment in individuals with symptomatic hydrocephalus and intratumoral hemorrhage. We describe four patients with symptomatic or asymptomatic hydrocephalus who were successfully treated with the mTOR inhibitor everolimus. We collected the clinical data of four consecutive patients presenting with symptomatic or asymptomatic hydrocephalus due to a growth of subependymal giant cell atrocytomas and who could not undergo surgery for different reasons. All patients experienced a clinically significant response to everolimus and an early shrinkage of the SEGA with improvement in ventricular dilatation. Everolimus was well tolerated by all individuals. Our clinical series demonstrate a possible expanding indication for mTOR inhibition in TSC, which can be considered in patients with asymptomatic hydrocephalus or even when the symptoms already appeared. It offers a significant therapeutic alternative to individuals that once would have undergone immediate surgery. Everolimus might also allow postponement of a neurosurgical resection, making it elective with an overall lower risk. Copyright © 2016 Elsevier Inc. All rights reserved.

  16. Acute infarction of corpus callosum due to transient obstructive hydrocephalus.

    Science.gov (United States)

    Kaymakamzade, Bahar; Eker, Amber

    2016-01-01

    Acute ischemia of the corpus callosum (CC) is not a well-known feature in patients with acute hydrocephalus. Herein, we describe a case with acute CC infarction due to another rare entity; transient obstructive hydrocephalus. A 66-year-old male was admitted with sudden onset right-sided hemiparesia. CT demonstrated a hematoma on the left basal ganglia with extension to all ventricles. The following day, the patient's neurological status progressed to coma and developed bilateral pyramidal signs. MRI demonstrated obstructive hydrocephalus and acute diffuse infarction accompanied by elevation of the CC. On the same day there was improvement in his neurological status with significant decrease in ventricular size and complete resolution of the clot in the third ventricle. The mechanism of signal abnormalities is probably related with the neural compression of the CC against the falx. Presumably, the clot causing obstruction in the third ventricle dissolved or decayed by the help of fibrinolytic activity of CSF, which was raised after IVH and caused spontaneous improvement of hydrocephalus. Bilateral neurological symptoms suggest diffuse axonal damage and normalization of the intracranial pressure should be performed on the early onset of clinical detorioration in order to prevent axonal injury. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  17. Nonverbal Learning Disability Explained: The Link to Shunted Hydrocephalus

    Science.gov (United States)

    Rissman, Barbara

    2011-01-01

    A nonverbal learning disability is believed to be caused by damage, disorder or destruction of neuronal white matter in the brain's right hemisphere and may be seen in persons experiencing a wide range of neurological diseases such as hydrocephalus and other types of brain injury (Harnadek & Rourke 1994). This article probes the relationship…

  18. Complication during Postacute Rehabilitation: Patients with Posttraumatic Hydrocephalus

    Science.gov (United States)

    Denes, Zoltan; Barsi, Peter; Szel, Istvan; Boros, Erzsebet; Fazekas, Gabor

    2011-01-01

    The objective of this study was to determine the incidence of posttraumatic hydrocephalus (PTH) among patients in our rehabilitation unit for traumatic brain injury. Furthermore, we aimed to assess the effect of shunt implantation and to identify the postoperative complication rate. This is a retrospective cohort study, in which between 2000 and…

  19. Importância da avaliação genético-clínica na hidrocefalia Importance of the clinical genetics evaluation on hydrocephalus

    Directory of Open Access Journals (Sweden)

    Marta Wey-Vieira

    2004-06-01

    Full Text Available Os objetivos deste estudo foram caracterizar a presença de possíveis quadros de etiologia genética entre portadores de hidrocefalia congênita de etiologia não anteriormente esclarecida e confirmar aqueles com etiologia identificada previamente. A casuística compôs-se de 16 pacientes portadores de hidrocefalia congênita. O protocolo de investigação incluiu anamnese, investigação de história familial, exame clínico-dismorfológico, tomografia computadorizada ou ressonância magnética de sistema nervoso central, radiografia vertebral simples, cariótipo e estudo dismorfológico. Para análise dos resultados, a casuística foi dividida em dois grupos. O Grupo I (3M:6F caracterizado por indivíduos com hidrocefalia e sinais clínicos inespecíficos; o Grupo II (7M, em que os indivíduos apresentavam hidrocefalia congênita e sinais sugestivos do espectro da doença L1. Orientação genética específica foi possível em 11 casos. Os resultados demonstram a heterogeneidade etiológica envolvida na hidrocefalia, evidenciando a necessidade de avaliação clínico-dismorfológica como instrumento complementar na investigação dessa condição clínica.The aim of this study was to characterize the possibility of genetic etiology in a group of individuals with congenital hydrocephalus in which the etiology was indeterminate and to confirm that earlier diagnosed. The casuistry was composed by 16 individuals with congenital hydrocephalus. Investigation protocol included anamnesis, familial investigation, physical examination, computerized tomography or magnetic resonance image of head, vertebral column X-ray, karyotype and dysmorphological study. Results were analyzed in two groups. In Group I (3M:9F was composed by hydrocephalus associated with unspecific signs. Group II (7 males had findings of epectrum of L1 disease. Genetic counseling could be offered in 11 cases. These results demonstrate the great etiological heterogeneity of congenital

  20. [Analyses of the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane].

    Science.gov (United States)

    Tang, Chaoying; Zhang, Jishuai; Han, Weiju; Shen, Weidong; Liu, Jun; Hou, Zhaohui; Dai, Pu; Yang, Shiming; Han, Dongyi

    2016-05-01

    To analyze the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane, and summarize the key diagnostic points, differential diagnosis and observe the effects of surgical treatment. We reviewed data from 82 patients with unilateral conductive hearing loss with intact tympanic membranes who accepted the exploratory tympanotomy from April 2011 to September 2013. There were 41 males and 41 females, aged from 7 to 66( averaged 26.5±13.7)years, with a history of one month to 50 years. The history, clinical symptoms, audiological evaluation, high resolution temporal bone CT, the results of surgical exploration and hearing reconstruction were analyzed. The exploratory tympanotomy revealed 43 cases of congenital middle ear malformations (52.4%), 22 cases of otosclerosis (26.8%), eight cases of congenital cholesteatoma (9.8%), six cases of trauma induced conductive hearing loss (7.3%), three cases of congenital ossicular malformations with congenital cholesteatoma (3.7%). Progressive hearing loss was common in patients with otosclerosis and congenital cholesteatoma, and patients with congenital middle ear malformations described their hearing loss since childhood. High resolution temporal bone CT of congenital middle ear malformation, trauma induced conductive hearing loss, congenital cholesteatoma diagnosis rate was 40.0%, 50.0%, and 83.3% respectively. The preoperative air-conductive threshold of patients with absence of the oval window were increased to (66.9±1.1)dBHL, the preoperative bone-conductive threshold achieved (28.3±10.4)dBHL at 2 000 Hz. While patients with stapes fixation and that with ossicular chain discontinuity were (27.2±9.7)dBHL and (17.8±8.8)dBHL(P=0.000)respectively. Through the tympanic exploration with endaural incision under the microscope, different hearing reconstruction were applied according to different lesions. After the operation, the hearing level of 52 patients with return visit were improved, the

  1. Dyke-Davidoff-Masson syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery

    OpenAIRE

    Piro, Ettore; Piccione, Maria; Marrone, Gianluca; Giuffr?, Mario; Corsello, Giovanni

    2013-01-01

    Prenatal ultrasonographic detection of unilateral cerebral ventriculomegaly arises suspicion of pathological condition related to cerebrospinal fluid flow obstruction or cerebral parenchimal pathology. Dyke-Davidoff-Masson syndrome is a rare condition characterized by cerebral hemiatrophy, calvarial thickening, skull and facial asymmetry, contralateral hemiparesis, cognitive impairment and seizures. Congenital and acquired types are recognized and have been described, mainly in late childhood...

  2. Endoscopic third ventriculostomy and choroid plexus cauterization in infants with hydrocephalus: a retrospective Hydrocephalus Clinical Research Network study.

    Science.gov (United States)

    Kulkarni, Abhaya V; Riva-Cambrin, Jay; Browd, Samuel R; Drake, James M; Holubkov, Richard; Kestle, John R W; Limbrick, David D; Rozzelle, Curtis J; Simon, Tamara D; Tamber, Mandeep S; Wellons, John C; Whitehead, William E

    2014-09-01

    The use of endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has been advocated as an alternative to CSF shunting in infants with hydrocephalus. There are limited reports of this procedure in the North American population, however. The authors provide a retrospective review of the experience with combined ETV + CPC within the North American Hydrocephalus Clinical Research Network (HCRN). All children (age within the HCRN. Thirty-six patients with ETV + CPC were included (13 with previous shunt). The etiologies of hydrocephalus were as follows: intraventricular hemorrhage of prematurity (9 patients), aqueductal stenosis (8), myelomeningocele (4), and other (15). There were no major intraoperative or early postoperative complications. There were 2 postoperative CSF infections. There were 2 deaths unrelated to hydrocephalus and 1 death from seizure. In 18 patients ETV + CPC failed at a median time of 30 days after surgery (range 4-484 days). The actuarial 3-, 6-, and 12-month success for ETV + CPC was 58%, 52%, and 52%. Time to treatment failure was slightly worse for the 36 patients with ETV + CPC compared with the 758 infants treated with shunts (p = 0.012). Near-complete CPC (≥ 90%) was achieved in 11 cases (31%) overall, but in 50% (10 of 20 cases) in 2012 versus 6% (1 of 16 cases) before 2012 (p = 0.009). Failure was higher in children with learning curve and appears to affect success, suggesting that surgeon training might improve results.

  3. Treatment of Class II subdivision malocclusion with congenitally missing upper lateral incisors: A case report

    OpenAIRE

    Siddharth Mehta; Ashima Valiathan; Arun Urala

    2014-01-01

    Orthodontic treatment for patients with unilateral or bilateral congenitally missing lateral incisor poses a challenge mainly with regard to treatment planning. The use of a diagnostic setup is one of the most important aids in the decision-making process. Two alternatives, orthodontic space closure or space opening for prosthetic replacement exist. The present case report shows use of the microimplant for unilateral upper molar distalization and space closure in a Class-II division 1 subdivi...

  4. Evaluation of mandibular condyles in children with unilateral posterior crossbite

    Directory of Open Access Journals (Sweden)

    Edson ILLIPRONTI-FILHO

    2015-01-01

    Full Text Available The relationship of mandibular condyle dimensions and its association with unilateral posterior crossbite (UPXB has been suggested in the literature. The purpose of this prospective study was to evaluate mandibular condyles on the left and right sides and between crossed and non-crossed sides in the sagittal and coronal planes, using cone-beam computed tomography (CBCT. Twenty CBCT images of 40 temporo mandibular joints (TMJs in individuals in mixed dentition phase, which included 9 males (mean 7.9 years and 11 females (mean 8.2 years, with unilateral posterior crossbite without premature contacts and functional mandibular shifts and with transverse maxillary deficiency. The criteria for sample exclusion included the presence of painful symptoms, facial trauma history, systemic diseases such as juvenile rheumatoid arthritis, mouth opening limitation (< 40 mm, congenital or genetic anomalies, and skeletal asymmetries that may result in TMJ disorders. Dimensional measurements of the condyles between the right and left sides and crossed and non-crossed sides in sagittal and coronal view were made. There was no significant difference between the measurements of the crossed and non-crossed sides in both sagittal and coronal view. These findings suggest that the presence of unilateral posterior crossbite in children with UPXB did not result in changes between the mandibular condyles in the right and left sides or between the crossed and non-crossed sides in the coronal or sagittal plane.

  5. Predictors and outcomes of shunt-dependent hydrocephalus in patients with aneurysmal sub-arachnoid hemorrhage

    Science.gov (United States)

    2012-01-01

    Background Hydrocephalus following spontaneous aneurysmal sub-arachnoid hemorrhage (SAH) is often associated with unfavorable outcome. This study aimed to determine the potential risk factors and outcomes of shunt-dependent hydrocephalus in aneurysmal SAH patients but without hydrocephalus upon arrival at the hospital. Methods One hundred and sixty-eight aneurysmal SAH patients were evaluated. Using functional scores, those without hydrocephalus upon arrival at the hospital were compared to those already with hydrocephalus on admission, those who developed it during hospitalization, and those who did not develop it throughout their hospital stay. The Glasgow Coma Score, modified Fisher SAH grade, and World Federation of Neurosurgical Societies grade were determined at the emergency room. Therapeutic outcomes immediately after discharge and 18 months after were assessed using the Glasgow Outcome Score. Results Hydrocephalus accounted for 61.9% (104/168) of all episodes, including 82 with initial hydrocephalus on admission and 22 with subsequent hydrocephalus. Both the presence of intra-ventricular hemorrhage on admission and post-operative intra-cerebral hemorrhage were independently associated with shunt-dependent hydrocephalus in patients without hydrocephalus on admission. After a minimum 1.5 years of follow-up, the mean Glasgow outcome score was 3.33 ± 1.40 for patients with shunt-dependent hydrocephalus and 4.21 ± 1.19 for those without. Conclusions The presence of intra-ventricular hemorrhage, lower mean Glasgow Coma Scale score, and higher mean scores of the modified Fisher SAH and World Federation of Neurosurgical grading on admission imply risk of shunt-dependent hydrocephalus in patients without initial hydrocephalus. These patients have worse short- and long-term outcomes and longer hospitalization. PMID:22765765

  6. Predictors and outcomes of shunt-dependent hydrocephalus in patients with aneurysmal sub-arachnoid hemorrhage

    Directory of Open Access Journals (Sweden)

    Wang Yi-Min

    2012-07-01

    Full Text Available Abstract Background Hydrocephalus following spontaneous aneurysmal sub-arachnoid hemorrhage (SAH is often associated with unfavorable outcome. This study aimed to determine the potential risk factors and outcomes of shunt-dependent hydrocephalus in aneurysmal SAH patients but without hydrocephalus upon arrival at the hospital. Methods One hundred and sixty-eight aneurysmal SAH patients were evaluated. Using functional scores, those without hydrocephalus upon arrival at the hospital were compared to those already with hydrocephalus on admission, those who developed it during hospitalization, and those who did not develop it throughout their hospital stay. The Glasgow Coma Score, modified Fisher SAH grade, and World Federation of Neurosurgical Societies grade were determined at the emergency room. Therapeutic outcomes immediately after discharge and 18 months after were assessed using the Glasgow Outcome Score. Results Hydrocephalus accounted for 61.9% (104/168 of all episodes, including 82 with initial hydrocephalus on admission and 22 with subsequent hydrocephalus. Both the presence of intra-ventricular hemorrhage on admission and post-operative intra-cerebral hemorrhage were independently associated with shunt-dependent hydrocephalus in patients without hydrocephalus on admission. After a minimum 1.5 years of follow-up, the mean Glasgow outcome score was 3.33 ± 1.40 for patients with shunt-dependent hydrocephalus and 4.21 ± 1.19 for those without. Conclusions The presence of intra-ventricular hemorrhage, lower mean Glasgow Coma Scale score, and higher mean scores of the modified Fisher SAH and World Federation of Neurosurgical grading on admission imply risk of shunt-dependent hydrocephalus in patients without initial hydrocephalus. These patients have worse short- and long-term outcomes and longer hospitalization.

  7. Treatment of Class II subdivision malocclusion with congenitally missing upper lateral incisors: A case report

    Directory of Open Access Journals (Sweden)

    Siddharth Mehta

    2014-01-01

    Full Text Available Orthodontic treatment for patients with unilateral or bilateral congenitally missing lateral incisor poses a challenge mainly with regard to treatment planning. The use of a diagnostic setup is one of the most important aids in the decision-making process. Two alternatives, orthodontic space closure or space opening for prosthetic replacement exist. The present case report shows use of the microimplant for unilateral upper molar distalization and space closure in a Class-II division 1 subdivision malocclusion case with bilateral congenitally missing upper lateral incisors.

  8. Congenital Synechia and Syngnathia: Two Case Reports.

    Science.gov (United States)

    Khan, Mohammad Nazir; Ibrahim, Muhammad Wasim; Niazi, Kausar Khan

    2018-02-01

    This case report presents two cases of rare congenital malformation, i.e syngnathia. First case is of 2-day infant with bilateral fusion of maxilla and mandible, leaving a small anterior portion. After consultation with other concerned specialties, early intervention was planned and fusion was released to facilitate feeding. The infant suffered from frequent respiratory tract infections and subsequently died at the age of ten months. The second case is of 8-month baby girl with unilateral congenital maxillomandibular bony fusion without any other anomaly. She underwent general anesthesia for thorough examination and release of soft tissue union. Second surgery was performed after few months for removal of bony fusion. Good mouth opening was seen on 1 month follow-up.

  9. [Congenital hypothyroidism].

    Science.gov (United States)

    Castilla Peón, María Fernanda

    Congenital hypothyroidism (CH) is a cause of preventable mental retardation; therefore, timely diagnosis and treatment by the primary care physician is very important. CH screening must be performed between the second and fifth days of life with capillary blood done with a heel prick and must be confirmed by measurement of thyroid hormones in venous blood. The most common cause of CH is thyroid dysgenesis, which may be identified by a thyroid scan carried out before initiating treatment. Treatment should be with levothyroxine (10-15μg/kg/day) and should not be delayed or suspended during the first 3 years of life due to the deleterious effect on neurodevelopment in case of low thyroid hormones during this time. Preterm or sick infants or those with Down syndrome require special consideration. This article provides diagnostic and therapeutic algorithms for CH. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  10. Fertility potential after unilateral orchiopexy

    DEFF Research Database (Denmark)

    Cortes, D; Thorup, J M; Lindenberg, S

    1996-01-01

    PURPOSE: We evaluated whether adult fertility potential was better when unilateral orchiopexy was done at ages 2 to 6 years or later, and we identified those at risk for infertility. MATERIALS AND METHODS: Unilateral orchiopexy was performed simultaneously with testicular biopsy in 11 patients 2...... similar fertility potentials were found in adulthood. Five of the 65 patients (7.7%, 95% confidence limits 2.5 to 17%) may experience infertility, representing 33% of both groups with less than 1% of the age matched number of spermatogonia per tubular transverse section (approximately no germ cells......) in the biopsy specimen at orchiopexy. CONCLUSIONS: Between ages 2 and 12 years the timing of unilateral orchiopexy may vary without an effect on subsequent fertility potential. When biopsy at surgery lacks germ cells, there is an approximately 33% age independent risk of subsequent infertility. Otherwise...

  11. First reported case of unilateral Graves' disease in the left lobe of a bilobar thyroid gland.

    Science.gov (United States)

    Chen, Louis C; Green, Jennifer B

    2011-06-01

    Unilateral Graves' disease is a rare disease variant that can occur in a bilobar thyroid gland. We report the first documented case of unilateral Graves' disease in the left lobe of a bilobar thyroid gland and review the pertinent literature. A 48-year-old man presented in June 2010 with thyrotoxicosis. I-131 radioisotope uptake was elevated at 33.4%, and scintigraphy revealed that uptake of the radioisotope was uniformly increased in the left lobe of the thyroid gland. Ultrasonography of the thyroid gland revealed a non-nodular, enlarged, and heterogeneous left lobe; Doppler investigation of the lobe showed hypervascularity classically seen in Graves' disease. The right lobe of the thyroid, on the other hand, appeared homogeneous and hypovascular on ultrasonography. Thyroid-stimulating immunoglobulin was significantly elevated at 191% (reference range disease was the most likely diagnosis. As has occasionally been described in the literature, unilateral involvement of the thyroid gland is a rare presentation of Graves' disease. Pre-existing functional or structural differences (either congenital or acquired) between the two lobes may contribute to this rare presentation. To our knowledge, this is the first reported case of unilateral Graves' disease presenting in the left lobe of a bilobar thyroid gland. Although the pathophysiology of unilateral Graves's disease has not been clearly elucidated, clinicians should be aware that Graves' disease can present unilaterally in either lobe of the thyroid gland.

  12. Unilateral hyperplasia of the left posterior arch and associated vertebral schisis at C6 level

    Energy Technology Data Exchange (ETDEWEB)

    Esposito, Giuseppe; Bonis, Pasquale de; Tamburrini, Gianpiero; Massimi, Luca; Rocco, Concezio di [Catholic University, School of Medicine, Department of Pediatric Neurosurgery, Rome (Italy); Byvaltsev, Vadim [Irkutsk Railway Clinical Hospital, Department of Neurosurgery, Irkutsk (Russian Federation); Leone, Antonio [Catholic University, School of Medicine, Department of Bioimaging and Radiological Sciences, Rome (Italy)

    2009-12-15

    We report on a 5-year-old girl with unilateral hyperplasia of the left posterior arch of C6 associated with spina bifida occulta at the same level. Anteroposterior and lateral radiographs of the cervical spine showed hypertrophy of the left lamina as well as overgrowth and elongation of the left spinous process of the sixth cervical vertebra. Computed tomography (CT) examination better depicted this congenital variant and clearly showed the associated schisis of the posterior arch at the same level. Magnetic resonance (MR) imaging examination ruled out other spinal anomalies. The neck pain, the young age of the patient and the local aesthetic abnormality contributed to the surgical indication. To the best of our knowledge, this is the first case in the English literature of unilateral hyperplasia of a posterior cervical arch. Only one previous study has reported a similar congenital anomaly, but it was limited to the left side of the spinous process. (orig.)

  13. Unilateral hyperplasia of the left posterior arch and associated vertebral schisis at C6 level

    International Nuclear Information System (INIS)

    Esposito, Giuseppe; Bonis, Pasquale de; Tamburrini, Gianpiero; Massimi, Luca; Rocco, Concezio di; Byvaltsev, Vadim; Leone, Antonio

    2009-01-01

    We report on a 5-year-old girl with unilateral hyperplasia of the left posterior arch of C6 associated with spina bifida occulta at the same level. Anteroposterior and lateral radiographs of the cervical spine showed hypertrophy of the left lamina as well as overgrowth and elongation of the left spinous process of the sixth cervical vertebra. Computed tomography (CT) examination better depicted this congenital variant and clearly showed the associated schisis of the posterior arch at the same level. Magnetic resonance (MR) imaging examination ruled out other spinal anomalies. The neck pain, the young age of the patient and the local aesthetic abnormality contributed to the surgical indication. To the best of our knowledge, this is the first case in the English literature of unilateral hyperplasia of a posterior cervical arch. Only one previous study has reported a similar congenital anomaly, but it was limited to the left side of the spinous process. (orig.)

  14. Congenital deformity of the distal extremities in three dogs

    Directory of Open Access Journals (Sweden)

    F. Di Dona

    2016-11-01

    Full Text Available Congenital limb deformities are very rare conditions and the knowledge about etiology, pathogenesis, clinical presentation and treatment is still poor. Moreover, many defects are still not reported in veterinary literature. This report documents clinical and radiographic findings in three dogs with congenital deformity involving the distal extremities. Case 1 was affected with bilateral aphalangia of the pedes, case 2 presented a combination of brachydactyly and syndactyly, whereas in case 3 a unilateral ectrodactyly was observed. To the authors’ knowledge, brachydactyly, as well as aphalangia, are very uncommon anomalies and have been rarely documented. Moreover, association between syndactyly and brachydactyly has still not been reported.

  15. Congenital anomalies of the pulmonary arteries: spectrum of findings on computed tomography.

    Science.gov (United States)

    Bueno, J; Flors, L; Mejía, M

    Congenital anomalies of the pulmonary arteries are uncommon. They can occur in isolation or in association with congenital heart defects. Isolated congenital anomalies remain undiscovered until they are reported as incidental findings on imaging tests, usually not until adolescence. We review the embryological development and normal anatomy of the pulmonary arteries as well as the spectrum of computed tomography findings for various congenital anomalies: unilateral interruption of the pulmonary artery, anomalous origin of the left pulmonary artery (pulmonary artery sling), idiopathic aneurysm of the pulmonary artery, and other anomalies associated with congenital heart defects. Congenital anomalies of the pulmonary arteries represent a diagnostic challenge for clinicians and radiologists. Computed tomography is useful for their diagnosis, and general radiologists need to be familiar with their imaging appearance because they are often discovered incidentally. Copyright © 2016 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. In need of a patch UP: Recurrent congenital diaphragmatic hernia presenting with a large pleural effusion

    Directory of Open Access Journals (Sweden)

    Farhana Shariff

    2014-10-01

    Full Text Available We report a case of recurrent congenital diaphragmatic hernia (CDH presenting with a large unilateral pleural effusion. A 12-year old boy who had a left sided CDH repaired in the neonatal period, presented with fever, lethargy, and non-productive cough. Chest radiograph demonstrated a loculated pleural effusion. Computed tomography scan revealed recurrent herniation of abdominal contents. To our knowledge, this is the first reported case of a recurrent congenital diaphragmatic hernia presenting with large pleural effusion.

  17. Congenital heart disease

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/001114.htm Congenital heart disease To use the sharing features on this page, please enable JavaScript. Congenital heart disease (CHD) is a problem with the heart's structure ...

  18. Unilateral submandibular gland aplasia masquerading as cancer nodal metastasis.

    Science.gov (United States)

    Shipchandler, Taha Z; Lorenz, Robert R

    2008-01-01

    Five reports have examined unilateral submandibular gland aplasia. The purposes of this report are to demonstrate submandibular gland aplasia leading to contralateral gland hypertrophy in the setting of oral cavity cancer and to discuss the corresponding diagnostic and management challenges. This study is a case report of a 60-year-old male who presented with pain on the right side of the mobile tongue. This report uses literature review. A 60-year-old male presented with pain on the right side of the mobile tongue. Subsequent results of punch biopsy revealed squamous cell carcinoma in situ with foci of microinvasion of the tongue. Head and neck examination revealed no abnormalities. The patient underwent a wide-local excision of the tongue lesion. Postoperative computed tomographic (CT) scan showed an asymmetric mass on the ipsilateral side of the cancer in the region of the submandibular gland. The gland was noted to be abnormally large. A diagnosis of contralateral submandibular gland aplasia was made. The patient is cancer-free at 2 years postlocal excision. Salivary gland aplasia is an extremely rare disorder and is often associated with various congenital syndromes. Unilateral submandibular gland aplasia is even rarer with ours representing the sixth reported case. Aplasia is believed to stem from a regional disturbance in early fetal development. Common symptoms can include dysphagia, dry mouth, decreased taste, and tooth decay. In the presence of a history of oral cavity cancer, unilateral submandibular gland aplasia poses a challenge during postoperative cancer follow-up. Unilateral submandibular gland aplasia in the setting of oral cavity cancer poses a unique challenge for cancer follow-up. Hypertrophy of the submandibular gland on the other side can masquerade as nodal metastasis. Head and neck examination as well as CT scan can be inconclusive. Regular confirmatory tests such as fine needle aspiration biopsy and positron emission tomography/CT for

  19. Gestational and congenital syphilis.

    Science.gov (United States)

    Wendel, G D

    1988-06-01

    The frequency of congenital syphilis continues to increase throughout the United States during the 1980s. Untreated maternal infection can lead to stillbirth, premature labor, congenital infection, and neonatal death. Preventive measures, based on control of early syphilis in women, prenatal care, improved diagnosis and diligent followup, can help to decrease the incidence of congenital syphilis. The clinical presentation and recommended regimens for therapy of the gravida with syphilis and neonates with suspected congenital syphilis are reviewed.

  20. Unilateral persistent hyperplastic primary vitreous: intensive management approach with excellent outcome beyond visual maturation.

    Science.gov (United States)

    Yusuf, Imran H; Patel, Chetan Kantibhai; Salmon, John F

    2015-01-06

    Persistent hyperplastic primary vitreous (PHPV) is an ocular developmental disorder resulting from incomplete apoptosis of the embryonic hyaloid vasculature. Unilateral PHPV is traditionally associated with a poor prognosis because of the challenges associated with managing progressive anisometropic amblyopia. We report a child with unilateral PHPV who underwent cataract extraction, primary posterior capsulotomy with anterior vitrectomy and intraocular lens implantation followed by combined trabeculectomy/trabeculotomy within the first 8 weeks of life. Intensive optometric and orthoptic input was required for many years to manage the increasing anisometropic amblyopia with final visual acuity of 20/40 unaided in the affected eye and without evidence of glaucomatous optic neuropathy. This case illustrates the excellent visual outcome possible in a child with complex, unilateral PHPV using an intensive management approach comprising: early surgical intervention for congenital cataract and secondary glaucoma, meticulous monitoring of refraction, visual acuity and intraocular pressure and motivated parents who engaged in the management. 2015 BMJ Publishing Group Ltd.

  1. Process Of Acceptance And Nurturing By Parents To Maintain Caring Affection To The Children With Hydrocephalus

    OpenAIRE

    Ageng Wirdhana, Sri; Naryoso, S.Sos, M.Si, Agus

    2017-01-01

    Based on data from The Ministry of Health Republic of Indonesia, there were 20 newborn babies suffered hydrocephalus from every 10.000 birth in Indonesia, Margaretha (Clinical Pshychology and Mental Health, 2014: Vol. 03, No.2). Hydrocephalus is a condition when the main characteristic is the excessive liquid accumulation in brain. This research aim to explain the process of acceptance and nurturing by parents to maintain caring affection to hydrocephalus children. This research used descript...

  2. Transient Obstructive Hydrocephalus in Patients with Intracerebral Hemorrhage: Report of Two Cases

    Science.gov (United States)

    Jergović, Ilija; Budinčević, Hrvoje; Planjar-Prvan, Miljenka; Bielen, Ivan

    2016-09-01

    Obstructive hydrocephalus is a relatively common complication of intraventricular hemorrhage resulting in high morbidity and mortality. We report two cases of transient obstructive hydrocephalus caused by obstruction of mesencephalic duct in patients that presented with altered consciousness which resolved spontaneously in a few hours. In very rare cases, obstructive hydrocephalus due to intraventricular hemorrhage may be transient and does not need neurosurgical or invasive procedures for lowering raised intracranial pressure, which otherwise are currently preferred treatment options.

  3. Communicating hydrocephalus due to cerebral venous sinus thrombosis treated with ventriculoperitoneal shunt

    OpenAIRE

    Rahul T Chakor; Sandeep Jakhere; Bhakti Yeragi Gavai; N S Santhosh

    2012-01-01

    Cerebral venous sinus thrombosis (CVT) is a rare cerebrovascular disease with variable presentation. CVT rarely causes hydrocephalus. Communicating hydrocephalus due to CVT is extremely rare. We describe a patient of CVT presenting with chronic headache and communicating hydrocephalus. The patient was successfully treated with ventriculoperitoneal (VP) shunt. A 40 year old man presented with moderate to severe headache since six months and progressive visual loss since two months. Head Comput...

  4. Comparison of CSF Distribution between Idiopathic Normal Pressure Hydrocephalus and Alzheimer Disease.

    Science.gov (United States)

    Yamada, S; Ishikawa, M; Yamamoto, K

    2016-07-01

    CSF volumes in the basal cistern and Sylvian fissure are increased in both idiopathic normal pressure hydrocephalus and Alzheimer disease, though the differences in these volumes in idiopathic normal pressure hydrocephalus and Alzheimer disease have not been well-described. Using CSF segmentation and volume quantification, we compared the distribution of CSF in idiopathic normal pressure hydrocephalus and Alzheimer disease. CSF volumes were extracted from T2-weighted 3D spin-echo sequences on 3T MR imaging and quantified semi-automatically. We compared the volumes and ratios of the ventricles and subarachnoid spaces after classification in 30 patients diagnosed with idiopathic normal pressure hydrocephalus, 10 with concurrent idiopathic normal pressure hydrocephalus and Alzheimer disease, 18 with Alzheimer disease, and 26 control subjects 60 years of age or older. Brain to ventricle ratios at the anterior and posterior commissure levels and 3D volumetric convexity cistern to ventricle ratios were useful indices for the differential diagnosis of idiopathic normal pressure hydrocephalus or idiopathic normal pressure hydrocephalus with Alzheimer disease from Alzheimer disease, similar to the z-Evans index and callosal angle. The most distinctive characteristics of the CSF distribution in idiopathic normal pressure hydrocephalus were small convexity subarachnoid spaces and the large volume of the basal cistern and Sylvian fissure. The distribution of the subarachnoid spaces in the idiopathic normal pressure hydrocephalus with Alzheimer disease group was the most deformed among these 3 groups, though the mean ventricular volume of the idiopathic normal pressure hydrocephalus with Alzheimer disease group was intermediate between that of the idiopathic normal pressure hydrocephalus and Alzheimer disease groups. The z-axial expansion of the lateral ventricle and compression of the brain just above the ventricle were the common findings in the parameters for differentiating

  5. Ehlers-Danlos syndrome type IV : unusual congenital anomalies in a mother and son with a COL3A1 mutation and a normal collagen III protein profile

    NARCIS (Netherlands)

    Kroes, HY; Pals, G; van Essen, AJ

    A mother and son with Ehlers-Danlos syndrome (EDS) type IV and unusual congenital anomalies are described. The congenital anomalies include, in the mother, amniotic band-like constrictions on one hand, a unilateral clubfoot, and macrocephaly owing to normal-pressure hydrocephaly and, in the son, an

  6. Transdermal hyoscine induced unilateral mydriasis.

    LENUS (Irish Health Repository)

    Hannon, Breffni

    2012-03-20

    The authors present a case of unilateral mydriasis in a teenager prescribed transdermal hyoscine hydrobromide (scopolamine) for chemotherapy induced nausea and vomiting. The authors discuss the ocular side-effects associated with this particular drug and delivery system and the potential use of transdermal hyoscine as an antiemetic agent in this group.

  7. Cryptococcal meningitis causing obstructive hydrocephalus in a patient on fingolimod.

    Science.gov (United States)

    Pham, Chengde; Bennett, Iwan; Jithoo, Rondhir

    2017-07-06

    Cryptococcosis is a recognised opportunistic infection in immunocompromised patients. The long-term adverse effect profile of fingolimod, an immunomodulating agent approved for use in multiple sclerosis in 2010, is only just emerging. We report the first case to our knowledge of a patient presenting with obstructive hydrocephalus secondary to cryptococcal meningitis in the setting of fingolimod therapy. Extensive posterior fossa leptomeningeal inflammation with associated cerebellar oedema resulted in effacement of the fourth ventricle and obstructive hydrocephalus requiring urgent ventriculostomy. Induction, consolidative and maintenance antifungal therapy was prescribed and subsequent conversion to a ventriculoperitoneal shunt was successful in relieving the patient's ventriculomegaly. Awareness of these rare, novel and life-threatening complications of fingolimod-associated immunocompromise is critical as the use of such drugs is expected to rise. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  8. Neuronal ceroid-lipofuscinosis and hydrocephalus in a chihuahua.

    Science.gov (United States)

    Kuwamura, M; Hattori, R; Yamate, J; Kotani, T; Sasai, K

    2003-05-01

    A two-year-old, female chihuahua presented with a six-month history of visual dysfunction. Computed tomography revealed dilation of the lateral ventricles in the central nervous system (CNS). The dog was tentatively diagnosed as having hydrocephalus and a month later was euthanased at the owner's request. The skull was expanded and dome-like in shape and an open fontanelle was observed on postmortem examination. Histologically, swollen neurons possessing yellowish pigment granules in the cytoplasm were observed throughout the CNS. These storage materials stained positively with periodic acid Schiff, Schmorl method for lipofuscin and oil red O for lipid, and showed autofluorescence under fluorescence microscopy. Ultrastructurally, the storage materials consisted of dense lamellar structures. This case was unique in having ceroid-lipofuscinosis in association with hydrocephalus.

  9. Diagnosis and differential diagnosis of hydrocephalus in adults; Diagnostik und Differenzialdiagnostik des Hydrozephalus beim Erwachsenen

    Energy Technology Data Exchange (ETDEWEB)

    Langner, Soenke; Mensel, Birger; Kuehn, Jens Peter; Kirsch, Michael [Univ. Medicine Greifswald (Germany). Inst. for Diagnostic Radiology and Neuroradiology; Fleck, Steffen; Baldauf, Joerg [Univ. Medicine Greifswald (Germany). Dept. of Neurosurgery

    2017-08-15

    Hydrocephalus is caused by an imbalance of production and absorption of cerebrospinal fluid (CSF) or obstruction of its pathways, resulting in ventricular dilatation and increased intracranial pressure. Imaging plays a crucial role in the diagnosis, differential diagnosis and planning of treatment. This review article presents the different types of hydrocephalus und their typical imaging appearance, describes imaging techniques, and discusses differential diagnoses of the different forms of hydrocephalus. Imaging plays a central role in the diagnosis of hydrocephalus. While magnetic resonance (MR) imaging is the first-line imaging modality, computed tomography (CT) is often the first-line imaging test in emergency patients.

  10. The Lhx9 homeobox gene controls pineal gland development and prevents postnatal hydrocephalus

    DEFF Research Database (Denmark)

    Yamazaki, Fumiyoshi; Møller, Morten; Fu, Cong

    2015-01-01

    stalk are also reduced in size. Although the brains of neonatal Lhx9 (-/-) mutant mice appear normal, severe hydrocephalus develops in about 70 % of the Lhx9 (-/-) mice at 5-8 weeks of age; these observations are the first to document that deletion of Lhx9 results in hydrocephalus and as such indicate...... that Lhx9 contributes to the maintenance of normal brain structure. Whereas hydrocephalus is absent in neonatal Lhx9 (-/-)mutant mice, the neonatal pineal gland in these animals is hypoplastic. Accordingly, it appears that Lhx9 is essential for early development of the mammalian pineal gland...... and that this effect is not secondary to hydrocephalus....

  11. Hydrocephalus due to extreme dilation of Virchow-Robin spaces

    Science.gov (United States)

    Revel, Frederic; Cotton, Francois; Haine, Max; Gilbert, Thomas

    2015-01-01

    Virchow-Robin spaces (VRS) are extensions of the subarachnoid space surrounding perforating blood vessels entering the brain parenchyma. VRS are fluid filled, but almost virtual and only visible on MRI of the brain when dilated. Such dilations are commonly asymptomatic. In rare cases, extreme dilations can be observed; the clinical repercussions of which remain unclear. We report the case of a patient presenting symptoms of normal pressure hydrocephalus due to extreme VRS mesencephalon dilations. PMID:25564639

  12. Visual outcome after endoscopic third ventriculostomy for hydrocephalus.

    Science.gov (United States)

    Jung, Ji-Ho; Chai, Yong-Hyun; Jung, Shin; Kim, In-Young; Jang, Woo-Youl; Moon, Kyung-Sub; Kim, Seul-Kee; Chong, Sangjoon; Kim, Seung-Ki; Jung, Tae-Young

    2018-02-01

    Hydrocephalus-related symptoms are mostly improved after successful endoscopic third ventriculostomy (ETV). However, visual symptoms can be different. This study was focused on visual symptoms. We analyzed the magnetic resonance images (MRI) of the orbit and visual outcomes. From August 2006 to November 2016, 50 patients with hydrocephalus underwent ETV. The male-to-female ratio was 33:17, and the median age was 61 years (range, 5-74 years). There were 18 pediatric and 32 adult patients. Abnormal orbital MRI findings included prominent subarachnoid space around the optic nerves and vertical tortuosity of the optic nerves. We retrospectively analyzed clinical symptoms, causes of hydrocephalus, ETV success score (ETVSS), ETV success rate, ETV complications, orbital MRI findings, and visual impairment score (VIS). The median duration of follow-up was 59 months (range, 3-113 months). The most common symptoms were headache, vomiting, and gait disturbance. Visual symptoms were found in 6 patients (12%). The most common causes of hydrocephalus were posterior fossa tumor in 13 patients, pineal tumor in 12, aqueductal stenosis in 8, thalamic malignant glioma in 7, and tectal glioma in 4. ETVSS was 70 in 3 patients, 80 in 34 patients, and 90 in 13 patients. ETV success rate was 80%. ETVSS 70 showed the trend in short-term survival compared to ETVSS 90 and 80. ETV complications included epidural hematoma requiring operation in one patient, transient hemiparesis in two patients, and infection in two patients. Preoperative abnormal orbital MRI findings were found in 18 patients and postoperative findings in 7 patients. Four of six patients with visual symptoms had abnormal MR findings. Three patients did not show VIS improvement, including two with severe visual symptoms. Patients with severe visual impairment were found to have bad outcomes. The visual symptoms related with increased intracranial pressure should be carefully monitored and controlled to improve outcomes.

  13. Tuberculous meningitis in an immunocompetent male complicated by hydrocephalus.

    Science.gov (United States)

    Dunphy, Louise; Shetty, Prashanth; Randhawa, Rabinder; Rani, Kharil Amir; Duodu, Yaw

    2016-10-07

    A 39-year-old man, born in India but resident in the UK for 10 years, was travelling in America when he became feverish with an altered mentation. He reported a 10-day history of fever, photophobia, headache and fatigue. His medical history included hypothyroidism and migraine. He was a non-smoker, did not consume alcohol and denied a history of drug use. He was transferred to the emergency department. Laboratory investigations confirmed hyponatraemia (sodium 128 mmol/L). A chest radiograph confirmed no focal consolidation. Further investigation with a CT brain was unremarkable. A lumbar puncture was suggestive of viral meningitis, with a raised white cell count, lymphocytosis, high protein and low glucose. His PCR was negative for enterovirus and herpes simplex virus. Further investigation with a CT thorax, abdomen and pelvis demonstrated bilateral upper-lobe infiltrations. A bronchoalveolar lavage was negative for acid alcohol fast bacilli (AAFB). A diagnosis of tuberculous meningitis was rendered following a repeat lumbar puncture. Gram stain revealed AAFB and PCR was also positive. He started antitubercular treatment and corticosteroids. A repeat CT brain demonstrated ventriculomegaly, suggestive of hydrocephalus and an MRI head revealed likely communicating hydrocephalus with basilar enhancement. He was repatriated to the UK. Eleven days post transfer, he became acutely confused and required external ventricular drain insertion. After surgical management of his hydrocephalus, there was no further neurological deterioration. He remains committed to his neurorehabilitation. 2016 BMJ Publishing Group Ltd.

  14. Acquired Encephalocele With Hydrocephalus and Pineal Region Epidermoid Cyst.

    Science.gov (United States)

    Toktaş, Zafer Orkun; Yilmaz, Baran; Ekşi, Murat Şakir; Bayoumi, Ahmed B; Akakin, Akin; Yener, Yasin; Demir, Mustafa Kemal; Kiliç, Türker

    2016-07-01

    A combination of trauma and a missed inflammatory response (nasal operation) concomitant with hydrocephalus and tumor in secondary encephalocele has not been described in the English literature yet. A 38-year-old man was admitted to the clinic with rhinorrhea that started 3 months ago. In his medical history, nothing abnormal was present except a nasal operation performed 1 year ago. Brain magnetic resonance imaging depicted left frontal encephalocele concomitant with obstructive hydrocephalus caused by an epidermoid cyst originated from the pineal region. A 2-staged surgery was planned. In the first stage, a ventriculoperitoneal shunt insertion was conveyed successfully. In the second-stage surgery, the herniated brain tissue was excised, and the frontal sinus was cleansed with serum saline combined with antibiotic. The bony defect and the dura defect were repaired. The patient's presenting complaint recovered fully, and he was discharged to home in a well condition. Acquired encephalocele is a rare entity. In case of rhinorrhea and encephalocele, even in the presence of prior history of nasal surgery, intracranial evaluation should be conveyed to exclude the presence of hydrocephalus and/or tumor. The cranial defect should be repaired to prevent future infections and brain tissue damage.

  15. Lumbar cisternography in evaluation of hydrocephalus in the preterm infant

    Energy Technology Data Exchange (ETDEWEB)

    Donn, S.M.; Roloff, D.W.; Keyes, J.W. Jr.

    1983-10-01

    Radionuclide lumbar cisternography using indium 111-diethylenetriamine pentaacetic acid (111In-DTPA) and a mobile gamma-camera with a converging collimator was utilized as a bedside procedure to evaluate CSF dynamics and the patency of the cerebral ventricular system in 30 preterm infants with hydrocephalus. Serial images of the brain were obtained at 0, 1, 2, 6, 24, and 48 hours after instillation of the isotope in the lumbar subarachnoid space. Three distinct patterns were seen. Infants with posthemorrhagic hydrocephalus displayed prompt ventricular filling but markedly delayed emptying with minimal flow over the cerebral convexities. Infants with ventriculomegaly secondary to suspected brain atrophy or periventricular leukomalacia demonstrated a pattern of prompt ventricular filling, delayed emptying, but with flow present over the convexities. An infant with noncommunicating hydrocephalus secondary to an Arnold-Chiari malformation showed a pattern of complete obstruction with no ventricular filling. Radionuclide lumbar cisternography appears to be a safe, well-tolerated procedure which produces images of sufficient resolution to provide valuable information about CSF dynamics, delineating basal cisternae, ventricles, and subarachnoid flow paths.

  16. Shunting outcomes in posthemorrhagic hydrocephalus: results of a Hydrocephalus Clinical Research Network prospective cohort study.

    Science.gov (United States)

    Wellons, John C; Shannon, Chevis N; Holubkov, Richard; Riva-Cambrin, Jay; Kulkarni, Abhaya V; Limbrick, David D; Whitehead, William; Browd, Samuel; Rozzelle, Curtis; Simon, Tamara D; Tamber, Mandeep S; Oakes, W Jerry; Drake, James; Luerssen, Thomas G; Kestle, John

    2017-07-01

    OBJECTIVE Previous Hydrocephalus Clinical Research Network (HCRN) retrospective studies have shown a 15% difference in rates of conversion to permanent shunts with the use of ventriculosubgaleal shunts (VSGSs) versus ventricular reservoirs (VRs) as temporization procedures in the treatment of hydrocephalus due to high-grade intraventricular hemorrhage (IVH) of prematurity. Further research in the same study line revealed a strong influence of center-specific decision-making on shunt outcomes. The primary goal of this prospective study was to standardize decision-making across centers to determine true procedural superiority, if any, of VSGS versus VR as a temporization procedure in high-grade IVH of prematurity. METHODS The HCRN conducted a prospective cohort study across 6 centers with an approximate 1.5- to 3-year accrual period (depending on center) followed by 6 months of follow-up. Infants with premature birth, who weighed less than 1500 g, had Grade 3 or 4 IVH of prematurity, and had more than 72 hours of life expectancy were included in the study. Based on a priori consensus, decisions were standardized regarding the timing of initial surgical treatment, upfront shunt versus temporization procedure (VR or VSGS), and when to convert a VR or VSGS to a permanent shunt. Physical examination assessment and surgical technique were also standardized. The primary outcome was the proportion of infants who underwent conversion to a permanent shunt. The major secondary outcomes of interest included infection and other complication rates. RESULTS One hundred forty-five premature infants were enrolled and met criteria for analysis. Using the standardized decision rubrics, 28 infants never reached the threshold for treatment, 11 initially received permanent shunts, 4 were initially treated with endoscopic third ventriculostomy (ETV), and 102 underwent a temporization procedure (36 with VSGSs and 66 with VRs). The 2 temporization cohorts were similar in terms of sex, race

  17. Communicating Hydrocephalus Associated with Small- to Medium-Sized Vestibular Schwannomas: Clinical Significance of the Tumor Apparent Diffusion Coefficient Map.

    Science.gov (United States)

    Taniguchi, Masaaki; Nakai, Tomoaki; Kohta, Masaaki; Kimura, Hidehito; Kohmura, Eiji

    2016-10-01

    The etiology of hydrocephalus associated with the small- to medium-sized vestibular schwannomas is still controversial. We investigated tumor-specific factors related to the association of hydrocephalus with small- to medium-sized vestibular schwannomas. Among the 77 patients with vestibular schwannoma smaller than 30 mm, 9 patients demonstrated associated communicating hydrocephalus. Patient medical records, radiologic data, and histopathologic specimens were reviewed retrospectively. The age of the patients, and size, mean apparent diffusion coefficient (ADC) value, and histologic features of the tumors were compared with those of patients without hydrocephalus. The symptoms related to hydrocephalus improved in all patients after tumor removal. Both the mean size and ADC values exhibited a statistically significant difference between the tumors with and without hydrocephalus (P hydrocephalus. The increased tumor ADC value was considered to be the result of degenerative change and suggested the involvement of protein sloughing in the etiology of the associated hydrocephalus. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Comparison between the incidence of right and left sided congenital torticollis

    International Nuclear Information System (INIS)

    Aslam, S.; Bashir, M.S.; Hussain, S.I.

    2013-01-01

    Congenital torticollis is an intriguing condition of unknown origin, characterized by unilateral shortening and tightness of the sternocleidomastoid muscle. Patients usually present with head tilt, facial asymmetry and plagiocephaly. A sternomastoid mass or tumor may or may not be clinically apparent. Untreated, cervical function and facial cosmesis may be severely compromised. Objective: My study is aimed at establishing a comparison between the incidence of right versus left sided congenital torticollis. Method: This observational study included 30 patients of congenital torticollis that completed the questionnaire. The data was collected from patients coming to the Physiotherapy and Orthopedic departments of Children Hospital, Lahore. Results: Results showed that right side was involved in 19 (63.3%) patients and left side was involved in 11 (36.7%) patients. Out of 30 patients, 14 (46.7%) were male, of which 8 had right sided congenital torticollis and 6 had left sided congenital torticollis, and 16 (53.3%) were female, of which right sided congenital torticollis and 5 had left sided congenital torticollis. Conclusion: Hence it is concluded that incidence of right sided congenital torticollis is more common than left sided congenital torticollis. The incidence of con-genital torticollis is higher in females than in males. (author)

  19. Disturbance of cerebral neuronal migration following congenital parvovirus B19 infection

    NARCIS (Netherlands)

    Pistorius, Lourens Rasmus; Smal, Jaime; de Haan, Timo Robert; Page-Christiaens, Godelieve C. M. L.; Verboon-Maciolek, Malgorzata; Oepkes, Dick; de Vries, Linda S.

    2008-01-01

    We describe the clinical course of an infant who presented with severe fetal anemia and fetal hydrops following congenital parvovirus B19 infection before 16 gestational weeks. The fetus was treated by cordocentesis and intrauterine transfusion at 18 weeks. The infant demonstrated mild unilateral

  20. CONGENITAL ANOMALIES OF THE KIDNEYS AND URINARY TRACT IN CHILDREN

    Directory of Open Access Journals (Sweden)

    Matjaž Kopač

    2015-03-01

    Full Text Available Congenital anomalies of the kidney and urinary tract are the commonest congenital anomalies in children, often detected prenatally with ultrasound. This method is useful for assesing the degree of dilatation of the collecting system, structure of the kidney parenchyma, amount of amniotic fluid and urinary bladder. Hydronephrosis is the most common among them. Anomalies can be bilateral or unilateral and different defects may coexist in an individual child. Anomalies of other organs and organ systems are often associated with anomalies of the kidneys and urinary tract, described in numerous syndromes. Congenital anomalies of the kidney and urinary tract can be divided in anomalies of the renal parencyma development, renal embryonic migration and position, cystic kidney diseases and anomalies of the urinary tract (collecting system of the kidneys, ureters, urinary bladder and urethra. They are the commonest cause of end-stage renal disease in children.

  1. Community Knowledge, Beliefs, Attitudes, and Practices towards Children with Spina Bifida and Hydrocephalus in Uganda

    Science.gov (United States)

    Bannink, Femke; Stroeken, Koenraad; Idro, Richard; van Hove, Geert

    2015-01-01

    This article describes the findings of a qualitative study on knowledge, beliefs, attitudes, and practices towards children with spina bifida and hydrocephalus in four regions of Uganda. Focus group discussions and semi-structured interviews were held with parents of children with spina bifida and hydrocephalus, policy-makers, and service…

  2. X-linked hydrocephalus : A novel missense mutation in the L1CAM gene

    NARCIS (Netherlands)

    Sztriha, L; Vos, YJ; Verlind, E; Johansen, J; Berg, B

    2002-01-01

    X-linked hydrocephalus is associated with mutations in the L1 neuronal cell adhesion molecule gene. L1 protein plays a key role in neurite outgrowth, axonal guidance, and pathfinding during the development of the nervous system. A male is described with X-linked hydrocephalus who had multiple small

  3. Impact of timing of cranioplasty on hydrocephalus after decompressive hemicraniectomy in malignant middle cerebral artery infarction.

    Science.gov (United States)

    Finger, Tobias; Prinz, Vincent; Schreck, Evelyn; Pinczolits, Alexandra; Bayerl, Simon; Liman, Thomas; Woitzik, Johannes; Vajkoczy, Peter

    2017-02-01

    Patients with malignant middle cerebral artery infarction frequently develop hydrocephalus after decompressive hemicraniectomy. Hydrocephalus itself and known shunt related complications after ventriculo-peritoneal shunt implantation may negatively impact patientś outcome. Here, we aimed to identify factors associated with the development of hydrocephalus after decompressive hemicraniectomy in malignant middle cerebral artery infarction. A total of 99 consecutive patients with the diagnosis of large hemispheric infarctions and the indication for decompressive hemicraniectomy were included. We retrospectively evaluated patient characteristics (gender, age and selected preoperative risk factors), stroke characteristics (side, stroke volume and existing mass effect) and surgical characteristics (size of the bone flap, initial complication rate, time to cranioplasty, complication rate following cranioplasty, type of implant, number of revision surgeries and mortality). Frequency of hydrocephalus development was 10% in our cohort. Patients who developed a hydrocephalus had an earlier time point of bone flap reimplantation compared to the control group (no hydrocephalus=164±104days, hydrocephalus=108±52days, phydrocephalus with a trend towards significance (p=0.08). Communicating hydrocephalus is frequent in patients with malignant middle cerebral artery infarction after decompressive hemicraniectomy. A later time point of cranioplasty might lead to a lower incidence of required shunting procedures in general as we could show in our patient cohort. Copyright © 2016 Elsevier B.V. All rights reserved.

  4. Hydrocephalus following severe traumatic brain injury in adults. Incidence, timing, and clinical predictors during rehabilitation

    DEFF Research Database (Denmark)

    Kammersgaard, Lars Peter; Linnemann, Mia; Tibæk, Maiken

    2013-01-01

    To investigate timing and clinical predictors that might predict hydrocephalus emerging during rehabilitation until 1 year following severe traumatic brain injury (TBI).......To investigate timing and clinical predictors that might predict hydrocephalus emerging during rehabilitation until 1 year following severe traumatic brain injury (TBI)....

  5. Morphological and Behavioral Changes in the Pathogenesis of a Novel Mouse Model of Communicating Hydrocephalus

    Science.gov (United States)

    McMullen, Allison B.; Baidwan, Gurlal S.; McCarthy, Ken D.

    2012-01-01

    The Ro1 model of hydrocephalus represents an excellent model for studying the pathogenesis of hydrocephalus due to its complete penetrance and inducibility, enabling the investigation of the earliest cellular and histological changes in hydrocephalus prior to overt pathology. Hematoxylin and eosin staining, immunofluorescence and electron microscopy were used to characterize the histopathological events of hydrocephalus in this model. Additionally, a broad battery of behavioral tests was used to investigate behavioral changes in the Ro1 model of hydrocephalus. The earliest histological changes observed in this model were ventriculomegaly and disorganization of the ependymal lining of the aqueduct of Sylvius, which occurred concomitantly. Ventriculomegaly led to thinning of the ependyma, which was associated with periventricular edema and areas of the ventricular wall void of cilia and microvilli. Ependymal denudation was subsequent to severe ventriculomegaly, suggesting that it is an effect, rather than a cause, of hydrocephalus in the Ro1 model. Additionally, there was no closure of the aqueduct of Sylvius or any blockages within the ventricular system, even with severe ventriculomegaly, suggesting that the Ro1 model represents a model of communicating hydrocephalus. Interestingly, even with severe ventriculomegaly, there were no behavioral changes, suggesting that the brain is able to compensate for the structural changes that occur in the pathogenesis of hydrocephalus if the disorder progresses at a sufficiently slow rate. PMID:22291910

  6. Mental Condition and Ventricular Size in Arrested Hydrocephalus: an Analysis of 29 Shunt‐independent Children

    NARCIS (Netherlands)

    HOLTZER, G.J.; de LANGE, S.A.; ORBAAN, I.J.C.; GELSEMA, R.

    1971-01-01

    textabstractMeasurement of the diameter of the ventricular system, in a series of 29 patients with arrested hydrocephalus who had become shunt‐independent, showed that enlargement of the ventricles does not necessarily play a part in the arrest of hydrocephalus, for in many of these cases the

  7. Acute hydrocephalus in a child with a third ventricle arachnoid cyst and coincidental enteroviral meningitis

    NARCIS (Netherlands)

    Jeltema, Hanne-Rinck; Kuijlen, Jos M. A.; Hoving, Eelco W.

    We present a 2.5-year-old child suffering from acute hydrocephalus. First, the child was diagnosed with aseptic viral meningitis. The PCR of the cerebrospinal fluid (CSF) was positive for enterovirus. Subsequently, MRI revealed that the hydrocephalus was caused by a cyst in the third ventricle.

  8. Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele.

    Science.gov (United States)

    Da Silva, Stephanie L; Jeelani, Yasser; Dang, Ha; Krieger, Mark D; McComb, J Gordon

    2015-04-01

    There is a known association of hydrocephalus with encephaloceles. Risk factors for hydrocephalus and neurological deficit were ascertained in a series of patients born with an encephalocele. A retrospective analysis was undertaken of patients treated for encephaloceles at Children's Hospital Los Angeles between 1994 and 2012. The following factors were evaluated for their prognostic value: age at presentation, sex, location of encephalocele, size, contents, microcephaly, presence of hydrocephalus, CSF leak, associated cranial anomalies, and neurological outcome. Seventy children were identified, including 38 girls and 32 boys. The median age at presentation was 2 months. The mean follow-up duration was 3.7 years. Encephalocele location was classified as anterior (n = 14) or posterior (n = 56) to the coronal suture. The average maximum encephalocele diameter was 4 cm (range 0.5-23 cm). Forty-seven encephaloceles contained neural tissue. Eight infants presented at birth with CSF leaking from the encephalocele, with 1 being infected. Six patients presented with hydrocephalus, while 11 developed progressive hydrocephalus postoperatively. On univariate analysis, the presence of neural tissue, cranial anomalies, encephalocele size of at least 2 cm, seizure disorder, and microcephaly were each positively associated with hydrocephalus. On multivariate logistic regression modeling, the single prognostic factor for hydrocephalus of borderline statistical significance was the presence of neural tissue (odds ratio [OR] = 5.8, 95% confidence interval [CI] = 0.8-74.0). Fourteen patients had severe developmental delay, 28 had mild/moderate delay, and 28 were neurologically normal. On univariate analysis, the presence of cranial anomalies, larger size of encephalocele, hydrocephalus, and microcephaly were positively associated with neurological deficit. In the multivariable model, the only statistically significant prognostic factor for neurological deficit was presence of

  9. Hydrocephalus and mucopolysaccharidoses: what do we know and what do we not know?

    Science.gov (United States)

    Dalla Corte, Amauri; de Souza, Carolina F M; Anés, Maurício; Giugliani, Roberto

    2017-07-01

    The precise incidence of hydrocephalus in patients with mucopolysaccharidoses (MPS) is hard to determine, because the condition lacks a formal, consensus-based definition. The diagnosis of hydrocephalus depends on symptom profile, presence of neuroimaging features, and the outcome of diagnostic tests. Although numerous techniques are used to identify MPS patients who are most likely to have hydrocephalus and respond to treatment, no definitive method exists to prove diagnosis. The authors propose an algorithm to aid in the diagnosis and management of hydrocephalus in MPS patients. The theory of venous hypertension associated with the morphological changes in the skull base and craniocervical junction indicate the need for future neuroimaging studies including cerebrospinal fluid (CSF) and venous flow measurements to monitor hydrocephalus progression and select therapeutic interventions in MPS patients. Preoperative planning should also be based on the increased risk of intraoperative and postoperative hemorrhagic complications.

  10. The influence of decompressive craniectomy on the development of hydrocephalus: a review

    Directory of Open Access Journals (Sweden)

    Jun Ding

    2014-09-01

    Full Text Available Decompressive craniectomy (DC is widely used to treat intracranial hypertension following traumatic brain injury (TBI or cerebral vascular disease. Many studies have discussed complications of this procedure, and hydrocephalus is a common complication of DC. To further evaluate the relationship between DC and hydrocephalus, a review of the literature was performed. Numerous complications may arise after DC, including contusion or hematoma expansion, epilepsy, herniation of the cortex through a bone defect, CSF leakage through the scalp incision, infection, subdural effusion, hydrocephalus and “syndrome of the trephined”. Several hydrocephalus predictors were identified; these included DC, distance from the midline, hygroma, age, injury severity, subarachnoid or intraventricular hemorrhage, delayed time to craniotomy, repeated operation, and duraplasity. However, results differed among studies. The impact of DC on hydrocephalus remains controversial.

  11. Unilateral renal agenesia in the angiographic material and renovascular hypertension

    International Nuclear Information System (INIS)

    Stojanov, D.; Lovasic, I.; Dujmovic, M.; Bobinac, D.

    1987-01-01

    Analysis of 1216 abdominal aortographies and selective renovasographies undertaken at the Institute of Radiology, Clinical Hospital Centre Rijeka during the period 1979-1985, was performed. 39 (3,2%) unilateral renal agenesias were established, a percentage that is significantly higher than reported by other authors. Analysis of all angiographic parameters of a single kidney was also worked out. A significanlty high percentage (66,7%) of hypertension was diagnosed in this group. Congenital renal failures make their appearance in the early embryological development and are discovered, if compatible with life, most frequently at an advanced age. Statistical data on the frequency during a lifetime are less reliable because a great number of anomalies are never discovered during a person's life. The etiology of variations and congenital failure of renal arteries is unknown in 90% of the cases, and most of the malformations are without characteristic symptomatology. Since the anatomic relations in anomalous kidney are disturbed, the sign of the disease can develop atypical forms causing frequent errors in diagnosis. The kidney is supplied by numerous lateral branches of the medial sacral artery, i.e. the aorta, during embryological development. Later, some of them degenerate or mutually connect themselves, and the definitive kidney has been usually penetrated by only one artery and one vein on the same side (1). If this is not so, i.e. when the obliterations of caudal metanephogenic arteries are only partially present, then the renal arteries emerge from the iliac or lumbar arteries (2,3). Bilateral renal agenesia is extreme and incompatible with life, and hence of small clinical importance. Unilateral agenesia makes its appearance according to various statistics and authors from the obductional materials in ratio 1:1000 (4,5), clinical ones 1:5000 (6, 7, 8) and the angiographically 1:76(9), more exactly 1:35 cases (10). (orig.)

  12. Incidence of environmental and genetic factors causing congenital cataract in Children of Lahore.

    Science.gov (United States)

    Naz, Shagufta; Sharif, Saima; Badar, Hafsa; Rashid, Farzana; Kaleem, Afshan; Iqtedar, Mehwish

    2016-07-01

    To check the incidence of environmental and genetic factors causing congenital cataract in infants. The descriptive study was conducted at Layton Rahmatullah Benevolent Trust, Lahore, Pakistan, from October 2013 to April 2014, and comprised children under 15 years of age who had rubella syndrome, herpes simplex, birth trauma, trisomy 21, Nance-Horan syndrome or Lowe's syndrome. Of the 38,000 cases examined, 120(0.3%) patients were diagnosed with congenital cataract. Of them, 52(43.33%)were aged between 2 and 5 years,22(18.33%) <11 years and 10(8.33%) ?15 years. Bilateral congenital cataract was observed in 91(75.83%) patients and unilateral congenital cataract in 29(24.17%). Environmental factors caused 72(62.07%) cases and genetic factors caused 44(37.93%).. Congenital cataract predominated in boys compared to girls. Early diagnosis and adequate therapy requires specific technology, as well as long-term and permanent care..

  13. Intelligence Quotient (IQ) in Congenital Strabismus*

    Science.gov (United States)

    Bagheri, Abbas; Fallahi, Mohammad Reza; Tamannaifard, Shima; Vajebmonfared, Sara; Zonozian, Saideh

    2013-01-01

    Purpose To evaluate intelligence quotient (IQ) in patients with congenital strabismus. Methods All patients with congenital strabismus scheduled for surgery were enrolled consecutively over a one year period in a cross-sectional study and were evaluated for verbal, performance and total IQ scores, and compared to the mean normal IQ of 100±15. Results During the study period, 109 patients with mean age of 18.4±10.5 (range, 4-63) years were included. Educational status in most patients (80%) was less than high-school. Most patients (80%) lived in urban areas and 46 patients (42.2%) had some degrees of unilateral or bilateral amblyopia. Mean verbal IQ was 87.2±19.6 (range, 45-127), performance IQ was 81±15.5 (range, 44-111) and total IQ was 83.5±18.3 (range, 40-120). Total IQ was significantly lower in comparison to the normal population (P0.05 for all comparisons). Conclusion Patients with congenital strabismus in this study had lower mean IQ scores than the normal population which may be due to genetic background or acquired causes secondary to strabismus. PMID:23943689

  14. Intelligence Quotient (IQ) in Congenital Strabismus.

    Science.gov (United States)

    Bagheri, Abbas; Fallahi, Mohammad Reza; Tamannaifard, Shima; Vajebmonfared, Sara; Zonozian, Saideh

    2013-04-01

    To evaluate intelligence quotient (IQ) in patients with congenital strabismus. All patients with congenital strabismus scheduled for surgery were enrolled consecutively over a one year period in a cross-sectional study and were evaluated for verbal, performance and total IQ scores, and compared to the mean normal IQ of 100±15. During the study period, 109 patients with mean age of 18.4±10.5 (range, 4-63) years were included. Educational status in most patients (80%) was less than high-school. Most patients (80%) lived in urban areas and 46 patients (42.2%) had some degrees of unilateral or bilateral amblyopia. Mean verbal IQ was 87.2±19.6 (range, 45-127), performance IQ was 81±15.5 (range, 44-111) and total IQ was 83.5±18.3 (range, 40-120). Total IQ was significantly lower in comparison to the normal population (PIQ levels. Verbal IQ was insignificantly higher in myopes than emmetropes and hyperopes. IQ was better with vertical deviations and was higher in esotropes than exotropes; however, these differences were not statistically significant (P>0.05 for all comparisons). Patients with congenital strabismus in this study had lower mean IQ scores than the normal population which may be due to genetic background or acquired causes secondary to strabismus.

  15. Post-hemispherectomy hydrocephalus: results of a comprehensive, multi-institutional review

    Science.gov (United States)

    Lew, Sean M.; Matthews, Anne E.; Hartman, Adam L.; Haranhalli, Neil

    2012-01-01

    SUMMARY Purpose Hemispherectomy surgery for medically intractable epilepsy is known to cause hydrocephalus in a subset of patients. Existing data regarding the incidence of, and risk factors for developing, post-hemispherectomy hydrocephalus has been limited by the relatively small number of cases performed by any single center. Our goal was to better understand this phenomenon and to identify risk factors that may predispose patients to developing hydrocephalus after hemispherectomy surgery. Methods Fifteen pediatric epilepsy centers participated in this study. A retrospective chart review was performed on all available patients who had hemispherectomy surgery. Data collected included surgical techniques, etiology of seizures, prior brain surgery, symptoms and signs of hydrocephalus, timing of shunt placement and basic demographics. Key findings Data were collected from 736 patients who underwent hemispherectomy surgery between 1986 and 2011. Forty-six patients had pre-existing shunted hydrocephalus and were excluded from analysis, yielding 690 patients for this study. One hundred sixty-two patients (23%) required hydrocephalus treatment. The timing of hydrocephalus ranged from the immediate post-operative period to 8.5 years after surgery, with 43 patients (27%) receiving shunts more than 90 days after surgery. Multivariate regression analysis revealed anatomic hemispherectomies (OR 4.1, phydrocephalus. There was a trend towards significance for the use of hemostatic agents (O.R. 2.2, p=0.07) and the involvement of basal ganglia or thalamus in the resection (O.R. 2.2, p=0.08) as risk factors. Significance Hydrocephalus is a common sequela of hemispherectomy surgery. Surgical technique and prior brain surgery influence the occurrence of post-hemispherectomy hydrocephalus. A significant portion of patients develop hydrocephalus on a delayed basis, indicating the need for long-term surveillance. PMID:23106378

  16. Epidemiology of adult-onset hydrocephalus: institutional experience with 2001 patients.

    Science.gov (United States)

    Bir, Shyamal C; Patra, Devi Prasad; Maiti, Tanmoy K; Sun, Hai; Guthikonda, Bharat; Notarianni, Christina; Nanda, Anil

    2016-09-01

    OBJECTIVE Adult-onset hydrocephalus is not commonly discussed in the literature, especially regarding its demographic distribution. In contrast to pediatric hydrocephalus, which is related to a primary CSF pathway defect, its development in adults is often secondary to other pathologies. In this study, the authors investigated the epidemiology of adult-onset hydrocephalus as it pertains to different etiologies and in reference to age, sex, and race distributions. METHODS The authors retrospectively reviewed the clinical notes of 2001 patients with adult-onset hydrocephalus who presented to Louisiana State University Health Sciences Center within a 25-year span. Significant differences between the groups were analyzed by a chi-square test; p hydrocephalus in this population was 77 ± 30 per year, with a significant increase in incidence in the past decade (55 ± 3 [1990-2003] vs 102 ± 6 [2004-2015]; p Hydrocephalus in a majority of the patients had a vascular etiology (45.5%) or was a result of a tumor (30.2%). The incidence of hydrocephalus in different age groups varied according to various pathologies. The incidence was significantly higher in males with normal-pressure hydrocephalus (p = 0.03) or head injury (p = 0.01) and higher in females with pseudotumor cerebri (p hydrocephalus was significantly higher in Caucasian patients (p = 0.0002) than in those of any other race. CONCLUSIONS Knowledge of the demographic variations in adult-onset hydrocephalus is helpful in achieving better risk stratification and better managing the disease in patients. For general applicability, these results should be validated in a large-scale meta-analysis based on a national population database.

  17. Clinical and Surgical Factors Associated With Increased Epilepsy Risk in Children With Hydrocephalus.

    Science.gov (United States)

    Tully, Hannah M; Kukull, Walter A; Mueller, Beth A

    2016-06-01

    Children with hydrocephalus are at risk for epilepsy both due to their underlying condition and as a consequence of surgical treatment; however, the relative contributions of these factors remain unknown. The authors sought to characterize epilepsy among children with infancy-onset hydrocephalus and to examine the risks of epilepsy associated with hydrocephalus subtype and with factors related to surgical treatment. We conducted a longitudinal cohort study of all children with infancy-onset hydrocephalus treated at a major regional children's hospital during 2002 to 2012, with follow-up to ascertain risk factors and epilepsy outcome through April 2015. Poisson regression was used to calculate adjusted risk ratios and 95% confidence intervals for associations. Among 379 children with hydrocephalus, 86 (23%) developed epilepsy (mean onset age = 2.7 years), almost one fifth of whom had a history of infantile spasms. Relative to spina bifida-associated hydrocephalus, children with other major hydrocephalus subtypes had fourfold higher risks of developing epilepsy. Among children who underwent surgery, surgical infection doubled the risk of epilepsy (risk ratio = 2.0, 95% confidence interval = 1.4 to 3.0). Epilepsy was associated with surgical failure for intracranial reasons but not extracranial reasons (risk ratio = 1.7, 95% confidence interval = 1.1 to 2.7; risk ratio = 1.1, 95% confidence interval = 0.7 to 1.9, respectively). Epilepsy is common among children with hydrocephalus. Compared with children with spina bifida-associated hydrocephalus, children with other major hydrocephalus subtypes have a markedly increased risk of epilepsy. Surgical infection doubles the risk of epilepsy. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Abnormal structural connectivity in the brain networks of children with hydrocephalus

    Directory of Open Access Journals (Sweden)

    Weihong Yuan

    2015-01-01

    Full Text Available Increased intracranial pressure and ventriculomegaly in children with hydrocephalus are known to have adverse effects on white matter structure. This study seeks to investigate the impact of hydrocephalus on topological features of brain networks in children. The goal was to investigate structural network connectivity, at both global and regional levels, in the brains in children with hydrocephalus using graph theory analysis and diffusion tensor tractography. Three groups of children were included in the study (29 normally developing controls, 9 preoperative hydrocephalus patients, and 17 postoperative hydrocephalus patients. Graph theory analysis was applied to calculate the global network measures including small-worldness, normalized clustering coefficients, normalized characteristic path length, global efficiency, and modularity. Abnormalities in regional network parameters, including nodal degree, local efficiency, clustering coefficient, and betweenness centrality, were also compared between the two patients groups (separately and the controls using two tailed t-test at significance level of p < 0.05 (corrected for multiple comparison. Children with hydrocephalus in both the preoperative and postoperative groups were found to have significantly lower small-worldness and lower normalized clustering coefficient than controls. Children with hydrocephalus in the postoperative group were also found to have significantly lower normalized characteristic path length and lower modularity. At regional level, significant group differences (or differences at trend level in regional network measures were found between hydrocephalus patients and the controls in a series of brain regions including the medial occipital gyrus, medial frontal gyrus, thalamus, cingulate gyrus, lingual gyrus, rectal gyrus, caudate, cuneus, and insular. Our data showed that structural connectivity analysis using graph theory and diffusion tensor tractography is sensitive to

  19. Cytomegalovirus Congenital Cataract

    Directory of Open Access Journals (Sweden)

    Ridha Wahyutomo

    2011-06-01

    Full Text Available Cytomegalovirus congenital infection is an infection caused by the the subfamily â Herpesviridae, during pregnancy. The incidence of infections among newborn infants is 1 %. One of the effects of congenitally acquired infection is the congenital cataract. A 6-year-old child complained to have a blurred vision diagnosed with cytomegalovirus congenital cataract. The diagnosis was confirmed by a positive serology testing for Ig M and Ig G CMV. The laboratory test using Giemsa staining to find inclusion bodies and a faster PCR could not be carried out (Sains Medika, 3(1:84-88.

  20. Emotional Perception of Music in Children with Unilateral Cochlear Implants

    Directory of Open Access Journals (Sweden)

    Sareh Shirvani

    2014-10-01

    Full Text Available Introduction: Cochlear implantation (CI improves language skills among children with hearing loss. However, children with CIs still fall short of fulfilling some other needs, including musical perception. This is often attributed to the biological, technological, and acoustic limitations of CIs. Emotions play a key role in the understanding and enjoyment of music. The present study aimed to investigate the emotional perception of music in children with bilaterally severe-to-profound hearing loss and unilateral CIs.   Materials and Methods: Twenty-five children with congenital severe-to-profound hearing loss and unilateral CIs and 30 children with normal hearing participated in the study. The children’s emotional perceptions of music, as defined by Peretz (1998, were measured. Children were instructed to indicate happy or sad feelings fostered in them by the music by pointing to pictures of faces showing these emotions.   Results: Children with CI obtained significantly lower scores than children with normal hearing, for both happy and sad items of music as well as in overall test scores (P

  1. Congenital Zika Virus Infection: Beyond Neonatal Microcephaly.

    Science.gov (United States)

    Melo, Adriana Suely de Oliveira; Aguiar, Renato Santana; Amorim, Melania Maria Ramos; Arruda, Monica B; Melo, Fabiana de Oliveira; Ribeiro, Suelem Taís Clementino; Batista, Alba Gean Medeiros; Ferreira, Thales; Dos Santos, Mayra Pereira; Sampaio, Virgínia Vilar; Moura, Sarah Rogéria Martins; Rabello, Luciana Portela; Gonzaga, Clarissa Emanuelle; Malinger, Gustavo; Ximenes, Renato; de Oliveira-Szejnfeld, Patricia Soares; Tovar-Moll, Fernanda; Chimelli, Leila; Silveira, Paola Paz; Delvechio, Rodrigo; Higa, Luiza; Campanati, Loraine; Nogueira, Rita M R; Filippis, Ana Maria Bispo; Szejnfeld, Jacob; Voloch, Carolina Moreira; Ferreira, Orlando C; Brindeiro, Rodrigo M; Tanuri, Amilcar

    2016-12-01

    Recent studies have reported an increase in the number of fetuses and neonates with microcephaly whose mothers were infected with the Zika virus (ZIKV) during pregnancy. To our knowledge, most reports to date have focused on select aspects of the maternal or fetal infection and fetal effects. To describe the prenatal evolution and perinatal outcomes of 11 neonates who had developmental abnormalities and neurological damage associated with ZIKV infection in Brazil. We observed 11 infants with congenital ZIKV infection from gestation to 6 months in the state of Paraíba, Brazil. Ten of 11 women included in this study presented with symptoms of ZIKV infection during the first half of pregnancy, and all 11 had laboratory evidence of the infection in several tissues by serology or polymerase chain reaction. Brain damage was confirmed through intrauterine ultrasonography and was complemented by magnetic resonance imaging. Histopathological analysis was performed on the placenta and brain tissue from infants who died. The ZIKV genome was investigated in several tissues and sequenced for further phylogenetic analysis. Description of the major lesions caused by ZIKV congenital infection. Of the 11 infants, 7 (63.6%) were female, and the median (SD) maternal age at delivery was 25 (6) years. Three of 11 neonates died, giving a perinatal mortality rate of 27.3%. The median (SD) cephalic perimeter at birth was 31 (3) cm, a value lower than the limit to consider a microcephaly case. In all patients, neurological impairments were identified, including microcephaly, a reduction in cerebral volume, ventriculomegaly, cerebellar hypoplasia, lissencephaly with hydrocephalus, and fetal akinesia deformation sequence (ie, arthrogryposis). Results of limited testing for other causes of microcephaly, such as genetic disorders and viral and bacterial infections, were negative, and the ZIKV genome was found in both maternal and neonatal tissues (eg, amniotic fluid, cord blood, placenta, and

  2. Genetics Home Reference: congenital hypothyroidism

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Congenital hypothyroidism Congenital hypothyroidism Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Congenital hypothyroidism is a partial or complete loss of function ...

  3. Kniest disease with Pierre Robin syndrome and hydrocephalus

    International Nuclear Information System (INIS)

    Silengo, M.C.; Davi, G.F.; Bianco, R.; DeMarco, A.; Franceschini, P.

    1983-01-01

    A 3-month-old male infant with clinical and radiological features of Kniest disease is reported. Additional findings were severe Pierre Robin syndrome and external hydrocephalus. The patient was retarded in mental and motor development. He died at 4 months of age from the complications of tracheostomy. The parents were both normal clinically and radiologically, thus the disease in the child was presumably due to a new mutation. The reported familial cases of Kniest disease suggest autosomal dominant inheritance. The differential diagnosis is discussed in detail. (orig.)

  4. A Rare Complication of Acute Otitis Media: Otitic Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Ahmet Mutlu

    2013-06-01

    Full Text Available Acute otitis media is very common disorder in childhood. In this article we present a 6-years old boy who applied with diplopia, dysfunction of lateral eye movements on left eye, nausea, at 10th day of acute otitis media treatment. After the radiological images case was described as an otitic hydrocephalus clinic. Patient underwent medical treatment, lomboperitoneal shunt operation and simple mastoidectomy. We wanted to share the evaluation and the management steps of this very rare complication of acute otitis media.

  5. The clinical application and nursing experience of adjustable shunt valve in treatment for patients with normal pressure hydrocephalus

    Directory of Open Access Journals (Sweden)

    YANG Li-rong

    2012-02-01

    Full Text Available Objective To introduce the application of adjustable shunt valve in treatment for patients with normal pressure hydrocephalus. Methods Twenty-four patients with normal pressure hydrocephalus implanted adjustable shunt valve underwent ventriculo-peritoneal shunt surgery and nursing care. Results After operation, cerebrospinal pressure was regulated for 0-6 (1.88 ± 1.52 times. Clinical symptoms were improved, especially in gait disturbance. Conclusion Treatment of normal pressure hydrocephalus with adjustable shunt valve can alleviate symptoms of hydrocephalus. It is especially suitable for patients with short course and secondary normal hydrocephalus patients.

  6. Mitochondrial disorders in congenital myopathies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of congenital myopathies: congenital muscular dystrophies and congenital structural myopathies. It describes changes in congenital muscular dystrophies with type VI collagen, in myodystrophy with giant mitochondria, in congenital central core myopathies, myotubular myopathy, etc. Clinical and experimental findings are presented. Approaches to therapy for energy disorders in congenital myopathies are depicted.

  7. Fertility potential after unilateral orchiopexy

    DEFF Research Database (Denmark)

    Cortes, D; Thorup, J M; Lindenberg, S

    1996-01-01

    biopsy simultaneously with unilateral orchiopexy. In adulthood 56 of the 87 patients agreed to measurement of serum follicle-stimulating hormone, luteinizing hormone, testosterone and testicular volume, and 54 provided specimens for semen analysis. RESULTS: The number of spermatogonia per tubular cross....... CONCLUSIONS: The age matched number of spermatogonia per tubular cross section is significant in regard to fertility potential, and a value of 1% or less (approximately Sertoli cells only) may predict low fertility potential. To our knowledge Sertoli cells only have not been observed before 15 months of life...

  8. Nitrofurantoin and congenital abnormalities

    DEFF Research Database (Denmark)

    Czeizel, A.E.; Rockenbauer, M.; Sørensen, Henrik Toft

    2001-01-01

    Objective: To study human teratogenic potential of oral nitrofurantoin treatment during pregnancy. Materials and Methods: Pair analysis of cases with congenital abnormalities and matched population controls in the population-based dataset of the Hungarian Case-Control Surveillance of Congenital...

  9. Congenital heat disease

    International Nuclear Information System (INIS)

    Higgins, C.B.; Silverman, N.H.; Kersting-Somerhoff, B.A.

    1990-01-01

    The book covers the tomographic anatomy of the normal and congenitally malformed heart and tomographic imaging of the normal heat. It then compares echocardiographic evaluation and the use of MR imaging in the diagnosis and evaluation of individual congenital cardiac malformations

  10. Comparative study of various models of experimental hydrocephalus.

    Science.gov (United States)

    Matsumoto, S; Hirayama, A; Yamasaki, S; Shirataki, K; Fujiwara, K

    1975-01-01

    The authors present morphological findings in the brains of rabbits, dogs, cats, rats, and mice that have been used as experimental hydrocephalic models. The methods used were as follows: (1) silicone oil injection into the cisterna magna and the neighboring basal cisterns in rabbits by the method of Wisniewski; (2) kaolin administration into the cisterna magna in rabbits and dogs (Dixon); (3) ballooning method with Foley's catheter into the 4th ventricle in rabbits (Milho-rat); (4) plug formation with small pieces of laminalia into the cisterna magna in rabbits by our method; (5) Hy-3 hereditary hydrocephalic mouse bred by Gruenberg; (6) ligation of the placental vessels of the pregnant rat at 13 days of gestation by our method, and (7) transplacental intraperitoneal administration of ethylnitrosourea in a pregnant rat at 9.5 days of gestation. The models with silicone oil, kaolin, laminalia, and ballooning methods produced obstructive hydrocephalus with various grades of ventricular dilatation. The models with the ethylnitrosourea-induced method, ligation of placental vessels, and Hy-3 mouse produced prenatal hydrocephalus. Dilatation of the ventricular system and histological abnormalities do not occur as a uniform process.

  11. Characteristics and Reversibility of Dementia in Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Priyanka Chaudhry

    2007-01-01

    Full Text Available Studies of the cognitive outcome after shunt insertion for treatment of Normal Pressure Hydrocephalus have reported widely mixed results. We prospectively studied performance of 60 patients with Normal Pressure Hydrocephalus on a comprehensive battery of neuropsychological tests before and after shunt surgery to determine which cognitive functions improve with shunt insertion. We also administered a subset of cognitive tests before and after temporary controlled drainage of cerebrospinal fluid to determine if change on this brief subset of tests after drainage could predict which patients would show cognitive improvement three to six months after shunt insertion. There was a significant improvement in learning, retention, and delayed recall of verbal memory three to six months after surgery (using paired t-tests. The majority (74% of patients showed significant improvement (by at least one standard deviation on at least one of the memory tests. Absence of improvement on verbal memory after temporary drainage of cerebrospinal fluid had a high negative predictive value for improvement on memory tests at 3–6 months after surgery (96%; p = 0.0005. Also, the magnitude of improvement from Baseline to Post-Drainage on few specific tests of learning and recall significantly predicted the magnitude of improvement after shunt surgery on the same tests (r2 = 0.32–0.58; p = 0.04–0.001. Results indicate that testing before and after temporary drainage may be useful in predicting which patients are less likely to improve in memory with shunting.

  12. A novel microneedle array for the treatment of hydrocephalus.

    Science.gov (United States)

    Oh, Jonghyun; Liu, Kewei; Medina, Tim; Kralick, Francis; Noh, Hongseok Moses

    2014-06-01

    We present a microfabricated 10 by 10 array of microneedles for the treatment of a neurological disease called communicating hydrocephalus. Together with the previously reported microvalve array, the current implantable microneedle array completes the microfabricated arachnoid granulations (MAGs) that mimic the function of normal arachnoid granulations (AGs). The microneedle array was designed to enable the fixation of the MAGs through dura mater membrane in the brain and thus provide a conduit for the flow of cerebrospinal fluid (CSF). Cone-shaped microneedles with hollow channels were fabricated using a series of microfabrication techniques: SU-8 photolithography for tapered geometry, reactive ion etching for sharpening the microneedles, 248 nm deep UV excimer laser machining for creating through-hole inside the microneedles, and metal sputtering for improved rigidity. Puncture tests were conducted using porcine dura mater and the results showed that the fabricated microneedle array is strong enough to pierce the dura mater. The in-vitro biocompatibility test result showed that none of the 100 outlets of the microneedles exposed to the bloodstream were clogged significantly by blood cells. We believe that these test results demonstrate the potential use of the microneedle array as a new treatment of hydrocephalus.

  13. Post-hemorrhagic hydrocephalus: Recent advances and new therapeutic insights.

    Science.gov (United States)

    Chen, Qianwei; Feng, Zhou; Tan, Qiang; Guo, Jing; Tang, Jun; Tan, Liang; Feng, Hua; Chen, Zhi

    2017-04-15

    Post-hemorrhagic hydrocephalus (PHH), also referred to as progressive ventricular dilatation, is caused by disturbances in cerebrospinal fluid (CSF) flow or absorption following hemorrhage in the brain. As one of the most serious complications of neonatal/adult intraventricular hemorrhage (IVH), subarachnoid hemorrhage (SAH), and traumatic brain injury (TBI), PHH is associated with increased morbidity and disability of these events. Common sequelae of PHH include neurocognitive impairment, motor dysfunction, and growth impairment. Non-surgical measures to reduce increased intracranial pressure (ICP) in PHH have shown little success and most patients will ultimately require surgical management, such as external ventricular drainage and shunting which mostly by inserting a CSF drainage shunt. Unfortunately, shunt complications are common and the optimum time for intervention is unclear. To date, there remains no comprehensive strategy for PHH management and it becomes imperative that to explore new therapeutic targets and methods for PHH. Over past decades, increasing evidence have indicated that hemorrhage-derived blood and subsequent metabolic products may play a key role in the development of IVH-, SAH- and TBI-associated PHH. Several intervention strategies have recently been evaluated and cross-referenced. In this review, we summarized and discussed the common aspects of hydrocephalus following IVH, SAH and TBI, relevant experimental animal models, clinical translation of in vivo experiments, and potential preventive and therapeutic targets for PHH. Copyright © 2017 Elsevier B.V. All rights reserved.

  14. Control volume based hydrocephalus research; a phantom study

    Science.gov (United States)

    Cohen, Benjamin; Voorhees, Abram; Madsen, Joseph; Wei, Timothy

    2009-11-01

    Hydrocephalus is a complex spectrum of neurophysiological disorders involving perturbation of the intracranial contents; primarily increased intraventricular cerebrospinal fluid (CSF) volume and intracranial pressure are observed. CSF dynamics are highly coupled to the cerebral blood flows and pressures as well as the mechanical properties of the brain. Hydrocephalus, as such, is a very complex biological problem. We propose integral control volume analysis as a method of tracking these important interactions using mass and momentum conservation principles. As a first step in applying this methodology in humans, an in vitro phantom is used as a simplified model of the intracranial space. The phantom's design consists of a rigid container filled with a compressible gel. Within the gel a hollow spherical cavity represents the ventricular system and a cylindrical passage represents the spinal canal. A computer controlled piston pump supplies sinusoidal volume fluctuations into and out of the flow phantom. MRI is used to measure fluid velocity and volume change as functions of time. Independent pressure measurements and momentum flow rate measurements are used to calibrate the MRI data. These data are used as a framework for future work with live patients and normal individuals. Flow and pressure measurements on the flow phantom will be presented through the control volume framework.

  15. Radiation doses to children with shunt-treated hydrocephalus

    Energy Technology Data Exchange (ETDEWEB)

    Holmedal, Lise J. [Helse Fonna, Department of Radiology, Stord Hospital, Stord (Norway); Friberg, Eva G.; Boerretzen, Ingelin; Olerud, Hilde [The Norwegian Radiation Protection Authority, Oesteraas (Norway); Laegreid, Liv [Haukeland University Hospital, Department of Paediatrics, Bergen (Norway); Rosendahl, Karen [University of Bergen, Department of Surgical Sciences, Radiology Section, Bergen (Norway); Great Ormond Street Hospital for Children, Department of Diagnostic Radiology, London (United Kingdom)

    2007-12-15

    Children with shunt-treated hydrocephalus are still followed routinely with frequent head CT scans. To estimate the effective dose, brain and lens doses from these examinations during childhood, and to assess dose variation per examination. All children born between 1983 and 1995 and treated for hydrocephalus between 1983 and 2002 were included. We retrospectively registered the number of examinations and the applied scan parameters. The effective dose was calculated using mean conversion factors from the CT dose index measured free in air, while doses to the lens and brain were estimated using tabulated CT dose index values measured in a head phantom. A total of 687 CT examinations were performed in 67 children. The mean effective dose, lens dose and brain dose to children over 6 months of age were 1.2 mSv, 52 mGy and 33 mGy, respectively, and the corresponding doses to younger children were 3.2 mSv, 60 mGy and 48 mGy. The effective dose per CT examination varied by a factor of 64. None of the children was exposed to doses known to cause deterministic effects. However, since the threshold for radiation-induced damage is not known with certainty, alternative modalities such as US and MRI should be used whenever possible. (orig.)

  16. Ongoing search for diagnostic biomarkers in idiopathic normal pressure hydrocephalus.

    Science.gov (United States)

    Tarnaris, Andrew; Toma, Ahmed K; Kitchen, Neil D; Watkins, Laurence D

    2009-12-01

    Idiopathic normal pressure hydrocephalus is a syndrome, which typically has a clinical presentation of gait/balance disturbance, often accompanied by cognitive decline and/or urinary incontinence. Its diagnosis is based on relevant history and clinical examination, appropriate imaging findings and physiological testing. The clinical picture of idiopathic normal pressure hydrocephalus may occasionally be difficult to distinguish from that of Alzheimer's dementia, subcortical ischemic vascular dementia and Parkinson's disease. The aim of this article is to systematically review the literature from the last 29 years in order to identify cerebrospinal fluid (CSF) or imaging biomarkers that may aid in the diagnosis of the syndrome. The authors concluded that no CSF or imaging biomarker is currently fulfilling the criteria required to aid in the diagnosis of the condition. However, a few studies have revealed promising CSF and imaging markers that need to be verified by independent groups. The reasons that the progress in this field has been slow so far is also commented on, as well as steps required to apply the current evidence in the design of future studies within the field.

  17. Hydrocephalus 2008, 17–20th September, Hannover Germany: a conference report

    Directory of Open Access Journals (Sweden)

    Klinge Petra M

    2008-12-01

    Full Text Available Abstract Hydrocephalus 2008 was held 17–20 September in Hannover, Germany, at the invitation of Petra M Klinge (President, co-hosted by Joachim K. Krauss (Vice President, and Madjid Samii (Honorary President. This meeting was a successor to Hydrocephalus 2006 held in Göteborg, Sweden, organised by Past-President, Carsten Wikkelso. The conference began with a general introductory session of six talks including three invited lectures, followed by eighteen parallel sessions. Subjects covered were hydrocephalus signs, symptoms and diagnosis, especially in normal pressure hydrocephalus; cerebrospinal fluid (CSF physics and dynamics; CSF function and modelling of function; dementia and quality of life, economy, health care and rehabilitation; neuropsychology, cognition and outcome assessment; neuroimaging, functional imaging and non-invasive diagnostics; paediatric and adolescent hydrocephalus; intelligent shunt and valve design (e.g. telemetry, adjustable and antimicrobial shunts; endoscopic third ventriculostomy; technical advances and image-guided surgical approaches in the treatment of hydrocephalus; brain metabolism, biomarkers and biophysics; co-morbidity, classification and aetiology; epidemiology, registries and clinical trials; experimental hydrocephalus; and pharmaceutical modulation of central nervous system function (CNS drug delivery. Each session began with introductory talks from the invited chairpersons followed by six to eight submitted oral presentations. Overall, 136 oral presentations and 18 posters were presented, the abstracts of which were published elsewhere 1. We present here an account of the introductory session, the invited chairperson's talks and the concluding remarks by Anthony Marmarou.

  18. Radionuclide cisternography in the diagnosis of hydrocephalus type in tuberculous meningitis in children

    International Nuclear Information System (INIS)

    Zafra B, R.M.

    1993-01-01

    The radionuclide cisternography permits an accurate diagnosis in hydrocephalus, this is an easy procedure, not traumatic nor expensive. Hydrocephalus is one of the most serious complications of tuberculous meningitis, that, without an opportune treatment, leads to severe sequel and death of the patient. The medical records of thirty patients with diagnosis of tuberculosis meningitis were reviewed, to whom the Neuro-Pediatric Service of the National Institute of Children Health solicited radionuclide cisternography to evaluate hydrocephalus and to determinate type, between january 1990 and october 1992. The hydrocephalus was demonstrated by radionuclide cisternography in 26/30 patients (86,7%) The most frequent age was in children under five years old 21/26 (80,7%) and pre-students in 65,38%. The most common finding was communicating hydrocephalus, pattern type IV in 20/30 patients (66,7%). It is concluded that the radionuclide cisternography is very useful for the diagnosis of hydrocephalus and its type patients with tuberculous meningitis. This exam is recommended for all patients with diagnosis of tuberculous meningitis, with no satisfactory evolution, in order to obtain an early diagnosis of hydrocephalus and its type, and get an opportune medical or surgical treatment. (author). 35 refs., 3 tab., 8 ills

  19. Large vestibular schwannomas and hydrocephalus: Lessons learnt from a single centre experience

    Directory of Open Access Journals (Sweden)

    Prakash Nair

    2014-01-01

    Full Text Available Aim: The aim of the following study is to analyze the outcome following surgery in 169 patients with large vestibular schwannoma (VS and to evaluate hydrocephalus as a prognostic factor in patients of the VSs. Subjects and Methods: Retrospective analysis of all cases of VSs admitted to our tertiary neurosurgical center from January 2005 to December 2010 was performed. Comparison of patients who underwent pre-operative cerebrospinal fluid (CSF diversion and those who underwent primary surgery was carried out for post-operative complications and delayed hydrocephalus. Results: A total of 169 patients of VS were seen. The mean age at presentation was 39.03 years (12-72 years. The most common symptom was hearing loss seen in 161 (95.2% cases. Giant VS was seen in 130 (75.5% and hydrocephalus was present in 110 (63.9%. Pre-operative CSF diversion was done in 23 (13.1% patients; 8 (4.6% patients developed gradually symptomatic hydrocephalus following surgery and underwent ventriculoperitoneal shunt. Total surgical excision was done in 92.9% patients and subtotal excision was done in 7% patients. Conclusions: Hydrocephalus occurs in longstanding untreated cases of VS. Hydrocephalus causes no statistically significant increase in post-operative complications like CSF leak and post-operative hematoma. Patients with hydrocephalus presenting with acute symptoms of raised intracranial pressure benefit from CSF diversion. In most patients, tumor resection will restore patency of the CSF pathway and CSF diversion can be avoided.

  20. Communicating Hydrocephalus Associated with Intracranial Schwannoma Treated by Gamma Knife Radiosurgery.

    Science.gov (United States)

    Park, Chang Kyu; Lee, Sung Ho; Choi, Man Kyu; Choi, Seok Keun; Park, Bong Jin; Lim, Young Jin

    2016-05-01

    Gamma knife radiosurgery (GKRS) has been established as an effective and safe treatment for intracranial schwannoma. However, serious complications can occur after GKRS, including hydrocephalus. The pathophysiology and risk factors of this disorder are not yet fully understood. The objective of the study was to assess potential risk factors for hydrocephalus after GKRS. We retrospectively reviewed the medical radiosurgical records of 244 patients who underwent GKRS to treat intracranial schwannoma. The following parameters were analyzed as potential risk factors for hydrocephalus after GKRS: age, sex, target volume, irradiation dose, prior tumor resection, treatment technique, and tumor enhancement pattern. The tumor enhancement pattern was divided into 2 groups: group A (homogeneous enhancement) and group B (heterogeneous or rim enhancement). Of the 244 patients, 14 of them (5.7%) developed communicating hydrocephalus. Communicating hydrocephalus occurred within 2 years after GKRS in most patients (92.8%). No significant association was observed between any of the parameters investigated and the development of hydrocephalus, with the exception of tumor enhancement pattern. Group B exhibited a statistically significant difference by univariate analysis (P = 0.002); this difference was also significant by multivariate analysis (P = 0.006). Because hydrocephalus is curable, patients should be closely monitored for the development of this disorder after GKRS. In particular, patients with intracranial schwannomas with irregular enhancement patterns or cysts should be meticulously observed. Copyright © 2015 Elsevier Inc. All rights reserved.

  1. The Effect of Gender on Acute Hydrocephalus after Experimental Subarachnoid Hemorrhage.

    Science.gov (United States)

    Shishido, Hajime; Zhang, Haining; Okubo, Shuichi; Hua, Ya; Keep, Richard F; Xi, Guohua

    2016-01-01

    Acute hydrocephalus is a common complication of subarachnoid hemorrhage (SAH). We investigated the effect of gender on acute hydrocephalus development in a rat SAH model. SAH was induced in adult male and female Sprague-Dawley rats using endovascular perforation. Sham rats underwent the same procedure without perforation. Magnetic resonance imaging (MRI) was performed 24 h after SAH to determine ventricular volume. Hydrocephalus was defined as a ventricular volume that was more than 3 standard deviations from the mean value in sham-operated animals. After MRI, animals were euthanized and the extent of SAH was assessed using a modified grading system. No sham animals died. Mortality rates after SAH induction in male and female animals were 27 and 22 %, respectively. SAH induced significant ventricular enlargement compared with sham-operated rats (p hydrocephalus 24 h after SAH was greater in female (75 %) than in male animals (47 %, p hydrocephalus occurred even though SAH severity grade and ventricular T2* hypointensity volumes were not significantly different between male and female animals. Our data demonstrate that gender influences acute hydrocephalus development in a rat SAH model. Future studies should determine the role of estrogen in SAH-induced hydrocephalus.

  2. Prolonged hydrocephalus induced by intraventricular hemorrhage in rats is reduced by curcumin therapy.

    Science.gov (United States)

    Qi, Zhihua; Zhang, Huiqin; Fu, Chuhua; Liu, Xiao; Chen, Bo; Dang, Yanwei; Chen, Huayun; Liu, Lijun

    2017-01-10

    Prolonged hydrocephalus is a major cause of severe disability and death of intraventricular hemorrhage (IVH) patients. However, the therapeutic options to minimize the detrimental effects of post-hemorrhagic hydrocephalus are limited. Curcumin has been reported to confer neuroprotective effects in numerous neurological diseases and injuries, but its role in IVH-induced hydrocephalus has not been determined. The aim of present study was to determine whether curcumin treatment ameliorates blood brain barrier (BBB) damage and reduces the incidence of post-hemorrhagic hydrocephalus in IVH rat model. Autologous blood intraventricular injection was used to establish the IVH model. Our results revealed that repeated intraperitoneal injection of curcumin ameliorated IVH-induced learning and memory deficits as determined by Morris water maze and reduced the incidence of post-hemorrhagic hydrocephalus in a dose-dependent manner at 28 d post-IVH induction. Further, the increased BBB permeability and brain edema induced by IVH were significantly reduced by curcumin administration. In summary, these findings highlighted the important role of curcumin in improving neurological function deficits and protecting against BBB disruption via promoting the neurovascular unit restoration, and thus it reduced the severity of post-hemorrhagic hydrocephalus in the long term. It is believed that curcumin might prove to be an effective therapeutic component in prevent the post-IVH hydrocephalus in the near future. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  3. Endoscopic third ventriculostomy for hydrocephalus in brainstem glioma: a case series.

    Science.gov (United States)

    Kobayashi, Natsuki; Ogiwara, Hideki

    2016-07-01

    A brainstem glioma is an incurable brain tumor that can be complicated by hydrocephalus. A ventriculoperitoneal (VP) shunt is generally performed for the control of hydrocephalus. This study aimed to reveal the safety and efficacy of an endoscopic third ventriculostomy (ETV) for hydrocephalus in brainstem gliomas. Six patients who had pontine glioma with hydrocephalus underwent an ETV between May 2010 and November 2015. In all the cases, there were one or more symptoms of hydrocephalus (headache, nausea, vomiting, or lethargy). Retrospective review of these patients was performed using the medical records and neuroimagings. The ETV was performed safely and there were no intraoperative complications in all patients. The mean follow-up period was 12.3 months. An immediate symptomatic relief of hydrocephalus and an adequate control of symptoms were achieved without a VP shunt in all patients. The ETV is considered to be an effective and safe procedure for the treatment of hydrocephalus in brainstem gliomas. Determining the ventriculostomy site according to the preoperative MRI in each case is considered to be important for the safe procedure.

  4. Unilateral antler combs from Romuliana

    Directory of Open Access Journals (Sweden)

    Petković Sofija

    2006-01-01

    Full Text Available In the course of investigations at Romuliana nine antler three-partite combs with a single row of teeth were found in the Late Roman horizons dating from the late 4th - mid 5th century. They were found in Tower 19, in the Palace II sector and in the Thermae sector. The combs can be classified as two types: three-partite unilateral combs with semicircular handle (Petković comb type VII and three-partite unilateral combs with triangular handle decorated with horse protomes (Petković comb type VI. Two groups of these finds were distinguished after more detailed analysis; the earlier one including specimens originating from the Chernyahov-Sîntana de Mureº culture and later one including specimens made under "barbarian"influence and produced in Romuliana. These finds confirm the continuity of settlement at Romuliana in the Late Roman period, from the final quarter of the 4th until the end of the 5th century and open up the question of the character of the settlement.

  5. Characteristics of intracranial pressure (ICP) waves and ICP in children with treatment-responsive hydrocephalus.

    Science.gov (United States)

    Sæhle, Terje; Eide, Per Kristian

    2015-06-01

    One important goal of modern treatment of pediatric hydrocephalus is to normalize the intracranial pressure (ICP) and ICP volume reserve capacity to optimize normal brain development. Better knowledge of the characteristics of ICP waves/ICP in pediatric hydrocephalus may provide new insight into the mechanisms behind modern hydrocephalus treatment. The aim of the present work was to characterize the ICP waves/ICP in children with either communicating or non-communicating hydrocephalus who improved clinically after surgery. The hydrocephalic children not treated surgically following ICP monitoring served as reference patients. The patient material includes all children with hydrocephalus and no previous surgical treatment who underwent diagnostic ICP wave/ICP monitoring during the period 2002-2011. We retrieved the information about the patients from the patient records and the digitally stored ICP waveforms. The ICP wave characteristics amplitude, rise time and rise time coefficient and the mean ICP were determined in the patients treated surgically for their hydrocephalus. The findings were compared with findings in children not treated surgically after ICP monitoring who served as reference patients. The patient material includes 58 patients. Thirty-one (53%) were treated surgically after ICP monitoring, of whom all improved clinically. As compared to the reference patients, patients treated surgically presented with increased ICP wave amplitudes (MWA) and mean ICP. Alterations were comparable in communicating and non-communicating hydrocephalus. We found no apparent association between the ICP wave/ICP scores and presence of symptoms, indices of ventricular size or age. Children with either communicating or non-communicating hydrocephalus improving clinically after surgery presented with elevated MWA and mean ICP. In particular, the levels of MWA were raised to a magnitude seen when intracranial compliance is impaired. Hence, the present observations may support

  6. [Hydrocephalus Associated with Small Clinoidal Meningioma that Resolved after Tumor Removal:A Case Report].

    Science.gov (United States)

    Fujiwara, Hidemoto; Aiba, Toyotaka; Watanabe, Toru; Hiraishi, Tetsuya; Fujii, Yukihiko

    2016-12-01

    Small meningiomas causing hydrocephalus without obstruction of the ventricular system are rare. Herein, we report a case of small clinoidal meningioma with communicating hydrocephalus, which resolved after tumor removal. A 70-year-old woman presented with a 1-month history of memory disturbance followed by gait disturbance. MR images revealed a right clinoidal meningioma, 2 cm in diameter, and dilatation of the ventricles suggesting communicating hydrocephalus. The cerebrospinal fluid(CSF)pressure was 130 mmH2O, as determined via a lumbar puncture. High concentrations of protein(65mg/dL)were detected in the lumbar CSF. The tumor was completely removed via a frontotemporal craniotomy. Higher protein concentrations(94mg/dL)were detected in the CSF obtained intraoperatively from the sylvian cistern. The histopathological diagnosis was meningothelial meningioma. The patient's symptoms improved markedly after surgery. Postoperative MR images revealed resolution of the hydrocephalus. The lumbar CSF protein concentration returned to normal(43mg/dL). Neither tumor recurrence nor progression of hydrocephalus has been observed for 4 years. Communicating hydrocephalus, associated with a small meningioma at the supratentorial region, has not been described. Previous studies have shown that patients with meningioma may develop communicating hydrocephalus after tumor removal or stereotactic radiosurgery. Thus, it is interesting that the small supratentorial meningioma in our case developed communicating hydrocephalus without any therapeutic intervention. Considering the CSF protein concentration, we speculate that the hydrocephalus was the result of CSF malabsorption associated with high CSF protein concentration and CSF pathway obstruction at the suprasellar cistern caused by the tumor.

  7. Long-term hydrocephalus alters the cytoarchitecture of the adult subventricular zone

    Science.gov (United States)

    Campos-Ordoñez, Tania; Herranz-Pérez, Vicente; Chaichana, Kaisorn L.; Rincon-Torroella, Jordina; Rigamonti, Daniele; García-Verdugo, Jose M.; Quiñones-Hinojosa, Alfredo; Gonzalez-Perez, Oscar

    2014-01-01

    Hydrocephalus can develop secondarily to a disturbance in production, flow and/or absorption of cerebrospinal fluid. Experimental models of hydrocephalus, especially subacute and chronic hydrocephalus, are few and limited, and the effects of hydrocephalus on the subventricular zone are unclear. The aim of this study was to analyze the effects of long-term obstructive hydrocephalus on the subventricular zone, which is the neurogenic niche lining the lateral ventricles. We developed a new method to induce hydrocephalus by obstructing the aqueduct of Sylvius in the mouse brain, thus simulating aqueductal stenosis in humans. In 120-day-old rodents (n = 18 per group), the degree of ventricular dilatation and cellular composition of the subventricular zone were studied by immunofluorescence and transmission electron microscopy. In adult patients (age > 18 years), the sizes of the subventricular zone, corpus callosum, and internal capsule were analyzed by magnetic resonance images obtained from patients with and without aqueductal stenosis (n=25 per group). Mice with 60-day hydrocephalus had a reduced number of Ki67+ and doublecortin+ cells on immunofluorescence, as well as decreased number of neural progenitors and neuroblasts in the subventricular zone on electron microscopy analysis as compared to non-hydrocephalic mice. Remarkably, a number of extracellular matrix structures (fractones) contacting the ventricular lumen and blood vessels were also observed around the subventricular zone in mice with hydrocephalus. In humans, the widths of the subventricular zone, corpus callosum, and internal capsule in patients with aqueductal stenosis were significantly smaller than age and gender-matched patients without aqueductal stenosis. In summary, supratentorial hydrocephalus reduces the proliferation rate of neural progenitors and modifies the cytoarchitecture and extracellular matrix compounds of the subventricular zone. In humans, this similar process reduces the

  8. External hydrocephalus (benign extra-axial collections of infancy) - review of 15 cases

    International Nuclear Information System (INIS)

    Machado Junior, Marcos Alberto da Costa; Matos, Paulo Engracio de; Barbosa, Veronica Aline; Vieira, Lauro Conceicao Nascimento; Puglio, Nadja; Bacelar, Aroldo

    1997-01-01

    The authors studied 15 cases of external hydrocephalus over a 7-year period (September 1987 to May 1995). External hydrocephalus is a condition in which infants with rapidly enlarging heads are found to have a MRI showing widening of subarachnoid space with mild or no ventricular dilatation. Some of the MRI were compared with those of patients with cerebral atrophy and some etiopathogenic hypothesis are presented. These findings indicate that external hydrocephalus is an age-related self-limited condition occurring in infants with open cranial sutures and that it usually resolves without intervention after the second or third year of age. (author)

  9. Obstructive Hydrocephalus Secondary to Enlarged Virchow-Robin Spaces: A Rare Cause of Pulsatile Tinnitus.

    Science.gov (United States)

    Donaldson, Christopher; Chatha, Gurkirat; Chandra, Ronil V; Goldschlager, Tony

    2017-05-01

    Obstructive hydrocephalus secondary to enlarged Virchow-Robin Spaces (VRS) is a rare entity, with only a few cases reported in the literature. Presenting symptoms vary widely from headaches to dizziness. We report a case of a 31-year-old man who presented with pulsatile tinnitus and magnetic resonance imaging showing obstructive hydrocephalus secondary to tumefactive VRS. After a cerebrospinal fluid diversion procedure in the form of an endoscopic third ventriculostomy, he had almost complete resolution of his symptoms. This is the first case of obstructive hydrocephalus secondary to enlarged VRS, presenting with pulsatile tinnitus. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. The other hip in unilateral hip dysplasia

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Rømer, Lone; Søballe, Kjeld

    2006-01-01

    with apparently unilateral right developmental dysplasia (left hip center-edge angles greater than 20 degrees), 26 patients with apparently unilateral left developmental dysplasia (right hip center-edge angles greater than 20 degrees), 68 patients with bilateral developmental dysplasia, and 34 patients...... "gold" standard]). See the Guidelines for Authors for a complete description of levels of evidence....

  11. Clinical and surgical management of unilateral prepubertal gynecomastia

    Directory of Open Access Journals (Sweden)

    Giuseppe Andrea Ferraro

    2014-01-01

    CONCLUSION: The exact mechanism of unilateral gynecomastia formation in our case is unclear. The evaluation of unilateral gynecomastia can therefore be complex. In conclusion, the surgical treatment of unilateral gynecomastia requires an individual approach, based on an appropriate diagnostic algorithm.

  12. ELECTROPHYSIOLOGICAL SUBSTANTIATION OF EFFICACY OF CONGENITAL CLUBFOOT SURGICAL TREATMENT

    Directory of Open Access Journals (Sweden)

    I. Y. Klychkova

    2012-01-01

    Full Text Available Purpose - a complex assessment of the neuromuscular apparatus in different age groups of children with congenital clubfoot before and after surgery. Material and methods. The study included 80 patients (23 girls and 57 boys with moderate and severe congenital clubfoot between the ages of 0 to 18 years. In 46% of patients a bilateral lesion was noted, in 54% - unilateral. All patients who received surgical treatment tendon-muscle plasty by the Sturm-Zatsepin method, modified in the Turner Institute, were carried out. Long-term results of surgical treatment were investigated in terms of 1 to 5 years. Neurophysiological studies were carried out on a multi-channel computer complex "Neuro-MEP-4" (Russia. Contractive ability of the tibial, peroneal, and gastrocnemius muscles of lower leg was assessed by global electromyography (EMG for the affected and healthy sides. In order to receive the normal age indexes of electrogenesis the identical study was conducted in 30 subjects in each age group without pathology of the lower extremities. Results. Significant abnormalities of the functional state of the neuro-muscular system in shin were revealed in patients with congenital clubfoot. The analysis of the EMG results in patients admitted for conservative treatment (group 1 showed that in unilateral disease there is a decrease of electrical activity of leg muscles on the affected side. If bilateral symmetrical lesions observed decreasing of muscle electrogenesis were noted compared with the age norm. There are quantitative and qualitative changes in electrogenesis, which are characterized by secondary changes in the muscles on the background of segmental abnormalities. After surgical intervention in patients 0-3 years the positive dynamics of the neuro-muscular functional state of the shin was achieved, which allows to recommend surgical treatment of congenital clubfoot in children in age till three years. Revealed violations of the conductivity on the

  13. CT manifestation of congenital toxoplasmosis infection of the brain (report of 42 cases)

    International Nuclear Information System (INIS)

    Wang Zhenyu; Li Shuxin; Feng Kun

    1997-01-01

    To improve the recognition and diagnosis of congenital toxoplasmosis infection of the brain, forty-two cases of congenital toxoplasmosis infection of the brain verified by serological tests and initially investigated by CT were retrospectively studied. The main diagnostic feature of the entity included: (1) Widely scattered small nodular or curvilinear calcifications involving the basal ganglia, subependymal region and the frontal or parietal lobes; (2) Small patches of low density foci located at the paraventricular and gray-white matter junction area with some enhancement surrounding the foci after contrast media administration; (3) Evidence of obstructive hydrocephalus and (4) Complications of CNS malformation or developmental problems. Conclusion: CT was one of the best methods for the diagnosis of this entity, however, it should be closely correlated with the results from serological tests

  14. Emotional Perception of Music in Children With Bimodal Fitting and Unilateral Cochlear Implant.

    Science.gov (United States)

    Shirvani, Sareh; Jafari, Zahra; Motasaddi Zarandi, Masoud; Jalaie, Shohre; Mohagheghi, Hamed; Tale, Mohammad Reza

    2016-06-01

    Biological, structural, and acoustical constraints faced by cochlear implant (CI) users can alter the perception of music. Bimodal fitting not only provides bilateral hearing but can also improve auditory skills. This study was conducted to assess the impact of this amplification style on the emotional perception of music among children with hearing loss (HL). Twenty-five children with congenital severe to profound HL and unilateral CIs, 20 children with bimodal fitting, and 30 children with normal hearing participated in this study. Their emotional perceptions of music were measured using a method where children indicated happy or sad feelings induced by music by pointing to pictures of faces showing these emotions. Children with bimodal fitting obtained significantly higher mean scores than children with unilateral CIs for both happy and sad music items and in overall test scores (P emotional perception of music compared to unilateral CI. Given the influence of music in neurological and linguistic development and social interactions, it is important to evaluate the possible benefits of bimodal fitting prescriptions for individuals with unilateral CIs. © The Author(s) 2015.

  15. Increased self-diffusion of brain water in hydrocephalus measured by MR imaging

    DEFF Research Database (Denmark)

    Gideon, P; Thomsen, C; Gjerris, F

    1994-01-01

    We used MR imaging to measure the apparent brain water self-diffusion in 5 patients with normal pressure hydrocephalus (NPH), in 2 patients with high pressure hydrocephalus (HPH), and in 8 age-matched controls. In all patients with NPH significant elevations of the apparent diffusion coefficients...... white matter, and in one patient reexamined one year after surgery, ADCs were unchanged in nearly all brain regions. The increased ADC values in hydrocephalus patients may be caused by factors such as changes in myelin-associated bound water, increased Virchow-Robin spaces, and increased extracellular...... brain water fraction. For further studies of brain water diffusion in hydrocephalus patients, echo-planar imaging techniques with imaging times of a few seconds may be valuable....

  16. Multiloculated hydrocephalus: a review of current problems in classification and treatment

    DEFF Research Database (Denmark)

    Andresen, Morten; Juhler, Marianne

    2012-01-01

    PURPOSE: Loculated hydrocephalus is a condition in which discrete fluid-filled compartments form in or in relation to the ventricular system of the brain. Both uni- and multiloculated variants exist, with marked differences in outcome. However, several competing and seemingly interchangeable...... of Systematic Reviews, and the U.S. NIH ClinicalTrials.gov database was carried out with the search terms: "multicystic," "multiloculated," "multicompartment," "uniloculated," and "loculated." All were used in conjunction with the search term "hydrocephalus." RESULTS: A single study with a control group......, evidence is in favor of the neuroendoscopic approach. CONCLUSIONS: In order to ensure a consistent nomenclature as well as to guide future research, we propose a new system of classification for loculated hydrocephalus. It acknowledges the differences between uniloculated and multiloculated hydrocephalus...

  17. Valved or valveless ventriculoperitoneal shunting in the treatment of post-haemorrhagic hydrocephalus

    DEFF Research Database (Denmark)

    Andreasen, Trine Hjorslev; Holst, Anders Vedel; Lilja, Alexander

    2016-01-01

    BACKGROUND: Implant infection and obstruction are major complications for ventriculoperitoneal shunts in patients with post-haemorrhagic hydrocephalus. In an effort to (1) reduce the incidence of these complications, (2) reduce the rate of shunt failure and (3) shorten the duration of neurosurgical...... hospitalisation, we have implemented valveless ventriculoperitoneal shunts at our department for adult patients with post-haemorrhagic hydrocephalus and haemorrhagic cerebrospinal fluid at the time of shunt insertion. METHODS: All adult patients (>18 years old) treated for post-haemorrhagic hydrocephalus.......3 %, p = 0.02), but a higher rate of overdrainage (10.3 % vs 2.6 %, p = 0.04). CONCLUSION: The use of a valveless shunting for patients with post-haemorrhagic hydrocephalus results in shorter duration of neurosurgical hospitalisation and lower rate of shunt infection, although these advantages should...

  18. Fever after aneurysmal subarachnoid hemorrhage: relation with extent of hydrocephalus and amount of extravasated blood

    NARCIS (Netherlands)

    Dorhout Mees, Sanne M.; Luitse, Merel J. A.; van den Bergh, Walter M.; Rinkel, Gabriel J. E.

    2008-01-01

    BACKGROUND AND PURPOSE: Fever after aneurysmal subarachnoid hemorrhage is associated with poor outcome. Because hydrocephalus and extravasated blood may influence thermoregulation, we determined whether these factors increase the risk for fever after subarachnoid hemorrhage. METHODS: Fever within 14

  19. Hydrocephalus after Subarachnoid Hemorrhage: Pathophysiology, Diagnosis, and Treatment

    Directory of Open Access Journals (Sweden)

    Sheng Chen

    2017-01-01

    Full Text Available Hydrocephalus (HCP is a common complication in patients with subarachnoid hemorrhage. In this review, we summarize the advanced research on HCP and discuss the understanding of the molecular originators of HCP and the development of diagnoses and remedies of HCP after SAH. It has been reported that inflammation, apoptosis, autophagy, and oxidative stress are the important causes of HCP, and well-known molecules including transforming growth factor, matrix metalloproteinases, and iron terminally lead to fibrosis and blockage of HCP. Potential medicines for HCP are still in preclinical status, and surgery is the most prevalent and efficient therapy, despite respective risks of different surgical methods, including lamina terminalis fenestration, ventricle-peritoneal shunting, and lumbar-peritoneal shunting. HCP remains an ailment that cannot be ignored and even with various solutions the medical community is still trying to understand and settle why and how it develops and accordingly improve the prognosis of these patients with HCP.

  20. Control volume based hydrocephalus research; analysis of human data

    Science.gov (United States)

    Cohen, Benjamin; Wei, Timothy; Voorhees, Abram; Madsen, Joseph; Anor, Tomer

    2010-11-01

    Hydrocephalus is a neuropathophysiological disorder primarily diagnosed by increased cerebrospinal fluid volume and pressure within the brain. To date, utilization of clinical measurements have been limited to understanding of the relative amplitude and timing of flow, volume and pressure waveforms; qualitative approaches without a clear framework for meaningful quantitative comparison. Pressure volume models and electric circuit analogs enforce volume conservation principles in terms of pressure. Control volume analysis, through the integral mass and momentum conservation equations, ensures that pressure and volume are accounted for using first principles fluid physics. This approach is able to directly incorporate the diverse measurements obtained by clinicians into a simple, direct and robust mechanics based framework. Clinical data obtained for analysis are discussed along with data processing techniques used to extract terms in the conservation equation. Control volume analysis provides a non-invasive, physics-based approach to extracting pressure information from magnetic resonance velocity data that cannot be measured directly by pressure instrumentation.

  1. [Quantitative volumetric determinations on MR tomograms in communicating hydrocephalus].

    Science.gov (United States)

    Langkowski, J H; Palmié, S G; von Koschitzky, H; Imme, M; Maas, R; Schmidt, K H; Heller, M

    1989-02-01

    For patients with communicating hydrocephalus the implantation of a cardioventricular shunt is mostly an approved therapy. However, indications are frequently problematic and therefore a shunt operation will not be always successful. The proof of periventricular oedemas in addition to clinical signs seems to be a criterion for an indication for implantation and for the selection of a special valve. With the aid of a newly developed computer programme volume estimation of the ventricular system and the periventricular oedemas has been effected for six patients on T2-weighted MR-tomograms (1.5 tesla). Quotients of the different volumes are correlated with cerebrospinal fluid (CSF) pressure obtained during shunt implantation or lumbar measurement. Distinct indications have been found for a correlation between the volume of the periventricular oedemas and the pressure measurements.

  2. Is There a Right Ear Advantage in Congenital Aural Atresia?

    Science.gov (United States)

    Reed, Robert; Hubbard, Matthew; Kesser, Bradley W

    2016-12-01

    To compare speech/language development and academic progress between children with right versus left congenital aural atresia (CAA). Case control survey and review of audiometric data. Tertiary care academic practice. Children with unilateral CAA. Demographic and audiometric data; rates of grade retention, use of any hearing or learning resource, and behavioral problems. No significant differences in grade retention rate, utilization of amplification, speech language therapy, use of an individualized education program, or frequency modulated system were found between children with right versus left CAA. Children with left CAA were significantly more likely to be enrolled in special education programs (p = 0.026). Differences in reported communication problems approached significance with more difficulty noted in the right ear group (p = 0.059). Left CAA patients were also more likely to have reported behavioral problems (p = 0.0039). Contrary to the hypothesis that a normal hearing right ear confers a language advantage in patients with unilateral hearing loss, children with left CAA (normal right ear) were statistically more likely to be enrolled in a special education program and have behavioral problems. Reported communication problems were more common in right CAA patients, but this did not reach statistical significance. No differences were found in use of amplification, frequency modulated system, individualized education program, or grade retention. Further investigation of both the clinical implications and underlying psychoacoustics of unilateral hearing loss and the identification and habilitation of "at risk" unilateral hearing loss children is warranted.

  3. Guidelines for management of idiopathic normal pressure hydrocephalus. Guidelines from the guidelines committee of idiopathic normal pressure hydrocephalus, the Japanese Society of Normal Pressure Hydrocephalus

    International Nuclear Information System (INIS)

    Ishikawa, Masatsune; Hashimoto, Masaaki; Kuwana, Nobumasa; Mori, Etsuro; Miyake, Hiroji; Wachi, Akihiko; Takeuchi, Totaro; Kazui, Hiroaki; Koyama, Hiroshi

    2008-01-01

    With the rapid aging of Japanese society, medical care of the elderly has become an important social issue. Among various disorders manifesting dementia, gait disturbance, and urinary incontinence in the elderly population, normal pressure hydrocephalus (NPH), especially of idiopathic type (iNPH), is becoming noteworthy. The Guidelines for management of iNPH in Japan are created in compliance with the evidence-based medicine methods and published in 2004. This English version is made to show the diagnosis and treatment of iNPH with reference to the socio-medical background in Japan and to promote the international research on iNPH. They propose three diagnostic levels; possible, probable, and definite. They indicate the diagnostic importance of high convexity tightness and dilated sylvian fissure with mild to moderate ventriculomegaly on coronal magnetic resonance imaging. The cerebrospinal fluid tap test is regarded as an important diagnostic test because of its simplicity to perform and high predictability of the shunt efficacy. The use of programmable valves at shunt surgeries is recommended. Flowcharts for diagnosis, preoperative assessment, and prevention for complications of shunt surgery are made to promote a wide use of them. (author)

  4. Syndrome of inappropriately low-pressure acute hydrocephalus (SILPAH).

    Science.gov (United States)

    Hamilton, Mark G; Price, Angel V

    2012-01-01

    Most patients with acute hydrocephalus have ventriculomegaly and high intracranial pressure (ICP). However, there is a subset of patients who are symptomatic with acute ventriculomegaly and inappropriately low ICP. Two patient groups were defined. Each patient presented with clinical deterioration that included a significant decrease in level of consciousness with new and significant ventriculomegaly. Patients in group 1 (n = 10) were managed without endoscopic third ventriculostomy (ETV). Group 2 was a series of patients (n = 10) managed with ETV. Treatment for both groups involved insertion of an external ventricular drain (EVD) with ICP <5 cmH(2)O. Further treatment consisted of either neck wrapping with a tensor bandage and/or lowering the EVD to negative levels to facilitate drainage of cerebrospinal fluid (CSF), which resulted in clinical improvement and resolution of ventriculomegaly. All 20 patients had anatomical obstruction to CSF flow into the subarachnoid space (SAS) confirmed by magnetic resonance imaging (MRI) with cine MRI studies. Group 1 patients were treated until shunt revision/insertion was possible (n = 7), ICP normalized, and the EVD could be removed (n = 2), or death (n = 1) occurred. Patients in group 2 all underwent ETV, and ICP patterns normalized in all. Group 2 patients were managed with an EVD until shunt revision/insertion was required (n = 2), ICP normalized and the EVD could be removed (n = 7), or death (n = 1) occurred. The syndrome of inappropriately low-pressure acute hydrocephalus (SILPAH) is an important entity in both children and adults. A possible hypothesis invokes loss of an effective SAS. ETV reestablishes communication between the SAS and ventricles, producing a rapid return of normal ICP dynamics and a significant decrease in the number of shunt-dependent patients.

  5. Pediatric posterior reversible encephalopathy syndrome presenting with isolated cerebellar edema and obstructive hydrocephalus.

    Science.gov (United States)

    Ettinger, Nicholas; Pearson, Matthew; Lamb, Fred S; Wellons, John C

    2014-10-01

    In this report, the authors describe the case of a teenage boy who presented with hypertensive emergency, posterior reversible encephalopathy syndrome, and hydrocephalus due to fourth ventricle outlet obstruction. Posterior reversible encephalopathy syndrome is a well-characterized but uncommon syndrome in children that is generally triggered by severe hypertension. The unusual clinical picture of this patient, who had isolated cerebellar edema leading to obstructive hydrocephalus, has been rarely described in children.

  6. Bedside transcranial sonography monitoring in a patient with hydrocephalus post subarachnoid hemorrhage

    OpenAIRE

    Najjar, Ahmed; Denault, André Y.; Bojanowski, Michel W.

    2017-01-01

    Background Development of hydrocephalus can occur after subarachnoid hemorrhage (SAH). Typically, it is diagnosed with computed tomography, CT, scan. However, transcranial sonography (TCS) can be used particularly in patients with craniotomy which removes the acoustic interference of the skull and allows a closer up visualization of brain structures through the skin. Case presentation We report a 73-year-old woman who was hospitalized for SAH and developed acute hydrocephalus requiring an ext...

  7. Adapted physical education for a student with spina bifida and hydrocephalus

    OpenAIRE

    Udir, Anita

    2016-01-01

    The purpose of the master's work was to determine the necessary adjustments and ways of integrating a student with spina bifida and hydrocephalus in Physical Education in the first grade of primary school. Whereas a student's progress in Physical Education is not being assessed, we wanted to evaluate student's progress and note what objectives are being achieved. The study include a student with spina bifida and hydrocephalus (spinal cord injury), who attends the first grade of primary sc...

  8. Pediatric Hydrocephalus in Ethiopia: Treatment Failures and Infections: A Hospital-Based, Retrospective Study.

    Science.gov (United States)

    Laeke, Tsegazeab; Tirsit, Abenezer; Biluts, Hagos; Murali, Deeptha; Wester, Knut

    2017-04-01

    Treatment of pediatric hydrocephalus in sub-Saharan countries is associated with significant complication rates. The objective of this study is to analyze the management of hydrocephalus and complication rates of surgical intervention in the Ethiopian setting to improve future quality. A retrospective cohort study was conducted in a neurosurgical teaching hospital. Two cohorts separated by 2.5 years were analyzed. A total of 128 (58.6% male and 41.4% female) children with isolated hydrocephalus, meningomyelocele (MMC)-related hydrocephalus, or MMC without hydrocephalus were included. Their age ranged from 1 day to 5 years, for a mean age of 7.2 months (median age 2 months). One hundred thirteen patients had hydrocephalus, of whom 57 (44.5.3%) had isolated hydrocephalus and 56 (43.8%) had hydrocephalus associated with MMC. Seventy-seven (74.7%) patients underwent ventriculoperitoneal (VP) shunting, whereas 24 (23.3%) underwent endoscopic third ventriculostomy (ETV). The incidence of shunt infection was 23.4%. Reoperation was needed in 54 (52.4%) patients, with the most common indication being shunt failure. ETV failed in 14 (58.3%) of the 24 patients undergoing ETV. VP shunt insertions had unacceptably high infection rate despite the presence of a protocol for the procedure. Intraoperative guidelines should be developed further and followed strictly to reduce infections. Such measures should include restricting the number of surgeons performing the procedure. In our opinion, one should avoid insertion of a VP shunt as the primary treatment. ETV has proved to be a good alternative in other studies and the decreasing pattern of ETV failure in our study also suggest ETV as a better alternative to VP shunt. Copyright © 2016 The Author(s). Published by Elsevier Inc. All rights reserved.

  9. [Hydrocephalus as initial presentation of a spinal cord tumour in a child

    DEFF Research Database (Denmark)

    Jorgensen, L.M.; Nysom, K.; Lavard, L.D.

    2008-01-01

    We report a previously healthy two-year-old girl who initially presented with signs of increased intracranial pressure of vomiting, lethargy and unstable gait. She had communicating hydrocephalus and a ventriculoperitoneal shunt was placed. Two years later the girl developed signs of myelopathy...... and was diagnosed with a spinal cord tumour between Th3 and Th9. We suggest that spinal cord tumour should be considered in patients with increased intracranial pressure or hydrocephalus of unknown origin Udgivelsesdato: 2008/9/15...

  10. Early variations of laboratory parameters predicting shunt-dependent hydrocephalus after subarachnoid hemorrhage.

    Science.gov (United States)

    Na, Min Kyun; Won, Yu Deok; Kim, Choong Hyun; Kim, Jae Min; Cheong, Jin Hwan; Ryu, Je Il; Han, Myung-Hoon

    2017-01-01

    Hydrocephalus is a frequent complication following subarachnoid hemorrhage. Few studies investigated the association between laboratory parameters and shunt-dependent hydrocephalus. This study aimed to investigate the variations of laboratory parameters after subarachnoid hemorrhage. We also attempted to identify predictive laboratory parameters for shunt-dependent hydrocephalus. Multiple imputation was performed to fill the missing laboratory data using Bayesian methods in SPSS. We used univariate and multivariate Cox regression analyses to calculate hazard ratios for shunt-dependent hydrocephalus based on clinical and laboratory factors. The area under the receiver operating characteristic curve was used to determine the laboratory risk values predicting shunt-dependent hydrocephalus. We included 181 participants with a mean age of 54.4 years. Higher sodium (hazard ratio, 1.53; 95% confidence interval, 1.13-2.07; p = 0.005), lower potassium, and higher glucose levels were associated with higher shunt-dependent hydrocephalus. The receiver operating characteristic curve analysis showed that the areas under the curve of sodium, potassium, and glucose were 0.649 (cutoff value, 142.75 mEq/L), 0.609 (cutoff value, 3.04 mmol/L), and 0.664 (cutoff value, 140.51 mg/dL), respectively. Despite the exploratory nature of this study, we found that higher sodium, lower potassium, and higher glucose levels were predictive values for shunt-dependent hydrocephalus from postoperative day (POD) 1 to POD 12-16 after subarachnoid hemorrhage. Strict correction of electrolyte imbalance seems necessary to reduce shunt-dependent hydrocephalus. Further large studies are warranted to confirm our findings.

  11. Hydrocephalus, skeletal anomalies, and mental disturbances in a mother and three daughters: A new syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Ferlini, A.; Zanetti, A.; Milan, M.; Calzolari, E. [Universita di Ferrara, London (United Kingdom)] [and others

    1995-12-04

    We report on a family in which a mother and her 3 daughters have delayed psychomotor development and/or psychosis, hydrocephalus with white matter alterations, arachnoid cysts, skeletal anomalies consisting of brachydactyly, and Sprengel anomaly. Biochemical and cytogenetic analyses were normal on all 4 patients. The pattern of inheritance, clinical manifestations, and variability of expression suggest that this is a new hydrocephalus syndrome possibly transmitted as an X-linked dominant trait. 24 refs., 6 figs., 1 tab.

  12. Congenital Ocular Motor Apraxia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2007-06-01

    Full Text Available The clinical and neuroradiological findings, and long-term intellectual prognosis in 10 patients (4 boys and 6 girls with congenital ocular motor apraxia (COMA are reviewed by researchers at Tottori University, Yonago, Japan.

  13. Congenital pulmonary vein stenosis.

    Science.gov (United States)

    Manzar, Shabih

    2007-06-01

    A case of a newborn infant is described who presented with severe cyanosis at birth with rapid deterioration. The infant died at six hours of life. The diagnosis was determined at autopsy as congenital pulmonary vein stenosis.

  14. Congenital anterior urethral diverticulum

    International Nuclear Information System (INIS)

    Jung, Hyun Sub; Chung, Young Sun; Suh, Chee Jang; Won, Jong Jin

    1985-01-01

    Two cases of congenital anterior urethral diverticular which have occurred in a 4 year old and one month old boy are presented. Etiology, diagnostic procedures, and its clinical results are briefly reviewed

  15. Congenital orbital teratoma

    Directory of Open Access Journals (Sweden)

    Shereen Aiyub

    2013-01-01

    Full Text Available We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  16. Giant congenital nevus

    Science.gov (United States)

    ... nevus; Giant hairy nevus; Giant pigmented nevus; Bathing trunk nevus; Congenital melanocytic nevus - large ... the spine) Involvement of the membranes of the brain and spinal cord when the nevus affects a ...

  17. Identification of congenital deafblindness

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2012-01-01

    For many reasons an accurate and straightforward identification of congenital deafblindness can be difficult. This article reports on the assessment procedures and experience in Denmark where medical examinations were combined with functional assessments performed through direct observation. The ...

  18. Evaluation of lung function changes before and after surfactant application during artificial ventilation in newborn rats with congenital diaphragmatic hernia

    NARCIS (Netherlands)

    E.C. Scheffers; H. IJsselstijn (Hanneke); R. Tenbrinck (Robert); B.F. Lachmann (Burkhard); J.C. de Jongste (Johan); J.C. Molenaar; D. Tibboel (Dick)

    1994-01-01

    textabstractPatients with congenital diaphragmatic hernia (CDH) have unilateral or bilateral hypoplasia of the lungs including delayed maturation of the terminal air sacs. Because these lungs are highly susceptible to barotrauma and oxygen toxicity, even in full-term newborns, continued research

  19. BIRTH INJURY RELATED UNILATERAL ANTERIOR NARES PARTIAL FIBROUS ATRESIA: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Jayagar

    2016-02-01

    Full Text Available Congenital atresia of anterior nares has been rarely reported and it may co-exist along with posterior choanal atresia. (1,2 In our case, birth injury caused by forceps delivery has caused unilateral anterior nares partial atresia. Twenty eight years old male patient came with the complaints of left nostril blocked sensation since birth, aggravated with attacks of upper respiratory tract infections. On examination he had left anterior nares partial atresia caused by fibrous bands as a result of birth injury due to instrumental delivery. Rhinoplasty performed to open-up left nostril and patient relieved of his symptoms and also on cosmetic appearance

  20. Case report: Isolated unilateral pulmonary vein atresia diagnosed on 128-slice multidetector CT

    Directory of Open Access Journals (Sweden)

    Rashmi Dixit

    2011-01-01

    Full Text Available Unilateral pulmonary venous atresia is an uncommon entity that is generally believed to be congenital. Most patients present in infancy or childhood with recurrent chest infections or hemoptysis. Pulmonary angiography is usually used for definitive diagnosis. However, the current multislice CT scanners may obviate the need for pulmonary angiography. We report two cases diagnosed using 128-slice CT angiography. On the CT angiography images both these cases demonstrated absent pulmonary veins on the affected side, with a small pulmonary artery and prominent bronchial or other systemic arterial supply.

  1. Congenital chylous ascites

    International Nuclear Information System (INIS)

    Romanska-Kita, J.; Borszewska-Kornacka, M. K.; Rudzinska, I.; Wawrzoniak, T.; Dobrzanska, A.; Czech-Kowalska, J.

    2011-01-01

    Congenital chylous ascites is a rare entity, conditioned by numerous factors and with changing dynamics of the disease. Because of the lack of therapeutic and diagnostic standards, this disease constitutes to be a medical challenge. This article presents current knowledge on pathogenesis, diagnostics and management of this disease, as well as a case of a newborn with primary congenital chylous ascites in the abdominal cavity. (authors)

  2. Congenital laryngeal anomalies,

    OpenAIRE

    Rutter, Michael J.

    2014-01-01

    Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the ma...

  3. Congenital gluteus maximus contracture.

    Science.gov (United States)

    Ganel, A; Blankstein, A

    1989-01-01

    Muscle contractures are infrequent in Israel. This report discusses one case of a congenital contracture associated with a skin dimple treated in Israel. A 3-year-old boy presented with difficulty in running, riding a bicycle, and squatting. Flexion of the right hip in adduction was impossible. The hip could be fully flexed in abduction. Congenital contracture of the right gluteus maximus muscle was successfully treated by surgical release.

  4. [Lower urinary tract dysfunction in normal pressure hydrocephalus: Review of the literature].

    Science.gov (United States)

    Bey, E; Nicot, B; Casez, O; Le Normand, L

    2016-12-01

    Lower urinary tract dysfunction in normal pressure hydrocephalus has received little attention from the scientific community. The aim of this review article was to discuss diagnostic and therapeutic options for these patients. A literature review of MedLine publications on urinary incontinence in normal pressure hydrocephalus was conducted. The following keywords were used: "hydrocephalus, normal pressure" and "bladder dysfunction" or "urinary incontinence" or "overactive bladder" or "urinary bladder, neurogenic". Prospective and retrospective studies as well as previous reviews were analyzed. Urinary symptoms in normal pressure hydrocephalus are mainly represented by overactive bladder, which is a significant burden for the concerned patients. Isolated overactive bladder is more frequent (64%) than urinary incontinence (57%). Detrusor overactivity is seen in 95.2% of the cases. Neuro-surgery is efficient on urinary symptoms for 61.5% of the patients. Bladder recovery after surgery relates with increased mid-cingulate perfusion, probably linked with a functional restoration of the mid-cingulate that normally inhibits the micturition reflex. Medical options, added or not to surgery, include anticholinergic drugs unable to pass through the blood-brain barrier, Transcutaneous Electrical Nerve Stimulation and sacral neuromodulation. There is actually an insufficient concern about urinary symptoms in normal pressure hydrocephalus. This article highlights the importance of a harmonization of neuro-urological practices in the pre-therapeutic evaluation of patients suffering from normal pressure hydrocephalus. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  5. Social media networking in pediatric hydrocephalus: a point-prevalence analysis of utilization.

    Science.gov (United States)

    Elkarim, Ghassan Awad; Alotaibi, Naif M; Samuel, Nardin; Wang, Shelly; Ibrahim, George M; Fallah, Aria; Weil, Alexander G; Kulkarni, Abhaya V

    2017-08-01

    OBJECTIVE A recent survey has shown that caregivers of children with shunt-treated hydrocephalus frequently use social media networks for support and information gathering. The objective of this study is to describe and assess social media utilization among users interested in hydrocephalus. METHODS Publicly accessible accounts and videos dedicated to the topic of hydrocephalus were comprehensively searched across 3 social media platforms (Facebook, Twitter, and YouTube) throughout March 2016. Summary statistics were calculated on standard metrics of social media popularity. A categorization framework to describe the purpose of pages, groups, accounts, channels, and videos was developed following the screening of 100 titles. Categorized data were analyzed using nonparametric tests for statistical significance. RESULTS The authors' search identified 30 Facebook pages, 213 Facebook groups, 17 Twitter accounts, and 253 YouTube videos. These platforms were run by patients, caregivers, nonprofit foundations, and patient support groups. Most accounts were from the United States (n = 196), followed by the United Kingdom (n = 31), Canada (n = 17), India (n = 15), and Germany (n = 12). The earliest accounts were created in 2007, and a peak of 65 new accounts were created in 2011. The total number of users in Facebook pages exceeded those in Facebook groups (p social media use in the topic of hydrocephalus. Users interested in hydrocephalus seek privacy for support communications and are attracted to treatment procedure and surgical products videos. These findings provide insight into potential avenues of hydrocephalus outreach, support, or advocacy in social media.

  6. Analysis of risk factors for hydrocephalus development in newborn infants with germinal matrix hemorrhage.

    Science.gov (United States)

    Romero, L; Ros, B; Arráez, M A; Ríus, F; González, L; Martín, A; Carrasco, A; Segura, M

    2015-10-01

    The aim of this study was to analyze risk factors for the development of hydrocephalus in newborn infants with germinal matrix (GM) hemorrhage. The study comprised 271 patients admitted to Carlos Haya University Hospital in Malaga with GM hemorrhage. The following data were recorded: gestational age, gender, twin birth, head circumference at birth, weight at birth, and Papile grade. Severe obstetrical (abruption, chorioamnionitis, pregnancy-induced hypertension, tocolytic treatment) and neonatal disorders (respiratory distress syndrome, neonatal infection, coagulation disorder, patent ductus arteriosus, necrotizing enterocolitis) were also recorded. Symptomatic hydrocephalus was diagnosed in the event of a progressive increase in head circumference and ventricular indices requiring shunting. Of the 271 patients, 139 (51%) developed posthemorrhagic ventriculomegaly; 47 patients (17%) developed symptomatic hydrocephalus and needed shunt implantation. We found a significant relationship between the development of symptomatic hydrocephalus and Papile grade, lower gestational age, lower birth weight, twin birth, and neonatal infection. Awareness of risk factors for the development of hydrocephalus in newborn infants with GM hemorrhage should be emphasized in order to enable an early diagnosis of ventriculomegaly and symptomatic hydrocephalus and thus make a correct therapeutic decision.

  7. Incidence of Intraventricular Hemorrhage and Post Hemorrhagic Hydrocephalus in Preterm Infants

    Directory of Open Access Journals (Sweden)

    Negar Sajjadian

    2010-07-01

    Full Text Available "nGerminal matrix-intraventricular hemorrhage (IVH is the most common variety of neonatal intracranial hemorrhage and is characteristics of the premature infant. The importance of the lesion relates not only to its high incidence but to their attendant complications (IC: hydrocephalus. Brain sonography is the procedure of choice in diagnosis of germinal matrix- intraventricular hemorrhage and hydrocephalus. In this study we have used brain sonography for detection of intraventricular hemorrhage and post hemorrhagic hydrocephalus and their incidences. The studied population was consisted of premature neonate (birth weight equal or less than 1500g and gestational age equal or less than 37 weeks who admitted in Mofid Hospital NICU (Tehran, Iran during a one year period. For all neonate (including criteria brain sonography in first week of life was done and in presence of IVH, serial Brain sonography was done weekly for detection of hydrocephalus. A total of 57 neonate entered the study. Intraventicular-germinal matrix hemorrhage was seen in 64.4% (35 patients. Forty percent of patients with intraventricular-germinal matrix hemorrhage had grade I, 11% grade II, 25.7% grade III, 2.8% grade VI. Hydrocephalus was detected in 20 percent of patients who had intraventricular-germinal matrix hemorrhage. That incidence of IVH in our study in comparison with other area and situation is higher. Hydrocephaly had direct relation with severity of IVH. This shows that with control of risk factor of IVH, we can control Post hemorrhagic hydrocephalus.

  8. Magnetic resonance velocity mapping of 3D cerebrospinal fluid flow dynamics in hydrocephalus: preliminary results

    Energy Technology Data Exchange (ETDEWEB)

    Stadlbauer, Andreas [Landesklinikum St. Poelten, MR Physics Group, Department of Radiology, St. Poelten (Austria); University of Erlangen-Nuremberg, Department of Neurosurgery, Erlangen (Germany); Salomonowitz, Erich [Landesklinikum St. Poelten, MR Physics Group, Department of Radiology, St. Poelten (Austria); Brenneis, Christian [Landesklinikum St. Poelten, Department of Neurology, St. Poelten (Austria); Ungersboeck, Karl [Landesklinikum St. Poelten, Department of Neurosurgery, St. Poelten (Austria); Riet, Wilma van der [European MRI Consultancy (EMRIC), Strasbourg (France); Buchfelder, Michael; Ganslandt, Oliver [University of Erlangen-Nuremberg, Department of Neurosurgery, Erlangen (Germany)

    2012-01-15

    To investigate the detectability of CSF flow alterations in the ventricular system of patients with hydrocephalus using time-resolved 3D MR velocity mapping. MR velocity mapping was performed in 21 consecutive hydrocephalus patients and 21 age-matched volunteers using a 3D phase-contrast (PC) sequence. Velocity vectors and particle path lines were calculated for visualisation of flow dynamics. CSF flow was classified as ''hypomotile flow'' if it showed attenuated dynamics and as ''hypermotile flow'' if it showed increased dynamics compared with volunteers. Diagnostic efficacy was compared with routine 2D cine PC-MRI. Seven patients showed hypomotile CSF flow: six had non-communicating hydrocephalus due to aqueductal stenosis. One showed oscillating flow between the lateral ventricles after craniotomy for intracranial haemorrhage. Seven patients showed normal flow: six had hydrocephalus ex vacuo due to brain atrophy. One patient who underwent ventriculostomy 10 years ago showed a flow path through the opening. Seven patients showed hypermotile flow: three had normal pressure hydrocephalus, three had dementia, and in one the diagnosis remained unclear. The diagnostic efficacy of velocity mapping was significantly higher except for that of aqueductal stenosis. Our approach may be useful for diagnosis, therapy planning, and follow-up of different kinds of hydrocephalus. (orig.)

  9. Hydrocephalus: the role of cerebral aquaporin-4 channels and computational modeling considerations of cerebrospinal fluid.

    Science.gov (United States)

    Desai, Bhargav; Hsu, Ying; Schneller, Benjamin; Hobbs, Jonathan G; Mehta, Ankit I; Linninger, Andreas

    2016-09-01

    Aquaporin-4 (AQP4) channels play an important role in brain water homeostasis. Water transport across plasma membranes has a critical role in brain water exchange of the normal and the diseased brain. AQP4 channels are implicated in the pathophysiology of hydrocephalus, a disease of water imbalance that leads to CSF accumulation in the ventricular system. Many molecular aspects of fluid exchange during hydrocephalus have yet to be firmly elucidated, but review of the literature suggests that modulation of AQP4 channel activity is a potentially attractive future pharmaceutical therapy. Drug therapy targeting AQP channels may enable control over water exchange to remove excess CSF through a molecular intervention instead of by mechanical shunting. This article is a review of a vast body of literature on the current understanding of AQP4 channels in relation to hydrocephalus, details regarding molecular aspects of AQP4 channels, possible drug development strategies, and limitations. Advances in medical imaging and computational modeling of CSF dynamics in the setting of hydrocephalus are summarized. Algorithmic developments in computational modeling continue to deepen the understanding of the hydrocephalus disease process and display promising potential benefit as a tool for physicians to evaluate patients with hydrocephalus.

  10. Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences.

    Science.gov (United States)

    Tully, Hannah M; Ishak, Gisele E; Rue, Tessa C; Dempsey, Jennifer C; Browd, Samuel R; Millen, Kathleen J; Doherty, Dan; Dobyns, William B

    2016-03-01

    Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed magnetic resonance images (MRIs) and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to 1 of 5 subtypes and compared on the basis of clinical characteristics and developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth; in addition, 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts, and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. Overall, 53% of surgically treated patients experienced at least 1 shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms. © The Author(s) 2015.

  11. Communicating Hydrocephalus and Vestibular Schwannomas: Etiology, Treatment, and Long-Term Follow-Up

    Science.gov (United States)

    Al Hinai, Qasim; Zeitouni, Anthony; Sirhan, Denis; Sinclair, David; Melancon, Denis; Richardson, John; Leblanc, Richard

    2013-01-01

    Background Large vestibular schwannomas (VSs) can cause hydrocephalus by obstructing the fourth ventricle. Little is known about the communicating hydrocephalus that is seen with a smaller VS. Methods The clinicopathological findings and follow up of three patients with communicating hydrocephalus associated with a small VS are presented. Results Four patients aged 40 to 66 years (mean: 57.7) presented with ataxia, dementia, and urinary incontinence. The VS were 2.0 to 2.4 cm. The cerebrospinal fluid (CSF) protein was elevated in three patients in whom it was measured (1.7 to 6 times normal). The VS was resected in two patients. All of the patients required ventriculoperitoneal shunting (VPS). All of the patients were asymptomatic or improved at follow-up at 9 months to 13 years. Conclusion Communicating hydrocephalus associated with a VS can occur in younger patients than was previously thought. An elevated CSF protein appears to be important, but other factors may be involved. A shunting procedure is often required to relieve the symptoms of hydrocephalus even if the tumor is resected. Possible etiological causes of communicating hydrocephalus in patients with a small VS are discussed. PMID:24436891

  12. Prepubertal Unilateral Gynecomastia: Report of 2 Cases

    Science.gov (United States)

    Demirbilek, Hüseyin; Bacak, Gökhan; Baran, Rıza Taner; Avcı, Yahya; Baran, Ahmet; Keleş, Ayşenur; Özbek, Mehmet Nuri; Alanay, Yasemin; Hussain, Khalid

    2014-01-01

    Prepubertal unilateral gynecomastia is an extremely rare condition. At present, its etiology and management strategy are not well known. Two unrelated prepubertal boys of ages 8 and 9 who presented with complaints of unilateral enlargement of breast tissue are reported. Physical examination, biochemical, hormonal and oncologic work-up findings were normal. Both patients were treated with peripheral liposuction successfully. Histopathological and immunohistochemical examinations showed benign fibroglandular gynecomastia and intensive (3+) estrogen receptor expression in 100% of periductal epithelial cells. Although an extremely rare and generally benign condition, patients with prepubertal unilateral gynecomastia should have a full endocrine and oncologic work-up. PMID:25541897

  13. Fibrosis unilateral congénita con enoftalmo y ptosis Congenital unilateral fibrosis with enophthalmos and ptosis

    OpenAIRE

    Yaimir Estévez Miranda; Rosa Maria Naranjo Fernández; Teresita de Jesús Méndez Sánchez; Raúl Rúa Martínez

    2011-01-01

    El síndrome de fibrosis congénita es definido como un grupo de desórdenes congénitos raros caracterizado por la restricción de los músculos extraoculares y el reemplazo de los músculos por el tejido fibroso. Muchos autores utilizan la clasificación en la que se manifiesta en cinco tipos diferentes. El caso que se presenta corresponde a un niño quien desde el nacimiento está imposibilitado de elevar su ojo izquierdo. Al examen oftalmológico resultó positivo una ptosis palpebral en su ojo izqui...

  14. Unilateral fusion of the odontoid process with the atlas in Klippel-Feil syndrome: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, So Young; Ryu, Kyung Nam; Park, Ji Seon; Suk, Kyung Soo; Han, Mi Young [Kyunghee Medical Center, Seoul (Korea, Republic of)

    2006-07-15

    Klippel-Feil syndrome (KFS) displays congenital fusion of the cervical vertebrae; it is a relatively common condition and has many associated malformations such as Sprengel's deformity, scoliosis, rib anomalies, congenital defects of the brain or spinal cord, renal anomalies, congenital heart disease, deafness, cleft palate, cranial and facial asymmetry, and enteric cysts. There are various types of cervical fusion observed in KFS. However, fusion of the odontoid process with the atlas is a very rare finding. We report here on a 4-year-old boy with unilateral fusion of a separated odontoid process with the lateral mass of the atlas, and this was associated with a spontaneously closed ventricular septal defect, a small patent ductus arteriosus and a horseshoe kidney.

  15. Congenital Hemifacial Hyperplasia: Clinical Presentation and Literature Review

    Directory of Open Access Journals (Sweden)

    Karpagavalli Shanmugasundaram

    2016-01-01

    Full Text Available Hemifacial hyperplasia is a rare congenital malformation characterized by noticeable unilateral excess development of hard and soft tissues of the face. Asymmetry in Congenital Hemifacial Hyperplasia (CHH is usually evident at birth and accentuated at the age of puberty. The affected side grows exponentially as compared to the unaffected side. Multiple tissue involvement has resulted due to etiological heterogeneity like heredity, chromosomal abnormalities, altered intrauterine environment, and endocrine dysfunctions. As this lesion is rarely seen in our routine clinical practice, we present a case of hemifacial hyperplasia with reported orofacial features that supplement existing clinical knowledge. This paper also adds knowledge to the readers regarding detailed investigation procedures which has complemented our diagnosis. Further emphasis has been placed on periodic approach to its diagnosis and multidisciplinary management following correct diagnosis.

  16. Unilateral traumatic oculomotor nerve paralysis

    International Nuclear Information System (INIS)

    Asari, Syoji; Satoh, Toru; Yamamoto, Yuji

    1982-01-01

    The present authors report a case of unilateral traumatic oculomotor nerve paralysis which shows interesting CT findings which suggest its mechanism. A 60-year-old woman was admitted to our hospital with a cerebral concussion soon after a traffic accident. A CT scan was performed soon after admission. A high-density spot was noted at the medial aspect of the left cerebral peduncle, where the oculomotor nerve emerged from the midbrain, and an irregular, slender, high-density area was delineated in the right dorsolateral surface of the midbrain. Although the right hemiparesis had already improved by the next morning, the function of the left oculomotor nerve has been completely disturbed for the three months since the injury. In our case, it is speculated that an avulsion of the left oculomotor nerve rootlet occurred at the time of impact as the mechanism of the oculomotor nerve paralysis. A CT taken soon after the head injury showed a high-density spot; this was considered to be a hemorrhage occurring because of the avulsion of the nerve rootlet at the medial surface of the cerebral peduncle. (J.P.N.)

  17. Clinical presentation and management of congenital ptosis

    Directory of Open Access Journals (Sweden)

    Marenco M

    2017-02-01

    Full Text Available Marco Marenco,1,* Ilaria Macchi,2,* Iacopo Macchi,3 Emilio Galassi,4 Mina Massaro-Giordano,5 Alessandro Lambiase1 1Department of Sense Organs, University of Rome “Sapienza”, 2Department of Ophthalmology, Campus Bio-Medico University of Rome, Rome, 3Department of Ophthalmology, University of Catania, Catania, 4Ophthalmic Clinic, Department of Ophthalmology, University of L’Aquila, L’Aquila, Italy; 5Department of Ophthalmology, Scheie Eye Institute, University of Pennsylvania, Philadelphia, PA, USA *These authors contributed equally to this work Abstract: Congenital ptosis is a rare condition characterized by lower positioning of the upper eyelid that is present at birth and is a clinical condition that is persistent if not treated. It may be unilateral or bilateral and may be associated with other ocular disorders or systemic conditions, including Marcus Gunn, Horner, and Duane syndromes. It is a benign condition but causes functional, cosmetic, and psychological problems in children. However, not all patients need to undergo surgery, and usually only patients at risk of amblyopia need a prompt surgical correction, while in other cases, surgery can be postponed. The grade of ptosis, the eyelid function, and the amblyopic risk are the parameters that affect the ophthalmologist’s decision on timing of surgery and the surgical technique to be used. In fact, there are several types of surgical techniques to correct a congenital ptosis, although very often more than one is needed to obtain an acceptable result. This paper reviews the causes of congenital ptosis and associated diseases. Particular emphasis is given to surgical management and different procedures available to correct the upper eyelid anomaly and avoid permanent damage to visual function. Keywords: ptosis, extraocular muscle development, neurologic dysfunction, surgical approach

  18. [Congenital toxoplasmosis: clinical manifestation, treatment and follow-up] [Article in Italian] • Il neonato con toxoplasmosi congenita: clinica, terapia e follow-up

    Directory of Open Access Journals (Sweden)

    Lina Bollani

    2014-01-01

    Full Text Available Toxoplamosis is a parasitic zoonosis which occurs worldwide, but is prevalent in Europe, South America and Africa. When infection occurs for the first time during pregnancy, mother to child transmission of the parasite can cause congenital toxoplasmosis. Rate of congenital infection ranges from less than 0.1 to approximately 1 per 1,000 live births. The risk of transmission depends on the gestational age at the time of maternal infection. A diagnosis of congenital toxoplasmosis is usually considered in infants who present: hydrocephalus, chorioretinitis, and intracranial calcifications, but this triade is very rare. Approximately 85% of the infants with congenital toxoplasmosis are clinically normal at birth; however, sequelae of infection may become apparent only months or even years later. Chorioretinitis is the main complication of congenital toxoplasmosis, late onset retinal lesions and relapse can appear many years after birth, but the overall ocular prognosis is satisfactory when infection is identified and treated accordingly. Fortunately, serious neonatal forms and severe neurological impairment have become rare, but prompt treatment of children with convulsions, abnormal muscle tone, hydrocephalus, may improve the prognosis and result in almost normal outcome. For infants who have congenital toxoplasmosis, treatment soon after birth for 1 year with pyrimetamine, sulfadiazine and leukoverin led to remarkable resolution of serious, active disease. A long follow-up is necessary to assess the long-term outcome of children and young adults with congenital toxoplasmosis, that is favourable for the majority of cases. Epidemiological surveillance needs to be improved in order to determine the effectiveness of prevention programs.Articoli Selezionati del “3° Convegno Pediatrico del Medio Campidano” · Guspini · 25 Maggio 2013Guest Editor: Roberto Antonucci

  19. Treatment of congenital tuberculosis.

    Science.gov (United States)

    Patel, Sonal; DeSantis, Evelyn R Hermes

    2008-11-01

    The diagnosis and treatment of congenital tuberculosis are discussed. Congenital tuberculosis is rare and fatal if left untreated. If a pregnant woman with tuberculosis is not treated, infection of the fetus can occur by hematogenous spread through the umbilical cord or by aspiration or ingestion of amniotic fluid. Signs and symptoms of congenital tuberculosis may be nonspecific, which may preclude early diagnosis and treatment. Criteria for the diagnosis of congenital tuberculosis require the infant to have a tuberculous lesion, as indicated by chest radiography or granulomas, and at least one of the following should be confirmed: (1) onset during the first week of life, (2) primary hepatic tuberculosis complex or caseating hepatic granulomas, (3) infection of the placenta or maternal genital tract, or (4) exclusion of postnatal transmission by a contact investigation. Since 2001, 21 cases of congenital tuberculosis have been reported in English-language medical journals, with the age of presentation ranging from day 1 to 90. Based on findings from published case reports, congenital tuberculosis should be considered in the differential diagnosis of newborns who have (1) nonresponsive, worsening pneumonia, especially in regions with high rates of tuberculosis, (2) nonspecific symptoms but have a mother diagnosed with tuberculosis, (3) high lymphocyte counts in the cerebrospinal fluid without an identified bacterial pathogen, or (4) fever and hepatosplenomegaly. Once diagnosed, it is essential to promptly begin treatment with isoniazid, rifampin, pyrazinamide, and streptomycin in order to decrease the mortality associated with the infection. Early diagnosis and treatment during the neonatal period are crucial in minimizing the fatality associated with congenital tuberculosis.

  20. Unilateral Condylar Hyperplasia of the Mandible

    Directory of Open Access Journals (Sweden)

    Malachovsky I

    2015-12-01

    Full Text Available Condylar hyperplasia (CH of the mandible is a rare pathology that occurs at the head of the condyle and can lead to facial asymmetry affecting occlusion and possible association with pain and dysfunction. Unilateral condylar hyperplasia is an uncommon condition of unknown aetiology, proper diagnosis of which has to be established, as the patients may look for surgical help. A rare case of unilateral condylar hyperplasia of the mandible is reported here.

  1. Hydrocephalus, Ventriculo-Peritoneal shunt and Cerebrospinal fluid ...

    African Journals Online (AJOL)

    CSF) ascites, Ventriculoatrial (VA) shunt. Résumé Un petit nombre de patients, avec congenital hydrocephale, qui ont été traite avec succès par shunts ventriculo-peritoneal, development une augmentation progressive de la sangle abdominale ...

  2. Congenital terminal transverse deformity of upper limb: clinical and radiological findings in a sporadic care.

    Science.gov (United States)

    Malik, Sajid; Afzal, Muhammad

    2013-03-01

    Congenital transverse limb anomalies are rare, which affect upper and/or lower limbs and may accompany several syndromic malformations. We present a sporadic male subject with congenital, unilateral transverse arrest of the left hand. The affected arm was observed to be short with reduced zeugopod and truncated palm. Fingers were represented by five bead-like nubbins. Roentgenographic examination revealed short radius and ulna with hypoplastic distal heads, absent carpals/metacarpals, and a hypoplastic bony island in each nubbin. Consanguinity was denied, and the subject had no symptoms in the orofacial, neurological and skeletal systems. Detailed clinical data with literature survey is presented.

  3. The association of congenital neuroblastoma and congenital heart disease

    International Nuclear Information System (INIS)

    Bellah, R.; D'Andrea, A.; Children's Hospital, Boston, MA; Darillis, E.; Fellows, K.E.

    1989-01-01

    Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma. (orig.)

  4. Congenital malaria in China.

    Directory of Open Access Journals (Sweden)

    Zhi-Yong Tao

    2014-03-01

    Full Text Available BACKGROUND: Congenital malaria, in which infants are directly infected with malaria parasites from their mother prior to or during birth, is a potentially life-threatening condition that occurs at relatively low rates in malaria-endemic regions. It is recognized as a serious problem in Plasmodium falciparum-endemic sub-Saharan Africa, where recent data suggests that it is more common than previously believed. In such regions where malaria transmission is high, neonates may be protected from disease caused by congenital malaria through the transfer of maternal antibodies against the parasite. However, in low P. vivax-endemic regions, immunity to vivax malaria is low; thus, there is the likelihood that congenital vivax malaria poses a more significant threat to newborn health. Malaria had previously been a major parasitic disease in China, and congenital malaria case reports in Chinese offer valuable information for understanding the risks posed by congenital malaria to neonatal health. As most of the literature documenting congenital malaria cases in China are written in Chinese and therefore are not easily accessible to the global malaria research community, we have undertaken an extensive review of the Chinese literature on this subject. METHODS/PRINCIPAL FINDINGS: Here, we reviewed congenital malaria cases from three major searchable Chinese journal databases, concentrating on data from 1915 through 2011. Following extensive screening, a total of 104 cases of congenital malaria were identified. These cases were distributed mainly in the eastern, central, and southern regions of China, as well as in the low-lying region of southwest China. The dominant species was P. vivax (92.50%, reflecting the malaria parasite species distribution in China. The leading clinical presentation was fever, and other clinical presentations were anaemia, jaundice, paleness, diarrhoea, vomiting, and general weakness. With the exception of two cases, all patients

  5. Congenital cranial dysinnervation disorders.

    Science.gov (United States)

    Singh, Anupam; Pandey, P K; Agrawal, Ajai; Mittal, Sanjeev Kumar; Rana, Kartik Maheshbhai; Bahuguna, Chirag

    2017-12-01

    The European Neuromuscular Centre (ENMC) derived the term Congenital Cranial Dysinnervation Disorders in 2002 at an international workshop for a group of congenital neuromuscular diseases. CCDDs are congenital, non-progressive ophthalmoplegia with restriction of globe movement in one or more fields of gaze. This group of sporadic and familial strabismus syndromes was initially referred to as the 'congenital fibrosis syndromes' because it was assumed that the primary pathologic process starts in the muscles of eye motility. Over the last few decades, evidence has accumulated to support that the primary pathologic process of these disorders is neuropathic rather than myopathic. This is believed that for normal development of extra ocular muscles and for preservation of muscle fiber anatomy, normal intra-uterine development of the innervation to these muscles is essential. Congenital dysinnervation to these EOMs can lead to abnormal muscle structure depending upon the stage and the extent of such innervational defects. Over last few years new genes responsible for CCDD have been identified, permitting a better understanding of associated phenotypes, which can further lead to better classification of these disorders. Introduction of high-resolution MRI has led to detailed study of cranial nerves courses and muscles supplied by them. Thus, due to better understanding of pathophysiology and genetics of CCDDs, various treatment modalities can be developed to ensure good ocular alignment and better quality of life for patients suffering from the same.

  6. Prevalence of congenital amusia.

    Science.gov (United States)

    Peretz, Isabelle; Vuvan, Dominique T

    2017-05-01

    Congenital amusia (commonly known as tone deafness) is a lifelong musical disorder that affects 4% of the population according to a single estimate based on a single test from 1980. Here we present the first large-based measure of prevalence with a sample of 20 000 participants, which does not rely on self-referral. On the basis of three objective tests and a questionnaire, we show that (a) the prevalence of congenital amusia is only 1.5%, with slightly more females than males, unlike other developmental disorders where males often predominate; (b) self-disclosure is a reliable index of congenital amusia, which suggests that congenital amusia is hereditary, with 46% first-degree relatives similarly affected; (c) the deficit is not attenuated by musical training and (d) it emerges in relative isolation from other cognitive disorder, except for spatial orientation problems. Hence, we suggest that congenital amusia is likely to result from genetic variations that affect musical abilities specifically.

  7. Hydrocephalus treatment in children: long-term outcome in 975 consecutive patients.

    Science.gov (United States)

    Beuriat, Pierre-Aurelien; Puget, Stephanie; Cinalli, Giuseppe; Blauwblomme, Thomas; Beccaria, Kevin; Zerah, Michel; Sainte-Rose, Christian

    2017-07-01

    OBJECTIVE Hydrocephalus remains one of the more common pathologies managed in pediatric neurosurgical units. Endoscopic third ventriculostomy (ETV) has an advantage over ventriculoperitoneal shunting as it enables patients to remain device free. Multiple shunt devices with various valve designs exist, with no one valve proven to be superior to another. The aim of this study was to describe the management of hydrocephalus and its long-term outcome. METHODS The authors retrospectively reviewed the medical records of all patients who had been treated for hydrocephalus at the Hôpital Necker-Enfants Malades in the period from 1985 to 1995. RESULTS Nine hundred seventy-five children had been treated for hydrocephalus. The mean follow-up was 11 ± 7.4 years (mean ± standard deviation). The most common cause of hydrocephalus was tumor related (32.3%), followed by malformative (24.5%) and inflammatory (20.9%) causes. Two hundred eighty patients underwent ETV as the first-line treatment. The procedure was effective in controlling hydrocephalus due to posterior fossa tumors and aqueductal stenosis. Six hundred ninety-five children had initial shunt insertion, with the majority receiving an Orbis-Sigma valve (OSV). The overall OSV shunt survival was 70% at 1 year, 58% at 10 years, and 49% at 20 years. The most common cause for mechanical shunt failure was obstruction (50.7%). Overall shunt survival was statistically different between the OSV and the differential-pressure valve (p = 0.009). CONCLUSIONS Endoscopic third ventriculostomy is effective in the management of childhood hydrocephalus. Its success is directly related to the underlying pathology. In the long term, the OSV has significantly higher event-free shunt survival than the classic differential-pressure valve systems.

  8. Hydrocephalus is a rare outcome in community-acquired bacterial meningitis in adults: a retrospective analysis

    Science.gov (United States)

    2013-01-01

    Background Community-acquired bacterial meningitis (CABM) continues to have a high mortality rate and often results in severe sequelae among survivors. Lately, an increased effort has been focused on describing the neurological complications of meningitis including hydrocephalus. To aid in this field of research we set out to ascertain the risk and outcome of hydrocephalus in patients with community-acquired bacterial meningitis (CABM) in North Denmark Region. Methods We conducted a retrospective population-based cohort study of CABM cases above 14 years of age. Cases diagnosed during a 13-year period, 1998 through 2010, were identified in a laboratory register and data were acquired through patient records. Cases not confirmed by culture met other strict inclusion criteria. The diagnosis of hydrocephalus relied upon the radiologists’ reports on cranial imaging. Outcome was graded according to the Glasgow Outcome Scale at discharge from the primary admission. Long-term sequelae were based upon any subsequent hospital contacts until the end of 2011. Results Hydrocephalus was diagnosed in five of 165 episodes (3%) and all were classified as communicating. Only 120 patients had cranial imaging done and in this group the rate was 4.2%. In three cases hydrocephalus was present at admission, while two cases were diagnosed on days 44 and 99, respectively, due to altered mental status. The aetiology was either Eschericia coli (n = 2) or Streptococcus pneumoniae (n = 3). Case fatality was 60% among cases with hydrocephalus and 17% among other cases. Case fatality was similar irrespective of whether patients had a cranial CT or not. Conclusions Hydrocephalus was diagnosed in 3% of adolescent and adult cases with CABM and had a high case fatality rate in spite of specialised medical care and neurosurgical interventions. Our findings are comparable with a recent Dutch national prospective study. PMID:23855442

  9. Congenital syphilis: literature review

    Directory of Open Access Journals (Sweden)

    Eduardo Chaida Sonda

    2013-01-01

    Full Text Available Syphilis is an infectious disease caused by Treponema pallidum and has high rates of vertical transmission, which can reach 100% depending on the maternal disease and stage of pregnancy. The diagnosis of gestational syphilis is simple and its screening is required during the prenatal period. However, this disease still has a high prevalence, affecting two million pregnant women worldwide. The procedures performed in newborns with congenital syphilis represent costs that are three-fold higher than the ones spent with a baby without this infection. The treatment is generally carried out with penicillin and must be extended to sexual partners. Inadequate or lack of treatment of congenital syphilis can result in miscarriage, premature birth, acute complications and other fetal sequelae. KEYWORDS: Congenital syphilis. Treponema pallidum. Vertical transmission.

  10. Congenital cataract screening

    Directory of Open Access Journals (Sweden)

    Zhale Rajavi

    2016-01-01

    Full Text Available Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender.

  11. Congenital optic nerve anomalies.

    Science.gov (United States)

    Martín-Begué, N; Saint-Gerons, M

    2016-12-01

    To update the current knowledge about congenital optic disc anomalies. A comprehensive literature search was performed in the major biomedical databases. Patients with these anomalies usually have poor vision in infancy. Refractive errors are common, and serous retinal detachment may develop in some of these anomalies. It is critically important to clinically differentiate between these congenital optic disc anomalies, as central nervous system malformations are common in some, whereas others may be associated with systemic anomalies. Congenital optic disc anomalies are a heterogeneous group of pathologies with characteristic fundus appearance and systemic associations. We should always try to make a correct diagnosis, in order to ask for specific tests, as well as to provide an adequate follow-up. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  12. Congenital and neonatal pneumonia.

    Science.gov (United States)

    Nissen, Michael D

    2007-09-01

    The greatest risk of death from pneumonia in childhood is in the neonatal period. It is estimated that pneumonia contributes to between 750000-1.2 million neonatal deaths annually, accounting for 10% of global child mortality. Congenital and neonatal pneumonias are often a difficult disease to identify and treat, with clinical manifestations often being non-specific. Many of the normal lung defences are compromised in the fetus and neonate, leading to an increased susceptibility to infection. The aetiology and epidemiology of congenital and neonatal pneumonias will depend on the clinical setting and population that the baby belongs to, the stage in the perinatal period, the gestational age of the baby and the definition of pneumonia. Diagnosis, treatment and prevention strategies are therefore also dependent on these factors, and will differ depending on the clinical setting. This review summarizes the current knowledge concerning congenital and neonatal pneumonia worldwide and discusses future directions in the prevention of the disease.

  13. Congenital Hemolytic Anemia.

    Science.gov (United States)

    Haley, Kristina

    2017-03-01

    Red blood cell (RBC) destruction can be secondary to intrinsic disorders of the RBC or to extrinsic causes. In the congenital hemolytic anemias, intrinsic RBC enzyme, RBC membrane, and hemoglobin disorders result in hemolysis. The typical clinical presentation is a patient with pallor, anemia, jaundice, and often splenomegaly. The laboratory features include anemia, hyperbilirubinemia, and reticulocytosis. For some congenital hemolytic anemias, splenectomy is curative. However, in other diseases, avoidance of drugs and toxins is the best therapy. Supportive care with transfusions are also mainstays of therapy. Chronic hemolysis often results in the formation of gallstones, and cholecystectomy is often indicated. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Congenital hearing impairment

    International Nuclear Information System (INIS)

    Robson, Caroline D.

    2006-01-01

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  15. [Congenital spinal malformations].

    Science.gov (United States)

    Ertl-Wagner, B B; Reiser, M F

    2001-12-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered.

  16. Congenital spinal malformations

    International Nuclear Information System (INIS)

    Ertl-Wagner, B.B.; Reiser, M.F.

    2001-01-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [de

  17. Vibrant Soundbridge®in preschool children with unilateral aural atresia: acceptance and benefit.

    Science.gov (United States)

    Leinung, M; Zaretsky, E; Lange, B P; Hoffmann, V; Stöver, T; Hey, C

    2017-01-01

    The aim of this pilot study was to determine the acceptance and benefit from the middle ear implant system Vibrant Soundbridge ® (VSB, MED-EL Corporation, Austria) by means of a questionnaire, compared to a previously used conventional bone conducting hearing device, in preschool children with unilateral congenital aural atresia. Prospective cohort study. All nine children with unilateral congenital aural atresia used the VSB and had previous experience with a bone conducting hearing device. The benefit from the VSB was evaluated by questionnaires concerning acceptance of hearing aids, handling, listening effort, behavior, quality of life, and the duration of daily use and compared to the experience with the bone conducting hearing device. In addition, to quantify the benefit from the VSB use, audiological assessment (pure-tone audiometry via free field testing, speech audiometry, and localization test) was performed with and without VSB. The questionnaires and audiological test results were compared pairwise. According to all questionnaire areas, children benefited significantly more from the VSB compared to bone conducting hearing device (ps atresia benefited significantly more from the VSB compared to a conventional bone conducting hearing device according to the parents' questionnaires and yielded better results in the audiometry and localization test with the VSB than without it.

  18. Isolated Unilateral Absent Branch Pulmonary Artery with Peripheral Pulmonary Stenosis and Coronary Artery Disease

    Directory of Open Access Journals (Sweden)

    Sunil Abhishek B

    2017-09-01

    Full Text Available Isolated Unilateral Absent Pulmonary Artery (UAPA is a rare congenital anomaly. It is usually associated with congenital heart defects. A 45 year old male patient presented with complaints of fever with cough and expectoration for 15 days and retrosternal chest discomfort for the previous 2 days. ECG showed diffuse ST segment depression with T wave inversion in the inferior and lateral leads. Coronary Angiogram done through the right femoral approach revealed diffusely diseased Left Anterior Descending (LAD artery that was totally cut off at the mid segment. The Left Circumflex (LCx artery was providing blood supply to the right middle and lower lung areas. There was another collateral arising from the Left Subclavian Artery supplying the right middle and lower lung areas. The left pulmonary artery was normal, but branches supplying the middle and lower lobes of the right lung were absent and the upper lobe branch had pulmonary stenosis. UAPA is a rare clinical entity; collaterals from coronaries are extremely rare in this condition and till now there has not been any case report of unilateral absent branch pulmonary artery with peripheral stenosis of other branches, on the affected side and associated coronary artery disease.

  19. Diffusion-weighted imaging in the diagnostic evaluation of the hydrocephalus in patients with acute or chronic increase in cerebral pressure; Diffusionsgewichtete Bildgebung in der Diagnostik des Hydrocephalus - Untersuchungen an Patienten mit akuter und ohne akute Hirndrucksymptomatik

    Energy Technology Data Exchange (ETDEWEB)

    Dorenbeck, U. [Abt. fuer Neuroradiologie, Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Inst. fuer Roentgendiagnostik, Universitaetsklinik Regensburg (Germany); Schlaier, J. [Klinik und Poliklinik fuer Neurochirurgie, Universitaetsklinik Regensburg (Germany); Feuerbach, S.; Seitz, J. [Inst. fuer Roentgendiagnostik, Universitaetsklinik Regensburg (Germany)

    2005-01-01

    Purpose: to investigate whether diffusion-weighted imaging (DWI) in magnetic resonance imaging (MRI) provides additional information about the periventricular white matter for the assessment of hydrocephalus. Materials and methods: sixteen MRI examinations (11 with acutely increased cerebral pressure, 5 without symptoms) on 15 patients with hydrocephalus (4 patients with communicating hydrocephalus and 11 patients with obstructive hydrocephalus) were analyzed. One symptomatic patient subsequently became asymptomatic. We investigated the ''apparent diffusion coefficient'' (ADC) in the subcortical and periventricular white matter. The ADCs of the study patients were compared with those of a healthy control group. Results: symptomatic patients with hydrocephalus, 6/11 showed periventricular edema and a significantly higher ADC values in the periventricular region than in the subcortical white matter. 5/11 symptomatic patients showed significantly higher ADC values even in the absence of periventricular interstitial edema (both groups contained patients with communicating and obstructive hydrocephalus). All 5 asymptomatic patients with hydrocephalus did not have a significantly higher ADC values in the periventricular region. Conclusion: in patients with hydrocephalus and acutely increased cerebral pressure, DWI showed a significantly higher ADC values in the periventricular region even without visible interstitial edema on conventional MRI sequences. (orig.)

  20. Evaluation of periventricular radiolucency in hydrocephalus by dynamic CT studies

    Energy Technology Data Exchange (ETDEWEB)

    Nakano, Hideki; Bandou, Kuniaki; Miyaoka, Makoto (Fujisawa City Hospital, Kanagawa (Japan))

    1994-06-01

    The association of periventricular radiolucency (PVL) in normal pressure hydrocephalus (NPH) on CT scan is a common finding. However, it is difficult to differentiate NPH from atrophy or infarctions in which shunt surgery does not reverse these conditions. To distinguish NPH from these, we examined PVL by means of dynamic CT (DCT) studies. We investigated 12 elderly patients presenting with gait disturbance, urinary incontinence and mental dysfunction who had both enlarged lateral ventricles and PVL. All patients had laboratory investigations, including radioisotope and CT cisternography and Xenon CBF studies. Nine patients were shunted because of abnormal CSF dynamics. Varying degrees of clinical improvement after surgery were observed in 6 cases. A difference of arrival time (AT) between PVL and thalamus obtained from time-density-curve was calculated in each patient. The AT difference was 6.2[+-]1.5 sec. in the shunt-effective group, and 1.4[+-]1.3 sec. in both, the shunt in effective and ineligible group, this was highly significant (p<0.01). We also measured peak time (PT) in PVL, of the thalamus and anterior cerebral artery, but no significant correlation was obtained. An AT difference between PVL and thalamus obtained from DCT studies is a clinically useful diagnostic tool for the evaluation of NPH. (author).

  1. Evaluation of periventricular radiolucency in hydrocephalus by dynamic CT studies

    International Nuclear Information System (INIS)

    Nakano, Hideki; Bandou, Kuniaki; Miyaoka, Makoto

    1994-01-01

    The association of periventricular radiolucency (PVL) in normal pressure hydrocephalus (NPH) on CT scan is a common finding. However, it is difficult to differentiate NPH from atrophy or infarctions in which shunt surgery does not reverse these conditions. To distinguish NPH from these, we examined PVL by means of dynamic CT (DCT) studies. We investigated 12 elderly patients presenting with gait disturbance, urinary incontinence and mental dysfunction who had both enlarged lateral ventricles and PVL. All patients had laboratory investigations, including radioisotope and CT cisternography and Xenon CBF studies. Nine patients were shunted because of abnormal CSF dynamics. Varying degrees of clinical improvement after surgery were observed in 6 cases. A difference of arrival time (AT) between PVL and thalamus obtained from time-density-curve was calculated in each patient. The AT difference was 6.2±1.5 sec. in the shunt-effective group, and 1.4±1.3 sec. in both, the shunt in effective and ineligible group, this was highly significant (p<0.01). We also measured peak time (PT) in PVL, of the thalamus and anterior cerebral artery, but no significant correlation was obtained. An AT difference between PVL and thalamus obtained from DCT studies is a clinically useful diagnostic tool for the evaluation of NPH. (author)

  2. Cerebrospinal Fluid Biomarkers in Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Ville Leinonen

    2011-01-01

    Full Text Available The diagnosis of idiopathic normal pressure hydrocephalus (iNPH is still challenging. Alzheimer's disease (AD, along with vascular dementia, the most important differential diagnosis for iNPH, has several potential cerebrospinal fluid (CSF biomarkers which might help in the selection of patients for shunt treatment. The aim of this study was to compare a battery of CSF biomarkers including well-known AD-related proteins with CSF from patients with suspected iNPH collected from the external lumbar drainage test (ELD. A total of 35 patients with suspected iNPH patients were evaluated with ELD. CSF was collected in the beginning of the test, and the concentrations of total tau, ptau181, Aβ42, NFL, TNF-α, TGFβ1, and VEGF were analysed by ELISA. Twenty-six patients had a positive ELD result—that is, their gait symptoms improved; 9 patients had negative ELD. The levels of all analyzed CSF biomarkers were similar between the groups and none of them predicted the ELD result in these patients. Contrary to expectations lumbar CSF TNF-α concentration was low in iNPH patients.

  3. Passive flow regulators for drug delivery and hydrocephalus treatment

    Science.gov (United States)

    Chappel, E.; Dumont-Fillon, D.; Mefti, S.

    2014-03-01

    Passive flow regulators are usually intended to deliver or drain a fluid at a constant rate independently from pressure variations. New designs of passive flow regulators made of a stack of a silicon membrane anodically bonded to a Pyrex substrate are proposed. A first design has been built for the derivation of cerebrospinal fluid (CSF) towards peritoneum for hydrocephalus treatment. The device allows draining CSF at the patient production rate independently from postural changes. The flow rate is regulated at 20 ml/h in the range 10 to 40 mbar. Specific features to adjust in vivo the nominal flow rate are shown. A second design including high pressure shut-off feature has been made. The intended use is drug delivery with pressurized reservoir of typically 100 to 300 mbar. In both cases, the membrane comprises several holes facing pillars in the Pyrex substrate. These pillars are machined in a cavity which ensures a gap between the membrane and the pillars at rest. The fluid in the pressurized reservoir is directly in contact with the top surface of the membrane, inducing its deflection towards Pyrex substrate and closing progressively the fluidic pathway through each hole of the membrane. Since the membrane deflection is highly non-linear, FEM simulations have been performed to determine both radial position and diameter of the membrane holes that ensure a constant flow rate for a given range of pressure.

  4. PTPRQ as a potential biomarker for idiopathic normal pressure hydrocephalus

    Science.gov (United States)

    Nagata, Yuki; Bundo, Masahiko; Sugiura, Saiko; Kamita, Masahiro; Ono, Masaya; Hattori, Kotaro; Yoshida, Sumiko; Goto, Yu-Ichi; Urakami, Katsuya; Niida, Shumpei

    2017-01-01

    Idiopathic normal pressure hydrocephalus (iNPH) is caused by the accumulation of cerebrospinal fluid (CSF) and is characterized by gait disturbance, urinary incontinence, and dementia. iNPH dementia is treatable by shunt operation; however, since the cognitive symptoms of iNPH are often similar to those of other dementias, including Alzheimer's disease (AD), accurate diagnosis of iNPH is difficult. To overcome this problem, the identification of novel diagnostic markers to distinguish iNPH and AD is warranted. Using comparative proteomic analysis of CSF from patients with iNPH and AD, protein tyrosine phosphatase receptor type Q (PTPRQ) was identified as a candidate biomarker protein for discriminating iNPH from AD. ELISA analysis indicated that the PTPRQ concentration in the CSF was significantly higher in patients with iNPH compared with those with AD. In addition, the PTPRQ concentration in the CSF of non-responders to shunt operation (SNRs) tended to be relatively lower compared with that in the responders. PTPRQ may be a useful biomarker for discriminating between patients with iNPH and AD, and may be a potential companion biomarker to identify SNRs among patients with iNPH. Additional large-scale analysis may aid in understanding the novel aspects of iNPH. PMID:28714010

  5. Spinal meningeal melanocytoma and hydrocephalus and intracranial superficial siderosis

    International Nuclear Information System (INIS)

    Das, A.; Ratnagopal, P.; Puvanendran, K.; Teo, J.G.C.

    2001-01-01

    Meningeal melanocytomas are uncommon tumours of the central nervous system; fewer than 50 cases have been reported in the English literature. We review the unique clinical presentation, radiological appearance and histological features of a rare case of meningeal melanocytoma. The patient was a 50-year-old man with a history of hypertension who presented with complaints of severe headache, nausea, vomiting and blurry vision for 2 days. Clinical examination revealed no hyperpigmentation marks and he had no history of regressed skin melanocytic lesions. Apart from mild terminal neck stiffness, his general medical examination was unremarkable. On funduscopic examination he had marked bilateral papilloedema and the blind spots were enlarged bilaterally. His neurological examination was otherwise unremarkable. A magnetic resonance imaging scan (MRI) of the brain showed hydrocephalus. On the precontrast T 2 -weighted images, there was hyperintensity of the meninges with little change after administration of gadolinium, which was suggestive of blood. On the T 2 -weighted sequences, there was evidence of hypo-i intensity on the surface of the pons and medulla, which was indicative of superficial siderosis

  6. A 2-D simulation of hydrocephalus in the Foramens of Monro of the human ventricular system

    International Nuclear Information System (INIS)

    Ammourah, S.; Aroussi, A.; Vloeberghs, M.

    2004-01-01

    This study investigates the Cerebrospinal fluid (CSF) flow behavior in a two-dimensional plane (2-D) of the human ventricular system when the hydrocephalus in the Foramens of Monro (F.O.M) occurs. In order to understand the CSF behaviour when the hydrocephalus occurs, it is essential to comprehend its normal flow dynamics i.e. the healthy case with no hydrocephalus. This had been done numerically by reconstructing the ventricular system geometry from the MRI scans and then made a 15 o degree cut in the surgical line to obtain the outlines of the 2-D plane. The healthy cases were solved for the CSF actual flow rate, which is between 100-500 ml/day in 100 incremental steps. The unhealthy cases were studied for the average flow rate, which is 300 ml/day but for blockage ratios of the Foramen diameter of 20%, 50% and 75%. These obstructions are designed to simulate the hydrocephalus. The results show that as the flow rate increases the pressure and the velocity values increases, but no changes in the flow pattern occurs. The maximum pressure on the normal cases occurred in the lateral ventricles and the maximum velocity occurred in the aqueduct neck. The results of the hydrocephalus cases show that as the obstruction degree increases the pressure in the lateral ventricle increases accompanied with a velocity increase in the obstructed Foramen, which may cause unwanted stress on the neighboring tissues. (author)

  7. Prediction of intracranial hypertension through noninvasive intracranial pressure waveform analysis in pediatric hydrocephalus.

    Science.gov (United States)

    Ballestero, Matheus Fernando Manzolli; Frigieri, Gustavo; Cabella, Brenno Caetano Troca; de Oliveira, Sergio Mascarenhas; de Oliveira, Ricardo Santos

    2017-09-01

    The purpose of this study is to evaluate a noninvasive device to assess intracranial pressure wave form in children with hydrocephalus. A prospective and non-experimental descriptive-analytic study was performed. Fifty-six patients were enrolled in this study. They were divided in four groups: group A, children with clinically compensated hydrocephalus; B, surgically treated hydrocephalus; C, patients with acute intracranial hypertension due to hydrocephalus; and D, children without neurological disease (control). Data were collected through the installation of an extracranial deformation sensor, coupled to the children's scalp, which allowed registration of noninvasive intracranial pressure curves. Parameters obtained were analyzed: P2/P1 ratio, "classification P1 and P2 and P1 slope. P2/P1 index and "classification of P1 and P2" had a sensitivity of 80% and specificity of 100% for predicting intracranial hypertension. "P1 slope" presented no statistical difference. This study showed a useful and noninvasive method for monitoring intracranial pressure, which was able to indicate the intracranial hypertension in children with hydrocephalus and, thus, should be further investigated for clinical applications.

  8. A 2-D simulation of hydrocephalus in the Foramens of Monro of the human ventricular system

    Energy Technology Data Exchange (ETDEWEB)

    Ammourah, S.; Aroussi, A. [Univ. of Nottingham, School of Mechanical, Materials, Manufacturing and Management, University Park, Nottingham (United Kingdom)]. E-mail: eaxsaka@nottingham.ac.uk; Vloeberghs, M. [Queen' s Medical Centre, Dept. of Child Health, Nottingham (United Kingdom)

    2004-07-01

    This study investigates the Cerebrospinal fluid (CSF) flow behavior in a two-dimensional plane (2-D) of the human ventricular system when the hydrocephalus in the Foramens of Monro (F.O.M) occurs. In order to understand the CSF behaviour when the hydrocephalus occurs, it is essential to comprehend its normal flow dynamics i.e. the healthy case with no hydrocephalus. This had been done numerically by reconstructing the ventricular system geometry from the MRI scans and then made a 15{sup o} degree cut in the surgical line to obtain the outlines of the 2-D plane. The healthy cases were solved for the CSF actual flow rate, which is between 100-500 ml/day in 100 incremental steps. The unhealthy cases were studied for the average flow rate, which is 300 ml/day but for blockage ratios of the Foramen diameter of 20%, 50% and 75%. These obstructions are designed to simulate the hydrocephalus. The results show that as the flow rate increases the pressure and the velocity values increases, but no changes in the flow pattern occurs. The maximum pressure on the normal cases occurred in the lateral ventricles and the maximum velocity occurred in the aqueduct neck. The results of the hydrocephalus cases show that as the obstruction degree increases the pressure in the lateral ventricle increases accompanied with a velocity increase in the obstructed Foramen, which may cause unwanted stress on the neighboring tissues. (author)

  9. Surgical management of arrested hydrocephalus: Case report, literature review, and 18-month follow-up.

    Science.gov (United States)

    Hong, Jennifer; Barrena, Benjamin G; Lollis, S Scott; Bauer, David F

    2016-12-01

    Arrested hydrocephalus is stable ventriculomegaly without evidence of neurologic deterioration or symptoms. Management of arrested hydrocephalus in asymptomatic adults is controversial, with little clinical data. This case highlights the potential for decompensation in adults with arrested hydrocephalus and reviews the literature regarding pathophysiology and management of this clinical entity. A 39 year-old gentleman with arrested hydrocephalus incidentally found during work-up for new-onset seizure and managed conservatively for ten years presented with increasing headache, memory loss, gait instability and urinary and fecal incontinence. Stable massive triventriculomegaly was documented on serial brain imaging, and ophthalmologic exam revealed no papilledema. The patient underwent endoscopic third ventriculostomy with immediate post-operative improvement of headache, resolution of incontinence, and cessation of seizures. At 15 months after surgery, neuropsychiatric testing demonstrated improvement in visuomotor skills, problem solving, verbal fluency and cognitive flexibility compared to his pre-operative baseline. At 18 months after surgery he remained seizure free with full continence and significant improvement in headaches. Early recognition of arrested hydrocephalus and its potential for decompensation may prompt surgical treatment and prevent neurologic deterioration. Copyright © 2016 Elsevier B.V. All rights reserved.

  10. A proposed role for efflux transporters in the pathogenesis of hydrocephalus

    Science.gov (United States)

    Krishnamurthy, Satish; Tichenor, Michael D.; Satish, Akhila G.; Lehmann, David B.

    2014-01-01

    Hydrocephalus is a common brain disorder that is treated only with surgery. The basis for surgical treatment rests on the circulation theory. However, clinical and experimental data to substantiate circulation theory have remained inconclusive. In brain tissue and in the ventricles, we see that osmotic gradients drive water diffusion in water-permeable tissue. As the osmolarity of ventricular CSF increases within the cerebral ventricles, water movement into the ventricles increases and causes hydrocephalus. Macromolecular clearance from the ventricles is a mechanism to establish the normal CSF osmolarity, and therefore ventricular volume. Efflux transporters, (p-glycoprotein), are located along the blood brain barrier and play an important role in the clearance of macromolecules (endobiotics and xenobiotics) from the brain to the blood. There is clinical and experimental data to show that macromolecules are cleared out of the brain in normal and hydrocephalic brains. This article summarizes the existing evidence to support the role of efflux transporters in the pathogenesis of hydrocephalus. The location of p-gp along the pathways of macromolecular clearance and the broad substrate specificity of this abundant transporter to a variety of different macromolecules are reviewed. Involvement of p-gp in the transport of amyloid beta in Alzheimer disease and its relation to normal pressure hydrocephalus is reviewed. Finally, individual variability of p-gp expression might explain the variability in the development of hydrocephalus following intraventricular hemorrhage. PMID:25165050

  11. Surgical management of hydrocephalus secondary to intraventricular hemorrhage in the preterm infant.

    Science.gov (United States)

    Christian, Eisha A; Melamed, Edward F; Peck, Edwin; Krieger, Mark D; McComb, J Gordon

    2016-03-01

    OBJECT Posthemorrhagic hydrocephalus (PHH) in the preterm infant remains a major neurological complication of prematurity. The authors first described insertion of a specially designed low-profile subcutaneous ventricular catheter reservoir for temporary management of hydrocephalus in 1983. This report presents the follow-up experience with the surgical management of PHH in this population and describes outcomes both in infants who were stable for permanent shunt insertion and those initially temporized with a ventricular reservoir (VR) prior to permanent ventriculoperitoneal (VP)/ventriculoatrial (VA) shunt placement. METHODS A retrospective review was undertaken of the medical records of all premature infants surgically treated for posthemorrhagic hydrocephalus (PHH) between 1997 and 2012 at Children's Hospital Los Angeles. RESULTS Over 14 years, 91 preterm infants with PHH were identified. Fifty neonates received temporizing measures via a VR that was serially tapped for varying time periods. For the remaining 41 premature infants, VP/VA shunt placement was the first procedure. Patients with a temporizing measure as their initial procedure had undergone CSF diversion significantly earlier in life than those who had permanent shunting as the initial procedure (29 vs 56 days after birth, p hydrocephalus and shunt infection did not statistically differ between the 2 groups. CONCLUSIONS Patients with initial VR insertion as a temporizing measure received a CSF diversion procedure significantly earlier than those who received a permanent shunt as their initial procedure. Otherwise, the outcomes with regard to shunt revisions, loculated hydrocephalus, and shunt infection were not different for the 2 groups.

  12. Changes in Rat Brain Tissue Microstructure and Stiffness during the Development of Experimental Obstructive Hydrocephalus

    Science.gov (United States)

    Jugé, Lauriane; Pong, Alice C.; Bongers, Andre; Sinkus, Ralph; Bilston, Lynne E.; Cheng, Shaokoon

    2016-01-01

    Understanding neural injury in hydrocephalus and how the brain changes during the course of the disease in-vivo remain unclear. This study describes brain deformation, microstructural and mechanical properties changes during obstructive hydrocephalus development in a rat model using multimodal magnetic resonance (MR) imaging. Hydrocephalus was induced in eight Sprague-Dawley rats (4 weeks old) by injecting a kaolin suspension into the cisterna magna. Six sham-injected rats were used as controls. MR imaging (9.4T, Bruker) was performed 1 day before, and at 3, 7 and 16 days post injection. T2-weighted MR images were collected to quantify brain deformation. MR elastography was used to measure brain stiffness, and diffusion tensor imaging (DTI) was conducted to observe brain tissue microstructure. Results showed that the enlargement of the ventricular system was associated with a decrease in the cortical gray matter thickness and caudate-putamen cross-sectional area (P hydrocephalus development, increased space between the white matter tracts was observed in the CC+PVWM (P hydrocephalus development. PMID:26848844

  13. Spontaneous third ventriculostomy 8 years after diagnosis of obstructive hydrocephalus.

    Science.gov (United States)

    Öğrenci, Ahmet; Ekşi, Murat Şakir; Koban, Orkun

    2016-09-01

    Spontaneous ventriculostomy is spontaneous rupture of membranes separating the ventricular system from the subarachnoid space in patients with chronic obstructive hydrocephalus that ends with resolution of symptoms. We present a case of spontaneous third ventriculostomy occurred in a 19-year-old girl 8 years after the initial diagnosis of hydrocephalus. An 11-year-old girl applied to the clinic with intermittent headaches. She was neurologically stable with no visual problems. On her brain MRI, obstructive hydrocephalus was observed. Cerebrospinal fluid diversion procedures were recommended, yet the family denied any interventional procedure. She had routine follow-ups with occasional clinical admissions because of ongoing intermittent headaches. On her last clinical visit, 8 years after the first one, she was in well condition with improvement in her headache in the last 4 months. Her new brain MRI showed an active CSF flow between the basal cistern and the third ventricle. In patients with aqueductal stenosis and without any other mass lesion, wait and see protocol might be conveyed in case of mild symptoms of hydrocephalus. However, there is need for large-scaled studies to make a more comprehensive statement for benign obstructive hydrocephalus cases.

  14. Ventriculoperitoneal (VP) Shunt Survival in Patients Developing Hydrocephalus After Cranial Surgery.

    Science.gov (United States)

    Khan, Farid; Rehman, Abdul; Shamim, Muhammad Shahzad; Bari, Muhammad Ehsan

    Ventriculoperitoneal (VP) shunt insertion remains the most popular method for the treatment of hydrocephalus despite its associated complications. We assessed VP shunt survival in a group of patients who had developed hydrocephalus following cranial surgery. A retrospective charts review was done over a 10-year period at our institution. Kaplan-Meier survival curves and Log-Rank (Cox-Mantel) test were used to analyze various factors affecting VP shunt survival. Among the 67 cases included, a total of 28 (46.3%) patients had undergone cranial surgery for brain tumors. The overall rate of shunt failure was 14.9% at a mean follow-up of 16 months. Shunt failure in pediatric patients (20%) was slightly higher than that in adult patients (13.5%). The median time to first shunt failure was adversely influenced by a history of brain tumor (p = 0.019), prolonged antibiotic therapy (p = 0.018) and administration of steroids (p = 0.004). Shunt survival was worse in patients who developed hydrocephalus following cranial surgery performed for brain tumors and those who received either steroids or prolonged antibiotic therapy. Thus post-cranial surgery hydrocephalus represents a unique subset of hydrocephalus.

  15. Contemporary management and surveillance strategy after shunt or endoscopic third ventriculostomy procedures for hydrocephalus.

    Science.gov (United States)

    Janjua, M Burhan; Hoffman, Caitlin E; Souweidane, Mark M

    2017-11-01

    The management of hydrocephalus can be challenging even in expert hands. Due to acute presentation, recurrence, accompanying complications, the need for urgent diagnosis; a robust management plan is an absolute necessity. We devised a novel time efficient surveillance strategy during emergency, and clinic follow up settings which has never been described in the literature. We searched all articles embracing management/surveillance protocol on pediatric hydrocephalus utilizing the terms "hydrocephalus follow up" or "surveillance protocol after hydrocephalus treatment". The authors present their own strategy based on vast experience in the hydrocephalus management at a single institution. The need for the diagnostic laboratory testing, age and presentation based radiological imaging, significance of neuro-opthalmological exam, and when to consider the emergent exploration have been discussed in detail. Moreover, a definitive triaging strategy has been described with the help of flow chart diagrams for clinicians, and the neurosurgeons in practice. The triage starts from detail history, physical exam, necessary labs, radiological imaging depending on the presentation, and the age of the child. A quick head CT scan helps after shunt surgery while, a FAST sequence MRI scan (fsMRI) is important in post ETV patients. The need for neuro-opthalmological exam, and the shunt series stays vital in asymptomatic patients during regular follow up. Copyright © 2017 Elsevier Ltd. All rights reserved.

  16. Unilateral Enlarged Vestibular Aqueduct Syndrome and Bilateral Endolymphatic Hydrops

    Directory of Open Access Journals (Sweden)

    Massimo Ralli

    2017-01-01

    Full Text Available Enlarged vestibular aqueduct (EVA syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH. EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH.

  17. Unilateral Enlarged Vestibular Aqueduct Syndrome and Bilateral Endolymphatic Hydrops.

    Science.gov (United States)

    Ralli, Massimo; Nola, Giuseppe; Sparvoli, Luca; Ralli, Giovanni

    2017-01-01

    Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH.

  18. Flow-regulated versus differential pressure-regulated shunt valves for adult patients with normal pressure hydrocephalus

    DEFF Research Database (Denmark)

    Ziebell, Morten; Wetterslev, Jørn; Tisell, Magnus

    2013-01-01

    Since 1965 many ventriculo-peritoneal shunt systems have been inserted worldwide to treat hydrocephalus. The most frequent indication in adults is normal pressure hydrocephalus (NPH), a condition that can be difficult to diagnose precisely. Surgical intervention with flow-regulated and differential...

  19. The congenital cranial dysinnervation disorders.

    Science.gov (United States)

    Gutowski, N J; Chilton, J K

    2015-07-01

    Congenital cranial dysinnervation disorders (CCDD) encompass a number of related conditions and includes Duane syndrome, congenital fibrosis of the external ocular muscles, Möbius syndrome, congenital ptosis and hereditary congenital facial paresis. These are congenital disorders where the primary findings are non-progressive and are caused by developmental abnormalities of cranial nerves/nuclei with primary or secondary dysinnervation. Several CCDD genes have been found, which enhance our understanding of the mechanisms involved in brain stem development and axonal guidance. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  20. EAMJ Congenital.indd

    African Journals Online (AJOL)

    2010-02-02

    Feb 2, 2010 ... Consultative Hemostasis and Thrombosis. Kitchens. S.C., Alving B.M, Kessler C.M. 2nd Edition, 2007,. Saundres Elsevier, Philadelphia, PA, USA. 2. International Registry of Rare Bleeding Disorders website: www.rbdd.org Last accessed on 29th. November 2009. 3. Menache, D. Congenital fibrinogen ...

  1. Congenital syphilis who risk?

    African Journals Online (AJOL)

    1989-08-05

    Aug 5, 1989 ... The prevalence of syphilis (or positive serology) in pregnant mothers delivering at ... with penicillin. The treponemes are as sensitive to penicillin today as they were when this therapy was introduced by. Mahney and Harris in 1934.1 Although congenital syphilis is a potentially preventable disease, it was ...

  2. Pseudoamblyopia in Congenital Cyclotropia

    Directory of Open Access Journals (Sweden)

    Antonio Frattolillo

    2017-01-01

    Full Text Available Purpose. To study the effect of surgery on amblyopia and suppression associated with congenital cyclovertical strabismus. Methods. The fixation pattern was investigated with microperimetry before and soon after surgery in ten consecutive children operated for congenital superior oblique palsy at the S. Martino Hospital, Belluno, Italy, between September 2014 and December 2015. Changes in visual performance in terms of best-corrected visual acuity (BCVA and stereopsis between the day before and one week after surgery were also evaluated. No other amblyopia treatment has been administered during the time study. Results. Surgical correction of the excyclodeviation in congenital SO palsy determined monocular and binocular sensory consequences: monocularly, in the cyclodeviated amblyopic eye, BCVA (0.46–0.03 LogMAR; p<0.0001 and the fixation pattern improved, as demonstrated by microperimetry examination. Binocularly, stereopsis improved or emerged while suppression at the Worth four-dot test disappeared. Conclusions. In the absence of further amblyopic factors such as coexisting constant vertical and/or horizontal deviation and anisometropia, the amblyopia encountered in congenital SO palsy may resolve soon after the surgical alignment. Therefore, it may be considered and defined “pseudoamblyopia.”

  3. EAMJ March -Congenital

    African Journals Online (AJOL)

    iMac User

    convulsions, cerebral cortical dysgenetic changes, bronchiectasis and chronic sinusitis is presented. She had features of both yellow nail syndrome and Hennekam syndrome. We think that our case might be a new congenital lymphoedema syndrome or an intermediate form between these syndromes. INTRODUCTION.

  4. Congenital extracranial meningioma

    International Nuclear Information System (INIS)

    Wong, H.F.; Ng, S.H.; Wai, Y.Y.; Wan, Y.L.; Kong, M.S.

    1995-01-01

    The authors report a case of congenital meningioma in a newborn. This tumour is extremely rare and only six cases have been reported in the literature. Those reported cases were mainly intracranial. This is the first case of a neonatal extracranial meningioma that was evaluated preoperatively by computed tomography and magnetic resonance imaging. (orig.)

  5. Congenital cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Chaudhry, A.K.; Azam, M.; Maqsood, R.; Naz, B.; Salam, A.

    2003-01-01

    This case report presents the clinical picture, diagnostic methodology and surgical treatment of a female child who presented with chronic cough and dyspnoea due to congenital malformation of lung. A discussion of diagnosis and management is presented at the end. (author)

  6. Congenital cystic adenomatoid malformation

    Directory of Open Access Journals (Sweden)

    Kamal M. Alshamiri

    2017-12-01

    Full Text Available Congenital pulmonary airway malformation of the lung is a rare lesion that commonly affects the lower respiratory tract, which manifests as progressive respiratory distress in the neonatal period. This case is highlighted to raise awareness of the variable evolving morphological and radiological appearance of this entity.

  7. EAMJ March -Congenital

    African Journals Online (AJOL)

    iMac User

    SUMMARY. A l0-year-old girl with facial anomalies, mental retardation, peripheral lymphoedema, convulsions, cerebral cortical dysgenetic changes, bronchiectasis and chronic sinusitis is presented. She had features of both yellow nail syndrome and Hennekam syndrome. We think that our case might be a new congenital ...

  8. The Nature of Hyperactivity in Children and Adolescents With Hydrocephalus: A Test of the Dual Pathway Model

    Science.gov (United States)

    Stevenson, Jim; Cate, Ineke Pit-ten

    2004-01-01

    To determine the strength and nature of the association between hydrocephalus and hyperactivity and to test the dual pathway model (DPM) of AD/HD, we compared a group of 51 children and adolescents with hydrocephalus with 57 normally developing controls from the general population on a battery of neuropsychological assessments. The mean hyperactivity scores were significantly greater in the group with hydrocephalus (effect size =0.94). This association was not just part of a general elevated rate of behavior problems and was not affected by sex or age. Variation in the clinical features of hydrocephalus was not related to the severity of hyperactivity. Path analysis was used to examine the relation between IQ, delay aversion, and executive function. In accordance with the DPM, the effect of hydrocephalus on hyperactivity was completely mediated via delay aversion and executive functions. PMID:15303302

  9. Neural stem cells: are they the hope of a better life for patients with fetal-onset hydrocephalus?

    Science.gov (United States)

    Guerra, Montserrat

    2014-01-01

    I was honored to be awarded the Casey Holter Essay Prize in 2013 by the Society for Research into Hydrocephalus and Spina Bifida. The purpose of the prize is to encourage original thinking in a way to improve the care of individuals with spina bifida and hydrocephalus. Having kept this purpose in mind, I have chosen the title: Neural stem cells, are they the hope of a better life for patients with fetal-onset hydrocephalus? The aim is to review and discuss some of the most recent and relevant findings regarding mechanisms leading to both hydrocephalus and abnormal neuro/gliogenesis. By looking at these outcome studies, it is hoped that we will recognize the potential use of neural stem cells in the treatment of hydrocephalus, and so prevent the disease or diminish/repair the associated brain damage.

  10. Congenitally corrected transposition

    Directory of Open Access Journals (Sweden)

    Debich-Spicer Diane

    2011-05-01

    Full Text Available Abstract Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has been reported to be around 1/33,000 live births, accounting for approximately 0.05% of congenital heart malformations. Associated malformations may include interventricular communications, obstructions of the outlet from the morphologically left ventricle, and anomalies of the tricuspid valve. The clinical picture and age of onset depend on the associated malformations, with bradycardia, a single loud second heart sound and a heart murmur being the most common manifestations. In the rare cases where there are no associated malformations, congenitally corrected transposition can lead to progressive atrioventricular valvar regurgitation and failure of the systemic ventricle. The diagnosis can also be made late in life when the patient presents with complete heart block or cardiac failure. The etiology of congenitally corrected transposition is currently unknown, and with an increase in incidence among families with previous cases of congenitally corrected transposition reported. Diagnosis can be made by fetal echocardiography, but is more commonly made postnatally with a combination of clinical signs and echocardiography. The anatomical delineation can be further assessed by magnetic resonance imaging and catheterization. The differential diagnosis is centred on the assessing if the patient is presenting with isolated malformations, or as part of a spectrum. Surgical management consists of repair of the associated malformations, or redirection of the systemic and pulmonary venous return associated with an arterial switch procedure, the so-called double switch approach. Prognosis is defined by the associated malformations, and on the timing and approach to palliative surgical care.

  11. Increased self-diffusion of brain water in hydrocephalus measured by MR imaging

    DEFF Research Database (Denmark)

    Gideon, P; Thomsen, C; Gjerris, F

    1994-01-01

    We used MR imaging to measure the apparent brain water self-diffusion in 5 patients with normal pressure hydrocephalus (NPH), in 2 patients with high pressure hydrocephalus (HPH), and in 8 age-matched controls. In all patients with NPH significant elevations of the apparent diffusion coefficients...... (ADC) of brain water were found within periventricular white matter, in the corpus callosum, in the internal capsule, within cortical gray matter, and in cerebrospinal fluid, whereas normal ADCs were found within the basal ganglia. In 2 patients with HPH elevated ADCs were found most prominently within...... white matter, and in one patient reexamined one year after surgery, ADCs were unchanged in nearly all brain regions. The increased ADC values in hydrocephalus patients may be caused by factors such as changes in myelin-associated bound water, increased Virchow-Robin spaces, and increased extracellular...

  12. NMR images of non-communicating hydrocephalus associated with Dandy-Walker variant and achondroplasia

    Energy Technology Data Exchange (ETDEWEB)

    Sato, Masaharu; Kuroda, Ryotaro; Watanabe, Masaru; Nakatani, Jiro; Ioku, Masahiko; Irisawa, Minoru; Hamada, Tatsumi; Ishida, Osamu

    1988-06-01

    Two cases of non-communicating hydrocephalus caused by a relatively rare etiology were reported. They were both diagnosed by means of nuclear magnetic resonance (NMR). The first patient, a 4-month-old boy, had Dandy-Walker variant, showing hypoplasia of the corpus callosum and the inferior vermis, and a large cyst behind the vermis, communicating with the subarachnoid space, as well as hydrocephalus; the fourth ventricle was partially reserved. The second patient, also a 4-month-old boy, had achondroplasia, resulting in a narrow foramen magnum; the disturbance of the outflow of the cerebro-spinal fluid around the cisterna magna was thought to be the cause of hydrocephalus in his case. The validity of NMR was demonstrated in the diagnoses of these conditions, for a high resolution was needed in examining the complicated structure of the posterior fossa.

  13. Endoscopic Removal of a Bullet That Migrated to the Third Ventricle Causing Hydrocephalus.

    Science.gov (United States)

    Aydoseli, Aydın; Unal, Tugrul Cem; Aras, Yavuz; Sabanci, Pulat Akın; Altunrende, Emre; Izgi, Nail

    2017-09-01

    Hydrocephalus caused by an intraventricular bullet is a rare event. We report a case of endoscopic removal of an intraventricular bullet. A 66-year-old man was admitted with a gunshot wound to the head after a suicide attempt. The bullet migrated from the frontal parenchyma to the third ventricle day 4 of admission. On day 21 of admission, the patient developed hydrocephalus with obstruction of the cerebral aqueduct. The bullet was accessed through an endoscopic third ventriculostomy and removed using an endoscope. Hydrocephalus may develop in patients with intraventricular foreign objects. When such objects must be removed, the endoscopic approach is a safe, efficient, and minimally invasive procedure. To our knowledge, this is the first case in the literature of foreign object removal from the ventricle via a transcortical endoscopic approach. Copyright © 2017 Elsevier Inc. All rights reserved.

  14. Subdural haematoma complicating shunting for normal pressure hydrocephalus in the setting of concomitant antiplatelet medication

    DEFF Research Database (Denmark)

    Birkeland, Peter; Lauritsen, Jens; Poulsen, Frantz Rom

    2016-01-01

    OBJECTIVE: To report on the occurrence and management of subdural haematoma after shunt implantation for normal pressure hydrocephalus and to determine the risk of recurrence in the setting of antiplatelet medication. METHODS: From a consecutive series of 80 patients implanted with a cerebrospinal...... fluid shunt for normal pressure hydrocephalus, records from 11 patients taking antiplatelet drugs, who subsequently had surgery for subdural haematoma were extracted and retrospectively reviewed. RESULTS: Patients were followed up for a mean of 1819 days after shunt implantation. Subdural haematomas...... reoperations done before the subdural collection disappeared. Only one patient had a late recurrence almost 11 years after shunt implantation. CONCLUSIONS: Subdural haematoma in the setting of a ventriculoperitoneal implantation for normal pressure hydrocephalus and concomitant antiplatelet medication can...

  15. A Mechanics-Based Framework Leading to Improved Diagnosis and Treatment of Hydrocephalus

    Science.gov (United States)

    Cohen, Benjamin; Soren, Vedels; Wagshul, Mark; Egnor, Michael; Voorhees, Abram; Wei, Timothy

    2007-11-01

    Hydrocephalus is defined as an accumulation of cerebrospinal fluid (CSF) in the cranium, at the expense of brain tissue. The result is a disruption of the normal pressure and/or flow dynamics of the intracranial blood and CSF. We seek to introduce integral control volume analysis to the study of hydrocephalus. The goal is to provide a first principles framework to integrate a broad spectrum of sometimes disparate investigations into a highly complex, multidisciplinary problem. The general technique for the implementation of control volumes to hydrocephalus will be presented. This includes factors faced in choosing control volumes and making the required measurements to evaluate mass and momentum conservation. In addition, the use of our Digital Particle Image Velocimetry (DPIV) processing program has been extended to measure the displacement of the ventricles' walls from Magnetic Resonance (MR) images. This is done to determine the volume change of the intracranial fluid spaces.

  16. Type III occipital condylar fracture presenting with hydrocephalus, vertebral artery injury and vasospasm: case report

    International Nuclear Information System (INIS)

    Menendez, J.A.; Baskaya, M.K.; Day, M.A.; Nanda, A.

    2001-01-01

    Occipital condylar fractures (OCF) are rare and have a high mortality rate. We report a patient with OCF who presented with acute hydrocephalus and died from diffuse vasospasm secondary to vertebral artery injury. A 45-year-old man fell 20 feet from a deer stand and landed on his head. CT showed a type III OCF continuing to the anterior rim of the foramen magnum on the left, with a bone fragment pushing into the medulla, causing hydrocephalus. The patient was stabilized, and a four-vessel arteriogram showed diffuse vasospasm with complete occlusion of the left vertebral artery at the level of the OCF. To our knowledge, this is the first documented case of the conjunction of OCF, hydrocephalus, and vasospasm. (orig.)

  17. Unilateral intracarotid amobarbital procedure for language lateralization.

    Science.gov (United States)

    Wellmer, Jörg; Fernández, Guillen; Linke, Detlef B; Urbach, Horst; Elger, Christian E; Kurthen, Martin

    2005-11-01

    The determination of language dominance as part of the presurgical workup of patients with pharmacoresistant epilepsies has experienced fundamental changes. With the introduction of noninvasive functional magnetic resonance imaging (fMRI), the number of patients receiving intracarotid amobarbital procedures (IAPs) for assessment of language dominance has decreased considerably. However, recent studies show that because of methodologic limitations of fMRI, IAP remains an important tool for language lateralization. The current study examines whether unilateral instead of bilateral IAP is an adequate way to apply IAP with reduced invasiveness. We retrospectively examine the predictive value of unilateral IAP for the results of bilateral IAP based on a sample of 75 patients with various types of language dominance. Target parameters are the prediction of the language-dominant hemisphere and the identification of patients with atypical language dominance. For language assessment based on unilateral IAP, we introduce the measure hemispheric language capacity (HLC). Unilateral IAP performed on the side of intended surgery quantifies language capacity contralateral to the intended surgery. It detects atypical (bilateral or right) language dominance in the majority of patients. Experience with a separate series of 107 patients requiring presurgical language lateralization shows that in >80%, bilateral IAPs are redundant. Unilateral IAP is principally sufficient for language lateralization in the presurgical evaluation of patients with pharmacoresistant epilepsies. Necessity of bilateral IAP is restricted to few indications (e.g., callosotomy). In times of noninvasive language lateralization, we propose unilateral IAP as the method of choice for the verification of doubtful (bilateral) fMRI activation patterns.

  18. Ventricular-subcutaneous shunt for the treatment of experimental hydrocephalus in young rats: technical note.

    Science.gov (United States)

    Santos, Marcelo Volpon; Garcia, Camila Araujo Bernardino; Jardini, Evelise Oliveira; Romeiro, Thais Helena; da Silva Lopes, Luiza; Machado, Hélio Rubens; de Oliveira, Ricardo Santos

    2016-08-01

    Hydrocephalus is a complex disease that affects cerebrospinal fluid (CSF) dynamics and is very common in children. To this date, CSF shunting is still the standard treatment for childhood hydrocephalus, but, nevertheless, the effects of such an operation on the developing brain are widely unknown. To help overcome this, experimental models of CSF shunts are surely very useful tools. The objective of this study was to describe a feasible and reliable technique of an adapted ventricular-subcutaneous shunt for the treatment of kaolin-induced hydrocephalus in young rats. We developed a ventricular-subcutaneous shunt (VSCS) technique which was used in 31 Wistar young rats with kaolin-induced hydrocephalus. Hydrocephalus was induced at 7 days of age, and shunt implantation was performed 7 days later. Our technique used a 0.7-mm gauge polypropylene catheter tunneled to a subcutaneous pocket created over the animal's back and inserted into the right lateral ventricle. All animals were sacrificed 14 days after shunt insertion. Twenty-four rats survived and remained well until the study was ended. No major complications were seen. Their weight gain went back to normal. They all underwent ambulatory behavioral testing prior and after VSCS, which showed improvement in their motor skills. We have also obtained magnetic resonance (MR) scans of 16 pups confirming reduction of ventricular size after shunting and indicating effective treatment. Histopathological analysis of brain samples before and after shunting showed reversion of ependymal and corpus callosum disruption, as well as fewer reactive astrocytes in shunted animals. An experimental CSF shunt technique was devised. Excessive CSF of hydrocephalic rats is diverted into the subcutaneous space where it can be resorbed. This technique has a low complication rate and is effective. It might be applied to various types of experimental studies involving induction and treatment of hydrocephalus.

  19. [Constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss].

    Science.gov (United States)

    Li, Youjin; Yang, Jun; Li, Yun

    2011-01-01

    To analyze the constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss(SNHL). Imaging and auditory data of 125 cases (225 ears) in 860 pediatric patients with congenital SNHL who referred to ENT department of Shanghai Children Medical Center from February 2005 to January 2010 were retrospectively studied. Congenital malformations of inner ear accounted for 14.5% in 860 pediatric patients with congenital SNHL. Bilateral ear was involved in 98 cases (78.4%), unilateral in 27 cases (21.6%). One hundred and sixty-seven ears (74.2%) were identified as profound deafness, 36 ears (16%) severe deafness and 22 ears (9.8%) moderate deafness in 225 ears, respectively. In present group, large vestibular aqueduct (75.6%) was the most common and next was vestibular deformity (32%), then cochleovestibular deformity (23.1%). Mondini deformity (55.8%) was the most common and common cavity deformity (28.9%) next in cochleovestibular deformity. Profound deafness in cochlea involved congenital malformations of inner ear was significantly more than those in no cochlea involved malformations. The results are of importance for understanding the constitute of congenital malformations of inner ear in pediatric patients with congenital SNHL in China, for etiological diagnosis of congenital SNHL, for intervention including hearing aids or cochlear implant and prognosis.

  20. Frequency of Congenital Anomalies in Newborns and Its Relation to Maternal Health in a Tertiary Care Hospital in Peshawar, Pakistan

    Directory of Open Access Journals (Sweden)

    Adnan Khan

    2015-03-01

    Full Text Available Background: Congenital anomalies are a major cause of perinatal and neonatal deaths, both in low- and high-income countries. They are relatively common worldwide, affecting 3% to 5% of live births. Methods: A cross-sectional study was conducted from January 2014 to June 2014 at the Khyber teaching hospital in Peshawar. Specific patient information was obtained from patient records at the beginning of the study. Those individuals found to have at least one birth defect were approached and their attendants (mothers were interviewed. Information regarding various risk factors was collected. Descriptive analyses were carried out. Results: Out of 1062 deliveries, 2.9% (31 of newborns had various congenital anomalies. Hydrocephalus (22.6%, anencephaly (12.9%, and spina bifida (9.7% were major anomalies. The maternal age ranged from 18 years to 46 years (mean: 30 ± 8. Most of the anomalies (35.5% were present in the 26-30 years age group. Out of 31 babies, 6.4% had multiple anomalies. The preponderance of various congenital anomalies was seen in parity 1 (35.4%; parities 2 to 4 had lower incidences (35.4%. The consanguinity rate was 67.7%; only 32.3% of patients were using folic acid. History of passive smoking was positive in 16.1% of cases. Conclusion: Anencephaly and hydrocephalus were the most prominent anomaly detected; early prenatal diagnosis may be helpful in decreasing mortality by offering early termination. Low intake of folic acid and a high consanguinity rate were the most common associated risk factors for congenital anomalies. These risk factors may be reduced by creating awareness regarding the avoidance of consanguineous marriage and promoting the use of folic acid during pregnancy.

  1. Diffusion tensor imaging with direct cytopathological validation: characterisation of decorin treatment in experimental juvenile communicating hydrocephalus.

    Science.gov (United States)

    Aojula, Anuriti; Botfield, Hannah; McAllister, James Patterson; Gonzalez, Ana Maria; Abdullah, Osama; Logan, Ann; Sinclair, Alexandra

    2016-05-31

    In an effort to develop novel treatments for communicating hydrocephalus, we have shown previously that the transforming growth factor-β antagonist, decorin, inhibits subarachnoid fibrosis mediated ventriculomegaly; however decorin's ability to prevent cerebral cytopathology in communicating hydrocephalus has not been fully examined. Furthermore, the capacity for diffusion tensor imaging to act as a proxy measure of cerebral pathology in multiple sclerosis and spinal cord injury has recently been demonstrated. However, the use of diffusion tensor imaging to investigate cytopathological changes in communicating hydrocephalus is yet to occur. Hence, this study aimed to determine whether decorin treatment influences alterations in diffusion tensor imaging parameters and cytopathology in experimental communicating hydrocephalus. Moreover, the study also explored whether diffusion tensor imaging parameters correlate with cellular pathology in communicating hydrocephalus. Accordingly, communicating hydrocephalus was induced by injecting kaolin into the basal cisterns in 3-week old rats followed immediately by 14 days of continuous intraventricular delivery of either human recombinant decorin (n = 5) or vehicle (n = 6). Four rats remained as intact controls and a further four rats served as kaolin only controls. At 14-days post-kaolin, just prior to sacrifice, routine magnetic resonance imaging and magnetic resonance diffusion tensor imaging was conducted and the mean diffusivity, fractional anisotropy, radial and axial diffusivity of seven cerebral regions were assessed by voxel-based analysis in the corpus callosum, periventricular white matter, caudal internal capsule, CA1 hippocampus, and outer and inner parietal cortex. Myelin integrity, gliosis and aquaporin-4 levels were evaluated by post-mortem immunohistochemistry in the CA3 hippocampus and in the caudal brain of the same cerebral structures analysed by diffusion tensor imaging. Decorin significantly

  2. Infantile holocord cellular ependymoma with communicating hydrocephalus: unusual presentation of a rare case.

    Science.gov (United States)

    Aryan, Saritha; Ghosal, Nandita; Aziz, Zarina A; Hegde, A S; Dadlani, Ravi

    2014-01-01

    We present a case of infantile holocord ependymoma in a 4-month-old boy who presented with infection of ventriculoperitoneal shunt done elsewhere for a communicating hydrocephalus. On magnetic resonance imaging, a diffuse holocord T2-hyperintense, T1-hypointense intramedullary bulky lesion with syringomyelia in the cervical level was seen. To the best of our knowledge, this is the first case of infantile holocord ependymoma. As the extent of morbidity associated with a spinal cord tumor is high, an increased level of suspicion and the need for a complete spinal cord screening in a case of infantile hydrocephalus without obvious clinical and radiological evidence of intracranial abnormality is emphasized.

  3. Numerical simulation of cerebrospinal fluid hydrodynamics in the healing process of hydrocephalus patients

    Science.gov (United States)

    Gholampour, S.; Fatouraee, N.; Seddighi, A. S.; Seddighi, A.

    2017-05-01

    Three-dimensional computational models of the cerebrospinal fluid (CSF) flow and brain tissue are presented for evaluation of their hydrodynamic conditions before and after shunting for seven patients with non-communicating hydrocephalus. One healthy subject is also modeled to compare deviated patients data to normal conditions. The fluid-solid interaction simulation shows the CSF mean pressure and pressure amplitude (the superior index for evaluation of non-communicating hydrocephalus) in patients at a greater point than those in the healthy subject by 5.3 and 2 times, respectively.

  4. Hydrocephalus is a rare outcome in community-acquired bacterial meningitis in adults

    DEFF Research Database (Denmark)

    Bodilsen, Jacob; Schønheyder, Henrik Carl; Nielsen, Henrik I

    2013-01-01

    BACKGROUND: Community-acquired bacterial meningitis (CABM) continues to have a high mortality rate and often results in severe sequelae among survivors. Lately, an increased effort has been focused on describing the neurological complications of meningitis including hydrocephalus. To aid...... in this field of research we set out to ascertain the risk and outcome of hydrocephalus in patients with community-acquired bacterial meningitis (CABM) in North Denmark Region. METHODS: We conducted a retrospective population-based cohort study of CABM cases above 14 years of age. Cases diagnosed during a 13...

  5. Opinions of Youngsters with Congenital Below-Elbow Deficiency, and Those of Their Parents and Professionals Concerning Prosthetic Use and Rehabilitation Treatment

    NARCIS (Netherlands)

    E. Vasluian (Ecaterina); I.G.M. de Jong (Ingrid); W.G.M. Janssen (Wim); M.J. Poelma (Margriet); I. van Wijk (Iris); H.A. Reinders-Messelink (Heleen); C.K. van der Sluis (Corry)

    2013-01-01

    textabstractBackground:Youngsters with unilateral congenital below-elbow deficiency (UCBED) seem to function well with or without a prosthesis. Reasons for rejecting prostheses have been reported earlier, but unfortunately not those of the children themselves. Furthermore, reasons for acceptance are

  6. Congenital CMV infection: prevalence in newborns and the impact on hearing deficit.

    Science.gov (United States)

    Engman, Mona-Lisa; Malm, Gunilla; Engstrom, Lotta; Petersson, Karin; Karltorp, Eva; Tear Fahnehjelm, Kristina; Uhlen, Inger; Guthenberg, Claes; Lewensohn-Fuchs, Ilona

    2008-01-01

    Congenital cytomegalovirus (CMV) infection is asymptomatic in 90% of infected newborns but approximately 10-20% of these infants are at risk of developing sequelae later, mostly hearing deficit. The aims of the study were to investigate the prevalence of congenital CMV infection in a Swedish population of newborns and investigate the relative risk of hearing deficit in newborns with congenital CMV infection. The dried blood spot (DBS) samples of 6060 newborns in southern Stockholm during 12 months (October 2003-June 2004; August 2004-October 2004) were analysed for CMV DNA by TaqMan based real-time PCR. Hearing deficit was assessed by otoacoustic emission (OAE) within a newborn screening programme. 12 infants out of 6060 or 0.2% (95% CI 0.1-0.3%) had congenital CMV infection. One boy among the 12 infected infants had unilateral hearing loss, indicating that the risk of hearing loss is greatly increased (about 20 times) in CMV infected infants. No child developed ocular complications such as chorioretinopathy during 3 y of follow-up. Congenital CMV has an impact on child health but can easily be overlooked due to lack of signs in the neonatal period. Surveillance for congenital CMV is important in addition to programmes for prevention and treatment.

  7. Congenital diaphramatic hernia

    International Nuclear Information System (INIS)

    Kline-Fath, Beth M.

    2012-01-01

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  8. Congenital diaphramatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    Kline-Fath, Beth M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Fetal Care Center of Cincinnati, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States)

    2012-01-15

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  9. Unilateral nodular adrenal hyperplasia: Case series

    African Journals Online (AJOL)

    A.F. Kotb

    2016-07-26

    Jul 26, 2016 ... Abstract. Introduction: Nodular adrenal hyperplasia is one of rare causes of adrenocortical hyperplasia. The disease usually presents bilaterally. Few publications discussed the possibility of unilateral disease, in association with hyperaldosteronism or Cushing syndrome. Case series: We are reporting 3 ...

  10. Unilateral vestibular loss impairs external space representation.

    Directory of Open Access Journals (Sweden)

    Liliane Borel

    Full Text Available The vestibular system is responsible for a wide range of postural and oculomotor functions and maintains an internal, updated representation of the position and movement of the head in space. In this study, we assessed whether unilateral vestibular loss affects external space representation. Patients with Menière's disease and healthy participants were instructed to point to memorized targets in near (peripersonal and far (extrapersonal spaces in the absence or presence of a visual background. These individuals were also required to estimate their body pointing direction. Menière's disease patients were tested before unilateral vestibular neurotomy and during the recovery period (one week and one month after the operation, and healthy participants were tested at similar times. Unilateral vestibular loss impaired the representation of both the external space and the body pointing direction: in the dark, the configuration of perceived targets was shifted toward the lesioned side and compressed toward the contralesioned hemifield, with higher pointing error in the near space. Performance varied according to the time elapsed after neurotomy: deficits were stronger during the early stages, while gradual compensation occurred subsequently. These findings provide the first demonstration of the critical role of vestibular signals in the representation of external space and of body pointing direction in the early stages after unilateral vestibular loss.

  11. CASE REPORT Unilateral axillary adenopathy with unremarkable ...

    African Journals Online (AJOL)

    of bursitis. As a local inflammatory cause for the unilateral axillary adenopathy was presumed, no biopsy was performed. Follow-up ultrasound was arranged for 4 weeks later. Diagnosis. After discussion with the patient's family physician, she attended for consultation the same day. A clinical diagnosis of elbow bursitis was.

  12. The ecological assessment of unilateral neglect.

    Science.gov (United States)

    Azouvi, Philippe

    2017-06-01

    Conventional paper-and-pencil tests of unilateral neglect are of limited ecological validity. To address this issue, a number of assessment procedures have been proposed to provide clinicians and researchers with more ecologically valid assessments of unilateral neglect, which may be useful to plan rehabilitation and to measure the generalization of the effects of rehabilitation to daily life. We present here an overview of the different assessment measures available in the literature. The most widely used scales are the Behavioural Inattention Test (BIT), the semi-structured scales for assessment of personal and extra-personal neglect, the Subjective Neglect Questionnaire, the Baking Tray Task, the wheelchair obstacle course, the ADL-based neglect battery, and the Catherine Bergego Scale (CBS). The CBS is probably, to date, the most widely used behavioural assessment instrument for unilateral neglect. It has been found to be reliable, valid, and sensitive to change during rehabilitation. It also enables the assessment of awareness of the consequences of unilateral neglect in daily life skills. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  13. Unilateral Sympathectomy for Primary Palmar Hyperhidrosis.

    Science.gov (United States)

    Ravari, Hassan; Rajabnejad, Ataollah

    2015-12-01

    Primary palmar hyperhidrosis that arises mostly during puberty and early adolescence has a tremendous impact on the quality of life in patients. This study aimed to investigate the effectiveness of unilateral video-assisted thoracic sympathectomy for dominant hand in these patients. From July 2010 to June 2013, 52 patients with primary palmar hyperhidrosis underwent unilateral video-assisted thoracoscopic sympathectomy for dominant hand. We analyzed the outcomes regarding the resolution of symptoms, occurrence of complications, recurrence rate, and compensatory hyperhidrosis, and need of operation for opposite side. All patients were followed up from 6 to 42 months. Palmar hyperhidrosis was completely alleviated and absolute dryness was achieved in all patients at the same hand after the operation. Palmar hyperhidrosis in the opposite hand was cured to a complete dryness in 24 (46.15%) patients. No change happened in the opposite hand in 22 (42.3%) patients, but an increase was seen in 6 (11.53%) patients. Only seven (13.46%) patients needed to undergo contralateral sympathectomy. Compensatory hyperhidrosis occurred in 13 patients (25%) after unilateral sympathectomy. Another five patients (totally 18, 34.6%) were involved with compensatory hyperhidrosis after contralateral sympathectomy. It was mainly on the trunk in all 18 patients. Unilateral dominant side thoracoscopic sympathectomy for patients with primary palmar hyperhidrosis is an effective, safe, and minimally invasive procedure. Only a small number of patients will eventually require a contralateral sympathectomy in nondominant hand. Georg Thieme Verlag KG Stuttgart · New York.

  14. Post-traumatic unilateral plantar hyperhidrosis.

    Science.gov (United States)

    Eren, Y; Yavasoglu, N G; Comoglu, S S

    2016-02-01

    Localized unilateral hyperhidrosis is rare and poorly understood, sometimes stemming from trauma. Feet, quite vulnerable to trauma are affected by disease-mediated plantar hyperhidrosis, usually bilaterally. This report describes partial hyperhidrosis developing post-traumatically on the left plantar region of a 52-year-old male.

  15. Unilateral hydronephrosis in two Ugandan patients

    African Journals Online (AJOL)

    child shows evidence of hypertrophy. Introduction. Hydronephrosis refers to dilatation of the renal pelvis and calyces, flattening of the renal papillae and ... revealed tubular dilation, glomerular atrophy and areas of fibrosis. Focal areas consisted of necrosis with accompanying inflammatory reaction. Discussion. Unilateral ...

  16. PICTORIAL INTERLUDE Unilateral membranous choanal atresia

    African Journals Online (AJOL)

    Images of a case of unilateral choanal atresia diagnosed in a 35-year- old woman are presented. This is an uncommon diagnosis in an adult patient, and a less frequently encountered form of the condition. In more than 90% of cases of choanal atresia, the abnormality is partly or completely osseous; pure membranous ...

  17. Possible rare congenital dysinnervation disorder: congenital ptosis associated with adduction.

    Science.gov (United States)

    Mendes, Sílvia; Beselga, Diana; Campos, Sónia; Neves, Arminda; Campos, Joana; Carvalho, Sílvia; Silva, Eduardo; Castro Sousa, João Paulo

    2015-01-01

    Ptosis is defined as an abnormally low position of the upper eyelid margin. It can be congenital or acquired, uni or bilateral, and isolated or associated with other ocular and nonocular defects. We report a case of a female child, aged 8 years, with congenital right ptosis increased on right adduction and with left ptosis on left adduction. There was no horizontal ocular movement limitation. Apparent underaction of the right inferior oblique muscle was also present. We believe that within the possible mechanisms it is more likely that it is a congenital innervation dysgenesis syndrome (CID)/congenital cranial dysinnervation disorder (CCDD).

  18. Congenital syphilis: literature review

    OpenAIRE

    Eduardo Chaida Sonda; Felipe Farias Richter; Graziela Boschetti; Marcela Pase Casasola; Candice Franke Krumel; Cristiane Pimentel Hernandes Machado

    2013-01-01

    Syphilis is an infectious disease caused by Treponema pallidum and has high rates of vertical transmission, which can reach 100% depending on the maternal disease and stage of pregnancy. The diagnosis of gestational syphilis is simple and its screening is required during the prenatal period. However, this disease still has a high prevalence, affecting two million pregnant women worldwide. The procedures performed in newborns with congenital syphilis represent costs that are three-fold higher ...

  19. Characterizing Congenital Amusia

    OpenAIRE

    Stewart, Lauren

    2011-01-01

    The ability to make sense of the music in our environment involves sophisticated cognitive mechanisms that, for most people, are acquired effortlessly and in early life. A special population of individuals, with a disorder termed congenital amusia, report lifelong difficulties in this regard. Exploring the nature of this developmental disorder provides a window onto the cognitive architecture of typical musical processing, as well as allowing a study of the relationship between processing of ...

  20. CONGENITAL ANTERIOR TIBIOFEMURAL SUBLUXATION

    Directory of Open Access Journals (Sweden)

    A. Shahla

    2008-06-01

    Full Text Available Congenital anterior tibiofemoral subluxation is an extremely rare disorder. All reported cases accompanied by other abnormalities and syndromes. A 16-year-old high school girl referred to us with bilateral anterior tibiofemoral subluxation as the knees were extended and reduced at more than 30 degrees flexion. Deformities were due to tightness of the iliotibial band and biceps femuris muscles and corrected by surgical release. Associated disorders included bilateral anterior shoulders dislocation, short metacarpals and metatarsals, and right calcaneuvalgus deformity.