Cadaveric Temporal Bone Dissection: Is It Obsolete Today?

Directory of Open Access Journals (Sweden)

Naik, Sulabha M.

2014-01-01

Full Text Available Introduction Traditionally, surgical training in otology, is imparted by dissecting harvested human cadaveric temporal bones. However, maintenance of a cadaveric temporal bone laboratory is expensive and carries risk of exposure to infection. In recent times, other modalities of training are gaining ground and are likely to eventually replace cadaveric temporal bone dissection altogether. Objectives Other alternative methods of training are emerging. New technology like simulation and virtual reality as high-fidelity, safer alternatives, are making rapid strides as teaching tools. Other options are the use of animal temporal bones as teaching tools. The advantages of these are compared. Data Synthesis None of these modalities can replicate the innumerable anatomical variations which are a characteristic feature of the human temporal bone. A novice surgeon not only needs exposure to surgical anatomy and it's variations but also needs to develop hand-eye coordination skills to gain expertise. Conclusion Deliberate practice on human cadaveric temporal bones only, will confer both mastery in anatomy and surgical technique. The human cadaveric temporal bone is ideal simulator for training in otology.

Temporal lobe developmental malformations and epilepsy: dual pathology and bilateral hippocampal abnormalities.

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Ho, S S; Kuzniecky, R I; Gilliam, F; Faught, E; Morawetz, R

1998-03-01

Temporal lobe developmental malformations (TLDM) with focal cortical dysplasia and balloon cells may coexist with mesial temporal sclerosis. The true incidence of this dual pathology is unknown. Our aim was to assess the frequency of amygdala (AM)-hippocampal abnormality in a homogeneous population with this specific developmental malformation. MRI-based volumetry of the AM and hippocampal formation (HF) in 30 patients with unilateral TLDM and intractable partial epilepsy was performed. A volume normalization process defined a normal range of HF and AM volumes in control subjects, and enabled the detection of bilateral volume loss. Normalized volumes detected HF atrophy in 26 patients (nine unilateral and 17 bilateral) and AM atrophy in 18 patients (three unilateral and 15 bilateral). Visual analysis detected unilateral HF abnormality in 21 patients and bilateral abnormality in two. When compared with a group of patients with temporal lobe epilepsy and pure hippocampal sclerosis (N = 92), where volumetry revealed bilateral HF atrophy in 18%, a significant difference in the frequency of bilateral HF atrophy was found (p Dual pathology is frequent in patients with TLDM (87%), and the AM-HF abnormality is often bilateral (57%). Our data suggest that more widespread and potentially epileptogenic lesions coexist with visibly detectable unilateral TLDM. This has implications for the selection of patients for temporal lobe surgery and may influence surgical strategies.

Middle and inner ear malformations in mutation-proven branchio-oculo-facial (BOF) syndrome: case series and review of the literature.

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Carter, Melissa T; Blaser, Susan; Papsin, Blake; Meschino, Wendy; Reardon, Willie; Klatt, Regan; Babul-Hirji, Riyana; Milunsky, Jeff; Chitayat, David

2012-08-01

Hearing impairment is common in individuals with branchio-oculo-facial (BOF) syndrome. The majority of described individuals have conductive hearing impairment due to malformed ossicles and/or external canal stenosis or atresia, although a sensorineural component to the hearing impairment in BOF syndrome is increasingly being reported. Sophisticated computed tomography (CT) of the temporal bone has revealed middle and inner ear malformations in three previous reports. We present middle and inner ear abnormalities in three additional individuals with mutation-proven BOF syndrome. We suggest that temporal bone CT imaging be included in the medical workup of a child with BOF syndrome, in order to guide management. Copyright © 2012 Wiley Periodicals, Inc.

Schneiderian papilloma of the temporal bone

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van der Putten, L.; Bloemena, E.; Merkus, P.; Hensen, E.F.

2013-01-01

Temporal bone Schneiderian papilloma may present as a primary tumour originating from the middle ear and mastoid process, or an extension from sinonasal disease. Both forms are rare, this being only the 18th case of primary temporal bone Schneiderian papilloma described to date. Although the current

Aspects of temporal bone anatomy and pathology in conjunction with cochlear implant surgery

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Stjernholm, Christina [Karolinska Inst., Stockholm (Sweden). Soedersjukhuset

2003-07-01

communicate. The second work is a radioanatomic study of one of the structures of the inner ear - the bony canal for the cochlear nerve. It involves measurements of the dimensions of the canal on 117 silicone rubber casts of the temporal bone (from a unique collection of casts at Uppsala temporal bone laboratory) and on 50 clinical CT-studies (100 ears). The purpose was to show the normal variation, which is of use in the appraisal of congenital temporal bone malformations on CT. Based on our results we propose that if the canal is less than 1.4 mm, as measured on CT, the possibility of cochlear nerve abnormality should be considered. This is of interest since aplasia of the cochlear nerve is a contraindication to cochlear implantation. If the canal is wider than 3.0 mm, then other anomalies may coexist, with the risk of CSF gusher when a cochleostomy or stape-dectomy is performed.

Aspects of temporal bone anatomy and pathology in conjunction with cochlear implant surgery

International Nuclear Information System (INIS)

Stjernholm, Christina

2003-01-01

. The second work is a radioanatomic study of one of the structures of the inner ear - the bony canal for the cochlear nerve. It involves measurements of the dimensions of the canal on 117 silicone rubber casts of the temporal bone (from a unique collection of casts at Uppsala temporal bone laboratory) and on 50 clinical CT-studies (100 ears). The purpose was to show the normal variation, which is of use in the appraisal of congenital temporal bone malformations on CT. Based on our results we propose that if the canal is less than 1.4 mm, as measured on CT, the possibility of cochlear nerve abnormality should be considered. This is of interest since aplasia of the cochlear nerve is a contraindication to cochlear implantation. If the canal is wider than 3.0 mm, then other anomalies may coexist, with the risk of CSF gusher when a cochleostomy or stape-dectomy is performed

3D Printed Pediatric Temporal Bone: A Novel Training Model.

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Longfield, Evan A; Brickman, Todd M; Jeyakumar, Anita

2015-06-01

Temporal bone dissection is a fundamental element of otologic training. Cadaveric temporal bones (CTB) are the gold standard surgical training model; however, many institutions do not have ready access to them and their cost can be significant: $300 to $500. Furthermore, pediatric cadaveric temporal bones are not readily available. Our objective is to develop a pediatric temporal bone model. Temporal bone model. Tertiary Children's Hospital. Pediatric patient model. We describe the novel use of a 3D printer for the generation of a plaster training model from a pediatric high- resolution CT temporal bone scan of a normal pediatric temporal bone. Three models were produced and were evaluated. The models utilized multiple colors (white for bone, yellow for the facial nerve) and were of high quality. Two models were drilled as a proof of concept and found to be an acceptable facsimile of the patient's anatomy, rendering all necessary surgical landmarks accurately. The only negative comments pertaining to the 3D printed temporal bone as a training model were the lack of variation in hardness between cortical and cancellous bone, noting a tactile variation from cadaveric temporal bones. Our novel pediatric 3D temporal bone training model is a viable, low-cost training option for previously inaccessible pediatric temporal bone training. Our hope is that, as 3D printers become commonplace, these models could be rapidly reproduced, allowing for trainees to print models of patients before performing surgery on the living patient.

Multi-material 3D Models for Temporal Bone Surgical Simulation.

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Rose, Austin S; Kimbell, Julia S; Webster, Caroline E; Harrysson, Ola L A; Formeister, Eric J; Buchman, Craig A

2015-07-01

A simulated, multicolor, multi-material temporal bone model can be created using 3-dimensional (3D) printing that will prove both safe and beneficial in training for actual temporal bone surgical cases. As the process of additive manufacturing, or 3D printing, has become more practical and affordable, a number of applications for the technology in the field of Otolaryngology-Head and Neck Surgery have been considered. One area of promise is temporal bone surgical simulation. Three-dimensional representations of human temporal bones were created from temporal bone computed tomography (CT) scans using biomedical image processing software. Multi-material models were then printed and dissected in a temporal bone laboratory by attending and resident otolaryngologists. A 5-point Likert scale was used to grade the models for their anatomical accuracy and suitability as a simulation of cadaveric and operative temporal bone drilling. The models produced for this study demonstrate significant anatomic detail and a likeness to human cadaver specimens for drilling and dissection. Simulated temporal bones created by this process have potential benefit in surgical training, preoperative simulation for challenging otologic cases, and the standardized testing of temporal bone surgical skills. © The Author(s) 2015.

A Novel Temporal Bone Simulation Model Using 3D Printing Techniques.

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Mowry, Sarah E; Jammal, Hachem; Myer, Charles; Solares, Clementino Arturo; Weinberger, Paul

2015-09-01

An inexpensive temporal bone model for use in a temporal bone dissection laboratory setting can be made using a commercially available, consumer-grade 3D printer. Several models for a simulated temporal bone have been described but use commercial-grade printers and materials to produce these models. The goal of this project was to produce a plastic simulated temporal bone on an inexpensive 3D printer that recreates the visual and haptic experience associated with drilling a human temporal bone. Images from a high-resolution CT of a normal temporal bone were converted into stereolithography files via commercially available software, with image conversion and print settings adjusted to achieve optimal print quality. The temporal bone model was printed using acrylonitrile butadiene styrene (ABS) plastic filament on a MakerBot 2x 3D printer. Simulated temporal bones were drilled by seven expert temporal bone surgeons, assessing the fidelity of the model as compared with a human cadaveric temporal bone. Using a four-point scale, the simulated bones were assessed for haptic experience and recreation of the temporal bone anatomy. The created model was felt to be an accurate representation of a human temporal bone. All raters felt strongly this would be a good training model for junior residents or to simulate difficult surgical anatomy. Material cost for each model was $1.92. A realistic, inexpensive, and easily reproducible temporal bone model can be created on a consumer-grade desktop 3D printer.

Establishing a temporal bone laboratory: considerations for ENT specialist training.

LENUS (Irish Health Repository)

Fennessy, B G

2012-02-01

Cadaveric temporal bone dissection in a temporal bone laboratory is a vital component in training safe, competent otorhinolaryngologists. Recent controversies pertaining to organ retention have resulted in a more limited supply of temporal bones. Consequently, current trainees are dissecting far fewer bones than their consultants. We discuss the establishment of a temporal bone laboratory in the Department of Anatomy in the University College Cork, from the timely preparation and preservation of the tissue to its disposal. Comparisons are drawn between our experience and that of the United States training schemes. The temporal bone laboratory in Cork is the only one in existence in Ireland. The exposure and experience obtained by registrars rotating through Cork, has resulted in noticeable improvements in their operative abilities. The temporal bone laboratory remains a core component to training. It is hoped that this article may facilitate other units overcoming obstacles to establish a temporal bone laboratory.

Unusual facies, arthrogryposis, advanced skeletal maturation and unique bone changes. A new congenital malformation syndrome

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Jequier, S.; Kozlowski, K.

1987-07-01

Two strikingly similar infant siblings showed the following pattern of anomalies: unusual cranio-facial appearance, arthrogryposis, advanced bone age of the hips and unique skeletal X-ray abnormalities. They represent a previously unrecognised, fatal malformation syndrome.

Chondroblastoma of temporal bone

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Tanohta, K.; Noda, M.; Katoh, H.; Okazaki, A.; Sugiyama, S.; Maehara, T.; Onishi, S.; Tanida, T.

1986-07-01

The case of a 55-year-old female with chondroblastoma arising from the left temporal bone is presented. Although 10 cases of temporal chondroblastoma have been reported, this is the first in which plain radiography, pluridirectional tomography, computed tomography (CT) and angiography were performed. We discuss the clinical and radiological aspects of this rare tumor.

Chondroblastoma of temporal bone

International Nuclear Information System (INIS)

Tanohta, K.; Noda, M.; Katoh, H.; Okazaki, A.; Sugiyama, S.; Maehara, T.; Onishi, S.; Tanida, T.

1986-01-01

The case of a 55-year-old female with chondroblastoma arising from the left temporal bone is presented. Although 10 cases of temporal chondroblastoma have been reported, this is the first in which plain radiography, pluridirectional tomography, computed tomography (CT) and angiography were performed. We discuss the clinical and radiological aspects of this rare tumor. (orig.)

Congenital malformations of the ear and cochlear implantation in children: review and temporal bone report of common cavity.

Science.gov (United States)

Graham, J M; Phelps, P D; Michaels, L

2000-01-01

The objective of this review is to analyze aspects of congenital malformation of the ear in relation to cochlear implantation in children. Having briefly described the in utero development of the ear and the classification of types of external, middle and inner ear malformation, five practical aspects of these malformations are discussed. It seems likely that the combination of bilateral profound sensorineural deafness with bilateral microtia severe enough to make a surgical approach to the cochlea difficult will be extremely uncommon. No such cases have been reported, although Klippel-Feil deformity seems the syndrome most likely to produce this set of circumstances. Abnormalities in the intratympanic course of the facial nerve have been associated with cochlear malformation, emphasizing the benefit of intra-operative facial nerve monitoring, and a technique suggested for safely avoiding an abnormally placed nerve. Fistulae of cerebrospinal fluid (CSF) and perilymph can complicate surgery and are relatively common in common cavity and Mondini malformations. Strategies for facilitating surgery in the presence of 'gushers', for measuring the pressure of a gusher and for placement of the cochlear implant electrode array are reviewed, with reports of fluctuating levels of electric current when implants lie in dysplastic cochleas. The relationship of implant performance to VIIIth nerve tissue in malformed cochleas is discussed, with a description of the histological findings in a common cavity cochlea. Techniques for identifying the absence of the cochlear nerve are reviewed. Stimulation of the facial nerve by cochlear implants has been described in cases of congenital malformation of the labyrinth but is relatively uncommon. Case reports of the benefit received by implanted children with congenital cochlear malformation have appeared since 1988. Most cases reported have not yet been followed for long enough to establish a clear picture of the outcome following

Bone scintigraphy in the diagnosis of fracture and infection of the temporal bone

International Nuclear Information System (INIS)

Djupesland, G.; Nakken, K.F.; Mueller, C.; Skjoerten, F.; Roehrt, T.; Eldevik, P.

1983-01-01

The sensivit of Tc99m-MDP-bone-scintiscanning in the diagnosis of temporal bone fracture was found to that of conventional radiography if the patients were examined 10 days after the trauma. Temporal bone osteomyelitis with concomitant moderate osteosclerosis was demonstrated by bone scintigraphy in 5 cases of mastoiditis with atypical symptoms. A case of apicitis was for the first time demonstrated by scintigraphy. A low sensivity of 67 Ga-scintigraphy was demonstrated by positive Tc99m-bone-scintigraphy and negative 67 Ga-scintigraphy in a patient with atypical mastoiditis. Tc99m-scintigraphy was negative in 5 cases of otitis media suppurative and in 3 cases of otitis media chronica cum cholesteatoma, all with slight degree of osteosclerosis in the mastoid. The sensitivity of Tc99m-bone-scintigraphy in fracture and osteomyelitis of the temporal bone seems to be a function of the amount of reactive new bone formed. (Authors)

Temporal Bone Fracture Causing Superior Semicircular Canal Dehiscence

Directory of Open Access Journals (Sweden)

Kevin A. Peng

2014-01-01

Full Text Available Importance. Superior semicircular canal dehiscence (SCD is a third window lesion of the inner ear causing symptoms of vertigo, autophony, tinnitus, and hearing loss. A “two-hit” hypothesis has traditionally been proposed, whereby thinly developed bone overlying the superior canal is disrupted by a sudden change in intracranial pressure. Although the symptoms of SCD may be precipitated by head injury, no previous reports have described a temporal bone fracture directly causing SCD. Observations. Two patients sustained temporal bone fractures after closed head trauma, and developed unilateral otologic symptoms consistent with SCD. In each instance, computed tomography imaging revealed fractures extending through the bony roof of the superior semicircular canal. Conclusions and Relevance. Temporal bone fractures, which are largely treated nonoperatively, have not previously been reported to cause SCD. As it is a potentially treatable entity, SCD resulting from temporal bone fracture must be recognized as a possibility and diagnosed promptly if present.

Bone Density Development of the Temporal Bone Assessed by Computed Tomography.

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Takahashi, Kuniyuki; Morita, Yuka; Ohshima, Shinsuke; Izumi, Shuji; Kubota, Yamato; Horii, Arata

2017-12-01

The temporal bone shows regional differences in bone development. The spreading pattern of acute mastoiditis shows age-related differences. In infants, it spreads laterally and causes retroauricular swelling, whereas in older children, it tends to spread medially and causes intracranial complications. We hypothesized that bone maturation may influence the spreading pattern of acute mastoiditis. Eighty participants with normal hearing, aged 3 months to 42 years, participated in this study. Computed tomography (CT) values (Hounsfield unit [HU]) in various regions of the temporal bone, such as the otic capsule (OC), lateral surface of the mastoid cavity (LS), posterior cranial fossa (PCF), and middle cranial fossa (MCF), were measured as markers of bone density. Bone density development curves, wherein CT values were plotted against age, were created for each region. The age at which the CT value exceeded 1000 HU, which is used as an indicator of bone maturation, was calculated from the development curves and compared between the regions. The OC showed mature bone at birth, whereas the LS, PCF, and MCF showed rapid maturation in early childhood. However, there were significant regional differences in the ages of maturation: 1.7, 3.9, and 10.8 years for the LS, PCF, and MCF, respectively. To our knowledge, this is the first report to show regional differences in the maturation of temporal bone, which could partly account for the differences in the spreading pattern of acute mastoiditis in individuals of different ages.

High resolution CT of temporal bone trauma

International Nuclear Information System (INIS)

Youn, Eun Kyung

1986-01-01

Radiographic studies of the temporal bone following head trauma are indicated when there is cerebrospinal fluid otorrhea or rhinorrhoea, hearing loss, or facial nerve paralysis. Plain radiography displays only 17-30% of temporal bone fractures and pluridirectional tomography is both difficult to perform, particularly in the acutely ill patient, and less satisfactory for the demonstration of fine fractures. Consequently, high resolution CT is the imaging method of choice for the investigation of suspected temporal bone trauma and allows special resolution of fine bony detail comparable to that attainable by conventional tomography. Eight cases of temporal bone trauma examined at Korea General Hospital April 1985 through May 1986. The results were as follows: Seven patients (87%) suffered longitudinal fractures. In 6 patients who had purely conductive hearing loss, CT revealed various ossicular chain abnormality. In one patient who had neuro sensory hearing loss, CT demonstrated intract ossicular with a fracture nearing lateral wall of the lateral semicircular canal. In one patient who had mixed hearing loss, CT showed complex fracture.

Primary pericranial Ewing's sarcoma on the temporal bone: A case report.

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Kawano, Hiroto; Nitta, Naoki; Ishida, Mitsuaki; Fukami, Tadateru; Nozaki, Kazuhiko

2016-01-01

Primary Ewing's sarcoma originating in the pericranium is an extremely rare disease entity. A 9-year-old female patient was admitted to our department due to a left temporal subcutaneous mass. The mass was localized under the left temporal muscle and attached to the surface of the temporal bone. Head computed tomography revealed a mass with bony spicule formation on the temporal bone, however, it did not show bone destruction or intracranial invasion. F-18 fluorodeoxyglucose positron emission tomography showed no lesions other than the mass on the temporal bone. Magnetic resonance imaging showed that the mass was located between the temporal bone and the pericranium. The mass was completely resected with the underlying temporal bone and the overlying deep layer of temporal muscle, and was diagnosed as primary Ewing's sarcoma. Because the tumor was located in the subpericranium, we created a new classification, "pericranial Ewing's sarcoma," and diagnosed the present tumor as pericranial Ewing's sarcoma. We herein present an extremely rare case of primary pericranial Ewing's sarcoma that developed on the temporal bone.

Severe Klippel-Feil syndrome with Mondini malformation of inner ear

OpenAIRE

Alaqeel, Aqeel Abdullah

2014-01-01

Klippel-Feil syndrome is defined as the fusion of cervical vertebra with associated congenital anomalies but was rarely reported to be associated with Mondini Malformation. We report a newborn girl with severe neck extension, computed tomography (CT) of the neck after birth showed fusion of the fifth, sixth, and seventh cervical vertebrae, compatible with Klippel-Feil Syndrome and CT temporal bone showed choclear dysplasia with incomplete number of turns that is compatible with Mondini Malfor...

Skeletal malformations in fetuses with Meckel syndrome

DEFF Research Database (Denmark)

Kjaer, K W; Fischer Hansen, B; Keeling, J W

1999-01-01

one foot was normal. Malformations of the cranial base (the basilar part of the occipital bone or the postsphenoid bone) occurred in five cases, and the vertebral bodies in the lumbar region of the spine were malformed (cleft) in three cases. It is proposed that a skeletal analysis be included...

Osteoradionecrosis of the temporal bone

International Nuclear Information System (INIS)

Fujimori, Masato; Koyama, Yukiko; Enomoto, Fuyuki; Ichikawa, Ginichiro

2002-01-01

We report a case of temporal bone necrosis that emerged after radiotherapy for epipharyngeal carcinoma performed 13 years ago. The patient was a 51-year-old male. His major complaint was left facial swelling. The patient underwent chemotherapy and radiotherapy (Co 60, 6120 rad), as the treatment of that period, for epipharyngeal carcinoma from September 30, 1986 to January 31, 1987. He also underwent lobectomy of the left temporal lobe in brain surgery for left temporal lobe necrosis in August, 1989. After that operation, we saw constriction in his left external acoustic meatus and continued the follow-up. On October 22, 1999 he felt a left facial swelling. We found skin defects and ulcer formation in the front part of his left ear. Although we administered an antiseptic and antibiotic to the diseased area, his condition did not improve. He was hospitalized for the purpose of undergoing medical treatment on January 6, 2000. We found extensive skin necrosis and defects in his left auricular area. The corrupted temporal bone reached the zygomatic, the bone department external acoustic meatus and the mastoid process was exposing. We performed debridement of the diseased area on January 19, 2000. On February 23, we performed reconstruction by left trapezius muscle flap after debridement once again. One year after the operation, the flap was completely incorporated. (author)

Osteoradionecrosis of the temporal bone

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Fujimori, Masato; Koyama, Yukiko; Enomoto, Fuyuki; Ichikawa, Ginichiro [Juntendo Univ., Tokyo (Japan). School of Medicine

2002-08-01

We report a case of temporal bone necrosis that emerged after radiotherapy for epipharyngeal carcinoma performed 13 years ago. The patient was a 51-year-old male. His major complaint was left facial swelling. The patient underwent chemotherapy and radiotherapy (Co 60, 6120 rad), as the treatment of that period, for epipharyngeal carcinoma from September 30, 1986 to January 31, 1987. He also underwent lobectomy of the left temporal lobe in brain surgery for left temporal lobe necrosis in August, 1989. After that operation, we saw constriction in his left external acoustic meatus and continued the follow-up. On October 22, 1999 he felt a left facial swelling. We found skin defects and ulcer formation in the front part of his left ear. Although we administered an antiseptic and antibiotic to the diseased area, his condition did not improve. He was hospitalized for the purpose of undergoing medical treatment on January 6, 2000. We found extensive skin necrosis and defects in his left auricular area. The corrupted temporal bone reached the zygomatic, the bone department external acoustic meatus and the mastoid process was exposing. We performed debridement of the diseased area on January 19, 2000. On February 23, we performed reconstruction by left trapezius muscle flap after debridement once again. One year after the operation, the flap was completely incorporated. (author)

A new classification for cochleovestibular malformations.

Science.gov (United States)

Sennaroglu, Levent; Saatci, Isil

2002-12-01

The report proposes a new classification system for inner ear malformations, based on radiological features of inner ear malformations reviewed in 23 patients. The investigation took the form of a retrospective review of computerized tomography findings relating to the temporal bone in 23 patients (13 male and 10 female patients) with inner ear malformations. The subjects were patients with profound bilateral sensorineural hearing loss who had all had high-resolution computed tomography (CT) with contiguous 1-mm-thick images obtained through the petrous bone in axial sections. The CT results were reviewed for malformations of bony otic capsule under the following subgroups: cochlear, vestibular, semicircular canal, internal auditory canal (IAC), and vestibular and cochlear aqueduct malformations. Cochlear malformations were classified as Michel deformity, common cavity deformity, cochlear aplasia, hypoplastic cochlea, incomplete partition types I (IP-I) and II (IP-II) (Mondini deformity). Incomplete partition type I (cystic cochleovestibular malformation) is defined as a malformation in which the cochlea lacks the entire modiolus and cribriform area, resulting in a cystic appearance, and there is an accompanying large cystic vestibule. In IP-II (the Mondini deformity), there is a cochlea consisting of 1.5 turns (in which the middle and apical turns coalesce to form a cystic apex) accompanied by a dilated vestibule and enlarged vestibular aqueduct. Four patients demonstrated anomalies involving only one inner ear component. All the remaining patients had diseases or conditions affecting more than one inner ear component. Eight ears had IP-I, and 10 patients had IP-II. Ears with IP-I had large cystic vestibules, whereas the amount of dilation was minimal in patients with IP-II. The majority of the semicircular canals (67%) were normal. Semicircular canal aplasia accompanied cases of Michel deformity, cochlear hypoplasia, and common cavity. In 14 ears, the IAC had a

The role of MRI in suspected inner ear malformations

International Nuclear Information System (INIS)

Koesling, S.; Juettemann, S.; Amaya, B.; Rasinski, C.; Bloching, M.; Koenig, E.

2003-01-01

Purpose: This is a prospective analysis of the value of MRI in suspected inner ear malformations. Materials and Methods: In 50 patients (43 children and young adults, 7 adults) with suspected inner ear malformation MRI (1.5 T) was performed. In addition, 42 of these patients underwent CT. For the analysis of the inner ear structures, the constructive interference in steady state (CISS) sequence with 0.7 mm slice thickness was used. Functional tests revealed a sensorineural hearing loss or deafness in 82 temporal bones (TB) and a combined hearing loss in 4 TB. The hearing loss was unilateral in 14 patients. MRI and CT findings were compared. Results: Imaging findings were normal in 58 TB. The pathological findings included inner ear malformations (35 TB), inflammatory changes (4 TB), partial obliteration of labyrinth (2 TB) and congenital aural atresia (1 TB). An isolated absence of the cochlear nerve (1 TB) could only be found by MRI. In the remaining cases, an inner ear malformation was diagnosed by MRI and CT with the same confidence but MRI was superior in displaying the fine details. Conclusions: MRI will become the method of choice in the diagnosis of inner ear malformations. (orig.) [de

Classification of temporal bone pneumatization based on sigmoid sinus using computed tomography

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Han, S.-J. [Department of Otorhinolaryngology, National Health Insurance Corporation Ilsan Hospital, Seoul (Korea, Republic of); Song, M.H. [Department of Otorhinolaryngology, Yonsei University College of Medicine, Kang-nam Gu, Do-gok Dong, 146-92, Seoul, Republic of Korea 135-720 (Korea, Republic of); Kim, J. [Department of Radiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Lee, W.-S. [Department of Otorhinolaryngology, Yonsei University College of Medicine, Kang-nam Gu, Do-gok Dong, 146-92, Seoul, Republic of Korea 135-720 (Korea, Republic of); Lee, H.-K. [Department of Otorhinolaryngology, Yonsei University College of Medicine, Kang-nam Gu, Do-gok Dong, 146-92, Seoul, Republic of Korea 135-720 (Korea, Republic of)], E-mail: hoki@yuhs.ac

2007-11-15

Aim: To analyse several reference structures using axial computed tomography (CT) imaging of the temporal bone, which may reflect pneumatization of the entire temporal bone by statistical correlation to the actual volume of the temporal bone measured using three-dimensional reconstruction. Materials and methods: One hundred and sixteen temporal bones were studied, comprising 48 with normal findings and 68 sides showing chronic otitis media or temporal bone fracture. After measuring the volume of temporal bone air cells by the volume rendering technique using three-dimensional reconstruction images, classification of temporal bone pneumatization was performed using various reference structures on axial images to determine whether significant differences in the volume of temporal bone air cells could be found between the groups. Results: When the sigmoid sinus at the level of the malleoincudal complex was used in the classification, there were statistically significant differences between the groups that correlated with the entire volume of the temporal bone. Grouping based on the labyrinth and the ascending carotid artery showed insignificant differences in volume. Furthermore, there was no significant correlation between the cross-sectional area of the antrum and the entire volume of the temporal bone. Conclusion: The degree of pneumatization of temporal bone can be estimated easily by the evaluation of the air cells around the sigmoid sinus on axial CT images.

Classification and Current Management of Inner Ear Malformations

Directory of Open Access Journals (Sweden)

Levent Sennaroğlu

2017-10-01

Full Text Available Morphologically congenital sensorineural hearing loss can be investigated under two categories. Majority of the congenital hearing loss (80% are membranous malformations. Here the pathology involves inner ear hair cells. There is no gross bony abnormality and therefore, in these cases, high resolution computerized tomography and MRI of the temporal bone reveal normal findings. Remaining 20% have various malformations involving the bony labyrinth and therefore, can be radiologically demonstrated by CT and MRI. The latter group involves surgical challenges as well as problems in decision making. Some cases may be managed by hearing aid, some need cochlear implantation while some cases are candidates for an auditory brainstem implantation. During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During the surgery for inner ear malformations, surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcome after various implantation methods is closely related to the status of cochlear nerve and a practical classification of the cochlear nerve deficiency is also provided

Chondrosarcoma of the temporal bone: a case report

International Nuclear Information System (INIS)

Park, Man Soo; Lee, Sang Youl; Chung, Jae Gul; Lee, Deok Hee; Jung, Seung Mun; Ryu, Dae Sik

2001-01-01

Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with chordoma, glomus jugulare tumor and meningioma, among other conditions, and due to its anatomic location, cranial nerve palsy is frequently observed. We report a case involving a 50-year-old woman with chondrosarcoma of the temporal bone

Chondrosarcoma of the temporal bone: a case report

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Park, Man Soo; Lee, Sang Youl; Chung, Jae Gul; Lee, Deok Hee; Jung, Seung Mun; Ryu, Dae Sik [Kang Nung Hospital, Ulsan Univ. Kangnung (Korea, Republic of)

2001-07-01

Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with chordoma, glomus jugulare tumor and meningioma, among other conditions, and due to its anatomic location, cranial nerve palsy is frequently observed. We report a case involving a 50-year-old woman with chondrosarcoma of the temporal bone.

Anatomical study of the pigs temporal bone by microdissection.

Science.gov (United States)

Garcia, Leandro de Borborema; Andrade, José Santos Cruz de; Testa, José Ricardo Gurgel

2014-01-01

Initial study of the pig`s temporal bone anatomy in order to enable a new experimental model in ear surgery. Dissection of five temporal bones of Sus scrofa pigs obtained from UNIFESP - Surgical Skills Laboratory, removed with hole saw to avoid any injury and stored in formaldehyde 10% for better conservation. The microdissection in all five temporal bone had the following steps: inspection of the outer part, external canal and tympanic membrane microscopy, mastoidectomy, removal of external ear canal and tympanic membrane, inspection of ossicular chain and middle ear. Anatomically it is located at the same position than in humans. Some landmarks usually found in humans are missing. The tympanic membrane of the pig showed to be very similar to the human, separating the external and the middle ear. The middle ear`s appearance is very similar than in humans. The ossicular chain is almost exactly the same, as well as the facial nerve, showing the same relationship with the lateral semicircular canal. The temporal bone of the pigs can be used as an alternative for training in ear surgery, especially due the facility to find it and its similarity with temporal bone of the humans.

Anomalous facial nerve canal with cochlear malformations.

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Romo, L V; Curtin, H D

2001-05-01

Anteromedial "migration" of the first segment of the facial nerve canal has been previously identified in a patient with a non-Mondini-type cochlear malformation. In this study, several patients with the same facial nerve canal anomaly were reviewed to assess for the association and type of cochlear malformation. CT scans of the temporal bone of 15 patients with anteromedial migration of the first segment of the facial nerve canal were collected from routine departmental examinations. In seven patients, the anomalous course was bilateral, for a total of 22 cases. The migration was graded relative to normal as either mild/moderate or pronounced. The cochlea in each of these cases was examined for the presence and size of the basilar, second, and apical turns. The turns were either absent, small, normal, or enlarged. The CT scans of five patients with eight Mondini malformations were examined for comparison. The degree of the facial nerve migration was pronounced in nine cases and mild/moderate in 13. All 22 of these cases had associated cochlear abnormalities of the non-Mondini variety. These included common cavity anomalies with lack of definition between the cochlea and vestibule (five cases), cochleae with enlarged basilar turns and absent second or third turns (five cases), and cochleae with small or normal basilar turns with small or absent second or third turns (12 cases). None of the patients with Mondini-type cochlear malformations had anteromedial migration of the facial nerve canal. Anteromedial migration of the facial nerve canal occurs in association with some cochlear malformations. It did not occur in association with the Mondini malformations. A cochlea with a Mondini malformation, being similar in size to a normal cochlea, may physically prohibit such a deviation in course.

Intra-temporal facial nerve centerline segmentation for navigated temporal bone surgery

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Voormolen, Eduard H. J.; van Stralen, Marijn; Woerdeman, Peter A.; Pluim, Josien P. W.; Noordmans, Herke J.; Regli, Luca; Berkelbach van der Sprenkel, Jan W.; Viergever, Max A.

2011-03-01

Approaches through the temporal bone require surgeons to drill away bone to expose a target skull base lesion while evading vital structures contained within it, such as the sigmoid sinus, jugular bulb, and facial nerve. We hypothesize that an augmented neuronavigation system that continuously calculates the distance to these structures and warns if the surgeon drills too close, will aid in making safe surgical approaches. Contemporary image guidance systems are lacking an automated method to segment the inhomogeneous and complexly curved facial nerve. Therefore, we developed a segmentation method to delineate the intra-temporal facial nerve centerline from clinically available temporal bone CT images semi-automatically. Our method requires the user to provide the start- and end-point of the facial nerve in a patient's CT scan, after which it iteratively matches an active appearance model based on the shape and texture of forty facial nerves. Its performance was evaluated on 20 patients by comparison to our gold standard: manually segmented facial nerve centerlines. Our segmentation method delineates facial nerve centerlines with a maximum error along its whole trajectory of 0.40+/-0.20 mm (mean+/-standard deviation). These results demonstrate that our model-based segmentation method can robustly segment facial nerve centerlines. Next, we can investigate whether integration of this automated facial nerve delineation with a distance calculating neuronavigation interface results in a system that can adequately warn surgeons during temporal bone drilling, and effectively diminishes risks of iatrogenic facial nerve palsy.

Intra-temporal facial nerve centerline segmentation for navigated temporal bone surgery

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Voormolen, E.H.J.; Stralen, van M.; Woerdeman, P.A.; Pluim, J.P.W.; Noordmans, H.J.; Regli, L.; Berkelbach van der Sprenkel, J.W.; Viergever, M.A.; Wong, K.H.; Holmes III, D.R.

2011-01-01

Approaches through the temporal bone require surgeons to drill away bone to expose a target skull base lesion while evading vital structures contained within it, such as the sigmoid sinus, jugular bulb, and facial nerve. We hypothesize that an augmented neuronavigation system that continuously

Post traumatic facial nerve palsy without temporal bone fracture

International Nuclear Information System (INIS)

Scuotto, A.; Cappabianca, S.; Capasso, R.; Porto, A.; D'Oria, S.; Rotondo, M.

2016-01-01

Facial nerve injury following head trauma is a frequent event with or without temporal bone fractures. Computed tomography is the imaging modality of choice for assessing the possible bone disruption of the facial nerve canal. Magnetic resonance is helpful in presence of a facial nerve paralysis, unexplained by computed tomography findings. We present a case of delayed post-traumatic facial nerve palsy without radiological evidence of temporal bone fractures, in which magnetic resonance was crucial for diagnosing the nerve impairment. Radiological findings in accordance both with electrodiagnostic tests and clinical presentation suggested the successful conservative management. - Highlights: • Facial nerve is more prone to damage than any other cranial nerve after trauma. • Facial nerve trauma is usually associated with temporal bone fractures. • MRI is mandatory in case of no evidence of bone disruption at CT.

Massive Cerebrospinal Fluid Leak of the Temporal Bone

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Giannicola Iannella

2016-01-01

Full Text Available Cerebrospinal fluid (CSF leakage of the temporal bone region is defined as abnormal communications between the subarachnoidal space and the air-containing spaces of the temporal bone. CSF leak remains one of the most frequent complications after VS surgery. Radiotherapy is considered a predisposing factor for development of temporal bone CSF leak because it may impair dural repair mechanisms, thus causing inadequate dural sealing. The authors describe the case of a 47-year-old man with a massive effusion of CSF which extended from the posterior and lateral skull base to the first cervical vertebrae; this complication appeared after a partial enucleation of a vestibular schwannoma (VS with subsequent radiation treatment and second operation with total VS resection.

CT features of fibrous dysplasia of the temporal bone

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Charrada-Ben Farhat, L.; Bourkhis, S.; Ben Yaacoub, I.; Dali, N.; Askri, A.; Hendaoui, L.

2006-01-01

Fibrous dysplasia is characterized by a progressive replacement of normal bone elements by fibrous tissue. The temporal bone is rarely involved. In this location, complications such as facial deformity, conductive hearing loss and facial peripheral neural involvement can occur. Positive diagnosis can be established with computerized tomography which also enables assessment of extension and detection of complications. We report a case of a 27-year-old man with extensive fibrous dysplasia of the right temporal bone presenting with conductive hearing loss secondary to progressive stenosis of the external auditory canal. Computerized tomography of the temporal region was performed. (authors)

Cranial base morphology and temporal bone pneumatization in Asian Homo erectus.

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Balzeau, Antoine; Grimaud-Hervé, Dominique

2006-10-01

The external morphological features of the temporal bone are used frequently to determine taxonomic affinities of fossils of the genus Homo. Temporal bone pneumatization has been widely studied in great apes and in early hominids. However, this feature is rarely examined in the later hominids, particularly in Asian Homo erectus. We provide a comparative morphological and quantitative analysis of Asian Homo erectus from the sites of Ngandong, Sambungmacan, and Zhoukoudian, and of Neandertals and anatomically modern Homo sapiens in order to discuss causes and modalities of temporal bone pneumatization during hominid evolution. The evolution of temporal bone pneumatization in the genus Homo is more complex than previously described. Indeed, the Zhoukoudian fossils have a unique pattern of temporal bone pneumatization, whereas Ngandong and Sambungmacan fossils, as well as the Neandertals, more closely resemble the modern human pattern. Moreover, these Chinese fossils are characterized by a wide midvault and a relatively narrow occipital bone. Our results support the point of view that cell development does not play an active role in determining cranial base morphology. Instead, pneumatization is related to available space and to temporal bone morphology, and its development is related to correlated morphology and the relative disposition of the bones and cerebral lobes. Because variation in pneumatization is extensive within the same species, the phyletic implications of pneumatization are limited in the taxa considered here.

A case of osteoradionecrosis of the temporal bone

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Okuno, Hideji; Saito, Yozo; Katori, Kimiaki; Hata, Yuko

1983-01-01

A case of osteoradionecrosis of the temporal bone was described. The patient had received radiotherapy for her nasopharyngeal cancer. Her symptoms of osteoradionecrosis of the temporal bone, otorrhea and earache, first appeared 5 years after her radiotherapy. Since then, conservative therapy was continued for 5 years, but her symptoms were not controlled with it. Ten years after her radiotherapy, the symptoms worsened rapidly, and the pain became intolerable, which necessitated mastoidectomy. After this surgery, she was free of symptoms until now. Based on this case, we discussed the pathologic, diagnostic and therapeutic aspects of osteoradionecrosis of the temporal bone. Emphasis was placed on the possibility of the occurence of this pathologic condition among all the patients who have received radiotherapy for malignant tumors of the head and neck or brain. (author)

Assessment of skills using a virtual reality temporal bone surgery simulator.

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Linke, R; Leichtle, A; Sheikh, F; Schmidt, C; Frenzel, H; Graefe, H; Wollenberg, B; Meyer, J E

2013-08-01

Surgery on the temporal bone is technically challenging due to its complex anatomy. Precise anatomical dissection of the human temporal bone is essential and is fundamental for middle ear surgery. We assessed the possible application of a virtual reality temporal bone surgery simulator to the education of ear surgeons. Seventeen ENT physicians with different levels of surgical training and 20 medical students performed an antrotomy with a computer-based virtual temporal bone surgery simulator. The ease, accuracy and timing of the simulated temporal bone surgery were assessed using the automatic assessment software provided by the simulator device and additionally with a modified Final Product Analysis Scale. Trained ENT surgeons, physicians without temporal bone surgical training and medical students were all able to perform the antrotomy. However, the highly trained ENT surgeons were able to complete the surgery in approximately half the time, with better handling and accuracy as assessed by the significant reduction in injury to important middle ear structures. Trained ENT surgeons achieved significantly higher scores using both dissection analysis methods. Surprisingly, there were no significant differences in the results between medical students and physicians without experience in ear surgery. The virtual temporal bone training system can stratify users of known levels of experience. This system can be used not only to improve the surgical skills of trained ENT surgeons for more successful and injury-free surgeries, but also to train inexperienced physicians/medical students in developing their surgical skills for the ear.

CT-diagnosis of temporal bone trauma

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Valavanis, A.; Stuckmann, G.; Antonucci, F.; Schubiger, O.

1986-02-01

73 patients with 78 fractures of the temporal bone were examined by high-resolution computed tomography (CT). Analysis of the CT-findings disclosed 55 longitudinal, 12 transverse, 8 combined and 3 atypical fractures. For determination of the fracture type, axial sections usually proved sufficient. However, for precise topographic analysis of the course of the fracture additional coronal sections were necessary in most of the cases. In the radiologic evaluation of temporal bone fractures detection of associated. Complications is clinically important since these can be surgically corrected. In this series 20 lesions of the ossicular chain were demonstrated by the combined performance of axial and coronal sections and sagittal reformations. High resolution CT demonstrated a lesion of the facial nerve canal in 79% of a patient group with traumatic facial nerve palsy. The most frequent site of injury of the facial nerve canal was the region of the geniculate ganglion. With the use of metrizaminde-CT-cisternography the site of cerebrospinal fluid leakage was demonstrated in 7 of 9 patients with liquorrhea. It is concluded that high-resolution CT is the radiologic method of choice for both topographic evaluation of temporal bone fractures and detection and precise localization of fracture-complications. (orig.).

Surgical anatomy of the temporal bone: an atlas

International Nuclear Information System (INIS)

Chan, L.L.; Manolidis, S.; Taber, K.H.; Hayman, L.A.

2001-01-01

This atlas demonstrates the usefulness of reconstructed high-resolution CT for planning temporal bone surgery. The first part focuses on a sagittal plane, the second on a rotated longitudinal plane, and the third on a rotated transverse plane. We believe knowledge of temporal bone anatomy in these planes facilitates surgical planning by showing anatomic relationships and providing a customized map for each patient. This decreases the likelihood of surgical mishap and improves teaching. (orig.)

Correlations of External Landmarks With Internal Structures of the Temporal Bone.

Science.gov (United States)

Piromchai, Patorn; Wijewickrema, Sudanthi; Smeds, Henrik; Kennedy, Gregor; O'Leary, Stephen

2015-09-01

The internal anatomy of a temporal bone could be inferred from external landmarks. Mastoid surgery is an important skill that ENT surgeons need to acquire. Surgeons commonly use CT scans as a guide to understanding anatomical variations before surgery. Conversely, in cases where CT scans are not available, or in the temporal bone laboratory where residents are usually not provided with CT scans, it would be beneficial if the internal anatomy of a temporal bone could be inferred from external landmarks. We explored correlations between internal anatomical variations and metrics established to quantify the position of external landmarks that are commonly exposed in the operating room, or the temporal bone laboratory, before commencement of drilling. Mathematical models were developed to predict internal anatomy based on external structures. From an operating room view, the distances between the following external landmarks were observed to have statistically significant correlations with the internal anatomy of a temporal bone: temporal line, external auditory canal, mastoid tip, occipitomastoid suture, and Henle's spine. These structures can be used to infer a low lying dura mater (p = 0.002), an anteriorly located sigmoid sinus (p = 0.006), and a more lateral course of the facial nerve (p external landmarks. The distances between these two landmarks and the operating view external structures were able to further infer the laterality of the facial nerve (p internal structures with a high level of accuracy: the distance from the sigmoid sinus to the posterior external auditory canal (p external landmarks found on the temporal bone. These relationships could be used as a guideline to predict challenges during drilling and choosing appropriate temporal bones for dissection.

Cognitive consequences of coexisting temporal lobe developmental malformations and hippocampal sclerosis.

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Martin, R; Dowler, R; Gilliam, F; Faught, E; Morawetz, R; Kuzniecky, R

1999-09-11

To characterize patterns of cognitive functioning in a well-defined group of patients with MRI-identified coexisting left temporal lobe developmental malformations (TLDM) and mesial temporal sclerosis (MTS), and to examine neuropsychological outcome in this dual-pathology group following epilepsy surgery. Cognitive functioning in patients with left TLDM and MTS (n = 15) was compared with patients with isolated left MTS (n = 40). TLDM and MTS were identified by high-quality MRI protocol. Patients were administered a battery of neuropsychology tests as part of their presurgical workup for possible epilepsy surgery. Unilateral temporal lobe resection was performed on 10 of the dual-pathology patients and 34 of the isolated MTS patients. Postoperative cognitive performance was also assessed. Both groups displayed impairments in verbal and visual memory, language, and academic achievement. Performance on measures of psychometric intelligence, executive function, and attention were not impaired and were similar between groups. Presence of dual pathology was associated with a significantly less efficient verbal encoding strategy on the word list learning task. Postoperatively, declines were noted for both groups across tasks of verbal memory and language. Groups were not different significantly in terms of neuropsychological outcome after surgery. Patients with coexisting TLDM and MTS have impaired cognitive functioning similar to MTS patients-in particular, with regard to episodic memory and language deficits. Temporal lobe resection produces similar cognitive changes in both groups.

Face and content validation of a virtual reality temporal bone simulator.

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Arora, Asit; Khemani, Sam; Tolley, Neil; Singh, Arvind; Budge, James; Varela, David A Diaz Voss; Francis, Howard W; Darzi, Ara; Bhatti, Nasir I

2012-03-01

To validate the VOXEL-MAN TempoSurg simulator for temporal bone dissection. Prospective international study. Otolaryngology departments of 2 academic health care institutions in the United Kingdom and United States. Eighty-five subjects were recruited consisting of an experienced and referent group. Participants performed a standardized familiarization session and temporal bone dissection task. Realism, training effectiveness, and global impressions were evaluated across 21 domains using a 5-point Likert-type scale. A score of 4 was the minimum threshold for acceptability. The experienced group comprised 25 otolaryngology trainers who had performed 150 mastoid operations. The referent group comprised 60 trainees (mean otolaryngology experience of 2.9 years). Familiarization took longer in the experienced group (P = .01). User-friendliness was positively rated (mean score 4.1). Seventy percent of participants rated anatomical appearance as acceptable. Trainers rated drill ergonomics worse than did trainees (P = .01). Simulation temporal bone training scored highly (mean score 4.3). Surgical anatomy, drill navigation, and hand-eye coordination accounted for this. Trainees were more likely to recommend temporal bone simulation to a colleague than were trainers (P = .01). Transferability of skills to the operating room was undecided (mean score 3.5). Realism of the VOXEL-MAN virtual reality temporal bone simulator is suboptimal in its current version. Nonetheless, it represents a useful adjunct to existing training methods and is particularly beneficial for novice surgeons before performing cadaveric temporal bone dissection. Improvements in realism, specifically drill ergonomics and visual-spatial perception during deeper temporal bone dissection, are warranted.

IgG4-Related Disease of Bilateral Temporal Bones.

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Li, Lilun; Ward, Bryan; Cocks, Margaret; Kheradmand, Amir; Francis, Howard W

2017-03-01

IgG4-related disease (IgG4-RD) is an idiopathic inflammatory condition that causes pseudotumor formation in single or multiple organs, including those of the head and neck. Temporal bone involvement is rare, with only 3 cases of unilateral temporal bone IgG4-RD described in the literature. We report the first known case of IgG4-RD of bilateral temporal bones and describe its clinical presentation, diagnosis, and treatment. The patient was a 52-year-old man with latent tuberculosis (TB) who presented with a 10-year history of bilateral profound hearing loss and vestibular dysfunction. Computed tomography and magnetic resonance imaging demonstrated bilateral labyrinthine destruction with invasion of the posterior fossa. Immunoglobulin level testing showed elevated total serum IgG levels with normal IgG4 levels. Bilateral mastoidectomies were performed, with biopsy samples demonstrating IgG4 staining with IgG4-positive plasma cells up to 40/HPF (high power field) on the right and 20/HPF on the left, consistent with bilateral IgG4-RD. IgG4-RD of bilateral temporal bones presents with chronic and progressive bilateral hearing loss and vestibular dysfunction. Clinical presentation and radiologic findings are nonspecific, and definitive diagnosis must be made with histopathology and immunostaining. Corticosteroids are therapeutic, but surgical resection may be necessary for temporal bone IgG4-RD to improve long-term remission.

Temporal bone radiography using the orthopantomograph

International Nuclear Information System (INIS)

Tatezawa, T.

1981-01-01

Temporal bone radiographs obtained with an Orthopantomograph were compared with conventional radiographs. In acoustic neurinoma, cholesteatoma, otitis media, and middle fossa tumors, both methods demonstrated the abnormalities well. In two cases with lesions extending beyond the range of conventional projections, the broad orthopantomographic coverage was very valuable. Mastoid air cells, the mastoid process, petrous ridge, and internal auditory meatus were well demonstrated by both techniques. Orthopantomography was found to be superior in the demonstration of the petrous apex, while the superior semicircular canal was better demonstrated on the conventional views. Bilateral symmetry was particularly good and because of fewer films, radiation exposure was considerably less with orthopantomography. For many applications, orthopantomography is an adequate convenient substitute for conventional methods of examining the temporal bones

Cartilage and bone malformations in the head of zebrafish (Danio rerio) embryos following exposure to disulfiram and acetic acid hydrazide

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Strecker, Ruben, E-mail: Ruben.Strecker@cos.uni-heidelberg.de [Aquatic Ecology and Toxicology Section, Center for Organismal Studies, University of Heidelberg, Im Neuenheimer Feld 230, D-69120 Heidelberg (Germany); Weigt, Stefan, E-mail: stefan.weigt@merckgroup.com [Institute of Toxicology, Merck KGaA, 64293 Darmstadt (Germany); Braunbeck, Thomas, E-mail: braunbeck@uni-hd.de [Aquatic Ecology and Toxicology Section, Center for Organismal Studies, University of Heidelberg, Im Neuenheimer Feld 230, D-69120 Heidelberg (Germany)

2013-04-15

In order to investigate teratogenic effects, especially on cartilage and bone formation, zebrafish embryos were exposed for 144 h to the dithiocarbamate pesticide disulfiram (20–320 μg/L) and acetic acid hydrazide (0.375–12 g/L), a degradation product of isoniazid. After fixation and full-mount staining, disulfiram could be shown to induce strong cartilage malformations after exposure to ≥ 80 μg/L, whereas acetic acid hydrazide caused cartilage alterations only from 1.5 g/L. Undulating notochords occurred after exposure to disulfiram even at the lowest test concentration of 20 μg/L, whereas at the two lowest concentrations of acetic acid hydrazide (0.375 and 0.75 g/L) mainly fractures of the notochord were observed. Concentrations of acetic acid hydrazide ≥ 1.5 g/L resulted in undulated notochords similar to disulfiram. Cartilages and ossifications of the cranium, including the cleithrum, were individually analyzed assessing the severity of malformation and the degree of ossification in a semi-quantitative approach. Cartilages of the neurocranium such as the ethmoid plate proved to be more stable than cartilages of the pharyngeal skeleton such as Meckel's cartilage. Hence, ossification proved significantly more susceptible than cartilage. The alterations induced in the notochord as well as in the cranium might well be of ecological relevance, since notochord malformation is likely to result in impaired swimming and cranial malformation might compromise regular food uptake. - Highlights: ► Disulfiram and acetic acid hydrazide as notochord, cartilage and bone teratogens ► Zebrafish embryos to model effects on single cartilages and bones in the head ► LC50 calculation and head length measurements after six days post-fertilization ► Lethality, head length and teratogenic effects are dose-dependent. ► Cartilages of the neurocranium are the most stable elements in the head.

Cartilage and bone malformations in the head of zebrafish (Danio rerio) embryos following exposure to disulfiram and acetic acid hydrazide

International Nuclear Information System (INIS)

Strecker, Ruben; Weigt, Stefan; Braunbeck, Thomas

2013-01-01

In order to investigate teratogenic effects, especially on cartilage and bone formation, zebrafish embryos were exposed for 144 h to the dithiocarbamate pesticide disulfiram (20–320 μg/L) and acetic acid hydrazide (0.375–12 g/L), a degradation product of isoniazid. After fixation and full-mount staining, disulfiram could be shown to induce strong cartilage malformations after exposure to ≥ 80 μg/L, whereas acetic acid hydrazide caused cartilage alterations only from 1.5 g/L. Undulating notochords occurred after exposure to disulfiram even at the lowest test concentration of 20 μg/L, whereas at the two lowest concentrations of acetic acid hydrazide (0.375 and 0.75 g/L) mainly fractures of the notochord were observed. Concentrations of acetic acid hydrazide ≥ 1.5 g/L resulted in undulated notochords similar to disulfiram. Cartilages and ossifications of the cranium, including the cleithrum, were individually analyzed assessing the severity of malformation and the degree of ossification in a semi-quantitative approach. Cartilages of the neurocranium such as the ethmoid plate proved to be more stable than cartilages of the pharyngeal skeleton such as Meckel's cartilage. Hence, ossification proved significantly more susceptible than cartilage. The alterations induced in the notochord as well as in the cranium might well be of ecological relevance, since notochord malformation is likely to result in impaired swimming and cranial malformation might compromise regular food uptake. - Highlights: ► Disulfiram and acetic acid hydrazide as notochord, cartilage and bone teratogens ► Zebrafish embryos to model effects on single cartilages and bones in the head ► LC50 calculation and head length measurements after six days post-fertilization ► Lethality, head length and teratogenic effects are dose-dependent. ► Cartilages of the neurocranium are the most stable elements in the head

A review of simulation platforms in surgery of the temporal bone.

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Bhutta, M F

2016-10-01

Surgery of the temporal bone is a high-risk activity in an anatomically complex area. Simulation enables rehearsal of such surgery. The traditional simulation platform is the cadaveric temporal bone, but in recent years other simulation platforms have been created, including plastic and virtual reality platforms. To undertake a review of simulation platforms for temporal bone surgery, specifically assessing their educational value in terms of validity and in enabling transition to surgery. Systematic qualitative review. Search of the Pubmed, CINAHL, BEI and ERIC databases. Assessment of reported outcomes in terms of educational value. A total of 49 articles were included, covering cadaveric, animal, plastic and virtual simulation platforms. Cadaveric simulation is highly rated as an educational tool, but there may be a ceiling effect on educational outcomes after drilling 8-10 temporal bones. Animal models show significant anatomical variation from man. Plastic temporal bone models offer much potential, but at present lack sufficient anatomical or haptic validity. Similarly, virtual reality platforms lack sufficient anatomical or haptic validity, but with technological improvements they are advancing rapidly. At present, cadaveric simulation remains the best platform for training in temporal bone surgery. Technological advances enabling improved materials or modelling mean that in the future plastic or virtual platforms may become comparable to cadaveric platforms, and also offer additional functionality including patient-specific simulation from CT data. © 2015 John Wiley & Sons Ltd.

Tympanic plate fractures in temporal bone trauma: prevalence and associated injuries.

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Wood, C P; Hunt, C H; Bergen, D C; Carlson, M L; Diehn, F E; Schwartz, K M; McKenzie, G A; Morreale, R F; Lane, J I

2014-01-01

The prevalence of tympanic plate fractures, which are associated with an increased risk of external auditory canal stenosis following temporal bone trauma, is unknown. A review of posttraumatic high-resolution CT temporal bone examinations was performed to determine the prevalence of tympanic plate fractures and to identify any associated temporal bone injuries. A retrospective review was performed to evaluate patients with head trauma who underwent emergent high-resolution CT examinations of the temporal bone from July 2006 to March 2012. Fractures were identified and assessed for orientation; involvement of the tympanic plate, scutum, bony labyrinth, facial nerve canal, and temporomandibular joint; and ossicular chain disruption. Thirty-nine patients (41.3 ± 17.2 years of age) had a total of 46 temporal bone fractures (7 bilateral). Tympanic plate fractures were identified in 27 (58.7%) of these 46 fractures. Ossicular disruption occurred in 17 (37.0%). Fractures involving the scutum occurred in 25 (54.4%). None of the 46 fractured temporal bones had a mandibular condyle dislocation or fracture. Of the 27 cases of tympanic plate fractures, 14 (51.8%) had ossicular disruption (P = .016) and 18 (66.6%) had a fracture of the scutum (P = .044). Temporomandibular joint gas was seen in 15 (33%) but was not statistically associated with tympanic plate fracture (P = .21). Tympanic plate fractures are commonly seen on high-resolution CT performed for evaluation of temporal bone trauma. It is important to recognize these fractures to avoid the preventable complication of external auditory canal stenosis and the potential for conductive hearing loss due to a fracture involving the scutum or ossicular chain.

Giant osteoblastoma of temporal bone: case report

Directory of Open Access Journals (Sweden)

FIGUEIREDO EBERVAL GADELHA

1998-01-01

Full Text Available Benign osteoblastoma is an uncommon bone tumor accounting for approximately 1% of all bone tumors. There are only 35 cases of skull osteoblastoma reported in the literature. We describe the case of a 23 year old male with a giant osteoblastoma of temporal bone submitted to a total removal of the tumor after an effective embolization of all external carotid branches. The authors discuss diagnostic and management aspects of this uncommon skull tumor.

Cerebrospinal otorrhoea--a temporal bone report.

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Walby, A P

1988-05-01

Spontaneous cerebrospinal otorrhoea is a rare complication of a cholesteatoma. The histological findings in a temporal bone from such a case are reported. The cholesteatoma had eroded deeply through the vestibule into the internal auditory meatus.

Content Validity of Temporal Bone Models Printed Via Inexpensive Methods and Materials.

Science.gov (United States)

Bone, T Michael; Mowry, Sarah E

2016-09-01

Computed tomographic (CT) scans of the 3-D printed temporal bone models will be within 15% accuracy of the CT scans of the cadaveric temporal bones. Previous studies have evaluated the face validity of 3-D-printed temporal bone models designed to train otolaryngology residents. The purpose of the study was to determine the content validity of temporal bone models printed using inexpensive printers and materials. Four cadaveric temporal bones were randomly selected and clinical temporal bone CT scans were obtained. Models were generated using previously described methods in acrylonitrile butadiene styrene (ABS) plastic using the Makerbot Replicator 2× and Hyrel printers. Models were radiographically scanned using the same protocol as the cadaveric bones. Four images from each cadaveric CT series and four corresponding images from the model CT series were selected, and voxel values were normalized to black or white. Scan slices were compared using PixelDiff software. Gross anatomic structures were evaluated in the model scans by four board certified otolaryngologists on a 4-point scale. Mean pixel difference between the cadaver and model scans was 14.25 ± 2.30% at the four selected CT slices. Mean cortical bone width difference and mean external auditory canal width difference were 0.58 ± 0.66 mm and 0.55 ± 0.46 mm, respectively. Expert raters felt the mastoid air cells were well represented (2.5 ± 0.5), while middle ear and otic capsule structures were not accurately rendered (all averaged bones for training residents in cortical mastoidectomies, but less effective for middle ear procedures.

Three-dimensional measurement of temporal bone by using personal computer

International Nuclear Information System (INIS)

Kimura, Hiroki; Murata, Kiyotaka; Isono, Michio; Azuma, Hiroji; Itou, Akihiko

1996-01-01

Measurement of anatomical indices in human temporal bone has been reported only sporadically using high resolution CT. We developed a method for measuring such indices by computer assisted processing of images obtained by high resolusion CT. Intensive measurement of distances between all anatomical points in the entire temporal bone structure became possible with this method. (author)

A case of a temporal bone meningioma presenting as a serous otitis media

International Nuclear Information System (INIS)

Nicolay, Simon; De Foer, Bert; Bernaerts, Anja; Van Dinther, Joost; Parizel, Paul M

2014-01-01

We report the imaging features of a case of a temporal bone meningioma extending into the middle ear cavity and clinically presenting as a serous otitis media. Temporal bone meningioma extending in the mastoid or the middle ear cavity, however, is very rare. In case of unexplained or therapy-resistant serous otitis media and a nasopharyngeal tumor being ruled out, a temporal bone computed tomography (CT) should be performed. If CT findings are suggestive of a temporal bone meningioma, a magnetic resonance imaging (MRI) examination with gadolinium will confirm diagnosis and show the exact extension of the lesion

X-ray diagnosis in temporal bone anomalies

International Nuclear Information System (INIS)

Schratter, M.; Canigiani, G.; Swoboda, H.; Brunner, E.

1988-01-01

The X-ray findings in temporal bone anomalies are reviewed. Radiological procedure and examination technique are presented, as are symptoms of important anomalies. The methods available are plain film X-ray of temporal bone, multi-directional tomography, and high-resolution CT. Although some of the abnormalities are visible even in plain films, consistent use of conventional tomography or CT is necessary for correct diagnosis. This procedure is indicated not only when an abnormality is clinically obvious, but also in all cases of unexplained hearing loss without evidence of acquired disease. The advantage of CT over conventional tomography is that soft tissue anomalies, such as primary cholesteatoma or tumor simulating vascular abnormalities, can be demonstrated. In these cases CT is obligatory. (orig.) [de

Preoperative diagnosis and surgical strategy in congenital auditory ossicular malformation of 26 ears

International Nuclear Information System (INIS)

Kanazawa, Yuji; Naito, Yasushi; Shinohara, Shogo; Fujiwara, Keizo; Kikuchi, Masahiro; Yamazaki, Hiroshi; Kurihara, Risa; Kishimoto, Ippei

2012-01-01

We retrospectively analyzed 26 ears of 21 subjects having auditory ossicular malformation and who had undergone auditory reconstruction between April 2004 and December 2010 at our clinic. We checked preoperative condition, pathological classification, surgical procedure, and hearing improvement. We could predict pathological conditions precisely from preoperative computed tomography (CT), including incudostapedial disconnection (9/12, 75%) and malleus and/or incus fixation (7/12, 58%), which tended to be present in external ear malformation, and stapes footplate fixation (0/12, 0%). We could not, however, predict complex malformation (0/8, 0%). Overall success was 90% (18/20) in the 20 ears observed for at least 1 year. In the 2 ears without improved hearing, the first had congenital cholesteatoma and no stapes superstructure, was treated with type IV tympanoplasty. The second had malleus, incus, and stapes fixation and discontinuity between the incus and stapes, and was treated with type III tympanoplasty and stapes mobilization. Preoperative diagnosis is difficult in mixed congenital auditory ossicular malformation, especially stapes footplate fixation, possibly requiring unexpected procedures, with a poor hearing outcome. Preoperative status must thus be evaluated precisely using hearing, tympanometry, acoustic reflex test, and CT. Temporal bone CT and external ear findings are useful in diagnosing middle-ear malformation. Subjects' informed consent should also be obtained due to the possible need for changing procedure based on findings during surgery. (author)

Face and content validation of a novel three-dimensional printed temporal bone for surgical skills development.

Science.gov (United States)

Da Cruz, M J; Francis, H W

2015-07-01

To assess the face and content validity of a novel synthetic, three-dimensional printed temporal bone for surgical skills development and training. A synthetic temporal bone was printed using composite materials and three-dimensional printing technology. Surgical trainees were asked to complete three structured temporal bone dissection exercises. Attitudes and impressions were then assessed using a semi-structured questionnaire. Previous cadaver and real operating experiences were used as a reference. Trainees' experiences of the synthetic temporal bone were analysed in terms of four domains: anatomical realism, usefulness as a training tool, task-based usefulness and overall reactions. Responses across all domains indicated a high degree of acceptance, suggesting that the three-dimensional printed temporal bone was a useful tool in skills development. A sophisticated three-dimensional printed temporal bone that demonstrates face and content validity was developed. The efficiency in cost savings coupled with low associated biohazards make it likely that the printed temporal bone will be incorporated into traditional temporal bone skills development programmes in the near future.

Suspected fetal skeletal malformations or bone diseases: how to explore

International Nuclear Information System (INIS)

Cassart, Marie

2010-01-01

Skeletal dysplasias are a heterogeneous and complex group of conditions that affect bone growth and development and result in various anomalies in shape and size of the skeleton. Although US has proved reliable for the prenatal detection of skeletal abnormalities, the precise diagnosis of a dysplasia is often difficult to make before birth (especially in the absence of a familial history) due to their various phenotypic presentations, the variability in the time at which they manifest and often, the lack of precise molecular diagnosis. In addition to the accuracy of the antenatal diagnosis, it is very important to establish a prognosis. This is a clinically relevant issue as skeletal dysplasias may be associated with severe disability and may even be lethal. We will therefore describe the respective role of two-dimensional (2-D) US, three-dimensional (3-D) US and CT in the antenatal assessment of skeletal malformations. (orig.)

CT of temporal bone - IV. inner ear

International Nuclear Information System (INIS)

Kwon, Jae Yoon; Sung, Kyu Bo; Youn, Eun Kyoung; Park, Youn Kyeung; Lee, Young Uk

1990-01-01

Temporal bone CT was done in 697 patients from April 1985 to October 1989. The abnormal findings were seen in 453 patients, which were chronic otitis media in 355 patients, fracture in 49 patients and congenital anomaly in 44 patients, etc. The abnormal findings of inner ear were observed on 46 patients. The results were summarized as follows : 1. The incidence of inner ear involvement by chronic otitis media was 7.3% (26/355 : labyrinthine fistula in 17 patients, labyrinthitis ossificans in 9 patients). Labyrinthine fistula was most commonly located on lateral semicircular canal (15/17, 88.2%). 2. Fusion of vestibule with lateral semicircular canal and formation of common cavity was demonstrated incidentally in 5 patients (0.7% of total number of temporal bone CT), and bilateral in 3 patients. 3. The incidence of inner ear anomaly in congenital ear anomaly was 11.4% (5/44). All cases were bilateral and three patients showed associated middle ear anomaly. 4. The incidence of involvement of bony labyrinth in temporal bone fracture was 10.2% (5/49). Labyrinthine fracture was seen all patients of transverse(3) and mixed fracture(1). In longitudinal fracture, labyrinthine fracture was seen in 2.2% (1/45). 5. Others were traumatic labyrinthitis ossificans(1), intracanalicular acoustic neuroma(3) and facial nerve neuroma(1)

Surgical Management and Long-Term Seizure Outcome After Surgery for Temporal Lobe Epilepsy Associated with Cerebral Cavernous Malformations.

Science.gov (United States)

Yang, Peng-Fan; Pei, Jia-Sheng; Jia, Yan-Zeng; Lin, Qiao; Xiao, Hui; Zhang, Ting-Ting; Zhong, Zhong-Hui

2018-02-01

Operative strategies for cerebral cavernous malformation (CCM)-associated temporal lobe epilepsy and timing of surgical intervention continue to be debated. This study aimed to establish an algorithm to evaluate the efficacy of surgical intervention strategies, to maximize positive surgical outcomes and minimize postsurgical neurologic deficits. 47 patients having undergone operation for CCM-associated temporal lobe epilepsy were retrospectively reviewed. They had received a diagnostic series for seizure localization, including long-term video electroencephalography (vEEG), high-resolution magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT). In patients with mesial temporal lobe CCMs, the involved structures (amygdala, hippocampus, or parahippocampal gyrus) were resected in addition to the lesions. Patients with neocortical epileptogenic CCM underwent extended lesionectomy guided by intraoperative electrocorticography; further performance of amygdalohippocampectomy depended on the extent of hippocampal epileptogenicity. The study cohort contained 28 patients with drug-resistant epilepsy (DRE), 12 with chronic epilepsy (CE), and 7 with sporadic seizure (SS). Normal temporal lobe metabolism was seen in 7/7 patients of the SS group. Hypometabolism was found in all patients with chronic disease except for those with posterior inferior and middle temporal gyrus cavernous malformations (CMs). Of the 31 patients with superficial neocortical CCM, 7 had normal PET without hippocampal sclerosis, 14 had ipsilateral temporal lobe hypometabolism without hippocampal sclerosis, and 10 had obvious hippocampal sclerosis and hypometabolism. Seizure freedom in DRE, CE, and SS was 82.1%, 75%, and 100%, respectively. A significant difference was found between lesion laterality and postoperative seizure control; the rate was lower in left-sided cases because of less aggressive resection. Our study demonstrates that the data from the

Anatomia do osso temporal de ovelhas sob aspectos didáticos Lambs' temporal bone anatomy under didactic aspects

Directory of Open Access Journals (Sweden)

André Gurr

2011-02-01

Full Text Available É difícil encontrar ossos temporais humanos para o ensino de cirurgia otológica. Ossos temporais de ovelhas podem representar uma possível alternativa. MATERIAIS E MÉTODOS: Os ossos temporais de ovelhas foram dissecados em um programa convencional de dissecação de osso temporal no laboratório. Incluímos mastoidectomia, abordagens endaurais, mas também analisamos a aparência externa, o meato acústico externo e o hipotímpano. Algumas etapas são diferentes das preparações de ossos humanos. Os resultados morfométricos foram comparados à anatomia conhecida de humanos para verificar se o osso temporal de ovelhas seria utilizável para o ensino de cirurgia otológica. RESULTADOS: O osso temporal de ovelhas parece menor do que o humano. Encontramos uma área bolhosa se estendendo ao hipotímpano abaixo do meato acústico externo. A membrana timpânica é muito semelhante à humana. O meato acústico externo é menor e mais curto. A cadeia ossicular exibe analogias para com a humana. DISCUSSÃO: Esse estudo mostra que especificamente o ouvido médio, a membrana timpânica e o conduto auditivo externo são morfologicamente semelhantes às suas contrapartidas encontradas nos ossos temporais humanos. A ovelha parece ser um modelo viável para o ensino da anatomia do ouvido. A menor escala de algumas estruturas, especialmente dos componentes externos do osso temporal representa uma desvantagem. CONCLUSÕES: A ovelha parece representar uma alternativa viável no ensino de cirurgia otológica.Human temporal bones in teaching ear surgery are rare. The lamb's temporal bone might be a possible alternative. MATERIAL AND METHODS: Temporal bones of the lamb were dissected with a typical temporal bone lab drilling program. We included a mastoidectomy, endaural approaches, but also analyzed the outer appearance, the external ear canal and the hypotympanon. Some steps differed from preparation done in humans. The morphometric results were compared to

High-resolution CT of temporal bone trauma: review of 38 cases

International Nuclear Information System (INIS)

Hiroual, M.R.; Zougarhi, A.; Cherif Idrissi El Ganouni, N.; Essadki, O.; Ousehal, A.; Tijani Adil, O.; Maliki, O.; Aderdour, L.; Raji, A.

2010-01-01

Purpose Temporal bone trauma is frequent but difficult to assess due to the diversity of clinical presentations and complex anatomy. We have sought to assess the different types of fractures and complications on high-resolution CT. Materials and methods Descriptive retrospective study over a 24 month period performed in the ENT radiology section of the Mohammed 6 university medical center in Marrakech. A total of 38 cases of temporal bone trauma were reviewed. All patients underwent ENT evaluation and high-resolution CT of the temporal bone using 1 mm axial and coronal sections. Results Mean patient age was 33 years (range: 14-55 years) with male predominance (sex ratio: 36/2). Clinical symptoms were mainly otorrhagia and conductive hearing loss. Oblique extra-labyrinthine fractures were most frequent. Two cases of pneumo-labyrinth were noted. Management was conservative in most cases with deafness in 3 cases. Conclusion High-resolution CT of the temporal bone provides accurate depiction of lesions explaining the clinical symptoms and helps guide management. MRI is complimentary to further assess the labyrinth and VII-VIII nerve complex. (author)

Statistical model estimating the occurrence of otitis media from temporal bone pneumatization

DEFF Research Database (Denmark)

Homøe, P; Lynnerup, N; Rasmussen, N

1994-01-01

In order to investigate the relationship between the pneumatization of temporal bones and the occurrence of otitis media in Greenlandic Inuit, 36 Greenlandic Inuit were examined by radiography of the temporal bones. The pneumatized cell area was measured planimetrically. All subjects answered...

Papercraft temporal bone in the first step of anatomy education.

Science.gov (United States)

Hiraumi, Harukazu; Sato, Hiroaki; Ito, Juichi

2017-06-01

(1) To compare temporal bone anatomy comprehension taught to speech therapy students with or without a papercraft model. (2) To explore the effect of papercraft simulation on the understanding of surgical approaches in first-year residents. (1) One-hundred and ten speech therapy students were divided into three classes. The first class was taught with a lecture only. The students in the second class were given a lecture and a papercraft modeling task without instruction. The third class modeled a papercraft with instruction after the lecture. The students were tested on their understanding of temporal bone anatomy. (2) A questionnaire on the understanding of surgical approaches was completed by 10 residents before and after the papercraft modeling. The papercraft models were cut with scissors to simulate surgical approaches. (1) The average scores were 4.4/8 for the first class, 4.3/8 for the second class, and 6.3/8 for the third class. The third class had significantly better results than the other classes (ppapercraft modeling and cutting were 2.6/7 and 4.9/7, respectively. The numerical rating scale score significantly improved (ppapercraft temporal bone model is effective in the first step of learning temporal bone anatomy and surgical approaches. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Utility of 3D printed temporal bones in pre-surgical planning for complex BoneBridge cases.

Science.gov (United States)

Mukherjee, Payal; Cheng, Kai; Flanagan, Sean; Greenberg, Simon

2017-08-01

With the advent of single-sided hearing loss increasingly being treated with cochlear implantation, bone conduction implants are reserved for cases of conductive and mixed hearing loss with greater complexity. The BoneBridge (BB, MED-EL, Innsbruck, Austria) is an active fully implantable device with no attenuation of sound energy through soft tissue. However, the floating mass transducer (FMT) part of the device is very bulky, which limits insertion in complicated ears. In this study, 3D printed temporal bones of patients were used to study its utility in preoperative planning on complicated cases. Computed tomography (CT) scans of 16 ears were used to 3D print their temporal bones. Three otologists graded the use of routine preoperative planning provided by MED-EL and that of operating on the 3D printed bone of the patient. Data were collated to assess the advantage and disadvantage of the technology. There was a statistically significant benefit in using 3D printed temporal bones to plan surgery for difficult cases of BoneBridge surgery compared to the current standard. Surgeons preferred to have the printed bones in theatre to plan their drill sites and make the transition of the planning to the patient's operation more precise. 3D printing is an innovative use of technology in the use of preoperative planning for complex ear surgery. Surgical planning can be done on the patient's own anatomy which may help to decrease operating time, reduce cost, increase surgical precision and thus reduce complications.

Aggressive osteoblastoma in mastoid process of temporal bone with facial palsy

Directory of Open Access Journals (Sweden)

Manoj Jain

2013-01-01

Full Text Available Osteoblastoma is an uncommon primary bone tumor with a predilection for posterior elements of spine. Its occurrence in temporal bone and middle ear is extremely rare. Clinical symptoms are non-specific and cranial nerve involvement is uncommon. The cytomorphological features of osteoblastoma are not very well defined and the experience is limited to only few reports. We report an interesting and rare case of aggressive osteoblastoma, with progressive hearing loss and facial palsy, involving the mastoid process of temporal bone and middle ear along with the description of cyto-morphological features.

Incidental internal carotid artery calcifications on temporal bone CT in children

International Nuclear Information System (INIS)

Koch, Bernadette; Jones, Blaise; Blackham, Aaron

2007-01-01

Incidental internal carotid artery (ICA) calcifications are occasionally noted on CT images of the brain and temporal bone. In adults, incidental calcifications have been correlated with increased incidence of hypercholesterolemia, cardiac disease, diabetes and carotid stenosis. To determine the incidence of incidental calcifications of the carotid siphon on temporal bone CT in children. We retrospectively reviewed 24 months of consecutive temporal bone CT examinations in children aged 18 years and younger. CT examinations on 663 patients were reviewed and the presence or absence of ICA calcifications was ranked as absent, questionable or definitive. In patients in whom definitive calcifications were identified, hospital charts were reviewed for evidence of diabetes mellitus, hypercholesterolemia, hypertriglyceridemia, hyperlipidemia and chronic renal disease as potential causes of early atherosclerosis. Of the 663 patients, 25% had definitive calcifications within the wall of the ICA: 6% of children younger than 2 years and 28% of children 12-19 years of age. Incidentally noted ICA calcifications are a common finding on temporal bone CT in children, most likely a physiologic response to turbulent flow at natural bends in the artery rather than secondary to underlying disease predisposing to early atherosclerotic calcification. (orig.)

Incidental internal carotid artery calcifications on temporal bone CT in children

Energy Technology Data Exchange (ETDEWEB)

Koch, Bernadette; Jones, Blaise [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Blackham, Aaron [University of Cincinnati College of Medicine, Cincinnati, OH (United States)

2007-02-15

Incidental internal carotid artery (ICA) calcifications are occasionally noted on CT images of the brain and temporal bone. In adults, incidental calcifications have been correlated with increased incidence of hypercholesterolemia, cardiac disease, diabetes and carotid stenosis. To determine the incidence of incidental calcifications of the carotid siphon on temporal bone CT in children. We retrospectively reviewed 24 months of consecutive temporal bone CT examinations in children aged 18 years and younger. CT examinations on 663 patients were reviewed and the presence or absence of ICA calcifications was ranked as absent, questionable or definitive. In patients in whom definitive calcifications were identified, hospital charts were reviewed for evidence of diabetes mellitus, hypercholesterolemia, hypertriglyceridemia, hyperlipidemia and chronic renal disease as potential causes of early atherosclerosis. Of the 663 patients, 25% had definitive calcifications within the wall of the ICA: 6% of children younger than 2 years and 28% of children 12-19 years of age. Incidentally noted ICA calcifications are a common finding on temporal bone CT in children, most likely a physiologic response to turbulent flow at natural bends in the artery rather than secondary to underlying disease predisposing to early atherosclerotic calcification. (orig.)

la dysplasie fibreuse du rocher fibrous dysplasia of the temporal bone

African Journals Online (AJOL)

view of the disease and its manifestations in the temporal bone. Ann. OtolRhinolLaryngol 1982;92(Suppl.): 1–52. 5- Papadakis CE, Skoulakis CE, Propakapis EP, et al. Fibrous dyspla- sia of the temporal bone: report of a case and review of its characteris- tics. Ear Nose Throat J, 2000;79:52–57. 6- Yang H, Chen S, Zheng Y, ...

Pre-operative simulation of pediatric mastoid surgery with 3D-printed temporal bone models.

Science.gov (United States)

Rose, Austin S; Webster, Caroline E; Harrysson, Ola L A; Formeister, Eric J; Rawal, Rounak B; Iseli, Claire E

2015-05-01

As the process of additive manufacturing, or three-dimensional (3D) printing, has become more practical and affordable, a number of applications for the technology in the field of pediatric otolaryngology have been considered. One area of promise is temporal bone surgical simulation. Having previously developed a model for temporal bone surgical training using 3D printing, we sought to produce a patient-specific model for pre-operative simulation in pediatric otologic surgery. Our hypothesis was that the creation and pre-operative dissection of such a model was possible, and would demonstrate potential benefits in cases of abnormal temporal bone anatomy. In the case presented, an 11-year-old boy underwent a planned canal-wall-down (CWD) tympano-mastoidectomy for recurrent cholesteatoma preceded by a pre-operative surgical simulation using 3D-printed models of the temporal bone. The models were based on the child's pre-operative clinical CT scan and printed using multiple materials to simulate both bone and soft tissue structures. To help confirm the models as accurate representations of the child's anatomy, distances between various anatomic landmarks were measured and compared to the temporal bone CT scan and the 3D model. The simulation allowed the surgical team to appreciate the child's unusual temporal bone anatomy as well as any challenges that might arise in the safety of the temporal bone laboratory, prior to actual surgery in the operating room (OR). There was minimal variability, in terms of absolute distance (mm) and relative distance (%), in measurements between anatomic landmarks obtained from the patient intra-operatively, the pre-operative CT scan and the 3D-printed models. Accurate 3D temporal bone models can be rapidly produced based on clinical CT scans for pre-operative simulation of specific challenging otologic cases in children, potentially reducing medical errors and improving patient safety. Copyright © 2015 Elsevier Ireland Ltd. All rights

Temporal bone dissection simulator for training pediatric otolaryngology surgeons

Science.gov (United States)

Tabrizi, Pooneh R.; Sang, Hongqiang; Talari, Hadi F.; Preciado, Diego; Monfaredi, Reza; Reilly, Brian; Arikatla, Sreekanth; Enquobahrie, Andinet; Cleary, Kevin

2017-03-01

Cochlear implantation is the standard of care for infants born with severe hearing loss. Current guidelines approve the surgical placement of implants as early as 12 months of age. Implantation at a younger age poses a greater surgical challenge since the underdeveloped mastoid tip, along with thin calvarial bone, creates less room for surgical navigation and can result in increased surgical risk. We have been developing a temporal bone dissection simulator based on actual clinical cases for training otolaryngology fellows in this delicate procedure. The simulator system is based on pre-procedure CT (Computed Tomography) images from pediatric infant cases (hospital. The simulator includes: (1) simulation engine to provide the virtual reality of the temporal bone surgery environment, (2) a newly developed haptic interface for holding the surgical drill, (3) an Oculus Rift to provide a microscopic-like view of the temporal bone surgery, and (4) user interface to interact with the simulator through the Oculus Rift and the haptic device. To evaluate the system, we have collected 10 representative CT data sets and segmented the key structures: cochlea, round window, facial nerve, and ossicles. The simulator will present these key structures to the user and warn the user if needed by continuously calculating the distances between the tip of surgical drill and the key structures.

Temporal bone trauma and complications: computed tomography findings

Energy Technology Data Exchange (ETDEWEB)

Costa, Ana Maria Doffemond; Gaiotti, Juliana Oggioni; Couto, Caroline Laurita Batista; Gomes, Natalia Delage; Diniz, Renata Lopes Furletti Caldeira; Motta, Emilia Guerra Pinto Coelho, E-mail: anadoffemond@yahoo.com.br [Hospital Mater Dei, Belo Horizonte, MG (Brazil). Unit of Radiology and Imaging Diagnosis

2013-03-15

Most temporal bone fractures result from high-energy blunt head trauma, and are frequently related to other skull fractures or to polytrauma. Fractures and displacements of ossicular chain in the middle ear represent some of the main complications of temporal bone injury, and hence they will be more deeply approached in the present article. Other types of injuries include labyrinthine fractures, dural fistula, facial nerve paralysis and extension into the carotid canal. Computed tomography plays a fundamental role in the initial evaluation of polytrauma patients, as it can help to identify important structural injuries that may lead to severe complications such as sensorineural hearing loss, conductive hearing loss, dizziness and balance dysfunction, perilymphatic fistulas, facial nerve paralysis, vascular injury and others. (author)

Temporal bone trauma and complications: computed tomography findings

International Nuclear Information System (INIS)

Costa, Ana Maria Doffemond; Gaiotti, Juliana Oggioni; Couto, Caroline Laurita Batista; Gomes, Natalia Delage; Diniz, Renata Lopes Furletti Caldeira; Motta, Emilia Guerra Pinto Coelho

2013-01-01

Most temporal bone fractures result from high-energy blunt head trauma, and are frequently related to other skull fractures or to polytrauma. Fractures and displacements of ossicular chain in the middle ear represent some of the main complications of temporal bone injury, and hence they will be more deeply approached in the present article. Other types of injuries include labyrinthine fractures, dural fistula, facial nerve paralysis and extension into the carotid canal. Computed tomography plays a fundamental role in the initial evaluation of polytrauma patients, as it can help to identify important structural injuries that may lead to severe complications such as sensorineural hearing loss, conductive hearing loss, dizziness and balance dysfunction, perilymphatic fistulas, facial nerve paralysis, vascular injury and others. (author)

Osteoradionecrosis of the temporal bone with cancer of the middle ear. A case report

International Nuclear Information System (INIS)

Ishida, Katsunori; Sakai, Makoto; Shinkawa, Atsushi

1999-01-01

Osteoradionecrosis of the temporal bone may occur as a result of radiation therapy for head and neck carcinomas or brain tumors. A 64-year-old female received radiation therapy for squamous cell carcinoma of the middle ear 20 years ago, and then she developed osteoradionecrosis of the temporal bone. The patient underwent extensive debridement with removal of sequestrations in the temporal bone and adjacent areas, and abscess drainage. Her postoperative course was satisfactory and there was no progression of the disease. (author)

Conventional diagnostic imaging of the temporal bone. A historical review

International Nuclear Information System (INIS)

Canigiani, G.

1997-01-01

The Viennese Medical School played an important role in the development of radiological examinations and signs of the temporal bone with conventional X-rays. Famous pioneers include E.G. Mayer (1893-1969) and L. Psenner (1910-1986). Nowadays conventional X-rays and tomography have lost their important role in diagnostic radiology of the temporal bone, but the basic principles established in those early years of radiology are still used now. This statement is correct not only for conventional X-rays, but particularly for 'poly'-tomography in comparison with CT. (orig.) [de

Intraosseous focal venous malformation of the mandibular body: Cone beam computed tomography planning followed by piezoelectric knife resection and free bone graft reconstruction

Directory of Open Access Journals (Sweden)

Nikolić Živorad S.

2017-01-01

Full Text Available Introduction. Intraosseous vascular malformation could be life-threatening due to uncontrolled hemorrhage after tooth extraction. According to biological behavior of this lesion, adequate diagnostic and treatment strategies are necessary in order to avoid possible complications. We reported cone beam computed tomography (CBCT planning of an urgent en bloc resection of an intraosseous venous malformation by piezoelectric knife. Case report. A 55-year-old man was submitted to CBCT planning followed by piezoelectric knife resection of an intraosseous focal venous malformation of the mandibular body. Immediate reconstruction of the defect using iliac bone free graft was performed. The surgical treatment was uneventful and a 2-year follow-up revealed no signs of recurrence. Conclusion. Piezoelectric knife could provide precise, safe and bloodless procedure which is especially important in this pathology. Advantages of this technique are: lower risk of damaging soft tissue structures, precise osteotomy and bloodless surgery. Moreover, using piezosurgery bone knife, blood transfusion and blood transmitted diseases could be avoided. This case highlights the importance of CBCT as planning tool for resection of the mandible, using piezoelectric knife as safe method to achieve bloodless surgery. [Project of the Serbian Ministry of Education, Science and Technological Development, Grant no. 175075

Semicircular canal dehiscence: Frequency and distribution on temporal bone CT and its relationship with the clinical outcomes

International Nuclear Information System (INIS)

Elmali, Muzaffer; Polat, Ahmet Veysel; Kucuk, Harun; Atmaca, Sinan; Aksoy, Ahmet

2013-01-01

Purpose: In this study, we aimed to investigate the frequency of SCD and its distribution and relationship with clinical outcomes on thin-section CT of the temporal bone. Materials and methods: Digital temporal bone CT images of 850 consecutive patients (1700 temporal bone CTs, 5100 SCs) who presented with a range of complaints such as vertigo, deafness, ear pain, fullness, and discharge between January 2008 and December 2011 were re-evaluated. Axial and oblique coronal reconstruction images of the temporal bone were made with a reconstruction thickness of 0.5 mm. Additionally, superior SC was evaluated in two perpendicular planes. Results: Out of 850 patients, 70 had completely normal temporal bone CT. Ninety-three patients had at least one SCD. In the temporal bone-based evaluation, 119 (26 bilateral, 67 unilateral) of 1700 temporal bones (7%) showed dehiscence. The SC-based evaluation revealed 125 SCD (2.5%) in 5100 SCs. The total number and rates of SCD were as follows: superior 103 (82.4%), posterior 13 (10.4%), and lateral nine (7.2%). Twenty of the 93 patients with SCD (21.5%) revealed no other findings on their temporal bone CTs. We determined a significant correlation between vestibular complaints, conductive hearing loss and SCD but there was no correlation between mixed, sensorineural hearing loss and SCD. Conclusion: We determined the frequency of SCD in 11% of patients and 7% of temporal bones. With regards to the distribution, the superior SC showed the highest dehiscence rate (82.4%). We found a significant correlation between vestibular symptoms, conductive hearing loss and SCD

Semicircular canal dehiscence: Frequency and distribution on temporal bone CT and its relationship with the clinical outcomes

Energy Technology Data Exchange (ETDEWEB)

Elmali, Muzaffer, E-mail: muzafel@yahoo.com.tr [Department of Radiology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey); Polat, Ahmet Veysel, E-mail: veyselp@hotmail.com [Department of Radiology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey); Kucuk, Harun, E-mail: hardrmd@yahoo.com [Department of Otorhinolaryngology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey); Atmaca, Sinan, E-mail: sinanatmaca@yahoo.com [Department of Otorhinolaryngology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey); Aksoy, Ahmet, E-mail: toxocara47@hotmail.com [Department of Otorhinolaryngology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey)

2013-10-01

Purpose: In this study, we aimed to investigate the frequency of SCD and its distribution and relationship with clinical outcomes on thin-section CT of the temporal bone. Materials and methods: Digital temporal bone CT images of 850 consecutive patients (1700 temporal bone CTs, 5100 SCs) who presented with a range of complaints such as vertigo, deafness, ear pain, fullness, and discharge between January 2008 and December 2011 were re-evaluated. Axial and oblique coronal reconstruction images of the temporal bone were made with a reconstruction thickness of 0.5 mm. Additionally, superior SC was evaluated in two perpendicular planes. Results: Out of 850 patients, 70 had completely normal temporal bone CT. Ninety-three patients had at least one SCD. In the temporal bone-based evaluation, 119 (26 bilateral, 67 unilateral) of 1700 temporal bones (7%) showed dehiscence. The SC-based evaluation revealed 125 SCD (2.5%) in 5100 SCs. The total number and rates of SCD were as follows: superior 103 (82.4%), posterior 13 (10.4%), and lateral nine (7.2%). Twenty of the 93 patients with SCD (21.5%) revealed no other findings on their temporal bone CTs. We determined a significant correlation between vestibular complaints, conductive hearing loss and SCD but there was no correlation between mixed, sensorineural hearing loss and SCD. Conclusion: We determined the frequency of SCD in 11% of patients and 7% of temporal bones. With regards to the distribution, the superior SC showed the highest dehiscence rate (82.4%). We found a significant correlation between vestibular symptoms, conductive hearing loss and SCD.

Unbiased Stereologic Estimation of the Spatial Distribution of Paget’s Disease in the Human Temporal Bone

DEFF Research Database (Denmark)

Bloch, Sune Land; Sørensen, Mads Sølvsten

2014-01-01

remodeling around the inner ear space and to compare it with that of otosclerosis in a contemporary context of temporal bone dynamics. MATERIALS AND METHODS: From the temporal bone collection of Massachusetts Eye and Ear Infirmary, 15 of 29 temporal bones with Paget's disease were selected to obtain...... an independent sample. All volume distributions were obtained along the normal axis of capsular bone remodeling activity by the use of vector-based stereology. RESULTS: Pagetic bone remodeling was distributed centrifugally around the inner ear space at the individual and the general level. This pattern...

Evaluation of temporal bone pneumatization on high resolution CT (HRCT) measurements of the temporal bone in normal and otitis media group and their correlation to measurements of internal auditory meatus, vestibular or cochlear aqueduct

International Nuclear Information System (INIS)

Nakamura, Miyako

1988-01-01

High resolution CT axial scans were made at the three levels of the temoral bone 91 cases. These cases consisted of 109 sides of normal pneumatization (NR group) and 73 of poor pneumatization resulted by chronic otitis (OM group). NR group included sensorineural hearing loss cases and/or sudden deafness on the side. Three levels of continuous slicing were chosen at the internal auditory meatus, the vestibular and the cochlear aqueduct, respectively. In each slice two sagittal and two horizontal measurements were done on the outer contour of the temporal bone. At the proper level, diameter as well as length of the internal acoustic meatus, the vestibular or the cochlear aqueduct were measured. Measurements of the temporal bone showed statistically significant difference between NR and OM groups. Correlation of both diameter and length of the internal auditory meatus to the temporal bone measurements were statistically significant. Neither of measurements on the vestibular or the cochlear aqueduct showed any significant correlation to that of the temporal bone. (author)

Temporal Bone Osteomyelitis: The Relationship with Malignant Otitis Externa, the Diagnostic Dilemma, and Changing Trends

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Jia-Cheng Chen

2014-01-01

Full Text Available Fifty-five patients hospitalized for osteomyelitis of the temporal bone between 1990 and 2011 were divided into two study groups: group 1 was patients collected from 1990 to 2001 and group 2 was composed of patients between 2002 and 2011. Clinical diagnostic criteria and epidemiologic data were analyzed to illustrate the altering features of osteomyelitis of the temporal bone. Group 1 patients were characterized by high prevalence of diabetes and more commonly suffered from otalgia, otitis externa and granulation tissue in the external auditory canal and higher positive culture for Pseudomonas aeruginosa. Noticeable changing trends were found between both groups, including declining prevalence of diabetes, fewer patients complaining of pain or presenting with otitis externa, and canal granulation, and increased variety of pathogens in group 2. We should highlight the index of clinical suspicion for osteomyelitis of the temporal bone, even in nondiabetic or immunocompetent patients. Painless otorrhea patients were also at risk of osteomyelitis of the temporal bone, especially patients with previous otologic operation. Increased multiplicity of pathogens amplified the difficulty of diagnosis for osteomyelitis of the temporal bone.

Evaluating the Effect of Virtual Reality Temporal Bone Simulation on Mastoidectomy Performance: A Meta-analysis.

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Lui, Justin T; Hoy, Monica Y

2017-06-01

Background The increasing prevalence of virtual reality simulation in temporal bone surgery warrants an investigation to assess training effectiveness. Objectives To determine if temporal bone simulator use improves mastoidectomy performance. Data Sources Ovid Medline, Embase, and PubMed databases were systematically searched per the PRISMA guidelines. Review Methods Inclusion criteria were peer-reviewed publications that utilized quantitative data of mastoidectomy performance following the use of a temporal bone simulator. The search was restricted to human studies published in English. Studies were excluded if they were in non-peer-reviewed format, were descriptive in nature, or failed to provide surgical performance outcomes. Meta-analysis calculations were then performed. Results A meta-analysis based on the random-effects model revealed an improvement in overall mastoidectomy performance following training on the temporal bone simulator. A standardized mean difference of 0.87 (95% CI, 0.38-1.35) was generated in the setting of a heterogeneous study population ( I 2 = 64.3%, P virtual reality simulation temporal bone surgery studies, meta-analysis calculations demonstrate an improvement in trainee mastoidectomy performance with virtual simulation training.

Evaluation of CT-scanning of the temporal bone in the diagnosis of ear diseases

International Nuclear Information System (INIS)

Murata, Kiyotaka; Isono, Michio; Nishimae, Tadahide; Tamaki, Katsuhiko; Hosoi, Hiroshi; Ohta, Fumihiko

1983-01-01

CT-scanning of 96 temporal bones was carried out to reveal the extension of cholesteatoma, periossicular drainage, fracture lines, enlarged internal acoustic meatus and anomalies of labyrinthine capsules and ossicles. The clinical aspects of CT-scanning of the temporal bone (CTTB) were as follows: 1) Inner ear anomalies were observed in 17 temporal bones of unilateral deafness, high tone loss from unknown origin and fluctuant hearing loss. CTTB may explain the pathology of deafness from unknown origin. 2) Inner ear anomalies may be classified into more detailed groups than before. 3) The extension of cholesteatoma, localization and size of labyrinthine fistula can be estimated prior to surgery. 4) Cholesteatoma in a mastoidectomy cavity may be detected. 5) The malleus and incus may be visualized, although the stapes can hardly be found. 6) Fracture lines of a temporal bone, destruction of the internal acoustic meatus may be clearly detected. (author)

Three-dimensional spiral CT of craniofacial malformations in children

International Nuclear Information System (INIS)

Binaghi, S.; Gudinchet, F.

2000-01-01

Objective. To assess the value of three-dimensional CT (3D CT) in the diagnosis and management of suspected paediatric craniofacial malformations. Materials and methods. Twenty-eight children (12 girls, 16 boys) with a mean age of 4 years, suffering from craniofacial or cervical malformations, underwent craniofacial spiral CT. 3D reformatting was performed using an independent workstation. Results. 3D CT allowed the preoperative evaluation of 16 patients with craniosynostosis and the post-surgical management of 2 patients. 3D CT clearly depicted malformations of the skull base involving the petrous bone in seven patients (four cases of Goldenhar-Gorlin syndrome, one case of Treacher-Collins syndrome and two cases of Crouzon's disease). Four patients with craniofacial clefts were also evaluated. Radiological findings were confirmed by the clinical and intraoperative findings in all patients that underwent surgical treatment. Movement artefacts and ''Lego effect'' related to abrupt change of cranial vault border were encountered and are discussed. Conclusions. 3D CT of the skull can safely and reliably identify paediatric craniofacial malformations involving bone, and it should be used as morphological mapping to help the surgeon in planning surgical treatment. (orig.)

Benign osteoblastoma of the temporal bone: case report and literature review

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Yoo, Hea Jung

2008-09-01

Full Text Available Introduction: Osteoblastoma is defined as a lesion of bone, which is rich in osteoblasts, well-vascularized, and affects mainly the spinal cord. Although it is benign, it presents malignant features and simulates osteosarcoma. It affects mainly young adults between 20 and 40 years old. It rarely compromises the temporal bone. Objective: To present a rare case in the temporal bone with clinical malignant features whose the anatomopathological study has revealed to be a benign tumor. Method: The patient presented a tumor which affected the middle ear cleft, the mastoid, and the right middle fossa. The patient underwent a surgery and, in association with otorhinolaryngology, the tumor was completely dried out from the middle fossa and the middle ear. Fascia lata was used to repair the dural impairment and an acrylic plate was used to cover the bone impairment. Result: Post-surgery evolved positively, however the acrylic plate has moved itself and has stenosed the right external acoustic meato, which had to be removed 3 years later. The patient has had a good followup, which is still being carried through. Conclusion: Osteoblastoma is a multiform tumor that might affect the temporal bone with malignant features, which simulates osteosarcoma, but, in histological terms, it does not present any malignant signals. However, there is a need for a long post-surgery follow-up.

Inflammatory Pseudotumor of the Temporal Bone: Three Cases and a Review of the Literature

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Huiqin Tian

2013-01-01

Full Text Available Inflammatory pseudotumor (IP is a clinically aggressive but histologically benign condition of unknown cause. Its appearance in the temporal bone is uncommon. We present clinical, radiological, and histopathologic findings of three cases originating in the temporal bone. In the first case, a simultaneous IP of the temporal bone and parotid gland was found with histopathologic confirmation. In the second case, an enlarged cervical node, which was also believed to be related to IP, was observed accompanied with the temporal lesion. While the third case presented with chronic suppurative otitis media. Two of them were treated by surgery alone with complete resolve of the diseases. Another one underwent tympanomastoidectomy in combination with oral steroids, radiation, and chemotherapy, but the IP still recurred. A comprehensive review of the literature on clinical features of the temporal pseudotumor was conducted.

Four variations of the Mondini inner ear malformations as seen in microdissections.

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Johnsson, L G; Hawkins, J E; Rouse, R C; Kingsley, T C

1984-01-01

Four variations and degrees of severity of the Mondini malformation were found in the temporal bones from two neonates, one with congenital heart disease and the other with trisomy D, and from one teenager with leukemia: 1) short cochlea and normal vestibular organs; 2) short cochlea and persistent horizontal canal anlage; 3) markedly shortened cochlea with no modiolus, wide internal auditory meatus, and persistent horizontal canal anlage; 4) same as variation 3, but with persistent anlagen in all semicircular canals. Variations 3 and 4 were from the case of trisomy D, in which the left cochlea had a normal hair cell population but few nerve fibers, and the intraganglionic spiral bundle was displaced from Rosenthal's canal to the osseous spiral lamina. The right ear had no cochlear nerve fibers; the organ of Corti was present, but hair cells were unusually small. In the case of trisomy D, both ears showed subtotal loss of vestibular nerve fibers. Although the rudimentary cristae of the right ear had numerous hair cells, the macular hair cells were fewer and malformed. No hydrops was present.

Transparent model of temporal bone and vestibulocochlear organ made by 3D printing.

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Suzuki, Ryoji; Taniguchi, Naoto; Uchida, Fujio; Ishizawa, Akimitsu; Kanatsu, Yoshinori; Zhou, Ming; Funakoshi, Kodai; Akashi, Hideo; Abe, Hiroshi

2018-01-01

The vestibulocochlear organ is composed of tiny complex structures embedded in the petrous part of the temporal bone. Landmarks on the temporal bone surface provide the only orientation guide for dissection, but these need to be removed during the course of dissection, making it difficult to grasp the underlying three-dimensional structures, especially for beginners during gross anatomy classes. We report herein an attempt to produce a transparent three-dimensional-printed model of the human ear. En bloc samples of the temporal bone from donated cadavers were subjected to computed tomography (CT) scanning, and on the basis of the data, the surface temporal bone was reconstructed with transparent resin and the vestibulocochlear organ with white resin to create a 1:1.5 scale model. The carotid canal was stuffed with red cotton, and the sigmoid sinus and internal jugular vein were filled with blue clay. In the inner ear, the internal acoustic meatus, cochlea, and semicircular canals were well reconstructed in detail with white resin. The three-dimensional relationships of the semicircular canals, spiral turns of the cochlea, and internal acoustic meatus were well recognizable from every direction through the transparent surface resin. The anterior semicircular canal was obvious immediately beneath the arcuate eminence, and the topographical relationships of the vestibulocochlear organ and adjacent great vessels were easily discernible. We consider that this transparent temporal bone model will be a very useful aid for better understanding of the gross anatomy of the vestibulocochlear organ.

Subdural hematoma from a cavernous malformation.

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Schmitt, Anne J; Mitha, Alim P; Germain, Rasha; Eschbacher, Jennifer; Spetzler, Robert F

2014-01-01

To present a case of a cavernous malformation presenting with a subdural hematoma. A 27-year-old woman was admitted with progressively worsening headache, vomiting, weakness, and word-finding difficulties 1 week after she was discharged from an outside hospital, where she was managed conservatively for a presumed traumatic subdural hematoma. Computed tomography revealed an enlarging subacute left hemispheric subdural hematoma for which she underwent drill craniostomy. Postprocedural magnetic resonance imaging showed a posterior left temporal lobe mass consistent with a cavernous malformation juxtaposed with the subdural hematoma. Craniotomy for resection of the lesion was performed. She had an uncomplicated postoperative course and experienced a good recovery. The signs and symptoms, diagnostic imaging, and intraoperative findings suggest that the subdural hematoma was caused by extralesional hemorrhage of the cavernous malformation, which is a rare finding associated with these malformations. The clinical course, radiologic, and intraoperative findings suggest that the subdural hemorrhage was caused by extralesional hemorrhage of the cavernous malformation. Copyright © 2014 Elsevier Inc. All rights reserved.

Fracture of the temporal bone in patients with traumatic brain injury

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Secchi, Myrian Marajó Dal

2012-01-01

Full Text Available Introduction: The fractures in the temporal bone are lesions that are observed in patients with traumatic brain injury (TBI. The computed tomography of high-resolution (CT allows evaluating the fracture and the complications. Objective: Evaluate patients with TBI and temporal bone fracture. Way of study: Retrospective study. Method: Were evaluated 28 patients interned by TBI with clinical evidence and/or radiologic from temporal bone fractures. Results: The age ranged from 3 to 75 years. The most affected side was the right side 50% (n=14, left side 36% (n=10 and both sides 14% (n=4. The etiology of the trauma was the falling 25% (n=7, accidents with motorcycles and bicycles 21% (n=6, physical aggression 14% (n=4, running over 11% (n=3, fall of object 4% (n=1 and other causes 25% (n=7. The clinical signs were: Otorrhagia 78%, otalgia 11% (n=3, otorrhea 7% (n=2, facial paralysis 7% (n=2 and hearing loss 7% (n=2. The otoscopic findings: otorrhagia 57% (n=16, laceration of external auditory canal 36% (n=10, hemotympanum 11% (n=3, normal 7% (n=2 and Battle signal 7% (n=2. The findings for CT of skull were: with no alterations 54% (n=15 and temporal fracture 7% (n=2 and the CT of temporal bones were: line of fracture 71% (n=20, opacification of the mastoid 25% (n=7, glenoid cavity air 14% (n=1, dislocation of the ossicular chain 7% (n=2 and veiling of the middle ear 4% (n=1. Conclusion: Patients with TBI must be submitted to the otorhinolaryngological evaluation and imaging, for the early diagnosis of the complications and treatment.

Primary Ewing's Sarcoma of the Temporal Bone: A Rare Case Report and Literature Review.

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Gupta, Divya; Gulati, Achal; Purnima

2017-09-01

Ewing's sarcoma is a malignant, round cell tumor arising from the bones and primarily affecting children and adolescent, accounting for 3 % of all childhood malignancies. Although the long bones and the trunk are typically affected, rare cases of it involving isolated bones throughout the body have been reported. Involvement of the skull bones is rare, constituting 1-6 % of the total Ewing's sarcoma cases but those affecting the cranial bones are rarer still, constituting only 1 %. We describe an 8 months old infant having Ewing sarcoma, of the petrous and mastoid parts of temporal bone along with the occipital bone, whose clinical presentation mimicked mastoiditis with facial nerve palsy. We discuss the clinical and therapeutic course of an extensive primary Ewing sarcoma of the temporal bone, which was treated without performing surgery and review this entity's literature in detail.

Imaging features of lower limb malformations above the foot.

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Bergère, A; Amzallag-Bellenger, E; Lefebvre, G; Dieux-Coeslier, A; Mezel, A; Herbaux, B; Boutry, N

2015-09-01

Lower limb malformations are generally isolated or sporadic events. However, they are sometimes associated with other anomalies of the bones and/or viscera in patients with constitutional syndromes or disorders of the skeleton. This paper reviews the main imaging features of these abnormalities, which generally exhibit a broad spectrum. This paper focuses on several different bone malformations: proximal focal femoral deficiency, congenital short femur and femoral duplication for the femur, tibial hemimelia (aplasia/hypoplasia of the tibia) and congenital bowing for the tibia, fibular hemimelia (aplasia/hypoplasia) for the fibula, and aplasia, hypoplasia and congenital dislocation for the patella. Copyright © 2015 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

Anatomic and Quantitative Temporal Bone CT for Preoperative Assessment of Branchio-Oto-Renal Syndrome.

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Ginat, D T; Ferro, L; Gluth, M B

2016-12-01

We describe the temporal bone computed tomography (CT) findings of an unusual case of branchio-oto-renal syndrome with ectopic ossicles that are partially located in the middle cranial fossa. We also describe quantitative temporal bone CT assessment pertaining to cochlear implantation in the setting of anomalous cochlear anatomy associated with this syndrome.

CT diagnosis and differential diagnosis of otodystrophic lesions of the temporal bone

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D' Archambeau, O.; Parizel, P.M.; Schepper, A.M. De (Antwerp University Hospital (Belgium). Department of Radiology); Koekelkoren, E.; Van De Heyning, P. (Antwerp University Hospital (Belgium). Department of E.N.T.)

The purpose of this study was to assess the diagnostic and differential diagnostic value of high-resolution computed tomography in the evaluation of temporal-bone dystrophies. The study group included 55 patients with osseous abnormalities of the temporal bone in general, and the labyrinthine capsule in particular. In 27 patients the CT scan revealed evidence of otodystrophic lesions. The CT findings in patients with otosclerosis (21 patients), osteogenesis imperfecta (two patients), fibrous dysplasia (one patient). Paget's disease (one patient) and osteoporosis (two patients) are described. The CT scans of 17 patients revealed secondary osseous lesions due to metastasis (five patients), post-inflammatory changes (10 patients) or labyrinthitis ossificans (two patients). Normal variants and congenital mineralization defects were diagnosed in nine patients, Down's syndrome in two. Our results indicate the importance of high-resolution computed tomography as the primary imaging modality in evaluating osseous lesions of the temporal bone and labyrinth. (author). 14 refs.; 13 figs; 2 tabs.

CT diagnosis and differential diagnosis of otodystrophic lesions of the temporal bone

International Nuclear Information System (INIS)

D'Archambeau, O.; Parizel, P.M.; Schepper, A.M. De; Koekelkoren, E.; Van De Heyning, P.

1990-01-01

The purpose of this study was to assess the diagnostic and differential diagnostic value of high-resolution computed tomography in the evaluation of temporal-bone dystrophies. The study group included 55 patients with osseous abnormalities of the temporal bone in general, and the labyrinthine capsule in particular. In 27 patients the CT scan revealed evidence of otodystrophic lesions. The CT findings in patients with otosclerosis (21 patients), osteogenesis imperfecta (two patients), fibrous dysplasia (one patient). Paget's disease (one patient) and osteoporosis (two patients) are described. The CT scans of 17 patients revealed secondary osseous lesions due to metastasis (five patients), post-inflammatory changes (10 patients) or labyrinthitis ossificans (two patients). Normal variants and congenital mineralization defects were diagnosed in nine patients, Down's syndrome in two. Our results indicate the importance of high-resolution computed tomography as the primary imaging modality in evaluating osseous lesions of the temporal bone and labyrinth. (author). 14 refs.; 13 figs; 2 tabs

Spatial and temporal variations of the callus mechanical properties during bone transport

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Mora-Macias, J.; Reina-Romo, E.; Pajares, A.; Miranda, P.; Dominguez, J.

2016-07-01

Nanoindentation allows obtaining the elastic modulus and the hardness of materials point by point. This technique has been used to assess the mechanical propeties of the callus during fracture healing. However, as fas as the authors know, the evaluation of mechanical properties by this technique of the distraction and the docking-site calluses generated during bone transport have not been reported yet. Therefore, the aim of this work is using nanoindentation to assess the spatial and temporal variation of the elastic modulus of the woven bone generated during bone transport. Nanoindentation measurements were carried out using 6 samples from sheep sacrificed at different stages of the bone transport experiments. The results obtained show an important heterogeneity of the elastic modulus of the woven bone without spatial trends. In the case of temporal variation, a clear increase of the mean elastic modulus with time after surgery was observed (from 7±2GPa 35 days after surgery to 14±2GPa 525 days after surgery in the distraction callus and a similar increase in the docking site callus). Comparison with the evolution of the elastic modulus in the woven bone generated during fracture healing shows that mechanical properties increase slower in the case of the woven bone generated during bone transport. (Author)

Differential diagnosis between chronic otitis media with and without cholesteatoma by temporal bone CT: focus on bone change and mass effect

International Nuclear Information System (INIS)

Jung, Cheol Kyu; Park, Dong Woo; Seong, Jin Yong; Lee, Kak Soo; Park Choong Ki; Lee, Seung Ro; Hahm, Chang Kok

2000-01-01

In order to determine specific differences, we compared the temporal bone CT findings of chronic otitis media (COM) with and without cholesteatoma, focusing on bone change. Between 1997 and 1998, 82 patients (84 cases) underwent temporal bone CT and were shown to have COM, with or without cholesteatoma after mastoidectomy and tympanoplasty. There were 36 cases of COM with cholesteatoma (26 patients, M:F =3D 11:15; age range, 16-61 (mean, 36,2) years), and 58 cases without chlesteatoma (56 patients, M:F =3D 25:31, age range, 15-61 (mean, 36.2) years). The findings of temporal bone CT were analysed at the point of bony changes including erosion and medial displacement of ossicles (malleus, incus, and stapes), erosion or destruction of the scutum, tegmen, facial canal, and lateral semicircular canal, and ballooning of the tympanic cavity and mastoid antrum. In addition, the soft tissue changes seen on temporal bone CT were analyzed at the site of lateral bulging of soft tissue in Prussak's space, perforation of the pars flaccida, tympanic membrane retraction, and tympanosclerosis. We retrospectively compared the findings of temporal bone CT with the surgical findings, and to assess statistical significance, the Chi-square test was used. Bone erosion or destruction was seen in 36.2% of COM cases without cholesteatoma, and in 96.2% of cases with cholesteatoma. Comparing COM with and without cholesteatoma, the erosion of ossicles including the malleus (81%, 24%), incus (88%, 14%), stapes (58%, 10%), scutum (88%, 10%), facial canal (8%, 0%), and lateral semicircular canal (8%, 0%), was more common in COM with cholesteatoma (p-value less than 0.05), with the exception of erosion of the tegmen (8%, 3%). Other bony changes including medial displacement of ossicles (27%, 3%), ballooning of tympanic cavity and mastoid antrum (96%, 16%), and the soft tissue changes including lateral bulging of soft tissue in Prussak's space (58%, 14%) and perforation of the pars flaccida (35

Differential diagnosis between chronic otitis media with and without cholesteatoma by temporal bone CT: focus on bone change and mass effect

Energy Technology Data Exchange (ETDEWEB)

Jung, Cheol Kyu; Park, Dong Woo; Seong, Jin Yong; Lee, Kak Soo; Park Choong Ki; Lee, Seung Ro; Hahm, Chang Kok [College of Medicine, Hanyang University, Seoul (Korea, Republic of)

2000-01-01

In order to determine specific differences, we compared the temporal bone CT findings of chronic otitis media (COM) with and without cholesteatoma, focusing on bone change. Between 1997 and 1998, 82 patients (84 cases) underwent temporal bone CT and were shown to have COM, with or without cholesteatoma after mastoidectomy and tympanoplasty. There were 36 cases of COM with cholesteatoma (26 patients, M:F =3D 11:15; age range, 16-61 (mean, 36,2) years), and 58 cases without chlesteatoma (56 patients, M:F =3D 25:31, age range, 15-61 (mean, 36.2) years). The findings of temporal bone CT were analysed at the point of bony changes including erosion and medial displacement of ossicles (malleus, incus, and stapes), erosion or destruction of the scutum, tegmen, facial canal, and lateral semicircular canal, and ballooning of the tympanic cavity and mastoid antrum. In addition, the soft tissue changes seen on temporal bone CT were analyzed at the site of lateral bulging of soft tissue in Prussak's space, perforation of the pars flaccida, tympanic membrane retraction, and tympanosclerosis. We retrospectively compared the findings of temporal bone CT with the surgical findings, and to assess statistical significance, the Chi-square test was used. Bone erosion or destruction was seen in 36.2% of COM cases without cholesteatoma, and in 96.2% of cases with cholesteatoma. Comparing COM with and without cholesteatoma, the erosion of ossicles including the malleus (81%, 24%), incus (88%, 14%), stapes (58%, 10%), scutum (88%, 10%), facial canal (8%, 0%), and lateral semicircular canal (8%, 0%), was more common in COM with cholesteatoma (p-value less than 0.05), with the exception of erosion of the tegmen (8%, 3%). Other bony changes including medial displacement of ossicles (27%, 3%), ballooning of tympanic cavity and mastoid antrum (96%, 16%), and the soft tissue changes including lateral bulging of soft tissue in Prussak's space (58%, 14%) and perforation of the pars

Surgical treatment of Chiari malformation complicated with basilar impression

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Yuan MA

2011-02-01

Full Text Available Objective To evaluate the therapeutic effect of small craniotomic posterior fossa decompression combined with occipital-cervical bone graft fusion and internal fixation on Chiari malformation complicated with basilar impression.Methods The clinical data of 16 cases(7 males and 9 females,aged 17 to 65 years,mean 36.4 of Chiari malformation complicated with basilar impression from 2006 to 2010 were retrospectively analyzed.The diagnoses for all the patients were confirmed by radiology.Small craniotomic posterior fossa decompression was performed in all patients,cerebellar tonsils were resected,and then one-stage occipital-cervical bone graft fusion using autogenous iliac bone and internal wiring fixation were performed.Neck support was used for 3 months after surgery.Results Symptoms were significantly improved in all cases after surgical operation.No patient died or infected.Cerebrospinal fluid leakage was found at draining site in one case.Transient pain of scapular and chest was found in one case and disappeared spontaneously.A 6-months follow-up showed that 6 patients were cured,9 improved and 1 unchanged according to Symon and Lavender standard.Postoperative MRI showed the reconstructed cisterna magna was clear in all patients,no cerebellar ptosis was found,and the occipital-cervical graft bone was fused.Conclusion In patients with Chiari malformation complicated with basilar impression,small craniotomic posterior fossa decompression combined with one-stage occipital-cervical bone graft fusion and internal wiring fixation has a clear and definite effect,it can increase the volume of posterior fossa and alleviate the ventral brain stem compression simultaneously,and reconstruct the stability of cranio-cervical junction.

Brain and bone abnormalities of thanatophoric dwarfism.

Science.gov (United States)

Miller, Elka; Blaser, Susan; Shannon, Patrick; Widjaja, Elysa

2009-01-01

The purpose of this article is to present the imaging findings of skeletal and brain abnormalities in thanatophoric dwarfism, a lethal form of dysplastic dwarfism. The bony abnormalities associated with thanatophoric dwarfism include marked shortening of the tubular bones and ribs. Abnormal temporal lobe development is a common associated feature and can be visualized as early as the second trimester. It is important to assess the brains of fetuses with suspected thanatophoric dwarfism because the presence of associated brain malformations can assist in the antenatal diagnosis of thanatophoric dwarfism.

Three cases of temporal bone osteoradionecrosis after nasopharyngeal carcinoma treatment

International Nuclear Information System (INIS)

Yamatodani, Takashi; Mizuta, Kunihiro; Nakanishi, Hiroshi; Takizawa, Yoshinori; Hosokawa, Kumiko; Hosokawa, Seiji; Mineta, Hiroyuki

2012-01-01

Osteoradionecrosis is most commonly caused by radiation-induced injury. We report on 3 cases of temporal bone necrosis that occurred after chemoradiotherapy for nasopharyngeal carcinoma performed more than 10 years previously. Case 1 was a 42-year-old woman who had nasopharyngeal carcinoma in 1991. The patient underwent chemoradiotherapy (70 Gy total) in 1991, and gamma knife irradiation (20 Gy) in 1998 for local recurrence. The bone in the posterior wall of the left external auditory canal began to be exposed in 2003. Otorrhea from the left ear increased and we found a skin defect and ulcer formation in the postauricular region. We performed radical mastoidectomy and debridement on April, 2010. The area of the defect was covered and filled in with a pedicle musculoperiosteal flap. The intra-aural skin became dry in 6 months, however, she lost consciousness due to a temporal lobe abscess and underwent an emergency operation on April, 2011. After operation, the patient recovered with no neurological symptoms and infections up to the present date. Case 2 was a 58-year-old man who had nasopharyngeal carcinoma in 2001. The patient underwent chemoradiotherapy (66 Gy total) in 2001, and X knife irradiation (15 Gy) 3 months later due to the remaining tumor. The left posterior ear canal wall collapsed and the tympanic membrane retracted with pooling epithelial debris appearing in 2007. Left facial nerve palsy was seen in December 2010. We performed a mastoidectomy on January, 2011. Cholesteatoma and necrotic granuloma with fragile bone filled the mastoid cavity, and a facial canal bone defect was seen. Bone necrosis with cholesteatoma and inflammatory granuloma was revealed by the pathological examination. The facial palsy improved after the operation. Case 3 was a 59-year-old man who had left abducens palsy with nasopharyngeal carcinoma invading the clivus. The patient underwent chemoradiotherapy (60 Gy total) in 2001, and X knife irradiation (24 Gy) 4 months later for

Endolymphatic Sac Tumors and Papillary Adenocarcinoma of the Temporal Bone:Role of MRI and CT

OpenAIRE

Mahmood F. Mafee; Hemant Shah

2003-01-01

Adenomatous Tumors of the temporal bone are rare. Benign adenomatous neoplasms (adenoma) of the middle ear are a distinctive benign tumor based on histological and clinical observations. Papillary adenocarcinomas of the temporal bone are invasive tumors. Although, the exact site of origin of these neoplasms is not identified, owing to the local bone destruction (usually centered at posterior petromastoid plate), the general consensus favors the endolymphatic sac as being the origin of these t...

Interactive atlas using web browser: CT and MRI of the temporal bone

International Nuclear Information System (INIS)

Chung, Eun Chul; Youn, Eun Kyung; Lee, Young Uk

2000-01-01

The purposes of this study were to construct an interactive atlas of the temporal bone using a web-browser and to provide a template for web-based teaching files, using free and shared applets and scripts on the internet. HRCT and MR images of the temporal bone including its normal anatomy, tumors, trauma, inflammation, anomalies and vascular diseases were used in this study. Acquired radiologic images were transformed to GIF/JPG formats and to achieve appropriate image quality, were retouched. Text and image files of normal anatomy and diseases were written by HTML. JavaScript and applets were inserted in the HTML files for the interactive display of images and texts. In order to review anatomic features and diseases, a search index was also attached to the last part of the file. Using interactive images and text, temporal bone anatomy and disorders were displayed. Scripts and applets were also useful for indicating specific points of interest when a mouse was placed over the anatomic sites. The atlas may be viewed in the form of a CD-ROM, or via the internet using any computer platform or web-browser. This web-based teaching file of the temporal bone offers dynamic and interactive education. It can be usefully employed as a template for the production of interactive educational materials, offering JavaScript and providing suitable input for classes. It can replace texts and imaging contents. (author)

Pattern of malformations in the axial skeleton in human trisomy 18 fetuses

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Kjaer, I. [Univ. of Copenhagen (Denmark); Hansen, B.F. [Hvidovre Univ. Hospital (Denmark); Keeling, J.W. [Royal Hospital for Sick Children, Edinburgh (United Kingdom)

1996-11-11

We examined and described the development and abnormalities of the axial skeleton in 10 human trisomy 18 fetuses. Whole-body radiographs and radiographs of midsagittal tissue blocks of the cranial base and the spine were studied. In 3 fetuses no spinal radiographs were available. Seven osseous regions or fields along the body axis were analyzed, four in the spine, and three in the cranial base and nasal bones. Malformations occurred in the occipital field in all fetuses. This was a characteristic notching, either unilateral or bilateral, of the basilar part of the occipital bone. Nasal bones were abnormal in 8 cases, either absent or hypoplastic. Malformations were found in the thoracic and/or lumbosacral field in 7 fetuses. A single abnormality was found in the cervical spine in one fetus. The pattern of axial skeletal malformation in trisomy 18 fetuses recorded in the present study has not been described previously. Axial skeletal radiography should be included in autopsies of fetuses when chromosome disorders are present or suspected. The methods applied here are unaffected by autolysis. 26 refs., 5 figs.

Locating the scala media in the fixed human temporal bone for therapeutic access: a preliminary study.

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Pau, H; Fagan, P; Oleskevich, S

2006-11-01

To investigate the location of the scala media in relation to the round window niche in human temporal bones. Ten human temporal bones were investigated by radical mastoidectomy and promontory drill-out. Temporal bone laboratory. The distance from the scala media to the anterior edge of the round window niche, measured by Fisch's stapedectomy measuring cylinders. The scala media was identified at the transection point of a vertical line 1.6 to 2.2 mm (mean=1.8 mm; standard deviation=0.2) anterior to the anterior edge of the round window niche and a horizontal line 0.2 mm inferior to the lower border of the oval window. This report demonstrates the point of entry into the scala media via the promontory in fixed temporal bone models, which may provide a site of entry for stem cells and gene therapy insertion.

Facial nerve palsy associated with a cystic lesion of the temporal bone.

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Kim, Na Hyun; Shin, Seung-Ho

2014-03-01

Facial nerve palsy results in the loss of facial expression and is most commonly caused by a benign, self-limiting inflammatory condition known as Bell palsy. However, there are other conditions that may cause facial paralysis, such as neoplastic conditions of the facial nerve, traumatic nerve injury, and temporal bone lesions. We present a case of facial nerve palsy concurrent with a benign cystic lesion of the temporal bone, adjacent to the tympanic segment of the facial nerve. The patient's symptoms subsided after facial nerve decompression via a transmastoid approach.

Malformations and abnormalities of the petrous portion of the temporal bone; Fehlbildungen und Missbildungen des Felsenbeins

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Reith, W.; Yilmaz, U. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany); Heumueller, I. [Westpfalzklinikum Kaiserslautern, Institut fuer Radiologie, Kaiserslautern (Germany)

2014-04-15

High-resolution computed tomography (HRCT) is the procedure of choice in the diagnostics of abnormalities of the middle and inner ear. It allows a detailed presentation of anatomical features and achieves the prerequisites for selection of the various therapeutic options. The highly diverse abnormalities can be described using detailed imaging analyses. Malformations with an abnormally developed modiolus are assumed to be early embryological defects, such as the classical Mondini dysplasia. The essential therapeutic option for middle ear deformities is still a cochlear implant. The domain of magnetic resonance imaging (MRI) is not only in the analysis of the cochlear nerve and for exclusion of fibrosis or ossification of the labyrinth but is also able to visualize details of isolated malformations, such as an extended vestibular aqueduct or subtle alterations to the vestibule or can visualize them better in comparison to CT. Radiological diagnostics are used not only for classification but also to recognize typical clinical problem situations and play a key role in the diagnostics of hearing disorders and selection of the optimal therapeutic procedure. (orig.) [German] Die hochaufloesende CT ist das Verfahren der Wahl in der Diagnostik von Fehlbildungen des Mittel- und Innenohrs. Sie erlaubt die detaillierte Darstellung anatomischer Details und schafft die Voraussetzung fuer die unterschiedlichen Therapieoptionen. Durch detaillierte bildgebenden Analysen koennen die unterschiedlichsten Fehlbildungen beschrieben werden. Fehlbildungen mit einem nicht normal ausgebildeten Modiolus werden als fruehere embryologische Schaedigungen als die klassische Mondini-Dysplasie angesehen. Die wesentliche therapeutische Option bei Mittelohrfehlbildung bleibt das Kochleaimplantat. Die Domaene der MRT liegt nicht nur in der Analyse des N. cochlearis und im Ausschluss von Fibrosierungen oder Ossifikationen des Labyrinths, sondern darueber hinaus ist sie in der Lage, Details einzelner

Chondroblastoma of the temporal bone

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Kobayashi, Yuko; Murakami, Ryusuke; Toba, Masahiro; Ichikawa, Taro [Dept. of Radiology, Nippon Medical School, Tokyo (Japan); Kanazawa, Ryuzaburo; Sanno, Naoko; Shimura, Toshiro [Dept. of Neurosurgery, Nippon Medical School, Tokyo (Japan); Sawada, Namie; Hosone, Masaru [Dept. of Pathology, Nippon Medical School, Tokyo (Japan); Kumazaki, Tatsuo [Dept. of Radiology, Nippon Medical School, Tokyo (Japan)

2001-12-01

A rare case of chondroblastoma arising from the temporal bone that occurred in a 60-year-old woman is reported. The tumor appeared well demarcated and osteolytic on the radiographs. CT scan clearly depicted marginal and central calcification in the tumor. MR imaging demonstrated two components in the tumor: a solid component with predominantly low signal intensities on both T1- and T2-weighted sequences, and a multilocular cystic component with T1- and T2-elongation and fluid-fluid levels on the T2-weighted images. Postcontrast MR imaging revealed marked enhancement in the solid component and the septa of the cystic component. (orig.)

Cholesterol granuloma of temporal bone: CT and MR findings

International Nuclear Information System (INIS)

Ruiz Jauriguizuria, J.C.; Ferrero Collado, A.; Ereno Ealo, M.J.; Grande Icaran, D.

1994-01-01

Three cases of cholesterol granuloma of the left temporal bone are presented, two located in the petrous apex and the other at the otomastoid level. One of the patients had a history of mastoid surgery and the other surgery of the bone ridge. The three cases were confirmed histologically following surgical resection. The etiopathogenesis of the lesion is discussed, as are the clinical, histological and radiological features, with a broad description of the computed tomography and magnetic resonance findings. The differential diagnosis is also dealt with. (Author)

Histology of sheep temporal bone A histologia do osso temporal do ovino

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Hormy Biavatti Soares

2011-06-01

Full Text Available Previous studies suggest that there is an excellent correlation between the morphology and dimensions of ear structures in sheep and human beings. AIM: To analyze and describe the histology of structures inside the temporal bone in sheep. MATERIAL AND METHODS: A total of 307 slides obtained from vertical and horizontal sections of the temporal bone of eight sheep were analyzed. Structures were classified as similar or not similar to human structures, based on cellularity and histological architecture parameters. STUDY DESIGN: Experimental. RESULTS: The study revealed similarities between sheep and humans in terms of type of epithelium, bone component, spaces in the auditory meatus, in addition to a marked histological resemblance of cellularity and that of the structures surrounding the ear. The main differences observed were the presence of an anatomic bulla, the absence of aeration in the mastoid and the inferior opening of the hypotympanum into the bulla in sheep. CONCLUSION: Based on these observations, it is possible to conclude that sheep represent an adequate option for training and research in otologic surgery.Resultados prévios apontam para uma ótima correlação entre a morfologia e as dimensões das estruturas anatômicas de ovelhas e seres humanos. OBJETIVO: Analisar e descrever a histologia das estruturas que compõem o osso temporal do ovino. MATERIAL E MÉTODO: Um total de 307 lâminas obtidas a partir de secções verticais e horizontais do osso temporal de oito ovelhas foi analisado. As estruturas foram caracterizadas como semelhantes ou não semelhantes às do ser humano, com base na celularidade e na arquitetura histológica das estruturas. DESENHO CIENTÍFICO: Experimental. RESULTADOS: Constatou-se semelhança quanto ao tipo de epitélio, componente ósseo, espaços da fenda auditiva e arquitetura, além da semelhança, em nível histológico, tanto dos componentes celulares como das estruturas contíguas ao ouvido. As

CT-MR image data fusion for computer assisted navigated neurosurgery of temporal bone tumors

International Nuclear Information System (INIS)

Nemec, Stefan Franz; Donat, Markus Alexander; Mehrain, Sheida; Friedrich, Klaus; Krestan, Christian; Matula, Christian; Imhof, Herwig; Czerny, Christian

2007-01-01

Purpose: To demonstrate the value of multi detector computed tomography (MDCT) and magnetic resonance imaging (MRI) in the preoperative work up of temporal bone tumors and to present, especially, CT and MR image fusion for surgical planning and performance in computer assisted navigated neurosurgery of temporal bone tumors. Materials and methods: Fifteen patients with temporal bone tumors underwent MDCT and MRI. MDCT was performed in high-resolution bone window level setting in axial plane. The reconstructed MDCT slice thickness was 0.8 mm. MRI was performed in axial and coronal plane with T2-weighted fast spin-echo (FSE) sequences, un-enhanced and contrast-enhanced T1-weighted spin-echo (SE) sequences, and coronal T1-weighted SE sequences with fat suppression and with 3D T1-weighted gradient-echo (GE) contrast-enhanced sequences in axial plane. The 3D T1-weighted GE sequence had a slice thickness of 1 mm. Image data sets of CT and 3D T1-weighted GE sequences were merged utilizing a workstation to create CT-MR fusion images. MDCT and MR images were separately used to depict and characterize lesions. The fusion images were utilized for interventional planning and intraoperative image guidance. The intraoperative accuracy of the navigation unit was measured, defined as the deviation between the same landmark in the navigation image and the patient. Results: Tumorous lesions of bone and soft tissue were well delineated and characterized by CT and MR images. The images played a crucial role in the differentiation of benign and malignant pathologies, which consisted of 13 benign and 2 malignant tumors. The CT-MR fusion images supported the surgeon in preoperative planning and improved surgical performance. The mean intraoperative accuracy of the navigation system was 1.25 mm. Conclusion: CT and MRI are essential in the preoperative work up of temporal bone tumors. CT-MR image data fusion presents an accurate tool for planning the correct surgical procedure and is a

Middle cranial fossa approach to repair tegmen defects assisted by three-dimensionally printed temporal bone models.

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Ahmed, Sameer; VanKoevering, Kyle K; Kline, Stephanie; Green, Glenn E; Arts, H Alexander

2017-10-01

To explore the perioperative utility of three-dimensionally (3D)-printed temporal bone models of patients undergoing repair of lateral skull base defects and spontaneous cerebrospinal fluid leaks with the middle cranial fossa approach. Case series. 3D-printed temporal bone models-based on patient-specific, high-resolution computed tomographic imaging-were constructed using inexpensive polymer materials. Preoperatively, the models demonstrated the extent of temporal lobe retraction necessary to visualize the proposed defects in the lateral skull base. Also preoperatively, Silastic sheeting was arranged across the modeled tegmen, marked, and cut to cover all of the proposed defect sites. The Silastic sheeting was then sterilized and subsequently served as a precise intraoperative template for a synthetic dural replacement graft. Of note, these grafts were customized without needing to retract the temporal lobe. Five patients underwent the middle cranial fossa approach assisted by 3D-printed temporal bone models to repair tegmen defects and spontaneous cerebrospinal fluid leaks. No complications were encountered. The prefabricated dural repair grafts were easily placed and fit precisely onto the middle fossa floor without any additional modifications. All defects were covered as predicted by the 3D temporal bone models. At their postoperative visits, all five patients maintained resolution of their spontaneous cerebrospinal fluid leaks. Inexpensive 3D-printed temporal bone models of tegmen defects can serve as beneficial adjuncts during lateral skull base repair. The models provide a panoramic preoperative view of all tegmen defects and allow for custom templating of dural grafts without temporal lobe retraction. 4 Laryngoscope, 127:2347-2351, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

Sclerosteosis involving the temporal bone: histopathologic aspects.

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Nager, G T; Hamersma, H

1986-01-01

Sclerosteosis is a rare, potentially lethal, autosomal recessive, progressive craniotubular sclerosing bone dysplasia with characteristic facial and skeletal features. The temporal bone changes include a marked increase in overall size, extensive sclerosis, narrowing of the external auditory canal, and severe constriction of the internal auditory meatus, fallopian canal, eustachian tube, and middle ear cleft. Attenuation of the bony canals of the 9th, 10th, and 11th cranial nerves, reduction in size of the internal carotid artery, and severe obliteration of the sigmoid sinus and jugular bulb also occur. Loss of hearing, generally bilateral, is a frequent symptom. It often manifests in early childhood and initially is expressed as sound conduction impairment. Later, a sensorineural hearing loss and loss of vestibular nerve function often develop. Impairment of facial nerve function is another feature occasionally present at birth. In the beginning, a unilateral intermittent facial weakness may occur which eventually progresses to a bilateral permanent facial paresis. The histologic examination of the temporal bones from a patient with sclerosteosis explains the mechanisms involved in the progressive impairment of sound conduction and loss of cochlear, vestibular, and facial nerve function. There is a decrease of the arterial blood supply to the brain and an obstruction of the venous drainage from it. The histopathology reveals the obstacles to decompression of the middle ear cleft, ossicular chain, internal auditory and facial canals, and the risks, and in many instances the contraindications, to such procedures. On the other hand, decompression of the sigmoid sinus and jugular bulb should be considered as an additional life-saving procedure in conjunction with the prophylactic craniotomy recommended in all adult patients.

Evaluation of a haptics-based virtual reality temporal bone simulator for anatomy and surgery training.

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Fang, Te-Yung; Wang, Pa-Chun; Liu, Chih-Hsien; Su, Mu-Chun; Yeh, Shih-Ching

2014-02-01

Virtual reality simulation training may improve knowledge of anatomy and surgical skills. We evaluated a 3-dimensional, haptic, virtual reality temporal bone simulator for dissection training. The subjects were 7 otolaryngology residents (3 training sessions each) and 7 medical students (1 training session each). The virtual reality temporal bone simulation station included a computer with software that was linked to a force-feedback hand stylus, and the system recorded performance and collisions with vital anatomic structures. Subjects performed virtual reality dissections and completed questionnaires after the training sessions. Residents and students had favorable responses to most questions of the technology acceptance model (TAM) questionnaire. The average TAM scores were above neutral for residents and medical students in all domains, and the average TAM score for residents was significantly higher for the usefulness domain and lower for the playful domain than students. The average satisfaction questionnaire for residents showed that residents had greater overall satisfaction with cadaver temporal bone dissection training than training with the virtual reality simulator or plastic temporal bone. For medical students, the average comprehension score was significantly increased from before to after training for all anatomic structures. Medical students had significantly more collisions with the dura than residents. The residents had similar mean performance scores after the first and third training sessions for all dissection procedures. The virtual reality temporal bone simulator provided satisfactory training for otolaryngology residents and medical students. Copyright © 2013. Published by Elsevier Ireland Ltd.

Ear malformations, hearing loss and hearing rehabilitation in children with Treacher Collins syndrome.

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Rosa, Francisco; Coutinho, Miguel Bebiano; Ferreira, João Pinto; Sousa, Cecilia Almeida

2016-01-01

The aim of this study was to assess the main ear malformations, hearing loss and auditory rehabilitation in children with Treacher Collins syndrome. We performed a retrospective study of 9 children with Treacher Collins syndrome treated in a central hospital between January 2003 and January 2013. This study showed a high incidence of malformations of the outer and middle ear, such as microtia, atresia or stenosis of the external auditory canal, hypoplastic middle ear cavity, dysmorphic or missing ossicular chain. Most patients had bilateral hearing loss of moderate or high degree. In the individuals studied, there was functional improvement in patients with bone-anchored hearing aids in relation to conventional hearing aids by bone conduction. Treacher Collins syndrome is characterized by bilateral malformations of the outer and middle ear. Hearing rehabilitation in these children is of utmost importance, and bone-anchored hearing aids is the method of choice. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

Validation of exposure visualization and audible distance emission for navigated temporal bone drilling in phantoms.

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Eduard H J Voormolen

Full Text Available BACKGROUND: A neuronavigation interface with extended function as compared with current systems was developed to aid during temporal bone surgery. The interface, named EVADE, updates the prior anatomical image and visualizes the bone drilling process virtually in real-time without need for intra-operative imaging. Furthermore, EVADE continuously calculates the distance from the drill tip to segmented temporal bone critical structures (e.g. the sigmoid sinus and facial nerve and produces audiovisual warnings if the surgeon drills in too close vicinity. The aim of this study was to evaluate the accuracy and surgical utility of EVADE in physical phantoms. METHODOLOGY/PRINCIPAL FINDINGS: We performed 228 measurements assessing the position accuracy of tracking a navigated drill in the operating theatre. A mean target registration error of 1.33±0.61 mm with a maximum error of 3.04 mm was found. Five neurosurgeons each drilled two temporal bone phantoms, once using EVADE, and once using a standard neuronavigation interface. While using standard neuronavigation the surgeons damaged three modeled temporal bone critical structures. No structure was hit by surgeons utilizing EVADE. Surgeons felt better orientated and thought they had improved tumor exposure with EVADE. Furthermore, we compared the distances between surface meshes of the virtual drill cavities created by EVADE to actual drill cavities: average maximum errors of 2.54±0.49 mm and -2.70±0.48 mm were found. CONCLUSIONS/SIGNIFICANCE: These results demonstrate that EVADE gives accurate feedback which reduces risks of harming modeled critical structures compared to a standard neuronavigation interface during temporal bone phantom drilling.

Creation of a 3D printed temporal bone model from clinical CT data.

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Cohen, Joss; Reyes, Samuel A

2015-01-01

Generate and describe the process of creating a 3D printed, rapid prototype temporal bone model from clinical quality CT images. We describe a technique to create an accurate, alterable, and reproducible rapid prototype temporal bone model using freely available software to segment clinical CT data and generate three different 3D models composed of ABS plastic. Each model was evaluated based on the appearance and size of anatomical structures and response to surgical drilling. Mastoid air cells had retained scaffolding material in the initial versions. This required modifying the model to allow drainage of the scaffolding material. External auditory canal dimensions were similar to those measured from the clinical data. Malleus, incus, oval window, round window, promontory, horizontal semicircular canal, and mastoid segment of the facial nerve canal were identified in all models. The stapes was only partially formed in two models and absent in the third. Qualitative feel of the ABS plastic was softer than bone. The pate produced by drilling was similar to bone dust when appropriate irrigation was used. We present a rapid prototype temporal bone model made based on clinical CT data using 3D printing technology. The model can be made quickly and inexpensively enough to have potential applications for educational training. Copyright © 2015 Elsevier Inc. All rights reserved.

Craniofacial and temporal bone CT findings in cleidocranial dysplasia

International Nuclear Information System (INIS)

Gonzalez, Guido E.; Caruso, Paul A.; Curtin, Hugh D.; Small, Juan E.; Jyung, Robert W.; Troulis, Maria J.

2008-01-01

Cleidocranial dysplasia (CCD) is a multistructural polyostotic genetic disorder that results from mutation of the CBFA1 gene. Hearing loss is a frequent finding in CCD. We describe the CT craniofacial findings in CCD and provide a comprehensive discussion of the CT temporal bone findings in these patients. (orig.)

Mixed reality temporal bone surgical dissector: mechanical design.

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Hochman, Jordan Brent; Sepehri, Nariman; Rampersad, Vivek; Kraut, Jay; Khazraee, Milad; Pisa, Justyn; Unger, Bertram

2014-08-08

The Development of a Novel Mixed Reality (MR) Simulation. An evolving training environment emphasizes the importance of simulation. Current haptic temporal bone simulators have difficulty representing realistic contact forces and while 3D printed models convincingly represent vibrational properties of bone, they cannot reproduce soft tissue. This paper introduces a mixed reality model, where the effective elements of both simulations are combined; haptic rendering of soft tissue directly interacts with a printed bone model. This paper addresses one aspect in a series of challenges, specifically the mechanical merger of a haptic device with an otic drill. This further necessitates gravity cancelation of the work assembly gripper mechanism. In this system, the haptic end-effector is replaced by a high-speed drill and the virtual contact forces need to be repositioned to the drill tip from the mid wand. Previous publications detail generation of both the requisite printed and haptic simulations. Custom software was developed to reposition the haptic interaction point to the drill tip. A custom fitting, to hold the otic drill, was developed and its weight was offset using the haptic device. The robustness of the system to disturbances and its stable performance during drilling were tested. The experiments were performed on a mixed reality model consisting of two drillable rapid-prototyped layers separated by a free-space. Within the free-space, a linear virtual force model is applied to simulate drill contact with soft tissue. Testing illustrated the effectiveness of gravity cancellation. Additionally, the system exhibited excellent performance given random inputs and during the drill's passage between real and virtual components of the model. No issues with registration at model boundaries were encountered. These tests provide a proof of concept for the initial stages in the development of a novel mixed-reality temporal bone simulator.

High-resolution computed tomography of the temporal bone. Part 2.: pathology

International Nuclear Information System (INIS)

Grzegorzewski, M.; Boron, Z.; Burzynska-Makuch, M.

1995-01-01

Selected cases of the temporal bone pathology on high resolution CT were demonstrated. Transverse and coronal sections of the cases were selected from 68 patients with various otological diseases. (author)

[Management of occult malformations at the lateral skull base].

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Bryson, E; Draf, W; Hofmann, E; Bockmühl, U

2005-12-01

Occult malformations of the lateral skull base are rare anomalies, but can cause severe complications such as recurrent meningitis. Therefore, they need to be precisely delineated and sufficient surgical closure is mandatory. Between 1986 and 2004 twenty patients (10 children and 10 adults) with occult malformations at the lateral skull base were treated surgically at the ENT-Department of the Hospital Fulda gAG. Of these 3 Mondini-malformations, 11 defects of the tegmen tympani or the mastoidal roof, 2 dural lesions to the posterior fossa and 4 malformations within the pyramidal apex have been found. Four patients have had multiple anomalies. Routing symptom was in all cases at least one previous meningitis. Radiological diagnostics included high-resolution computed tomography (CT) and magnetic resonance imaging (MRI) as well as CT- or MR-cisternography. Depending on type and localisation of the defect the following surgical algorithm was carried out: The trans-mastoidal approach was used in all cases of Mondini-malformation (including obliteration of the ear), in case of lesions to the posterior fossa as well as partly in anomalies at the tegmen tympani and mastoidal roof, respectively. Defects of the pyramidal apex should be explored via the trans-mastoidal way if the lesion is located caudally to the inner auditory canal (IAC), whereas the trans-temporal approach should be used if the lesion is situated ventral to the IAC and dorso-medially to the internal carotid artery (ICA). The trans-temporal approach was also performed in large defects of the tegmen tympani and mastoidal roof as well as in recurrences. In all cases of recurrent meningitis caused by agents of the upper airway tract the basic principle should be to search for occult skull base malformations radiologically as well as by sodium fluorescein endoscopy as long as the anomaly is detected.

Primary Ewing's Sarcoma of the temporal bone in an infant.

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Goudarzipour, Kourosh; Shamsian, Shahin; Alavi, Samin; Nourbakhsh, Kazem; Aghakhani, Roxana; Eydian, Zahra; Arzanian, Mohammad Taghi

2015-04-01

Introduction : Ewing's sarcoma is the second most common primary malignant tumor of bone found in children after Osteosarcoma. It accounts for 4-9% of primary malignant bone tumors and it affects bones of the skull or face in only 1-4% of cases. Hence it rarely affects the head and neck. Subject and Method : In this case report, we describe a case of primary Ewing's sarcoma occurring in the temporal bone. The tumor was surgically excised, and the patient underwent chemotherapy for ten months. Results : Neither recurrence nor distant metastasis was noted in these 10 months after surgery but about 18 months after surgery our patient was expired. Conclusion : Although the prognosis of Ewing's sarcoma is generally poor because of early metastasis to the lungs and to other bones, a review of the article suggested that Ewing's sarcoma occurring in the skull can often be successfully managed by intensive therapy with radical excision and chemotherapy. This result was supported by the case reported here.

Displasia fibrosa do osso temporal: relato de dois casos Fibrous dysplasia of the temporal bone: report of two cases

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Roberto Claudio B. Oliveira

2004-10-01

Full Text Available A displasia fibrosa do osso temporal é uma doença de etiologia ainda controversa, manifestando-se principalmente por estenose progressiva do conduto auditivo externo e pela perda condutiva da audição. Outras manifestações incluem abaulamento na região temporal ou retroauricular, otorréia, otalgia e disacusia sensório-neural. A incidência é maior no sexo masculino e acomete principalmente a raça branca. O exame radiológico característico demonstra um aspecto de "vidro-fosco" homogêneo envolvido por uma concha de tecido cortical denso, embora existam outros padrões radiológicos desta enfermidade. O exame microscópico demonstra um trabeculado ósseo semelhante aos caracteres chineses. Este estudo relata dois casos de displasia fibrosa do osso temporal que se destacam, pois ultrapassaram o osso temporal, acometendo a região zigomática, sendo que no segundo caso houve também comprometimento do osso esfenóide e o pterigóide. Os pacientes foram submetidos à mastoidectomia radical modificada e tiveram boa evolução.Fibrous dysplasia of the temporal bone (FDTB is a disorder which etiology is still controversial. Its main clinical feature is a progressive narrowing of the external auditory canal following by conductive hearing loss. Temporal or retroauricular enlargement, ear discharge, otalgia, and sensorineural hearing loss are additional findings. Women and Caucasians are more affected. The prominent finding is a homogeneous radiodense "grounded glass" like image shell surrounded by dense cortical tissue. However, other radiological patterns of this disease may be displayed. Microscopically, a trabecular of bone in "Chinese letter" configuration is found. The two cases of FDTB herein reported are particularly special for a far beyond temporal commitment reaching the zygomatic area in the first case and sphenoid and pterygoid bones in the second one. This infrequent clinical feature with unusual radiological findings made these

Visualization of subtle temporal bone structures. Comparison of cone beam CT and MDCT

International Nuclear Information System (INIS)

Pein, M.K.; Plontke, S.K.; Brandt, S.; Koesling, S.

2014-01-01

The purpose of this study was to compare the visualization of subtle, non-pathological temporal bone structures on cone beam computed tomography (CBCT) and multi-detector computed tomography (MDCT) in vivo. Temporal bone studies of images from 38 patients archived in the picture archiving and communication system (PACS) were analyzed (slice thickness MDCT 0.6 mm and CBCT 0.125 mm) of which 23 were imaged by MDCT and 15 by CBCT using optimized standard protocols. Inclusion criteria were normal radiological findings, absence of previous surgery and anatomical variants. Images were evaluated blind by three trained observers. Using a five-point scale the visualization of ten subtle structures of the temporal bone was analyzed. Subtle middle ear structures showed a tendency to be more easily distinguishable by CBCT with significantly better visualization of the tendon of the stapedius muscle and the crura of the stapes on CBCT (p = 0.003 and p = 0.033, respectively). In contrast, inner ear components, such as the osseus spiral lamina and the modiolus tended to be better detectable on MDCT, showing significant differences for the osseous spiral lamina (p = 0.001). The interrater reliability was 0.73 (Cohen's kappa coefficient) and intraobserver reliability was 0.89. The use of CBCT and MDCT allows equivalent and excellent imaging results if optimized protocols are chosen. With both imaging techniques subtle temporal bone structures could be visualized with a similar degree of definition. In vivo differences do not seem to be as large as suggested in several previous studies. (orig.) [de

Congenital ossicular chain malformations with mobile stapes in children: Results in 17 cases.

Science.gov (United States)

Vincent, Robert; Wegner, Inge; Derks, Laura S M; Grolman, Wilko

2016-03-01

To prospectively evaluate surgical findings and hearing results in children undergoing surgery for congenital malformations of the ossicular chain with a mobile stapes footplate (Teunissen and Cremers class III malformations). A nonrandomized, nonblinded, prospective case series. Fifteen consecutive pediatric patients who underwent 17 surgical procedures for congenital ossicular malformations with a mobile stapes at a tertiary referral center were included. In 16 cases, postoperative pure-tone audiometry was available. The surgical technique for repair of the ossicular chain was dictated by the surgical findings at the time of surgery. The majority of the cases underwent ossiculoplasty. A Teflon piston, partial ossicular replacement prosthesis, or total ossicular replacement prosthesis was used in these cases. Associated surgical techniques included malleus relocation, Silastic banding, drilling out of a bony bridge, and a combination of these techniques. Hearing loss was evaluated using preoperative and postoperative four-frequency (0.5, 1, 2, and 4 kHz) audiometry. Air-conduction thresholds, bone-conduction thresholds, and air-bone gaps were measured. Postoperative audiometry was performed at 3, 6, 9, 12, 18, and 24 months after surgery and at a yearly interval thereafter. Overall, a postoperative air-bone gap closure to 10 dB or less was achieved in 63% of the included cases. A postoperative air-bone gap closure to 20 dB or less was achieved in 75%. Postoperative sensorineural hearing loss did not occur in any of the cases. Ossicular reconstruction is a feasible treatment option in children with congenital malformations of the ossicular chain with a mobile stapes footplate. 4. Laryngoscope, 126:682-688, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Intraoperative cone-beam computed tomography and multi-slice computed tomography in temporal bone imaging for surgical treatment.

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Erovic, Boban M; Chan, Harley H L; Daly, Michael J; Pothier, David D; Yu, Eugene; Coulson, Chris; Lai, Philip; Irish, Jonathan C

2014-01-01

Conventional computed tomography (CT) imaging is the standard imaging technique for temporal bone diseases, whereas cone-beam CT (CBCT) imaging is a very fast imaging tool with a significant less radiation dose compared with conventional CT. We hypothesize that a system for intraoperative cone-beam CT provides comparable image quality to diagnostic CT for identifying temporal bone anatomical landmarks in cadaveric specimens. Cross-sectional study. University tertiary care facility. Twenty cadaveric temporal bones were affixed into a head phantom and scanned with both a prototype cone-beam CT C-arm and multislice helical CT. Imaging performance was evaluated by 3 otologic surgeons and 1 head and neck radiologist. Participants were presented images in a randomized order and completed landmark identification questionnaires covering 21 structures. CBCT and multislice CT have comparable performance in identifying temporal structures. Three otologic surgeons indicated that CBCT provided statistically equivalent performance for 19 of 21 landmarks, with CBCT superior to CT for the chorda tympani and inferior for the crura of the stapes. Subgroup analysis showed that CBCT performed superiorly for temporal bone structures compared with CT. The radiologist rated CBCT and CT as statistically equivalent for 18 of 21 landmarks, with CT superior to CBCT for the crura of stapes, chorda tympani, and sigmoid sinus. CBCT provides comparable image quality to conventional CT for temporal bone anatomical sites in cadaveric specimens. Clinical applications of low-dose CBCT imaging in surgical planning, intraoperative guidance, and postoperative assessment are promising but require further investigation.

Spontaneous evolution of an unusual cortical malformation in SOX2 anophthalmia syndrome

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Jay Desai

2013-01-01

Full Text Available Brain malformations such as agenesis and dysgenesis of corpus callosum, pituitary hypoplasia, hypothalamic hamartoma, mesial temporal periventricular heterotopia, and abnormally oriented and misshapen hippocampi have been described with SOX2 gene mutations. A neocortical malformation is presented here in association with SOX2 deletion that over time underwent spontaneous evolution and decrease in size.

Screening of SLC26A4, FOXI1, KCNJ10, and GJB2 in bilateral deafness patients with inner ear malformation.

Science.gov (United States)

Chen, Kaitian; Wang, Xianren; Sun, Liang; Jiang, Hongyan

2012-06-01

Bilateral nonsyndromic sensorineural hearing loss associated with inner ear malformation is closely related to genetics. SLC26A4 is considered to be the major involved gene. Recently, FOXI1 and KCNJ10 mutations have been linked to enlarged vestibular aqueducts and GJB2 mutations linked to temporal bone malformation. The authors aimed to investigate the mutation spectrums of these genes in Chinese patients with bilateral hearing impairment associated with inner ear malformation. Cross-sectional study. Affiliated hospital of the university. The authors analyzed the GJB2, SLC26A4, FOXI1, and KCNJ10 gene sequences in 43 patients presenting with bilateral hearing impairment associated with inner ear malformation using pyrosequencing and direct DNA sequencing. In total, 74.4% (32/43) of patients carried at least 1 of 14 pathogenic SLC26A4 mutations, including 6 novel mutations and 4 polymorphisms. Patients with enlarged vestibular aqueducts had a higher rate of SLC26A4 mutation than Mondini dysplasia patients. No FOXI1 or KCNJ10 potential pathogenic mutation was present, and GJB2 biallelic pathogenic mutations were uncommon (2.3%; 1/43). No significant correlation was observed between the genotype and phenotype of SLC26A4 mutations. SLC26A4 accounts for 74.4% of inner ear malformations in our cohort, whereas FOXI1, KCNJ10, and GJB2 mutations are not common. Other possible genes or external factors may contribute to this multibranch abnormality.

Bone dysplasia with optic atrophy, vascular malformation and seizures in a 14-year-old girl - a case report

International Nuclear Information System (INIS)

Kuleta-Bosak, E.; Kluczewska, E.; Gibinska, E.; Jamroz, E.; Augustyn, D.; Gluszkiewicz, E.

2007-01-01

The heritable skeletal dysplasias or osteochondrodysplasias are a large heterogeneous group of disorders associated with abnormal shape, growth, or integrity of bones. Osteopetrosis is a collective term for a range of sclerosing bone diseases with various degree of defective remodeling. Increased bone density is the predominant radiologic feature. The skull is often involved with basal sclerosis and the sinuses are obliterated. The most serious consequences of the osteopetroses are seen in the nervous system. Because of perturbed remodeling of the skull bones, many aspects of the brain and cranial nerve function are endangered. Cranial nerves, blood vessels and the spinal cord may be compressed by progressive occlusion of cranial foramina. Carious, misplaced teeth, dysplastic fingernails, tendency to pathologic fractures are the other clinical manifestations. The authors present a 14-year-old girl with dysmorphic features, optic atrophy, CNS vessel malformation, pathologic fractures and seizures. The girl had a wide range of clinical and radiographic symptoms of bone dysplasias together with a giant left internal carotid artery aneurysm and epilepsy. On the basis of clinical and radiological features, a disease belonging to the group of skeletal dysplasias was recognized in our patient. The configuration of the presented symptoms does not allow at the moment strict classification to hitherto determined forms of dysplasia. This leads to the necessity of extending diagnostics, especially by molecular tests, and further long-lasting observations, which perhaps would allow classification of the presented syndrome to one of the known groups, or determination of a new clinical entity. (author)

Congenital vascular malformations in scintigraphic evaluation

International Nuclear Information System (INIS)

Pilecki, Stanisław; Gierach, Marcin; Gierach, Joanna; Świętaszczyk, Cyprian; Junik, Roman; Lasek, Władysław

2014-01-01

Congenital vascular malformations are tumour-like, non-neoplastic lesions caused by disorders of vascular tissue morphogenesis. They are characterised by a normal cell replacement cycle throughout all growth phases and do not undergo spontaneous involution. Here we present a scintigraphic image of familial congenital vascular malformations in two sisters. A 17-years-old young woman with a history of multiple hospitalisations for foci of vascular anomalies appearing progressively in the upper and lower right limbs, chest wall and spleen. A Parkes Weber syndrome was diagnosed based on the clinical picture. Due to the occurrence of new foci of malformations, a whole-body scintigraphic examination was performed. A 12-years-old girl reported a lump in the right lower limb present for approximately 2 years, which was clinically identified as a vascular lesion in the area of calcaneus and talus. Phleboscintigraphy visualized normal radiomarker outflow from the feet via the deep venous system, also observed in the superficial venous system once the tourniquets were released. In static and whole-body examinations vascular malformations were visualised in the area of the medial cuneiform, navicular and talus bones of the left foot, as well as in the projection of right calcaneus and above the right talocrural joint. People with undiagnosed disorders related to the presence of vascular malformations should undergo periodic follow-up to identify lesions that may be the cause of potentially serious complications and to assess the results of treatment. Presented scintigraphic methods may be used for both diagnosing and monitoring of disease progression

Rectal and splenic vascular malformation in klippel trenaunay weber syndrome: A case report

Energy Technology Data Exchange (ETDEWEB)

Kim, Ha Youn; Chang, Yun Woo; Lee, Dong Hwan [Soonchunhyang Univ. Hospital, Seoul (Korea, Republic of)

2012-10-15

Klippel Trenaunay Weber syndrome (KTWS) is a rare congenital disorder, characterized by a cutaneous vascular nevus of the involved extremity, vascular malformations, bone and soft tissue hypertrophy of the extremity. We present the case of an 18 year old female patient with KTWS, showing a marked rectosigmoid wall thickening and phlebolith, and also variable sized cystic masses in the spleen, as a result of vascular malformations.

[Diagnostic value of high-resolution computed tomography imaging in congenital inner ear malformations].

Science.gov (United States)

Sun, Xiaowei; Ding, Yuanping; Zhang, Jianji; Chen, Ying; Xu, Anting; Dou, Fenfen; Zhang, Zihe

2007-02-01

To observe the inner ear structure with volume rendering (VR) reconstruction and to evaluate the role of high-resolution computed tomography (HRCT) in congenital inner ear malformations. HRCT scanning was performed in 10 patients (20 ears) without ear disease (control group) and 7 patients (11 ears) with inner ear malformations (IEM group) and the original data was processed with VR reconstruction. The inner ear osseous labyrinth structure in the images generated by these techniques was observed respectively in the normal ears and malformation ears. The inner ear osseous labyrinth structure and the relationship was displayed clearly in VR imaging in the control group,meanwhile, characters and degree of malformed structure were also displayed clearly in the IEA group. Of seven patients (11 ears) with congenital inner ear malformations, the axial, MPR and VR images can display the site and degree in 9 ears. VR images were superior to the axial images in displaying the malformations in 2 ears with the small lateral semicircular canal malformations. The malformations included Mondini deformity (7 ears), vestibular and semicircular canal malformations (3 ears), vestibular aqueduct dilate (7 ears, of which 6 ears accompanied by other malformations) , the internal auditory canal malformation (2 ears, all accompanied by other malformations). HRCT can display the normal structure of bone inner ear through high quality VR reconstructions. VR images can also display the site and degree of the malformations three-dimensionally and intuitively. HRCT is valuable in diagnosing the inner ear malformation.

Temporal bone fracture following blunt trauma caused by a flying fish.

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Goldenberg, D; Karam, M; Danino, J; Flax-Goldenberg, R; Joachims, H Z

1998-10-01

Blunt trauma to the temporal region can cause fracture of the skull base, loss of hearing, vestibular symptoms and otorrhoea. The most common causes of blunt trauma to the ear and surrounding area are motor vehicle accidents, violent encounters, and sports-related accidents. We present an obscure case of a man who was struck in the ear by a flying fish while wading in the sea with resulting temporal bone fracture, sudden deafness, vertigo, cerebrospinal fluid otorrhoea, and pneumocephalus.

Dual-time-point FDG-PET/CT Imaging of Temporal Bone Chondroblastoma: A Report of Two Cases

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Akira Toriihara

2015-07-01

Full Text Available Temporal bone chondroblastoma is an extremely rare benign bone tumor. We encountered two cases showing similar imaging findings on computed tomography (CT, magnetic resonance imaging (MRI, and dual-time-point 18F-fluorodeoxyglucose (18F-FDG positron emission tomography (PET/CT. In both cases, CT images revealed temporal bone defects and sclerotic changes around the tumor. Most parts of the tumor showed low signal intensity on T2- weighted MRI images and non-uniform enhancement on gadolinium contrast-enhanced T1-weighted images. No increase in signal intensity was noted in diffusion-weighted images. Dual-time-point PET/CT showed markedly elevated 18F-FDG uptake, which increased from the early to delayed phase. Nevertheless, immunohistochemical analysis of the resected tumor tissue revealed weak expression of glucose transporter-1 and hexokinase II in both tumors. Temporal bone tumors, showing markedly elevated 18F-FDG uptake, which increases from the early to delayed phase on PET/CT images, may be diagnosed as malignant bone tumors. Therefore, the differential diagnosis should include chondroblastoma in combination with its characteristic findings on CT and MRI.

Theoretical analysis of the spatio-temporal structure of bone multicellular units

International Nuclear Information System (INIS)

Buenzli, P R; Pivonka, P; Gardiner, B S; Smith, D W; Dunstan, C R; Mundy, G R

2010-01-01

Bone multicellular units (BMUs) maintain the viability of the skeletal tissue by coordinating locally the sequence of bone resorption and bone formation performed by cells of the osteoclastic and osteoblastic lineage. Understanding the emergence and the net bone balance of such structured microsystems out of the complex network of biochemical interactions between bone cells is fundamental for many bone-related diseases and the evaluation of fracture risk. Based on current experimental knowledge, we propose a spatio-temporal continuum model describing the interactions of osteoblastic and osteoclastic cells. We show that this model admits travelling-wave-like solutions with well-confined cell profiles upon specifying external conditions mimicking the environment encountered in cortical bone remodelling. The shapes of the various cell concentration profiles within this travelling structure are intrinsically linked to the parameters of the model such as differentiation, proliferation, and apoptosis rates of bone cells. The internal structure of BMUs is reproduced, allowing for experimental calibration. The spatial distribution of the key regulatory factors can also be exhibited, which in diseased states could give hints as to the biochemical agent most accountable for the disorder.

Bilateral Non-Hodgkin’s Lymphoma of the Temporal Bone: A Rare and Unusual Presentation

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Sanjay Vaid

2016-01-01

Full Text Available Primary lymphoma of the temporal bone is an unusual finding in clinical practice and bilateral affection is even more rare. To the best of our knowledge, there are no reports of bilateral primary temporal bone lymphoma without middle ear involvement in the English medical literature so far. We report, for the first time, a case of primary lymphoma involving both temporal bones which presented with left-sided infranuclear facial palsy. A combination of contrast enhanced magnetic resonance imaging (MRI and high resolution computed tomography (HRCT was used to characterize and to map the extent of the lesion, as well as to identify the exact site of facial nerve affection. An excision biopsy and immunohistochemistry revealed diffuse large B-cell non-Hodgkin’s lymphoma (DLBCL. Whole body fluorodeoxyglucose (FDG positron emission tomography-computed tomography study (PET-CT was performed to stage the disease. The patient was treated with chemotherapy and radiation therapy and is now on regular follow-up. The patient is alive and asymptomatic without disease progression for the last twenty months after initial diagnosis.

European status on temporal bone training

DEFF Research Database (Denmark)

Frithioff, Andreas; Sørensen, Mads Sølvsten; Andersen, Steven Arild Wuyts

2018-01-01

laboratory facilities for training seems to be decreasing. Alternatives to traditional training can consist of drilling artificial models made of plaster or plastic but also virtual reality (VR) simulation. Nevertheless, the integration and availability of these alternatives into specialist training programs...... training modality. CONCLUSIONS: VR simulation and artificial models are reported to be used at many leading training departments already. Decreasing availability of cadavers, lower costs of VR simulation and artificial models, in addition to established evidence for a positive effect on the trainees......' competency, were reported as the main reasons. Most remaining departments expect to implement VR simulation and artificial models for temporal bone training into their residency programs in the near future....

Inflammation and tumors of the temporal bone; Entzuendungen und Tumoren des Schlaefenbeins

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Burian, M. [Universitaetsklinik fuer Hals-, Nasen- und Ohrenkrankheiten, Allgemeines Krankenhaus, Wien (Austria)

1997-12-01

The term `inflammation of the middle ear` covers a couple of deseases which range from the acute otitis media to the middle ear cholesteatoma. However, a clear characterization of a certain pathology is essential for any further treatment. Therefore this article presents a short overview about the different types of infections and their clinical manifestation. The tumors of the temporal bone show a great variety in their incidence. Even if tumors like the acoustic neurinoma or the paraganglioma are compareable common, the chondroblastoma of the temporal bone is absolutely rare. In spite of these differences the individual temporal bone neoplasias are shortly mentioned herein. (orig.) [Deutsch] Der Begriff Mittelohrentzuendung umfasst ein weites Spektrum von Krankheiten welches von der akuten Mittelohrentzuendung bis hin zum Cholesteatom reicht. Es soll in diesem Artikel eine kurze Uebersicht ueber die verschiedenen Entzuendungen gegeben werden, wobei vor allem auf eine klare Begriffsdefinition der einzelnen Entzuendungsformen und deren klinisches Erscheinungsbild geachtet wurde. Bei den Tumoren des Schlaefenbeins ist ein grosser Unterschied in der Inzidenz der einzelnen Tumoren gegeben. Waehrend Neubildungen wie das Akustikusneurinom oder das Paragangliom vergleichsweise haeufig im klinischen Alltag zu sehen sind, stellen Veraenderungen wie das Chondroblastom eine Raritaet dar. Trotz dieses Unterschieds im Vorkommen der verschiedenen Tumoren, wurde versucht, einen kurzen Gesamtueberblick ueber die Tumore des Mittel- und Innenohres zu geben. (orig.)

Genetics Home Reference: capillary malformation-arteriovenous malformation syndrome

Science.gov (United States)

... Institute: How the Heart Works National Institute of Neurological Disorders and Stroke: Arteriovenous Malformation Educational Resources (7 links) Boston Children's Hospital: Arteriovenous Malformation Boston Children's Hospital: Capillary Malformation ...

Temporal Bone Fractures and its Classification: Retrospective Study of Incidence, Causes, Clinical Features, Complications and Outcome

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Umamaheshwari Basavaraju

2017-10-01

Full Text Available Introduction: Temporal bone fracture is usually associated with high energy head injury and can cause potentially severe complications. Immediate detection of temporal bone fracture and its complications helps in providing early and effective treatment, which if left untreated can have drastic consequences. Aim: The main objective of the study is to document the frequency and most prevalent type of temporal bone fracture, co-existing complications and to establish association between them. Materials and Methods: One year (2015-2016 retrospective study of head injured patients presented to the Emergency Department, Mysore Medical College And Research Institute was conducted. Age and gender distribution, cause of injury, radiological findings, otorhinolaryngological clinical presentations and treatment given were analyzed. The results were tabulated and were evaluated by Microsoft Excel 2013. Results: Out of 1450 patients evaluated for head injury 154 patients were positive for temporal bone fracture. Incidence of the study was 10.6%. Majority of the patients were male (66.2% and were between 30 to 40 years (50.1%. The major cause of injury was motor vehicle accidents (84.48%. Right side was involved (58.4% more than the left side (41.5%. Most common clinical presentation was otorrhea 68.8%, followed by otalgia (35.04% and otorhinorrhea (24.67%. Longitudinal type fracture was most frequent 56.25%. Otic capsule involvement was present in 35.93%. Most of the fractures were managed conservatively whereas surgery was required in 12 patients (7.7%. Conclusion: Temporal bone fractures were frequently associated with severe traumatic brain injury leading to serious long term morbidity and sequelae. CT-scan is of utmost importance in detection of fractures and its complications.

Cetuximab with radiotherapy as an alternative treatment for advanced squamous cell carcinoma of the temporal bone.

Science.gov (United States)

Ebisumoto, Koji; Okami, Kenji; Hamada, Masashi; Maki, Daisuke; Sakai, Akihiro; Saito, Kosuke; Shimizu, Fukuko; Kaneda, Shoji; Iida, Masahiro

2018-06-01

The prognosis of advanced temporal bone cancer is poor, because complete surgical resection is difficult to achieve. Chemoradiotherapy is one of the available curative treatment options; however, its systemic effects on the patient restrict the use of this treatment. A 69-year-old female (who needed peritoneal dialysis) presented at our clinic with T4 left external auditory canal cancer and was treated with cetuximab plus radiotherapy (RT). The primary lesion showed complete response. The patient is currently alive with no evidence of disease two years after completion of the treatment and does not show any late toxicity. This is the first advanced temporal bone cancer patient treated with RT plus cetuximab. Cetuximab plus RT might be a treatment alternative for patients with advanced temporal bone cancer. Copyright © 2017 Elsevier B.V. All rights reserved.

Radiation injury to the temporal bone

International Nuclear Information System (INIS)

Guida, R.A.; Finn, D.G.; Buchalter, I.H.; Brookler, K.H.; Kimmelman, C.P.

1990-01-01

Osteoradionecrosis of the temporal bone is an unusual sequela of radiation therapy to the head and neck. Symptoms occur many years after the radiation is administered, and progression of the disease is insidious. Hearing loss (sensorineural, conductive, or mixed), otalgia, otorrhea, and even gross tissue extrusion herald this condition. Later, intracranial complications such as meningitis, temporal lobe or cerebellar abscess, and cranial neuropathies may occur. Reported here are five cases of this rare malady representing varying degrees of the disease process. They include a case of radiation-induced necrosis of the tympanic ring with persistent squamous debris in the external auditory canal and middle ear. Another case demonstrates the progression of radiation otitis media to mastoiditis with bony sequestration. Further progression of the disease process is seen in a third case that evolved into multiple cranial neuropathies from skull base destruction. Treatment includes systemic antibiotics, local wound care, and debridement in cases of localized tissue involvement. More extensive debridement with removal of sequestrations, abscess drainage, reconstruction with vascularized tissue from regional flaps, and mastoid obliteration may be warranted for severe cases. Hyperbaric oxygen therapy has provided limited benefit

Clinical significance of temporal bone CT with regard to hearing preservation in acoustic neurinoma removal

Energy Technology Data Exchange (ETDEWEB)

Umezu, Hiromichi; Seki, Yojirou; Aiba, Tadashi

1988-02-01

Since Rand and Kurze discussed the possibility of the anatomic preservation of the cochlear nerve via the posterior fossa transmeatal approach for the acoustic neurinoma, there have been increasingly many reports on the preservation of cochlear nerve function. With recent advances in diagnostic tools, today's neurosurgeon is able to make an earlier and more precise diagnosis of acoustic neurinomas even when the patient has good hearing. If useful hearing is to be kept, the cochlear nerve and blood supply of the labyrinth have to be preserved. In addition, surgical entry into the labyrinth, upon the removal of the posterior wall of the internal auditory canal, must be avoided, since it is likely to result in permanent hearing loss. Because of its superior contrast and spatial resolution, thin-section and high-resolution computed tomography of the temporal bone has the great advantage of demonstrating the exact relationship of the internal auditory canal to the posterior semicircular canal, the vestibule, and the common crus. In this study, thin-section, high-resolution computed tomography of the temporal bone was performed in 11 cases of acoustic neurinoma pre- and postoperatively. At operation, the lateral limit of the bone removal of the posterior internal auditory canal was determined on the basis of preoperative computed tomography of the temporal bone. As a result, inadvertent entry into the labyrinth was avoided, and useful hearing was preserved in 8 cases. In order to preserve useful hearing following total tumor removal, it is essential to plan the operative strategy meticulously on the basis of the findings of the preoperative investigation, Including temporal bone CT.

The temporal bones from Sima de los Huesos Middle Pleistocene site (Sierra de Atapuerca, Spain). A phylogenetic approach.

Science.gov (United States)

Martínez, I; Arsuaga, J L

1997-01-01

Three well-preserved crania and 22 temporal bones were recovered from the Sima de los Huesos Middle Pleistocene site up to and including the 1994 field season. This is the largest sample of hominid temporal bones known from a single Middle Pleistocene site and it offers the chance to characterize the temporal bone morphology of an European Middle Pleistocene population and to study the phylogenetic relationships of the SH sample with other Upper and Middle Pleistocene hominids. We have carried out a cladistic analysis based on nine traits commonly used in phylogenetic analysis of Middle and Late Pleistocene hominids: shape of the temporal squama superior border, articular eminence morphology, contribution of the sphenoid bone to the median glenoid wall, postglenoid process projection, tympanic plate orientation, presence of the styloid process, mastoid process projection, digastric groove morphology and anterior mastoid tubercle. We have found two autapomorphies on the Home erectus temporal bone: strong reduction of the postglenoid process and absence of the styloid process. Modern humans, Neandertals and the Middle Pleistocene fossils from Europe and Africa constitute a clade characterized by a convex superior border of the temporal squama. The European Middle Pleistocene fossils from Sima de los Huesos, Petralona, Steinheim, Bilzingsleben and Castel di Guido share a Neandertal apomorphy: a relatively flat articular eminence. The fossils from Ehringsdorf, La Chaise Suardi and Biache-Saint-Vaast also display another Neandertal derived trait: an anteriorly obliterated digastric groove. Modern humans and the African Middle Pleistocene fossils share a synapomorphy: a sagittally orientated tympanic plate.

Primary giant myxoma of the temporal bone with major intracranial extension: presenting with hearing impairment and ear polyp

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Satyarthee Guru Dutta

2016-12-01

Full Text Available Myxomas are mesenchymal origin, benign tumor, constituting approximately half of the benign cardiac tumors. Occasionally, it may also occurs at other locations, though the intracranial location of a myxoma is considered exceptionally rare. Only isolated few cases of intracranial myxoma are reported in the literature, almost all were locally confined within the originating bone. The extensive Pubmed and Medline search yielded only eight cases of primary myxoma arising in the temporal bone with extension into intracranial compartment. However intracranial extension is limited as early detection, however, Osterdock et al reported a case also arising from temporal bone with extensive intracranial extension. Author report an interesting case of intracranial myxoma in 27- year- old- male, involving the temporal bone associated with extensive bony erosion and also extending into infratemporal fossa, mastoid, and frontoparietal region and a polypoidal mass protruding into external ear. To the best of knowledge of authors, temporal myxoma presenting with external ear polypoidal mass, which underwent successful surgical excision is not reported and represent first case in the world literature.

Microsurgical and Endoscopic Anatomy for Intradural Temporal Bone Drilling and Applications of the Electromagnetic Navigation System: Various Extensions of the Retrosigmoid Approach.

Science.gov (United States)

Matsushima, Ken; Komune, Noritaka; Matsuo, Satoshi; Kohno, Michihiro

2017-07-01

The use of the retrosigmoid approach has recently been expanded by several modifications, including the suprameatal, transmeatal, suprajugular, and inframeatal extensions. Intradural temporal bone drilling without damaging vital structures inside or beside the bone, such as the internal carotid artery and jugular bulb, is a key step for these extensions. This study aimed to examine the microsurgical and endoscopic anatomy of the extensions of the retrosigmoid approach and to evaluate the clinical feasibility of an electromagnetic navigation system during intradural temporal bone drilling. Five temporal bones and 8 cadaveric cerebellopontine angles were examined to clarify the anatomy of retrosigmoid intradural temporal bone drilling. Twenty additional cerebellopontine angles were dissected in a clinical setting with an electromagnetic navigation system while measuring the target registration errors at 8 surgical landmarks on and inside the temporal bone. Retrosigmoid intradural temporal bone drilling expanded the surgical exposure to allow access to the petroclival and parasellar regions (suprameatal), internal acoustic meatus (transmeatal), upper jugular foramen (suprajugular), and petrous apex (inframeatal). The electromagnetic navigation continuously guided the drilling without line of sight limitation, and its small devices were easily manipulated in the deep and narrow surgical field in the posterior fossa. Mean target registration error was less than 0.50 mm during these procedures. The combination of endoscopic and microsurgical techniques aids in achieving optimal exposure for retrosigmoid intradural temporal bone drilling. The electromagnetic navigation system had clear advantages with acceptable accuracy including the usability of small devices without line of sight limitation. Copyright © 2017 Elsevier Inc. All rights reserved.

A metastatic glomus jugulare tumor. A temporal bone report

International Nuclear Information System (INIS)

El Fiky, F.M.; Paparella, M.M.

1984-01-01

The clinicopathologic findings in the temporal bone of a patient with a highly malignant metastasizing glomus jugulare tumor are reported. The patient exhibited all the symptoms of primary malignant tumors of the ear, including facial paralysis, otorrhea, pain, hearing loss, tinnitus, dizziness, and vertigo. He was treated with cobalt irradiation followed by radium implant in the ear canal for a residual tumor; then a left-sided radical mastoidectomy was performed

Bilateral Facial Paralysis Caused by Bilateral Temporal Bone Fracture: A Case Report and a Literature Review

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Sultan Şevik Eliçora

2015-01-01

Full Text Available Bilateral facial paralysis caused by bilateral temporal bone fracture is a rare clinical entity, with seven cases reported in the literature to date. In this paper, we describe a 40-year-old male patient with bilateral facial paralysis and hearing loss that developed after an occupational accident. On physical examination, House-Brackmann (HB facial paralysis of grade 6 was observed on the right side and HB grade 5 paralysis on the left. Upon temporal bone computed tomography (CT examination, a fracture line exhibiting transverse progression was observed in both petrous temporal bones. Our patient underwent transmastoid facial decompression surgery of the right ear. The patient refused a left-side operation. Such patients require extensive monitoring in intensive care units because the presence of multiple injuries means that facial functions are often very difficult to evaluate. Therefore, delays may ensue in both diagnosis and treatment of bilateral facial paralysis.

Pneumatization of the Temporal Bones in a Greenlandic Inuit Anthropological Material

DEFF Research Database (Denmark)

Homøe, P; Lynnerup, N

1991-01-01

The degree of pneumatization of the temporal bones correlates with exposure during childhood and adolescence to infectious middle ear diseases (IMED), both acute and chronic. The pneumatized area as seen on cranial X-rays can be measured. This was applied to an anthropological material in order...

Study of a temporal bone of Homo heildelbergensis.

Science.gov (United States)

Urquiza, Rafael; Botella, Miguel; Ciges, Miguel

2005-05-01

The characteristic features of the Hh specimen conformed to those of other Pleistocene human fossils, indicating strong cranial structures and a heavy mandible. The mastoid was large and suggested a powerful sternocleidomastoid muscle. The inner ear and tympanic cavities were similar in size and orientation, suggesting that their functions were probably similar. Our observations suggest that the left ear of this Hh specimen was healthy. The large canaliculo-fenestral angle confirms that this ancestor was bipedal. It also strongly suggests that Hh individuals were predisposed to develop certain pathologies of the labyrinth capsule associated with bipedalism, in particular otosclerosis. We studied a temporal bone of Homo heidelbergensis (Hh) in order to investigate the clinical and physiological implications of certain morphological features, especially those associated with the evolutionary reorganization of the inner ear. The bone, found in a breach of a cave near MAáaga in southern Spain, together with Middle Upper Pleistocene faunal remains, is >300000 years old. Four analytical methods were employed. A 3D high-resolution surface laser scan was used for anatomical measurements. For the sectional analysis of the middle and inner ears of Hh we used high-resolution CT, simultaneously studying a normal temporal bone from Homo sapiens sapiens (Hss). To study the middle and inner ear spaces we used 3D reconstruction CT preceded by an intra-bone air shielding technique. To examine the tympanic cavities and measure the canaliculo fenestral angle, we used a special minimally invasive endoscopic procedure. The surface, sectional and 3D CT examinations showed that the Hh specimen was generally more robust and larger than the Hss specimen. It had a large glenoid fossa. The external meatus was wide and deep. The middle ear, and especially the mastoid, was large and widely pneumatized. There were no appreciable differences in the position and size of the labyrinthine spaces

Morphometrical Study of the Temporal Bone and Auditory Ossicles in Guinea Pig

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Ahmadali Mohammadpour

2011-03-01

Full Text Available In this research, anatomical descriptions of the structure of the temporal bone and auditory ossicles have been performed based on dissection of ten guinea pigs. The results showed that, in guinea pig temporal bone was similar to other animals and had three parts; squamous, tympanic and petrous .The tympanic part was much better developed and consisted of oval shaped tympanic bulla with many recesses in tympanic cavity. The auditory ossicles of guinea pig concluded of three small bones; malleus, incus and stapes but the head of the malleus and the body of incus were fused and forming a malleoincudal complex. The average of morphometric parameters showed that the malleus was 3.53 ± 0.22 mm in total length. In addition to head and handle, the malleus had two distinct process; lateral and muscular. The incus had a total length 1.23 ± 0.02mm. It had long and short crus although the long crus was developed better than short crus. The lenticular bone was a round bone that articulated with the long crus of incus. The stapes had a total length 1.38 ± 0.04mm. The anterior crus(0.86 ± 0.08mm was larger than posterior crus (0.76 ± 0.08mm. It is concluded that, in the guinea pig, the malleus and the incus are fused, forming a junction called incus-malleus, while in the other animals these are separate bones. The stapes is larger and has a triangular shape and the anterior and posterior crus are thicker than other rodents. Therefore, for otological studies, the guinea pig is a good lab animal.

Large capillary hemangioma of the temporal bone with a dural tail sign: A case report

KAUST Repository

YANG, GUANG

2014-05-13

The present study reports a rare case of large capillary hemangioma of the temporal bone with a dural tail sign. A 57-year-old female presented with pulsatile tinnitus and episodic vertigo associated with a ten-year history of an intermittent faint headache. Magnetic resonance imaging revealed a mass in the right petrous bone, which was hypointense on T1-weighted images and heterogeneously hyperintense on T2-weighted images, and showed a dural tail sign following gadolinium administration. Pre-operatively, this tumor was believed to be a meningioma. During surgery, the vascular tumor was removed by a modified pterional approach. A histopathological examination indicated that the tumor was a capillary hemangioma. Although intraosseous capillary hemangiomas are rare, they most frequently affect the temporal bone. Hemangiomas of the temporal bone may mimic other more common basal tumors. The diagnosis is most often made during surgical resection. The dural tail sign is not specific for meningioma, as it also occurs in other intracranial or extracranial tumors. The treatment of intratemporal hemangiomas is complete surgical excision, with radiotherapy used for unresectable lesions. To the best of our knowledge, the present study is the fourth case of intraosseous intracranial capillary hemangioma, but the largest intratemporal hemangioma to be reported in the literature to date.

Large capillary hemangioma of the temporal bone with a dural tail sign: A case report

KAUST Repository

YANG, GUANG; LI, CHENGUANG; CHEN, XIN; LIU, YAOHUA; HAN, DAYONG; Gao, Xin; KAWAMOTO, KEIJI; ZHAO, SHIGUANG

2014-01-01

The present study reports a rare case of large capillary hemangioma of the temporal bone with a dural tail sign. A 57-year-old female presented with pulsatile tinnitus and episodic vertigo associated with a ten-year history of an intermittent faint headache. Magnetic resonance imaging revealed a mass in the right petrous bone, which was hypointense on T1-weighted images and heterogeneously hyperintense on T2-weighted images, and showed a dural tail sign following gadolinium administration. Pre-operatively, this tumor was believed to be a meningioma. During surgery, the vascular tumor was removed by a modified pterional approach. A histopathological examination indicated that the tumor was a capillary hemangioma. Although intraosseous capillary hemangiomas are rare, they most frequently affect the temporal bone. Hemangiomas of the temporal bone may mimic other more common basal tumors. The diagnosis is most often made during surgical resection. The dural tail sign is not specific for meningioma, as it also occurs in other intracranial or extracranial tumors. The treatment of intratemporal hemangiomas is complete surgical excision, with radiotherapy used for unresectable lesions. To the best of our knowledge, the present study is the fourth case of intraosseous intracranial capillary hemangioma, but the largest intratemporal hemangioma to be reported in the literature to date.

Temporal bone CT analysis of congenital ear anomalies

International Nuclear Information System (INIS)

Hwang, Jung Won; Moon, Min Joo; Sung, Kyu Bo

1988-01-01

Authors analysed the CT findings of the congenital ear anomalies of twenty-nine patients for 2 years and 3 months. The results were as follows: 1. Most of the patients were under the age of 20 (82.7%) and prevalent in male (72.4%). 2. Clinically, congenital ear anomalies were detected in 20 patients (68.9%), conductive hearing loss in 4, sensorineural hearing loss in 1, and the remained 4 patients were detected incidentally without clinical symptom. 3. In the cases of unilateral involvement of 20 patients, right ear was more common (12/20). Eight of 9 bilateral involvement showed similar degree. 4. The middle ear malformations were found in 22 patients (75.9%) and bilateral in 4 patients. 26 cases of middle ear malformations had been classified by Frey into 4 groups; Group I in 5, Group II in 9, Graoup III in 9 and Group IV in 3. 5. Incidentally found ear anomaly was lateral semicircular canal formed a single cavity with the vestibule in all patients (5 pts.). 6. Inner ear malformations accompanying sensorineural hearing loss were found in 3 patients with bilateral involvement and middle ear malformations were accompanied in 2 patients. The degree of involvement of labyrinth was variable.

Chemodectomas arising in temporal bone structures

International Nuclear Information System (INIS)

Dickens, W.J.; Million, R.R.; Cassisi, N.J.; Singleton, G.T.

1982-01-01

Eighteen patients with chemodectomas arising in temporal bone structures were evaluated and treated at the University of Florida. Seventeen patients have each been followed a minimum of 3 years. Patients were retrospectively staged as having ''local'' or ''advanced'' disease, depending on the presence or absence of bone destruction and/or cranial nerve involvement. Fourteen of the patients received radiation therapy as all or part of their therapy; 6 patients were treated with radiation therapy alone, 3 patients were irradiated immediately postoperatively for residual disease, and 5 patients had radiation therapy for recurrence after operation. They were treated with cobalt-60 radiation with doses ranging from 3760 to 5640 rad. All irradiated patients demonstrated evidence of tumor regression, and none have had tumor recurrence with followup of 3-12 years. Of the 8 patients with cranial nerve paralysis prior to therapy, 5 had return of function of 1 or more cranial nerves. One of 6 patients treated initially with radiation therapy had a complication, while 6 of 8 patients irradiated postoperatively had complications. None of the complications were fatal. Three patients treated by operation for early disease limited to the hypotympanum had the disease controlled for 11-12 years. Guidelines for the selection of initial therapy are discussed

CT and MRI characteristica of tumours of the temporal bone and the cerebello-pontine angle

International Nuclear Information System (INIS)

Imhof, H.; Henk, C.B.; Dirisamer, A.; Czerny, C.; Gstoettner, W.

2003-01-01

Tumours lesions of the temporal bone and of the cerebello-pontine angle are rare.This tumours can be separated into benign and malignant lesions. In this paper the CT and MRI characteristica of tumours of the temporal bone and the cerebello-pontane angle will be demonstrated. High resolution CT (HRCT) as usually performed in the axial plane are using a high resolution bone window level setting, coronal planes are the reconstructed from the axial data set or will be obtained directly. With the MRI FLAIR sequence in the axial plane the whole brain will be scanned either to depict or exclude a tumour invasion into the brain. After this,T2-weighted fast spin echo sequences or fatsuppressed inversion recovery sequences in high resolution technique in the axial plane will be obtained from the temporal bone and axial T1-weighted spinecho sequences before and after the intravenous application of contrast material will be obtained of this region. Finally T1-weighted spinecho sequences in high resolution technique with fatsuppression after the intravenous application of contrast material will be performed in the coronal plane. HRCT and MRI are both used to depict the most exact tumorous borders. HRCT excellently depicts the osseous changes for example exostosis of the external auditory canal, while also with HRCT osseous changes maybe characterized into more benign or malignant types. MRI has a very high soft tissue contrast and may therefore either characterize vascular space-occupying lesions for example glomus jugulare tumours or may differentiate between more benign or malignant lesions. In conclusion HRCT and MRI of the temporal bone are excellent methods to depict and mostly characterize tumour lesions and can help to differentiate between benign and malignant lesion. These imaging methods shall be used complementary and may have a great impact for the therapeutic planning. (orig.) [de

Congenital external auditory canal atresia and stenosis: temporal bone CT findings

Energy Technology Data Exchange (ETDEWEB)

Lee, Dong Hoon; Kim, Bum Soo; Jung, So Lyung; Kim, Young Joo; Chun, Ho Jong; Choi, Kyu Ho; Park, Shi Nae [College of Medicine, Catholic Univ. of Korea, Seoul (Korea, Republic of)

2002-04-01

To determine the computed tomographic (CT) findings of atresia and stenosis of the external auditory canal (EAC), and to describe associated abnormalities in surrounding structures. We retrospectively reviewed the axial and coronal CT images of the temporal bone in 15 patients (M:F=8:7;mean age, 15.8 years) with 16 cases of EAC atresia (unilateral n=11, bilateral n=1) and EAC stenosis (unilateral n=3). Associated abnormalities of the EAC, tympanic cavity, ossicles, mastoid air cells, eustachian tube, facial nerve course, mandibular condyle and condylar fossa, sigmoid sinus and jugular bulb, and the base of the middle cranial fossa were evaluated. Thirteen cases of bony EAC atresia (one bilateral), with an atretic bony plate, were noted, and one case of unilateral membranous atresia, in which a soft tissue the EAC. A unilateral lesion occurred more frequently on the right temporal bone (n=8, 73%). Associated abnormalities included a small tympanic cavity (n=8, 62%), decreased mastoid pneumatization (n=8, 62%), displacement of the mandibular condyle and the posterior wall of the condylar fossa (n=7, 54%), dilatation of the Eustachian tube (n=7, 54%), and inferior displacement of the temporal fossa base (n=8, 62%). Abnormalities of ossicles were noted in the malleolus (n=12, 92%), incus (n=10, 77%) and stapes (n=6, 46%). The course of the facial nerve was abnormal in four cases, and abnormality of the auditory canal was noted in one. Among three cases of EAC stenosis, ossicular aplasia was observed in one, and in another the location of the mandibular condyle and condylar fossa was abnormal. In the remaining case there was no associated abnormality. Atresia of the EAC is frequently accompanied by abnormalities of the middle ear cavity, ossicles, and adjacent structures other than the inner ear. For patients with atresia and stenosis of this canal, CT of the temporal bone is essentially helpful in evaluating these associated abnormalities.

Transmastoid approach to temporal bone cerebrospinal fluid leaks.

Science.gov (United States)

Oliaei, Sepehr; Mahboubi, Hossein; Djalilian, Hamid R

2012-01-01

The aim of the study was to evaluate various presentations and treatment options for spontaneous cerebrospinal fluid (CSF) leakage originating in the temporal bone. Clinical data and imaging results for 18 ears (15 patients) presenting with spontaneous CSF leakage originating in the temporal bone were reviewed. Average follow-up period was 13.5 months. The main outcome measure was presence of persistent CSF leak postoperatively. A standard postauricular mastoidectomy was performed. Fifteen patients diagnosed with spontaneous CSF leakage over an 8-year period including 3 treated for bilateral disease were included in the study. The age ranged between 33 and 83 years. Presenting symptoms included serous otitis media (44%), persistent otorrhea after tympanostomy tube placement (28%), and meningitis (28%). Preoperative diagnosis was made using imaging studies and was substantiated by observation of CSF leakage and dural herniation intraoperatively. Treatment was eustachian tube plugging (5%), mastoidectomy with fat obliteration (61%), middle fossa approach with extradural (17%), intradural repair (5%), or combined middle fossa and transmastoid (TM) approach (11%). Successful treatment was obtained in 17 of the 18 cases. The last 9 patients in the series underwent TM approach alone for repair with no treatment failures. Repair of defects in tegmen mastoideum and posterior fossa can be successfully achieved on an outpatient basis without regard to size and multitude of defects via TM approach. This approach obviates the need for a craniotomy or lumbar drain. Copyright © 2012 Elsevier Inc. All rights reserved.

Determination of a facial nerve safety zone for navigated temporal bone surgery

NARCIS (Netherlands)

Voormolen, E.H.J.; Stralen, van M.; Woerdeman, P.A.; Pluim, J.P.W.; Noordmans, H.J.; Viergever, M.A.; Regli, L.; Berkelbach van der Sprenkel, J.W.

2012-01-01

Transtemporal approaches require surgeons to drill the temporal bone to expose target lesions while avoiding the critical structures within it, such as the facial nerve and other neurovascular structures. We envision a novel protective neuronavigation system that continuously calculates the drill

Development of a robot for surgery of temporal bone

International Nuclear Information System (INIS)

Komune, Shizuo

2011-01-01

Described was development of a robot for drill-outing the mastoid process, the first essential step purposing such otologic surgery of temporal bone as tympanoplasty, cochlear implantation and tumor resection of auditory nerve, etc. A model of the bone prototyped by CT 3D data (Ono and Co., Ltd., Tokyo) was used for getting the trace of the drill-outing procedure by an expert, and information of the trace and bone position was registered by STAMP (surface template-assisted marker positioning), which was then integrated with a navigation system 3D slicer (a free, open source software) with use of data from position sensors of optical Polaris (NDI, Canada) and magnetic Aurora (NDI) on the drill tip. The sensors were also usable for recording the trace after the surgery as a log by MRI. The robot system was made to have thus 3 parts of drill-outing, operative navigation and control unit based on anatomical information. The drill-outing mechanic was made to have 6 degrees of freedom. Comparison of logs of the procedure conducted in the phantom bone by the robot and by an otologic operator gave agreement within error of 0.9 mm. More mechanical preciseness was thought desirable for reproducible operation. (author)

Successful Function-Preserving Therapy for Chondroblastoma of the Temporal Bone Involving the Temporomandibular Joint

Directory of Open Access Journals (Sweden)

Junkichi Yokoyama

2011-02-01

Full Text Available We present a case involving a late diagnosis of chondroblastoma of the temporal skull base involving the temporomandibular joint (TMJ. Following an initial misdiagnosis and unsuccessful treatment over a period of 5 years, the patient was referred to our department for further evaluation and possible surgical intervention for occlusal abnormalities, trismus, clicking of the TMJ, and hearing impairment. Based on preoperative immunochemical studies showing positive reaction of multinucleated giant cells for S-100 protein, the final diagnosis was chondroblastoma. The surgical approach – postauricular incision and total parotidectomy, with complete removal of the temporal bone, including the TMJ via the extended middle fossa – was successful in preserving facial nerves and diminishing clinical manifestations. This study highlights a misdiagnosed case in an effort to underline the importance of medical examinations and accurate differential diagnosis in cases involving any tumor mass in the temporal bone.

Cerebellar arteriovenous malformations in children

International Nuclear Information System (INIS)

Griffiths, P.D.; Humphreys, R.P.

1998-01-01

We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.)

Cerebellar arteriovenous malformations in children

Energy Technology Data Exchange (ETDEWEB)

Griffiths, P.D. [Sheffield Univ. (United Kingdom). Acad. Dept. of Radiol.; Blaser, S.; Armstrong, D.; Chuang, S.; Harwood-Nash, D. [Division of Neuroradiology, The Hospital for Sick Children and University of Toronto, Toronto (Canada); Humphreys, R.P. [Division of Neurosurgery, The Hospital for Sick Children and University of Toronto, Toronto (Canada)

1998-05-01

We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.) With 4 figs., 4 tabs., 23 refs.

The clinical significance of temporal bone CT with regard to hearing preservation in acoustic neurinoma removal

International Nuclear Information System (INIS)

Umezu, Hiromichi; Seki, Yojirou; Aiba, Tadashi

1988-01-01

Since Rand and Kurze discussed the possibility of the anatomic preservation of the cochlear nerve via the posterior fossa transmeatal approach for the acoustic neurinoma, there have been increasingly many reports on the preservation of cochlear nerve function. With recent advances in diagnostic tools, today's neurosurgeon is able to make an earlier and more precise diagnosis of acoustic neurinomas even when the patient has good hearing. If useful hearing is to be kept, the cochlear nerve and blood supply of the labyrinth have to be preserved. In addition, surgical entry into the labyrinth, upon the removal of the posterior wall of the internal auditory canal, must be avoided, since it is likely to result in permanent hearing loss. Because of its superior contrast and spatial resolution, thin-section and high-resolution computed tomography of the temporal bone has the great advantage of demonstrating the exact relationship of the internal auditory canal to the posterior semicircular canal, the vestibule, and the common crus. In this study, thin-section, high-resolution computed tomography of the temporal bone was performed in 11 cases of acoustic neurinoma pre- and postoperatively. At operation, the lateral limit of the bone removal of the posterior internal auditory canal was determined on the basis of preoperative computed tomography of the temporal bone. As a result, inadvertent entry into the labyrinth was avoided, and useful hearing was preserved in 8 cases. In order to preserve useful hearing following total tumor removal, it is essential to plan the operative strategy meticulously on the basis of the findings of the preoperative investigation, Including temporal bone CT. (author)

Evaluation of the utility of temporal subtraction images in successive whole-body bone scans: a prospective clinical study

International Nuclear Information System (INIS)

Shiraishi, J.; Appelbaum, D.; Pu, Y.; Engelmann, R.; Li Qiang; Doi, K.

2007-01-01

We have begun a prospective clinical study for evaluating the clinical utility of temporal subtraction images in successive whole-body bone scans. The computerized temporal subtraction technique has been developed in order to highlight interval changes of abnormal lesions due to skeletal metastases, primary bone tumors, osteomyelitis, and fractures. In our initial preliminary results of the prospective study which was started on November 22, 2006 in our hospital, radiologists reported some interval changes which were not recognized in the initial standard readings, but were obvious when temporal subtraction images were viewed. The usefulness of the temporal subtraction images will be investigated in terms of its clinical utility by the prospective clinical study. (orig.)

Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations.

Science.gov (United States)

Elmaleh-Bergès, M; Baumann, C; Noël-Pétroff, N; Sekkal, A; Couloigner, V; Devriendt, K; Wilson, M; Marlin, S; Sebag, G; Pingault, V

2013-01-01

Waardenburg syndrome, characterized by deafness and pigmentation abnormalities, is clinically and genetically heterogeneous, consisting of 4 distinct subtypes and involving several genes. SOX10 mutations have been found both in types 2 and 4 Waardenburg syndrome and neurologic variants. The purpose of this study was to evaluate both the full spectrum and relative frequencies of inner ear malformations in these patients. Fifteen patients with Waardenburg syndrome and different SOX10 mutations were studied retrospectively. Imaging was performed between February 2000 and March 2010 for cochlear implant work-up, diagnosis of hearing loss, and/or evaluation of neurologic impairment. Eleven patients had both CT and MR imaging examinations, 3 had MR imaging only, and 1 had CT only. Temporal bone abnormalities were bilateral. The most frequent pattern associated agenesis or hypoplasia of ≥1 semicircular canal, an enlarged vestibule, and a cochlea with a reduced size and occasionally an abnormal shape, but with normal partition in the 13/15 cases that could be analyzed. Three patients lacked a cochlear nerve, bilaterally in 2 patients. In addition, associated abnormalities were found when adequate MR imaging sequences were available: agenesis of the olfactory bulbs (7/8), hypoplastic or absent lacrimal glands (11/14), hypoplastic parotid glands (12/14), and white matter signal anomalies (7/13). In the appropriate clinical context, bilateral agenesis or hypoplasia of the semicircular canals or both, associated with an enlarged vestibule and a cochlear deformity, strongly suggests a diagnosis of Waardenburg syndrome linked to a SOX10 mutation.

Imaging of congenital anomalies of the temporal bone.

Science.gov (United States)

Benton, C; Bellet, P S

2000-02-01

This article briefly presents the embryology of the ear and discusses the external auditory canal and middle ear, including first branchial cleft anomalies, external auditory canal atresia and stenoses, congenital cholesteatoma, and congenital teratoma. Topics related to the labyrinths include aplasia, the common-chamber anomaly, the pseudo-Mondini and Mondini malformations, semicircular canal dysplasia, and the large vestibular aqueduct. Vascular malformations and variations also are presented, including the absent and aberrant internal carotid artery, the persistent stapedial artery, and high jugular bulb; cerebrospinal fluid and perilymph fistulas are the subjects of the final section.

Case analysis of temporal bone lesions with facial paralysis as main manifestation and literature review.

Science.gov (United States)

Chen, Wen-Jing; Ye, Jing-Ying; Li, Xin; Xu, Jia; Yi, Hai-Jin

2017-08-23

This study aims to discuss clinical characteristics, image manifestation and treatment methods of temporal bone lesions with facial paralysis as the main manifestation for deepening the understanding of such type of lesions and reducing erroneous and missed diagnosis. The clinical data of 16 patients with temporal bone lesions and facial paralysis as main manifestation, who were diagnosed and treated from 2009 to 2016, were retrospectively analyzed. Among these patients, six patients had congenital petrous bone cholesteatoma (PBC), nine patients had facial nerve schwannoma, and one patient had facial nerve hemangioma. All the patients had an experience of long-term erroneous diagnosis. The lesions were completely excised by surgery. PBC and primary facial nerve tumors were pathologically confirmed. Facial-hypoglossal nerve anastomosis was performed on two patients. HB grade VI was recovered to HB grade V in one patient. The anastomosis failed due to severe facial nerve fibrosis in one patient. Hence, HB remained at grade VI. Postoperative recovery was good for all patients. No lesion recurrence was observed after 1-6 years of follow-up. For the patients with progressive or complete facial paralysis, imaging examination should be perfected in a timely manner. Furthermore, PBC, primary facial nerve tumors and other temporal bone space-occupying lesions should be eliminated. Lesions should be timely detected and proper intervention should be conducted, in order to reduce operation difficulty and complications, and increase the opportunity of facial nerve function reconstruction.

Neutrophil Extracellular Traps and Fibrin in Otitis Media: Analysis of Human and Chinchilla Temporal Bones.

Science.gov (United States)

Schachern, Patricia A; Kwon, Geeyoun; Briles, David E; Ferrieri, Patricia; Juhn, Steven; Cureoglu, Sebahattin; Paparella, Michael M; Tsuprun, Vladimir

2017-10-01

Bacterial resistance in acute otitis can result in bacterial persistence and biofilm formation, triggering chronic and recurrent infections. To investigate the middle ear inflammatory response to bacterial infection in human and chinchilla temporal bones. Six chinchillas underwent intrabullar inoculations with 0.5 mL of 106 colony-forming units (CFUs) of Streptococcus pneumoniae, serotype 2. Two days later, we counted bacteria in middle ear effusions postmortem. One ear from each chinchilla was processed in paraffin and sectioned at 5 µm. The opposite ear was embedded in epoxy resin, sectioned at a thickness of 1 µm, and stained with toluidine blue. In addition, we examined human temporal bones from 2 deceased donors with clinical histories of otitis media (1 with acute onset otitis media, 1 with recurrent infection). Temporal bones had been previously removed at autopsy, processed, embedded in celloidin, and cut at a thickness of 20 µm. Sections of temporal bones from both chinchillas and humans were stained with hematoxylin-eosin and immunolabeled with antifibrin and antihistone H4 antibodies. Histopatological and imminohistochemical changes owing to otitis media. Bacterial counts in chinchilla middle ear effusions 2 days after inoculation were approximately 2 logs above initial inoculum counts. Both human and chinchilla middle ear effusions contained bacteria embedded in a fibrous matrix. Some fibers in the matrix showed positive staining with antifibrin antibody, others with antihistone H4 antibody. In acute and recurrent otitis media, fibrin and neutrophil extracellular traps (NETs) are part of the host inflammatory response to bacterial infection. In the early stages of otitis media the host defense system uses fibrin to entrap bacteria, and NETs function to eliminate bacteria. In chronic otitis media, fibrin and NETs appear to persist.

Human multipotent mesenchymal stromal cells in the treatment of postoperative temporal bone defect: an animal model

Czech Academy of Sciences Publication Activity Database

Školoudík, L.; Chrobok, V.; Kalfert, D.; Kočí, Zuzana; Syková, Eva; Chumak, Tetyana; Popelář, Jiří; Syka, Josef; Laco, J.; Dědková, J.; Dayanithi, Govindan; Filip, S.

2016-01-01

Roč. 25, č. 7 (2016), s. 1405-1414 ISSN 0963-6897 R&D Projects: GA MŠk(CZ) LO1309 Institutional support: RVO:68378041 Keywords : Human bone marrow * Human mesenchymal stromal cells (hMSCs) * Middle ear surgery * Temporal bone Subject RIV: FP - Other Medical Disciplines Impact factor: 3.006, year: 2016

High-resolution computed tomography of the temporal bone. Part 1.: normal anatomy

International Nuclear Information System (INIS)

Grzegorzewski, M.; Boron, Z.; Burzynska-Makuch, M.

1995-01-01

Normal anatomy of the temporal bone in transverse and coronal sections was presented. CT studies were performed using high-resolution program. The images of an asymptomatic ear of 2 patients were selected from 68 cases examined on account of various otological diseases. All the sections showed as many as 68 anatomic structures. (author)

Functional Magnetic Resonance Imaging in the Presurgical Evaluation of Brain Vascular Malformations

International Nuclear Information System (INIS)

Montes, Natalia; Herrera, Diego A; Vargas Sergio A

2010-01-01

Objective: To describe our experience in presurgical evaluation of intracranial vascular malformations by means of functional magnetic resonance (fMRI). Method: To evaluate eight patients with cerebral vascular malformations (seven arterio-venous malformation [AVM ] and one cavernous malformation) to send to the eloquent cortex with RMf pre-surgical mapping is assessed. Used a technique that is dependent on the level of oxygen (BOLD) to locate these areas in the cerebral vascular malformation, by applying different paradigms. Results: We found one AVM at the right temporal lobe with activation of the parahipocampal gyrus at the contralateral side using a memory paradigm; another patient with an AVM at the right mesotemporal lobe showed activation of visual and spatial memory of the contralateral hippocampus and parahippocampus. One patient with an AVM at the left parietal lobe without compromise of sensorial and motor cortex; a cavernous malformation at the left angular gyrus with hemispheric language dominance in that side; one right thalamic AVM, one periventricular AVM bilateral language dominance; one left occipital AVM with decreased activation in visual association cortex; one temporoccipital AVM with left language dominance and neurovascular uncoupling. Conclusion: fMRI can delineate anatomically the relationship between the lesion and eloquent cortex, providing useful information for presurgical planning and allowing risk estimation of intervention.

Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation

Energy Technology Data Exchange (ETDEWEB)

Moore, Hannah E. [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States); Moore, Kevin R. [University of Utah School of Medicine, Department of Radiology, Salt Lake City, UT (United States); Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States)

2014-11-15

Complex Chiari malformation is a subgroup of Chiari 1 malformation with distinct imaging features. Children with complex Chiari malformation are reported to have a more severe clinical phenotype and sometimes require more extensive surgical treatment than those with uncomplicated Chiari 1 malformation. We describe reported MR imaging features of complex Chiari malformation and evaluate the utility of craniometric parameters and qualitative anatomical observations for distinguishing complex Chiari malformation from uncomplicated Chiari 1 malformation. We conducted a retrospective search of the institutional imaging database using the keywords ''Chiari'' and ''Chiari 1'' to identify children imaged during the 2006-2011 time period. Children with Chiari 2 malformation were excluded after imaging review. We used the first available diagnostic brain or cervical spine MR study for data measurement. Standard measurements and observations were made of obex level (mm), cerebellar tonsillar descent (mm), perpendicular distance to basion-C2 line (pB-C2, mm), craniocervical angle (degrees), clivus length, and presence or absence of syringohydromyelia, basilar invagination and congenital craniovertebral junction osseous anomalies. After imaging review, we accessed the institutional health care clinical database to determine whether each subject clinically met criteria for Chiari 1 malformation or complex Chiari malformation. Obex level and craniocervical angle measurements showed statistically significant differences between the populations with complex Chiari malformation and uncomplicated Chiari 1 malformation. Cerebellar tonsillar descent and perpendicular distance to basion-C2 line measurements trended toward but did not meet statistical significance. Odontoid retroflexion, craniovertebral junction osseous anomalies, and syringohydromyelia were all observed proportionally more often in children with complex Chiari malformation than in

T-bone plastique for treatment of brachy-turricephaly.

Science.gov (United States)

Donauer, E; Bernardy, M; Neuenfeldt, D

1993-01-01

The "T-Bone Plastique", which is presented in this paper, allows a surgical correction even of extreme cases of brachy-turricephaly together with malformations of the occipital region in one operative session. Brachy-turricephaly is characterized by abnormal vertical height of the skull and a shortening of its anterior-posterior length, frequently combined with malformations of the occipital region. Resection of the prematurely closed coronal suture, bi-parietal trepanations with 90 degrees rotation and side-exchange of the parietal bone flaps, double transverse trepanation of the occipital bone and outward bending and shifting of the bone fragments enable a bony remodeling and normalization of the deformed skull. Using this operative technique in three children we achieved a significant improvement of the skull form with an aesthetically pleasing result, without any neurologic sequelae and with normal development of the children during follow-up.

Post-marketing surveillance of CustomBone Service implanted in children under 7 years old.

Science.gov (United States)

Frassanito, Paolo; Tamburrini, Gianpiero; Massimi, Luca; Di Rocco, Concezio; Nataloni, Angelo; Fabbri, Greta; Caldarelli, Massimo

2015-01-01

The CustomBone Service is a bioceramic implant suitable for cranial repair in both adults and children, although there are no clinical data about its use in children under 7 years of age. This surveillance study investigates the outcome in this age group. Twenty-eight children under 7 years old (range, 2.5-6 years) received CustomBone Service from July 2006 to May 2013 in 16 international hospitals. Data of 23 children (12 males and 11 females), harboring 24 prosthesis, were available with a minimum follow-up of 1 year. Sites of the cranial defect were frontal or parietal (20.8 % each), parieto-temporal (16.7 %), fronto-parietal or occipital (12.5 % each), fronto-parieto-temporal or fronto-temporal (8.3 % each). Initial diseases were trauma (54.2 %), malformation (37.5 %), or tumor of the bone/skin (8.3 %). Rupture of the implant occurred in a single case during the implant (1/26 surgeries, 3.8 %) and the cranial repair was achieved by means of the back-up prosthesis. Five adverse events were registered during the follow-up period consisting of three cases of fracture and two of exposure/infection of the prosthesis. All cases required the removal of the device (20.8 %). The failure rate of CustomBone Service under 7 years of age was higher than reported in adults and children over 7 years old (20.8 vs. 3.8 %), However, CustomBone Service may be considered a valid option under 7 years old since other materials are burdened by more significant rates of complications in the long-term period. Due to specific properties of this material, indication to CustomBone Service in toddlers should be carefully evaluated by the surgeon on a case-by-case basis.

17p13.3 microduplications are associated with split-hand/foot malformation and long-bone deficiency (SHFLD).

Science.gov (United States)

Armour, Christine M; Bulman, Dennis E; Jarinova, Olga; Rogers, Richard Curtis; Clarkson, Kate B; DuPont, Barbara R; Dwivedi, Alka; Bartel, Frank O; McDonell, Laura; Schwartz, Charles E; Boycott, Kym M; Everman, David B; Graham, Gail E

2011-11-01

Split-hand/foot malformation with long-bone deficiency (SHFLD) is a relatively rare autosomal-dominant skeletal disorder, characterized by variable expressivity and incomplete penetrance. Although several chromosomal loci for SHFLD have been identified, the molecular basis and pathogenesis of most SHFLD cases are unknown. In this study we describe three unrelated kindreds, in which SHFLD segregated with distinct but overlapping duplications in 17p13.3, a region previously linked to SHFLD. In a large three-generation family, the disorder was found to segregate with a 254 kb microduplication; a second microduplication of 527 kb was identified in an affected female and her unaffected mother, and a 430 kb microduplication versus microtriplication was identified in three affected members of a multi-generational family. These findings, along with previously published data, suggest that one locus responsible for this form of SHFLD is located within a 173 kb overlapping critical region, and that the copy gains are incompletely penetrant.

Vascular malformations in pediatrics

International Nuclear Information System (INIS)

Reith, W.; Shamdeen, M.G.

2003-01-01

Vascular malformations are the cause of nearly all non-traumatic intracranial hemorrhage in children beyond the neonatal stage. Therefore, any child presenting with spontaneous intracranial hemorrhage should be evaluated for child abuse and for vascular malformations. Intracerebral malformations of the cerebral vasculature include vein of Galen malformations, arteriovenous malformation (AVM), cavernomas, dural arteriovenous fistulas, venous anomalies (DVA), and capillary teleangiectasies. Although a few familial vascular malformation have been reported, the majority are sporadic. Clinical symptoms, diagnostic and therapeutic options are discussed. (orig.) [de

Pneumatization of the zygomatic process of temporal bone on computed tomograms

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Friedrich, Reinhard E.

2016-06-01

Full Text Available Purpose: Zygomatic air cells (ZAC are a variant of temporal bone pneumatization that needs no treatment. However, ZAC can have an impact on surgical procedures in the temporo-mandibular joint region. Recent reports suggest that computed tomography will disclose more ZAC than can be diagnosed on panoramic radiography. The aim of this study was to analyze ZAC prevalence on CT in a population that was not pre-selected by admission to a dental clinic. Furthermore, an extensive literature review was performed to assess the prevalence of ZAC and to address the impact of imaging technique on the definition of the item.Material and methods: Digitalized cranial CTs of 2007 patients were retrospectively analyzed. The Frankfort horizontal was used to define a ZAC on sagittal CTs. Results: In this study group, 806 were female (40.16% and 1,201 were male (59.84%. Mean age was 49.96 years in the whole group (female: 55.83 years, male: 46.01 years. A ZAC was diagnosed in 152 patients (female: 66, male: 86. Unilateral ZAC surpasses bilateral findings (115 vs. 37 patients. ZAC were diagnosed in children 5 years of age and older. Sectional imaging techniques show a better visualization of the region of interest. However, presently an increase of ZAC prevalence attributable to imaging technique cannot conclusively be derived from the current literature. The normal finding of a ZAC on radiograms is a sharply defined homogenous transparent lesion restricted to the zygomatic process of the temporal bone that has no volume effect on the shape of the process.Conclusion: ZAC is an anatomical variant of the temporal bone that has come into focus of maxillofacial radiology due to its noticeable aspect on panoramic radiograms. The harmless variant can be expected in about one in thirteen individuals undergoing facial radiology. Panoramic radiograms appear to be sufficient to present ZAC of relevant size. However, in preparation for surgical procedures affecting the

Comparison of cadaveric and isomorphic three-dimensional printed models in temporal bone education.

Science.gov (United States)

Hochman, Jordan B; Rhodes, Charlotte; Wong, Dana; Kraut, Jay; Pisa, Justyn; Unger, Bertram

2015-10-01

Current three-dimensional (3D) printed simulations are complicated by insufficient void spaces and inconsistent density. We describe a novel simulation with focus on internal anatomic fidelity and evaluate against template/identical cadaveric education. Research ethics board-approved prospective cohort study. Generation of a 3D printed temporal bone was performed using a proprietary algorithm that deconstructs the digital model into slices prior to printing. This supplemental process facilitates removal of residual material from air-containing spaces and permits requisite infiltrative access to the all regions of the model. Ten otolaryngology trainees dissected a cadaveric temporal bone (CTB) followed by a matched/isomorphic 3D printed bone model (PBM), based on derivative micro-computed tomography data. Participants rated 1) physical characteristics, 2) specific anatomic constructs, 3) usefulness in skill development, and 4) perceived educational value. The survey instrument employed a seven-point Likert scale. Trainees felt physical characteristics of the PBM were quite similar to CTB, with highly ranked cortical (5.5 ± 1.5) and trabecular (5.2 ± 1.3) bone drill quality. The overall model was considered comparable to CTB (5.9 ± 0.74), with respectable air cell reproduction (6.1 ± 1.1). Internal constructs were rated as satisfactory (range, 4.9-6.2). The simulation was considered a beneficial training tool for all types of mastoidectomy (range, 5.9-6.6), posterior tympanotomy (6.5 ± 0.71), and skull base approaches (range, 6-6.5). Participants believed the model to be an effective training instrument (6.7 ± 0.68), which should be incorporated into the temporal bone lab (7.0 ± 0.0). The PBM was thought to improve confidence (6.7 ± 0.68) and operative performance (6.7 ± 0.48). Study participants found the PBM to be an effective platform that compared favorably to CTB. The model was considered a valuable adjunctive

Quantifying temporal bone morphology of great apes and humans: an approach using geometric morphometrics

Science.gov (United States)

Lockwood, Charles A; Lynch, John M; Kimbel, William H

2002-01-01

The hominid temporal bone offers a complex array of morphology that is linked to several different functional systems. Its frequent preservation in the fossil record gives the temporal bone added significance in the study of human evolution, but its morphology has proven difficult to quantify. In this study we use techniques of 3D geometric morphometrics to quantify differences among humans and great apes and discuss the results in a phylogenetic context. Twenty-three landmarks on the ectocranial surface of the temporal bone provide a high level of anatomical detail. Generalized Procrustes analysis (GPA) is used to register (adjust for position, orientation and scale) landmark data from 405 adults representing Homo, Pan, Gorilla and Pongo. Principal components analysis of residuals from the GPA shows that the major source of variation is between humans and apes. Human characteristics such as a coronally orientated petrous axis, a deep mandibular fossa, a projecting mastoid process, and reduced lateral extension of the tympanic element strongly impact the analysis. In phenetic cluster analyses, gorillas and orangutans group together with respect to chimpanzees, and all apes group together with respect to humans. Thus, the analysis contradicts depictions of African apes as a single morphotype. Gorillas and orangutans lack the extensive preglenoid surface of chimpanzees, and their mastoid processes are less medially inflected. These and other characters shared by gorillas and orangutans are probably primitive for the African hominid clade. PMID:12489757

Anatomic variations of the cochlea and relations to other temporal bone structures

Energy Technology Data Exchange (ETDEWEB)

Dimopoulos, P.; Muren, C. (Akademiska Sjukhuset, Uppsala (Sweden). Dept. of Diagnostic Radiology Sabbatsberg' s Sjukhus, Stockholm (Sweden). Dept. of Diagnostic Radiology)

1990-09-01

The size and shape of the human cochlea and the normal ranges of variation of its dimensions were evaluated in 95 plastic casts, prepared from temporal bone specimens. The normal range of variation is fairly small, and is not age-dependent. Obvious digression from this range, associated with pertinent clinical symptoms, indicates an abnormality. (orig./MG).

Repair of Temporal Bone Encephalocele following Canal Wall Down Mastoidectomy

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Sarantis Blioskas

2014-01-01

Full Text Available We report a rare case of a temporal bone encephalocele after a canal wall down mastoidectomy performed to treat chronic otitis media with cholesteatoma. The patient was treated successfully via an intracranial approach. An enhanced layer-by-layer repair of the encephalocele and skull base deficit was achieved from intradurally to extradurally, using temporalis fascia, nasal septum cartilage, and artificial dural graft. After a 22-month follow-up period the patient remains symptom free and no recurrence is noted.

Does microtia predict severity of temporal bone CT abnormalities in children with persistent conductive hearing loss?

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Tekes, Aylin; Ishman, Stacey L; Baugher, Katherine M; Brown, David J; Lin, Sandra Y; Tunkel, David E; Unalp-Arida, Aynur; Huisman, Thierry A G M

2013-07-01

This study aimed to determine the spectrum of temporal bone computed tomography (CT) abnormalities in children with conductive hearing loss (CHL) with and without microtia. From 1993 to 2008, a total of 3396 pediatric records including CHL were reviewed at our institution and revealed 180 cases of persistent CHL, 46 of whom had diagnostic temporal bone CT examinations. All of these examinations were systematically reviewed by two pediatric neuroradiologists, working in consensus, who had 5 and 18 years, respectively, of dedicated pediatric neuroradiology experience. Of the 46 children, 16 were boys and 30 were girls (age: 0.2-16 years; mean: 5 years). Also, 21 (46%) children had microtia and 25 (54%) children did not, as determined by clinical evaluation. External auditory canal atresia/stenosis (EAC-A/S) was the most common anomaly in both microtia and non-microtia groups. Two or more anomalies were observed in 18/21 children with microtia. The frequency of EAC-A/S was greater in children with microtia versus those without it (86% versus 32%, respectively; P = 0.0003). Syndromic diagnoses were also significantly more frequently made in children with microtia versus those without microtia (76% versus 20%, respectively; P = 0.0001). Temporal bone CT scans were normal in 10 children (22%) with persistent CHL. Microtia is an important finding in children with CHL. EAC and middle ear/ossicle anomalies were significantly more frequently seen in children with microtia, and multiple anomalies and bilateral microtia were more common in children with syndromic associations. These findings highlight the importance of understanding the embryological development of the temporal bone. The presence of one anomaly should raise suspicion of the possibility of other anomalies, especially in the setting of microtia. Bilateral microtia and multiple anomalies should also raise suspicion of genetic syndromes. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Cerebral palsy and congenital malformations

DEFF Research Database (Denmark)

Garne, Ester; Dolk, Helen; Krägeloh-Mann, Inge

2007-01-01

AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were...... classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP...... were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children...

Modifications to a 3D-printed temporal bone model for augmented stapes fixation surgery teaching.

Science.gov (United States)

Nguyen, Yann; Mamelle, Elisabeth; De Seta, Daniele; Sterkers, Olivier; Bernardeschi, Daniele; Torres, Renato

2017-07-01

Functional outcomes and complications in otosclerosis surgery are governed by the surgeon's experience. Thus, teaching the procedure to residents to guide them through the learning process as quickly as possible is challenging. Artificial 3D-printed temporal bones are replacing cadaver specimens in many institutions to learn mastoidectomy, but these are not suitable for middle ear surgery training. The goal of this work was to adapt such an artificial temporal bone to aid the teaching of otosclerosis surgery and to evaluate this tool. We have modified a commercially available 3D-printed temporal bone by replacing the incus and stapes of the model with in-house 3D-printed ossicles. The incus could be attached to a 6-axis force sensor. The stapes footplate was fenestrated and attached to a 1-axis force sensor. Six junior surgeons (residents) and seven senior surgeons (fellows or consultants) were enrolled to perform piston prosthesis placement and crimping as performed during otosclerosis surgery. The time required to perform the tasks and the forces applied to the incus and stapes were collected and analyzed. No statistically significant differences were observed between the junior and senior groups for time taken to perform the tasks and the forces applied to the incus during crimping and placement of the prosthesis. However, significantly lower forces were applied to the stapes by the senior surgeons in comparison with the junior surgeons during prosthesis placement (junior vs senior group, 328 ± 202.9 vs 80 ± 99.6 mN, p = 0.008) and during prosthesis crimping (junior vs senior group, 565 ± 233 vs 66 ± 48.6 mN, p = 0.02). We have described a new teaching tool for otosclerosis surgery based on the modification of a 3D-printed temporal bone to implement force sensors on the incus and stapes. This tool could be used as a training tool to help the residents to self-evaluate their progress with recording of objective measurements.

Simplified Summative Temporal Bone Dissection Scale Demonstrates Equivalence to Existing Measures.

Science.gov (United States)

Pisa, Justyn; Gousseau, Michael; Mowat, Stephanie; Westerberg, Brian; Unger, Bert; Hochman, Jordan B

2018-01-01

Emphasis on patient safety has created the need for quality assessment of fundamental surgical skills. Existing temporal bone rating scales are laborious, subject to evaluator fatigue, and contain inconsistencies when conferring points. To address these deficiencies, a novel binary assessment tool was designed and validated against a well-established rating scale. Residents completed a mastoidectomy with posterior tympanotomy on identical 3D-printed temporal bone models. Four neurotologists evaluated each specimen using a validated scale (Welling) and a newly developed "CanadaWest" scale, with scoring repeated after a 4-week interval. Nineteen participants were clustered into junior, intermediate, and senior cohorts. An ANOVA found significant differences between performance of the junior-intermediate and junior-senior cohorts for both Welling and CanadaWest scales ( P .05). Cohen's kappa found strong intrarater reliability (0.711) with a high degree of interrater reliability of (0.858) for the CanadaWest scale, similar to scores on the Welling scale of (0.713) and (0.917), respectively. The CanadaWest scale was facile and delineated performance by experience level with strong intrarater reliability. Comparable to the validated Welling Scale, it distinguished junior from senior trainees but was challenged in differentiating intermediate and senior trainee performance.

Supratentorial CNS malformations

International Nuclear Information System (INIS)

Zlatareva, D.

2012-01-01

Full text: Clinical suspicion of a developmental anomaly of the central nervous system (CNS) is a frequent indication for performing and magnetic resonance imaging (MRI) examination of the brain. Classification systems for malformation of the CNS are constantly revised according to newer scientific research. Developmental abnormalities can be classified in two main types. The first category consists of disorders of organogenesis in which genetic defects or any ischemic, metabolic, toxic or infectious insult to the developing brain can cause malformation. These malformations result from abnormal neuronal and glial proliferation and from anomalies of neuronal migration and or cortical organization. They are divided into supra- and infratentorial and may involve grey or white matter or both. The second category of congenital brain abnormalities is disorders of histogenesis which result from abnormal cell differentiation with a relatively normal brain appearance. Supratentorial CNS malformations could be divided into anomalies in telencephalic commissure, holoprosencephalies and malformations in cortical development. There are three main telencephalic commissures: the anterior commissure, the hippocampal commissure and the corpus callosum. Their morphology (hypoplasia, hyperplasia, agenesis, dysgenesis, even atrophy) reflects the development of the brain. Their agenesis, complete or partial, is one of the most commonly observed features in the malformations of the brain and is a part of many syndromes. Malformations of cortical development (MCD) are heterogeneous group of disease which result from disruption of 3 main stages of cortical development. The common clinical presentation is refractory epilepsy and or developmental delay. The most common MCD are heterotopias, focal cortical dysplasia, polymicrogyria, schizencephaly, pachygyria and lizencephaly. The exact knowledge of the brain anatomy and embryology is mandatory to provide a better apprehension of the

Three dimensional CT of stapes. Stapedial imagings in dry temporal bone and clinical applications

Energy Technology Data Exchange (ETDEWEB)

Edamatsu, Hideo; Kubota, Osamu; Yamashita, Koichi [Kanazawa Medical Univ., Ishikawa (Japan)

1995-03-01

This study was performed to evaluate the usefulness and limitations of three dimensional (3-D) imagings of stapes in the middle ear by high speed helical CT. One dissected human temporal bone, ten normal and diseased ears were scanned with a slice of 1.0 mm and reconstructed in a thickness of 0.2-0.5 mm. Every specimen of 3-D can be observed in any plane and from any direction. Ossicular imagings of the temporal bone in 3-D were reconstructed as if the malleus, incus and stapes were observed under microscope. The whole structure of stapes was impossible to be represented by two dimensional CT heretofore in use, but 3-D in our study showed the head, crus and foot plate of the stapes in detail. Stapedial imagings of 3-D CT in normal ears showed the same findings as those recorded in temporal bone. Preoperative diagnostic findings of ossicles in the affected ears were very useful. Especially in ossicular anomalies, 3-D CT was positive in diagnosis and its accuracies were confirmed with operative observation. For the postoperative evaluation concerning the ossicular reconstruction, i.e. TORP and PORP, 3-D CT was also important method. It could present an anatomical relation between those prosthesis and the oval window. High speed helical CT can scan an object more quickly and clearly than formerly used CT, and its biological damage for human is less than that of the others. 3-D CT can be more clearly reconstructed with helical CT than former CT. (author).

Radiological features of childhood giant cavernous malformations

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Ozgen, Burce; Senocak, Efsun; Oguz, Kader K. [Hacettepe University, Department of Radiology, Faculty of Medicine, Ankara (Turkey); Soylemezoglu, Figen [Hacettepe University, Department of Pathology, School of Medicine, Ankara (Turkey); Akalan, Nejat [Hacettepe University, Department of Neurosurgery, School of Medicine, Ankara (Turkey)

2011-04-15

Giant cavernous malformations (GCM) are very large, low-flow vascular malformations, which usually have atypical imaging features and are commonly misdiagnosed preoperatively as neoplasms or vascular malformations. These lesions have mostly been reported in children. As cavernomas show different features in children compared to adults, we evaluated the imaging features of pediatric GCMs in order to help in the preoperative diagnosis of these malformations. Brain MR studies of nine children (mean age of 4 years; 8 months-9 years) with biopsy-proven GCM were retrospectively evaluated. We defined GCMs as cavernomas of {>=}4 cm. Lesions were evaluated regarding their size, location, signal characteristics, general appearance (uni/multilocular) as well as regarding the presence of mass effect, edema, and fluid-fluid levels and were classified according to the Mottolese classification of pediatric cavernomas. Lesion locations were parietal (n = 5), frontal (n = 2), temporal, and intraventricular. Seven lesions were in the periventricular region (with five in the periatrial region). Six patients had T1 hyperintense multilobulated lesions with ''bubbles of blood'' appearance and three patients had heterogeneous lesions with reticular core. All lesions had mass effect, edema (marked in four cases), and peripheral hemosiderin rim. Fluid-fluid levels were also common (n = 7). Most of our lesions (six of nine) were classified as type IIIA, two as type IIIC, and one as type IA. In children, a GCM should be considered in case of very large hemorrhagic intra-axial mass with ''bubbles of blood'' multicystic appearance, surrounding hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect, especially in the periatrial location. (orig.)

Chiari Malformation

Science.gov (United States)

... the back of the skull, just above the spine. When a child has a Chiari malformation, the space for the ... a portion of the top vertebra in the spine to reduce pressure in that area. If a child with a Chiari malformation also has hydrocephalus, the ...

CT and MRI findings of temporal bone anomaly in patients with tinnitus

International Nuclear Information System (INIS)

Wang Bing; Xian Junfang; Wang Zhenchang; Liu Zhaohui

2011-01-01

Objective: To study high resolution CT (HRCT) and MRI findings of temporal hone anomaly in patients with tinnitus and identify the optimal examination method in the detection of the anomaly. Methods: The HRCT and MRI data were analyzed retrospectively in 1015 patients including 145 patients with pulsatile tinnitus (PT) and 870 patients with nonpulsatile tinnitus (NPT). The positive rates of HRCT and MRI in the identification of temporal bone anomaly were analyzed and the efficiency of various examination methods was compared in revealing the anomaly. Data were tested by Chi-square test analysis. Results: Among 1015 patients, anomaly was seen in 767 cases (75.57%). High jugular bulb was found in 414 patients, accounting for 40.79%. Sigmoid sinus anomaly was detected in 387 patients (38.13%), while otitis media was found in 148 cases (14.58%), and low middle cranial fossa in 70 cases (6.90%). The positive rate of HRCT in the detection of high jugular bulb was 54.89% (365/665), which was significantly higher than those of other methods (P 2 =56.537, P<0.01). The fast imaging employing steady-state acquisition (FIESTA) sequence was the best examination method in displaying the vessel within the internal auditory canal (42/42,100%). Conclusions: High jugular bulb and sigmoid sinus anomaly were the most frequent abnormal findings of temporal bone in patients with tinnitus. Enhanced HRCT was the choice of modality in patients with PT. Plain HRCT was recommended for NPT. FIESTA sequence was the best in the evaluation of the vessel within the internal auditory canal. (authors)

Field guide to malformations of frogs and toads: with radiographic interpretations

Science.gov (United States)

Meteyer, Carol U.

2000-01-01

In 1995, students found numerous malformed frogs on a field trip to a Minnesota pond. Since that time, reports of malformed frogs have increased dramatically. Malformed frogs have now been reported in 44 states in 38 species of frogs, and 19 species of toads. Estimates as high as 60% of the newly metamorphosed frog populations have had malformations at some ponds (NARCAM, ’99). The wide geographic distribution of malformed frogs and the variety of malformations are a concern to resource managers, research scientists and public health officials. The potential for malformations to serve as a signal of ecosystem disruption, and the affect this potential disruption might have on other organisms that share those ecosystems, has not been resolved. Malformations represent an error that occurred early in development. The event that caused the developmental error is temporally distant from the malformation we see in the fully developed animal. Knowledge of normal developmental principles is necessary to design thoughtful investigations that will define the events involved in abnormal development in wild frog populations.Development begins at the time an egg is fertilized and progresses by chemical communication between cells and cell layers. This communication is programmed through gene expression. Malformations represent primary errors in development, errors in chemical communication or translation of genetic information. Deformations arise later in development and usually result from the influence of mechanical factors (such as amputation) that alter shape or anatomy of a structure that has developed normally. The occurrence and the type of malformations are influenced by the type of error or insult as well as the timing of the error (the developmental stage at which the error occurred). The appearance of the malformation can therefore provide clues that suggest when the error may have occurred. If the malformation is an incomplete organ, such as an incomplete limb, the

Brain Malformations Associated with Knobloch Syndrome – Review of Literature, Expanding Clinical Spectrum and Identification of Novel Mutations

Science.gov (United States)

Caglayan, Ahmet Okay; Baranoski, Jacob F.; Aktar, Fesih; Han, Wengi; Tuysuz, Beyhan; Guzel, Aslan; Guclu, Bulent; Kaymakcalan, Hande; Aktekin, Berrin; Akgumus, Gozde Tugce; Murray, Phillip B.; Omay, E. Zeynep Erson; Caglar, Caner; Bakircioglu, Mehmet; Sakalar, Yildirim Bayezit; Guzel, Ebru; Demir, Nihat; Tuncer, Oguz; Senturk, Senem; Ekici, Baris; Minja, Frank J.; Šestan, Nenad; Yasuno, Katsuhito; Bilguvar, Kaya; Caksen, Huseyin; Gunel, Murat

2014-01-01

BACKGROUND Knobloch syndrome is a rare, autosomal recessive, developmental disorder characterized by stereotyped ocular abnormalities with or without occipital skull deformities (encephalocele, bone defects, cutis aplasia). Although there is clear heterogeneity in clinical presentation, central nervous system malformations, aside from the characteristic encephalocele, have not typically been considered a component of the disease phenotype. METHODS Four patients originally presented for genetic evaluation of symptomatic structural brain malformations. Whole-genome genotyping, whole-exome sequencing, and confirmatory Sanger sequencing were performed. Using immunohistochemical analysis, we investigated the protein expression pattern of COL18A1 in the mid-fetal and adult human cerebral cortex and then analyzed the spatial and temporal changes in the expression pattern of COL18A1 during human cortical development using the Human Brain Transcriptome database. RESULTS We identified two novel homozygous deleterious frame-shift mutations in the COL18A1 gene. Upon further investigation of these patients and their families, we found that many exhibited certain characteristics of Knobloch syndrome, including pronounced ocular defects. Our data strongly support an important role for COL18A1 in brain development and this report contributes to an enhanced characterization of the brain malformations that can result from deficiencies of collagen XVIII. CONCLUSIONS This case series highlights the diagnostic power and clinical utility of whole-exome sequencing technology – allowing clinicians and physician scientists to better understand the pathophysiology and presentations of rare diseases. We suggest that patients who are clinically diagnosed with Knobloch syndrome and/or found to have COL18A1 mutations via genetic screening should be investigated for potential structural brain abnormalities even in the absence of encephaloceles. PMID:25456301

The incidence of Arnold–Chiari malformation in neurological practice

Directory of Open Access Journals (Sweden)

E. A. Kantimirova

2015-01-01

Full Text Available Arnold-Chiari malformation (ACM is a group of congenital hindbrain malformations affecting the structural relationships between the cerebellum, brain stem, top cervical spinal cord, and bones of the skull base. In clinical practice, ACM types 0 and 1 are more common and types 2, 3, and 4 belong to rare severe (often fatal congenital malformations.Objective: to study gender differences in the incidence of ACM types 0 and 1 in outpatient neurological practice.Patients and methods. A total 2039 case records of outpatients who had visited a neurologist of the Krasnoyarsk University clinic in 2008–2014 were analyzed. Neurological and neuroradiological (1.5 Tesla brain magnetic resonance imaging, phase-contrast spinal cerebral fluid flow imaging diagnostic techniques were used. 3.4% (70/2039 of cases were randomized according to the criteria of inclusion and exception. An entire sample included 70 ACM patients (median age, 25 [17; 34] years (30 (42.8–7.1% men and 40 (57.2–7.1% women.Results. There was an increase in the incidence of ACM type 1 in the women than in the men. Conclusion. It is necessary to develop a new strategy for the prophylactic medical examination of patients with ACM to improve primary and specialized outpatient health care.

Creating an Optimal 3D Printed Model for Temporal Bone Dissection Training.

Science.gov (United States)

Takahashi, Kuniyuki; Morita, Yuka; Ohshima, Shinsuke; Izumi, Shuji; Kubota, Yamato; Yamamoto, Yutaka; Takahashi, Sugata; Horii, Arata

2017-07-01

Making a 3-dimensional (3D) temporal bone model is simple using a plaster powder bed and an inkjet printer. However, it is difficult to reproduce air-containing spaces and precise middle ear structures. The objective of this study was to overcome these problems and create a temporal bone model that would be useful both as a training tool and for preoperative simulation. Drainage holes were made to remove excess materials from air-containing spaces, ossicle ligaments were manually changed to bony structures, and small and/or soft tissue structures were colored differently while designing the 3D models. The outcomes were evaluated by 3 procedures: macroscopic and endoscopic inspection of the model, comparison of computed tomography (CT) images of the model to the original CT, and assessment of tactile sensation and reproducibility by 20 surgeons performing surgery on the model. Macroscopic and endoscopic inspection, CT images, and assessment by surgeons were in agreement in terms of reproducibility of model structures. Most structures could be reproduced, but the stapes, tympanic sinus, and mastoid air cells were unsatisfactory. Perioperative tactile sensation of the model was excellent. Although this model still does not embody perfect reproducibility, it proved sufficiently practical for use in surgical training.

Hind limb malformations in free-living northern leopard frogs (Rana pipiens) from Maine, Minnesota, and Vermont suggest multiple etiologies

Science.gov (United States)

Meteyer, C.U.; Loeffler, I.K.; Fallon, J.F.; Converse, K.A.; Green, E.; Helgen, J.C.; Kersten, S.; Levey, R.; Eaton-Poole, L.; Burkhart, J.G.

2000-01-01

Background Reports of malformed frogs have increased throughout the North American continent in recent years. Most of the observed malformations have involved the hind limbs. The goal of this study was to accurately characterize the hind limb malformations in wild frogs as an important step toward understanding the possible etiologies. Methods During 1997 and 1998, 182 recently metamorphosed northern leopard frogs (Rana pipiens) were collected from Minnesota, Vermont, and Maine. Malformed hind limbs were present in 157 (86%) of these frogs, which underwent necropsy and radiographic evaluation at the National Wildlife Health Center. These malformations are described in detail and classified into four major categories: (1) no limb (amelia); (2) multiple limbs or limb elements (polymelia, polydactyly, polyphalangy); (3) reduced limb segments or elements (phocomelia, ectromelia, ectrodactyly, and brachydactyly; and (4) distally complete but malformed limb (bone rotations, bridging, skin webbing, and micromelia). Results Amelia and reduced segments and/or elements were the most common finding. Frogs with bilateral hind limb malformations were not common, and in only eight of these 22 frogs were the malformations symmetrical. Malformations of a given type tended to occur in frogs collected from the same site, but the types of malformations varied widely among all three states, and between study sites within Minnesota. Conclusions Clustering of malformation type suggests that developmental events may produce a variety of phenotypes depending on the timing, sequence, and severity of the environmental insult. Hind limb malformations in free-living frogs transcend current mechanistic explanations of tetrapod limb development.

Radiosurgery for brain arteriovenous malformations

International Nuclear Information System (INIS)

Latorzeff, I.; Schlienger, M.; Sabatier, J.; Borius, P.Y.; Lotterie, J.A.; Bourdin, S.; Menegalli, D.; Cognard, C.; Januel, A.C.; Desal, H.; Debono, B.; Blond, S.

2012-01-01

Radiosurgery as treatment for arteriovenous malformations has shown a good efficacy in reducing intracranial bleeding due to rupture. The choice of therapeutic modalities is based on evolutive risk and arteriovenous malformations volume, patient profile and risks stratification following therapeutic techniques (microsurgery, radiosurgery, embolization). Nidus size, arteriovenous malformations anatomical localization, prior embolization or bleeding, distributed dose are predictive factors for radio-surgery's good results and tolerance. This review article will highlight arteriovenous malformations radiosurgery indications and discuss recent irradiation alternatives for large arteriovenous malformation volumes. (authors)

Bilateral cochlear implantation in a patient with bilateral temporal bone fractures.

Science.gov (United States)

Chung, Jae Ho; Shin, Myung Chul; Min, Hyun Jung; Park, Chul Won; Lee, Seung Hwan

2011-01-01

With the emphasis on bilateral hearing nowadays, bilateral cochlear implantation has been tried out for bilateral aural rehabilitation. Bilateral sensorineural hearing loss caused by head trauma can get help from cochlear implantation. We present the case of a 44-year-old man with bilateral otic capsule violating temporal bone fractures due to head trauma. The patient demonstrated much improved audiometric and psychoacoustic performance after bilateral cochlear implantation. We believe bilateral cochlear implantation in such patient can be a very effective tool for rehabilitation. Copyright © 2011 Elsevier Inc. All rights reserved.

Destructive, granulating lesion in the temporal bone after elevated plasma homocysteine

DEFF Research Database (Denmark)

Bonding, Per; Skriver, Elisabeth; Helin, Pekka

2004-01-01

lesion in the left temporal bone was discovered; repeated histologic examination only showed simple granulation tissue. After 6 months, a part of the bony cochlea was extruded. With approximately 8 months' delay and after the patient had had postoperative lung embolism, plasma homocysteine was found...... to be significantly elevated, a condition known as an independent risk factor for thromboembolic lesions. In the acquired form, it is most often caused by nutritional deficiency of vitamin B cofactors. Accordingly, the patient was treated with folic acid, which rapidly normalized plasma homocysteine. Subsequently...

MRI phenotypes of localized intravascular coagulopathy in venous malformations

Energy Technology Data Exchange (ETDEWEB)

Koo, Kevin S.H. [Boston Children' s Hospital, Department of Radiology, Boston, MA (United States); Dowd, Christopher F.; Hess, Christopher P. [University of California, San Francisco, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States); Mathes, Erin F.; Frieden, Ilona J. [University of California, San Francisco, Department of Dermatology, San Francisco, CA (United States); Rosbe, Kristina W. [University of California, San Francisco, Department of Otolaryngology, San Francisco, CA (United States); Hoffman, William Y. [University of California, San Francisco, Department of Surgery, San Francisco, CA (United States)

2015-10-15

The incidence of localized intravascular coagulopathy (LIC) in venous malformations varies with lesion size and location, as well as the presence of palpable phleboliths. The development of LIC can cause pain and hemorrhage and can progress to disseminated intravascular coagulopathy (DIC) and thromboembolic disease resulting in death in some cases. Early recognition of LIC can relieve symptoms and prevent progression to life-threatening complications. The aim of this work was to identify MRI features of venous malformation associated with LIC. We hypothesized that venous malformations with larger capacitance, slower flow and less physiological compression (greater stasis) were more likely to be associated with LIC. In this HIPAA-compliant and IRB-approved study, we retrospectively reviewed clinical records and MRI for consecutive patients undergoing evaluation of venous malformations at our multidisciplinary Birthmarks and Vascular Anomalies Center between 2003 and 2013. Inclusion required consensus diagnosis of venous malformation and availability of laboratory data and MRI; patients on anticoagulation or those previously undergoing surgical or endovascular treatment were excluded. LIC was diagnosed when D-dimer exceeded 1,000 ng/mL and/or fibrinogen was less than 200 mg/dL. Two board-certified radiologists assessed the following MRI features for each lesion: morphology (spongiform vs. phlebectatic), presence of phleboliths, size, location (truncal vs. extremity), and tissue type(s) involved (subcutis, muscle, bone and viscera). Univariate logistic regression analyses were used to test associations between LIC and MRI findings, and stepwise regression was applied to assess the significance of the individual imaging predictors. Seventy patients, 37 with LIC, met inclusion criteria during the 10-year study period (age: 14.5 +/- 13.6 years [mean +/- standard deviation]; 30 male, 40 female). Both elevated D-dimer and low fibrinogen were associated with the presence of

MRI phenotypes of localized intravascular coagulopathy in venous malformations

International Nuclear Information System (INIS)

Koo, Kevin S.H.; Dowd, Christopher F.; Hess, Christopher P.; Mathes, Erin F.; Frieden, Ilona J.; Rosbe, Kristina W.; Hoffman, William Y.

2015-01-01

The incidence of localized intravascular coagulopathy (LIC) in venous malformations varies with lesion size and location, as well as the presence of palpable phleboliths. The development of LIC can cause pain and hemorrhage and can progress to disseminated intravascular coagulopathy (DIC) and thromboembolic disease resulting in death in some cases. Early recognition of LIC can relieve symptoms and prevent progression to life-threatening complications. The aim of this work was to identify MRI features of venous malformation associated with LIC. We hypothesized that venous malformations with larger capacitance, slower flow and less physiological compression (greater stasis) were more likely to be associated with LIC. In this HIPAA-compliant and IRB-approved study, we retrospectively reviewed clinical records and MRI for consecutive patients undergoing evaluation of venous malformations at our multidisciplinary Birthmarks and Vascular Anomalies Center between 2003 and 2013. Inclusion required consensus diagnosis of venous malformation and availability of laboratory data and MRI; patients on anticoagulation or those previously undergoing surgical or endovascular treatment were excluded. LIC was diagnosed when D-dimer exceeded 1,000 ng/mL and/or fibrinogen was less than 200 mg/dL. Two board-certified radiologists assessed the following MRI features for each lesion: morphology (spongiform vs. phlebectatic), presence of phleboliths, size, location (truncal vs. extremity), and tissue type(s) involved (subcutis, muscle, bone and viscera). Univariate logistic regression analyses were used to test associations between LIC and MRI findings, and stepwise regression was applied to assess the significance of the individual imaging predictors. Seventy patients, 37 with LIC, met inclusion criteria during the 10-year study period (age: 14.5 +/- 13.6 years [mean +/- standard deviation]; 30 male, 40 female). Both elevated D-dimer and low fibrinogen were associated with the presence of

Osteodistrofias do Osso Temporal: Revisão dos Conceitos Atuais, Manifestações Clínicas e Opções Terapêuticas Osteodysplasia of the Temporal Bone: Up-date Concepts, Clinical Presentations and Therapeutic Options

Directory of Open Access Journals (Sweden)

Oswaldo Laércio M. Cruz

Full Text Available Sob a designação de osteodistrofias do osso temporal, podemos encontrar uma série de doenças que apresentam em comum a desorganização da arquitetura ou da composição do tecido ósseo. A otospongiose é, com larga margem, a osteodistrofia mais comum nessa localização e suas alterações, repercussões clínicas e tratamentos são amplamente discutidos na literatura. Entretanto, formas menos freqüentes, como a displasia fibrosa e a osteogênese imperfeita, não são entidades raras e merecem atenção. Este artigo tem como objetivo discutir essas formas menos comuns de osteodistrofia do temporal através de uma revisão sobre os conceitos atuais dessas entidades, da apresentação de três exemplos clínicos e a discussão sobre opções de tratamento.Osteodysplasia of the temporal bone included a significant amount of osseous diseases sharing bone matrix structural and composition damage. Otospongiosis is, by far, the most frequent form of this involvement in the temporal bone. Nevertheless, fibrous dysplasia and osteogenesis imperfecta are not rare and deserve attention. In this article, the authors present a discussion about the recent concepts of those less frequent forms of osteodysplasia of temporal bone, its options of treatment, illustrated with three clinical cases.

A complex craniovertebral junction malformation in a patient with late onset glycogenosis 2

Directory of Open Access Journals (Sweden)

Mariasofia Cotelli

2014-01-01

Full Text Available Glycogenosis II (GSDII is an autosomal recessive lysosomal storage disorder resulting from deficiency of acid alpha-glucosidase and subsequent lysosomal accumulation of glycogen in skeletal, cardiac and smooth muscles. The late-onset form is characterized by wide variability of the phenotypical spectrum. Clinical findings may include muscle weakness, respiratory insufficiency, vascular abnormalities, low bone mineral density and higher risk of developing osteoporosis. Craniovertebral junction (CVJ malformations have never been described so far. We here report on a GSDII 43-year-old woman who harbored the mutations IVS1-13T>G and c.2237G>A in the acid alpha-glucosidase gene. She recurrently suffered from headache, neck pain and dizziness. Brain MRI and CT scan showed the presence of a very rare complex CVJ malformation composed of basilar invagination, basiocciput hypoplasia, partial C1 assimilation, C1 posterior arch aplasia and C1 lateral mass hypoplasia and offset. Although we cannot rule out their coincidental occurrence, the rarity of multiple CVJ malformations in the general population as well as the well-known GSDII multisystem involvement should suggest to study the CVJ in the diagnostic process of GSDII patients in order to assess the CVJ malformation frequency in GSDII population and verify a possible relationship between these two conditions.

Venous malformations: MR imaging features that predict skin burns after percutaneous alcohol embolization procedures

International Nuclear Information System (INIS)

Fayad, Laura M.; Hazirolan, Tuncay; Carrino, John A.; Bluemke, David A.; Mitchell, Sally

2008-01-01

To examine the value of magnetic resonance (MR) imaging for predicting the occurrence of skin burns in patients with venous malformations who undergo percutaneous alcohol embolization was the objective of the study. Pre-procedural MR imaging at 1.5 T from 40 patients with venous malformations who had undergone percutaneous alcohol embolization was retrospectively reviewed by two observers for these features: anatomic location, definition (well-defined or ill-defined), and the presence of skin, subcutaneous tissue, muscle, tendon, bone, joint, and deep venous system involvement. One observer recorded the length of skin involvement and volume of the malformation. Univariate and multivariate analysis tests were used to determine whether an association between the occurrence of skin burns and MR imaging features existed. The anatomic locations of the venous malformations were the lower extremity (20 out of 40), upper extremity (11 out of 40), trunk (four out of 40), head/neck (three out of 40) and pelvis (two out of 40). Of the 40 subjects, 15% (six out of 40) experienced skin burns. There was a significant association between the absence of muscle involvement (p=0.0198) as well as the length of skin involvement (p=0.027), with the occurrence of skin burns. Malformation size and all other features were not significantly associated with skin burns. Skin burns in patients with venous malformations treated with alcohol embolization are associated with the length of skin involvement and with the absence of deeper tissue involvement, as depicted on MR imaging. (orig.)

Petrified ears in a patient with Keutel syndrome: temporal bone CT findings

International Nuclear Information System (INIS)

Parmar, Hemant; Blaser, Susan; Yoo, Shi-Joon; Unger, Sheila; Papsin, Blake

2006-01-01

We present unusual imaging findings of petrified ears in a 9-year-old girl with Keutel syndrome. The patient presented for a temporal bone study for hearing loss. CT scan showed middle and inner ear abnormalities along with extensive and unsuspected calcification of the external ears and ossicular ligaments. On further investigation, the patient was found to have diffuse cartilage calcification in the larynx and tracheobronchial tree, brachytelephalangism and peripheral pulmonary stenosis suggestive of Keutel syndrome. Confirmation was obtained by mutation analysis. (orig.)

Transverse axial plane anatomy of the temporal bone employing high spatial resolution computed tomography

International Nuclear Information System (INIS)

Russell, E.J.; Koslow, M.; Lasjaunias, P.; Bergeron, R.T.; Chase, N.

1982-01-01

Anatomical relationships of temporal bone structures are demonstrated by thin section edge detection computed tomography. Many otic structures are best appreciated in axial view, but reorientation to anatomy as seen in this plane is needed for optimal diagnosis. A level by level review of key structure is presented toward this end. The limitations and advantages of computed tomography are discussed. (orig.)

Improving temporal bone dissection using self-directed virtual reality simulation: results of a randomized blinded control trial.

Science.gov (United States)

Zhao, Yi Chen; Kennedy, Gregor; Yukawa, Kumiko; Pyman, Brian; O'Leary, Stephen

2011-03-01

A significant benefit of virtual reality (VR) simulation is the ability to provide self-direct learning for trainees. This study aims to determine whether there are any differences in performance of cadaver temporal bone dissections between novices who received traditional teaching methods and those who received unsupervised self-directed learning in a VR temporal bone simulator. Randomized blinded control trial. Royal Victorian Eye and Ear Hospital. Twenty novice trainees. After receiving an hour lecture, participants were randomized into 2 groups to receive an additional 2 hours of training via traditional teaching methods or self-directed learning using a VR simulator with automated guidance. The simulation environment presented participants with structured training tasks, which were accompanied by real-time computer-generated feedback as well as real operative videos and photos. After the training, trainees were asked to perform a cortical mastoidectomy on a cadaveric temporal bone. The dissection was videotaped and assessed by 3 otologists blinded to participants' teaching group. The overall performance scores of the simulator-based training group were significantly higher than those of the traditional training group (67% vs 29%; P < .001), with an intraclass correlation coefficient of 0.93, indicating excellent interrater reliability. Using other assessments of performance, such as injury size, the VR simulator-based training group also performed better than the traditional group. This study indicates that self-directed learning on VR simulators can be used to improve performance on cadaver dissection in novice trainees compared with traditional teaching methods alone.

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

Application of digital tomosynthesis to radiographic diagnosis of the temporal bone. Studies on visualization in normal subjects

International Nuclear Information System (INIS)

Kawai, Takashi

1995-01-01

To examine the usefulness of digital tomosynthesis for conducting radiographic diagnosis of the temporal bone, visualization of various aural structures such as the semicircular canals, cochlea, vestibular apparatus, ossicles of the ear and facial nerve canal was examined in 18 volunteers. The visualization of temporal bone specimens by digital tomosynthesis and CT images (slice thickness: 1.5 mm) was compared. The results showed that this system (Digital Tomosynthesis) produced clear images of bony labyrinthine structures such as the semicircular canals, cochlea, and vestibular apparatus. Visualization of the ossicles was also clear, and their continuity could be comprehended better than on CT images. This system also provided good visualization of the labyrinthine and tympanic parts of the facial nerve canal, although CT images had greater sharpness. Visualization of the lower half of the mastoid part was poor with this system. (author)

Pediatric central nervous system vascular malformations

Energy Technology Data Exchange (ETDEWEB)

Burch, Ezra A. [Brigham and Women' s Hospital, Department of Radiology, Boston, MA (United States); Orbach, Darren B. [Boston Children' s Hospital, Neurointerventional Radiology, Boston, MA (United States)

2015-09-15

Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

Pediatric central nervous system vascular malformations

International Nuclear Information System (INIS)

Burch, Ezra A.; Orbach, Darren B.

2015-01-01

Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

Dandy-Walker Malformation

African Journals Online (AJOL)

rme

Dandy-Walker malformation is a rare congenital malformation and involves the cerebellum and fourth ventricle. The condition is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. A large number of concomitant problems may be ...

Surgical management of venous malformations.

Science.gov (United States)

Loose, D A

2007-01-01

Among vascular malformations, the predominantly venous malformations represent the majority of cases. They form a clinical entity and therefore need clear concepts concerning diagnosis and treatment. This paper presents an overview of contemporary classification as well as tactics and techniques of treatment. According to the Hamburg Classification, predominantly venous malformations are categorized into truncular and extratruncular forms, with truncular forms distinguished as obstructions and dilations, and extratruncular forms as limited or infiltrating. The tactics of treatment represent surgical and non-surgical methods or combined techniques. Surgical approaches utilize different tactics and techniques that are adopted based on the pathologic form and type of the malformation: (I) operation to reduce the haemodynamic activity of the malformation; (II) operation to eliminate the malformation; and (III) reconstructive operation. As for (I), a type of a tactic is the operation to derive the venous flow. In (II), the total or partial removal of the venous malformation is demonstrated subdivided into three different techniques. In this way, the infiltrating as well as the limited forms can be treated. An additional technique is dedicated to the treatment of a marginal vein. Approach (III) involves the treatment of venous aneurysms, where a variety of techniques have been successful. Long-term follow-up demonstrates positive results in 91% of the cases. Congenital predominantly venous malformations should be treated according to the principles developed during the past decades in vascular surgery, interventional treatment and multidisciplinary treatment. The days of predominantly conservative treatment should be relegated to the past. Special skills and experiences are necessary to carry out appropriate surgical strategy, and the required operative techniques should be dictated by the location and type of malformation and associated findings.

Evaluation on temporal bone CT findings of cholesteatoma

International Nuclear Information System (INIS)

Lee, Kun Won; Lee, Nam Joon; Kang, Eun Young; Chung, Kyoo Byung; Suh, Won Hyuck

1989-01-01

Cholesteatomas are thought to result from ingrowth of keratinizing squamous epithelium from external ear to middle ear. The cholesteatomas are usually diagnosed by clinical symptoms and signs, otoscopy, and plain radiograms. But various view points are emphasized radiologically before operation, leading to examine by computed tomography (CT), especially in complicated cases. We retrospectively reviewed the CT findings of cholesteatomas in 25 surgically proven cases during the period from May, 1983 to Aug, 1988. The results were as follows: 1. Most cholesteatomas showed soft tissue mass and bony erosion of ossicles (88%), attic wall and mastoid antrum (84%) on temporal bone CT scan. 2. The CT members of cholesteatomas ranged from 25 to 50 HU (avg. 33 HU). 3. Involved sites were attic (16%), antrum (28%), and both attic and antrum (56%). Other sites were middle ear cavity and external auditory canal. 4. Extra-tympanomastoid extension of cholesteatoma was intracranial abscess (8%), exposure of dural sinus (8%), and extension along with neck (4%)

Analysis of Vibrant Soundbridge placement against the round window membrane in a human cadaveric temporal bone model.

NARCIS (Netherlands)

Pennings, R.J.E.; Ho, A.; Brown, J.; Wijhe, R.G. van; Bance, M.

2010-01-01

OBJECTIVE: To evaluate optimal placement of the Floating Mass Transducer of the Vibrant Soundbridge (Med-El, Innsbruck, Austria) against the round window membrane, particularly the impact of interposed coupling fascia and of covering materials. METHOD: : Six fresh human cadaveric temporal bones were

Primary chondroid chordoma arising from the petrous temporal bone: a case report

Energy Technology Data Exchange (ETDEWEB)

Lee, Young Uk; Youn, Eun Kyung [Koryo General Hospital, Seoul (Korea, Republic of)

1991-01-15

Chordomas are uncommon tumors which arise from remnants of the primitive notochord. They are situated chiefly in the anterior spinal axis with a predilection for the sacrococcygeal region and the basiocciput. About 50% of chordomas are sacrococcygeal, 35% are intracranial, and 15% arise from a vertebral body. As a histologic variant of chordoma, /chondroid chordoma' was first described by Heffelfinger et al. We present a rare case of primary chondroid chordoma arising from the petrous temporal bone. To our knowledge, only two other cases of this type have been reported earlier.

Primary chondroid chordoma arising from the petrous temporal bone: a case report

International Nuclear Information System (INIS)

Lee, Young Uk; Youn, Eun Kyung

1991-01-01

Chordomas are uncommon tumors which arise from remnants of the primitive notochord. They are situated chiefly in the anterior spinal axis with a predilection for the sacrococcygeal region and the basiocciput. About 50% of chordomas are sacrococcygeal, 35% are intracranial, and 15% arise from a vertebral body. As a histologic variant of chordoma, /chondroid chordoma' was first described by Heffelfinger et al. We present a rare case of primary chondroid chordoma arising from the petrous temporal bone. To our knowledge, only two other cases of this type have been reported earlier

[Identification of risk factors for congenital malformations].

Science.gov (United States)

Canals C, Andrea; Cavada C, Gabriel; Nazer H, Julio

2014-11-01

The relative importance of congenital malformations as a cause of death in the first year of life is increasing along with the control of preventable causes of perinatal mortality. To identify risk factors for congenital malformations. Retrospective case-control study of births registered in the database of The Latin American Collaborative Study of Congenital Malformations (ECLAMC), in the period 2001-2010. Birth weight and gestational age were significantly lower in cases than controls, behaving as risk factors and associated with a greater severity of congenital malformations. The risk and severity of congenital malformations increased along with mother's age. Fetal growth retardation, a history of congenital malformations in the family, physical factors and acute illnesses of the mother in the first trimester of pregnancy were also significant risk factors for congenital malformations and their severity. The educational level of the mother was a protective factor for congenital malformations and their severity. Variables previously identified as risk factors for congenital malformations, were significantly related with the occurrence of congenital malformations and their severity.

Malformations of the first ray of the foot in children: diagnosis, clinical picture, treatment

Directory of Open Access Journals (Sweden)

Mikhail Pavlovich Konyukhov

2015-06-01

Full Text Available Malformations of the first ray of the foot vary widely and are divided into simple and complex. Complex malformations include abnormality of development of the first metatarsal or the main phalanx and refer to atypical adducted foot deformity. They are also characterized by varus location of the first ray of varying severity. The cause of deformation is a damage of longitudinal epiphyseal growth plate area of the first metatarsal - “longitudinal epiphyseal bracket” or the so-called delta phalanx. Over the past five years, we treated 37 patients (53 feet aged from 6 months to 17 years old with developmental disabilities of the first ray of the foot. The spectrum of pathology is very diverse. In the majority of cases surgical treatment was multi-staged. It was revealed that the removal of deformity at the first stage of treatment should be complete, with maximum use of the bone to restore the length and shape of the affected bone. In treating combined deformities the good effect is guaranteed only with the removal of all the elements, including excision of the fibrous bridle along the inner surface of the first ray.

Temporal bone trauma: correlative study between CT findings and clinical manifestations

Energy Technology Data Exchange (ETDEWEB)

Kim, Jung Hee; Kim, Hyung Jin; Kim, Jae Hyoung [College of Medicine, Gyeongsang National University, Jinju (Korea, Republic of)

1994-11-15

To assess how accurately computed tomography (CT) can demonstrate the abnormal findings which are believed to cause the clinical signs and symptoms of hearing loss (HL), vertigo and facial paralysis (FP) in patients with temporal bone trauma. The authors studied CT scans of 39 ears in 35 patients with temporal bone trauma. CT scans were performed with 1-1.5 mm slice thickness and table incrementation. Both axial and coronal scans were obtained in 32 patients and in three patients only axial scans were obtained. We analyzed CT with special reference to the structural abnormalities of the external auditory canal, middle ear cavity, bony labyrinth, and facial nerve canal, and correlated these findings with the actual clinical signs and symptoms. As to hearing loss, we evaluated 32 ears in which pure tone audiometry or brainstem evoked response audiometry had been performed. With respect to the specific types of HL, CT accurately showed the abnormalities in 84% (16/19) in conductive HL, 100% (2/2) in sensorineural HL, and 25% (2/8) for mixed HL. When we categorized HL simply as conductive and sensorineural, assuming that mixed be the result of combined conductive and sensorineural HL, CT demonstrated the abnormalities in 89% (24/27) for conductive HL and 50% (5/10) for sensorineural HL. Concerning vertigo and FP, CT demonstrated abnormalities in 67%(4/6), and 29% (4/14), respectively. Except for conductive HL, CT seems to have a variable degree of limitation for the demonstration of the structural abnormalities resulting sensorineural HL, vertigo or facial paralysis. It is imperative to correlate the CT findings with the signs and symptoms in those clinical settings.

Temporal bone trauma: correlative study between CT findings and clinical manifestations

International Nuclear Information System (INIS)

Kim, Jung Hee; Kim, Hyung Jin; Kim, Jae Hyoung

1994-01-01

To assess how accurately computed tomography (CT) can demonstrate the abnormal findings which are believed to cause the clinical signs and symptoms of hearing loss (HL), vertigo and facial paralysis (FP) in patients with temporal bone trauma. The authors studied CT scans of 39 ears in 35 patients with temporal bone trauma. CT scans were performed with 1-1.5 mm slice thickness and table incrementation. Both axial and coronal scans were obtained in 32 patients and in three patients only axial scans were obtained. We analyzed CT with special reference to the structural abnormalities of the external auditory canal, middle ear cavity, bony labyrinth, and facial nerve canal, and correlated these findings with the actual clinical signs and symptoms. As to hearing loss, we evaluated 32 ears in which pure tone audiometry or brainstem evoked response audiometry had been performed. With respect to the specific types of HL, CT accurately showed the abnormalities in 84% (16/19) in conductive HL, 100% (2/2) in sensorineural HL, and 25% (2/8) for mixed HL. When we categorized HL simply as conductive and sensorineural, assuming that mixed be the result of combined conductive and sensorineural HL, CT demonstrated the abnormalities in 89% (24/27) for conductive HL and 50% (5/10) for sensorineural HL. Concerning vertigo and FP, CT demonstrated abnormalities in 67%(4/6), and 29% (4/14), respectively. Except for conductive HL, CT seems to have a variable degree of limitation for the demonstration of the structural abnormalities resulting sensorineural HL, vertigo or facial paralysis. It is imperative to correlate the CT findings with the signs and symptoms in those clinical settings

Aortic arch malformations

Energy Technology Data Exchange (ETDEWEB)

Kellenberger, Christian J. [University Children' s Hospital, Department of Diagnostic Imaging, Zuerich (Switzerland)

2010-06-15

Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring. In this article, the development and imaging appearance of the aortic arch system and its various malformations are reviewed. (orig.)

Aortic arch malformations

International Nuclear Information System (INIS)

Kellenberger, Christian J.

2010-01-01

Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring. In this article, the development and imaging appearance of the aortic arch system and its various malformations are reviewed. (orig.)

MRI of the brain: malformations

International Nuclear Information System (INIS)

Treguier, C.; Heautot, J.F.; Gandon, Y.; Carsin, M.; Buhe, T.; Weppe, V.

1990-01-01

Brain malformations include occlusion, diverticula, neuronal migration abnormalities, cystic malformations, and histogenetic disorders in neurocutaneous syndromes. The multiple planes of section, excellent white/gray matter differentiation and accurate delineation of the brain cortex available with magnetic resonance imaging make it a tool of choice for the diagnosis of malformations, as well as for prognosis and genetic counseling [fr

RASA1 analysis guides management in a family with capillary malformation-arteriovenous malformation.

Science.gov (United States)

Flore, Leigh Anne; Leon, Eyby; Maher, Tom A; Milunsky, Jeff M

2012-06-01

Capillary malformation-arteriovenous malformation (CM-AVM; MIM 60354) is an autosomal dominant disorder characterized by multifocal cutaneous capillary malformations, often in association with fast-flow vascular lesions, which may be cutaneous, subcutaneous, intramuscular, intraosseus, or cerebral arteriovenous malformations or arteriovenous fistulas. CM-AVM results from heterozygous mutations in the RASA1 gene. Capillary malformations of the skin are common, and clinical examination alone may not be able to definitively diagnose-or exclude- CM-AVM. We report a family in which the proband was initially referred for a genetic evaluation in the neonatal period because of the presence of a cardiac murmur and minor dysmorphic features. Both he and his mother were noted to have multiple capillary malformations on the face, head, and extremities. Echocardiography revealed dilated head and neck vessels and magnetic resonance imaging and angiography of the brain revealed a large infratentorial arteriovenous fistula, for which he has had two embolization procedures. RASA1 sequence analysis revealed a heterozygous mutation, confirming his diagnosis of CM-AVM. We established targeted mutation analysis for the proband's mother and sister, the latter of whom is a healthy 3-year-old whose only cutaneous finding is a facial capillary malformation. This revealed that the proband's mother is also heterozygous for the RASA1 mutation, but his sister is negative. Consequently, his mother will undergo magnetic resonance imaging and angiography screening for intracranial and spinal fast-flow lesions, while his sister will require no imaging or serial evaluations. Targeted mutation analysis has been offered to additional maternal family members. This case illustrates the benefit of molecular testing in diagnosis and making screening recommendations for families with CM-AVM.

Fibrous Dysplasia of the Temporal Bone with External Auditory Canal Stenosis and Secondary Cholesteatoma.

Science.gov (United States)

Liu, Yu-Hsi; Chang, Kuo-Ping

2016-04-01

Fibrous dysplasia is a slowly progressive benign fibro-osseous disease, rarely occurring in temporal bones. In these cases, most bony lesions developed from the bony part of the external auditory canals, causing otalgia, hearing impairment, otorrhea, and ear hygiene blockade and probably leading to secondary cholesteatoma. We presented the medical history of a 24-year-old woman with temporal monostotic fibrous dysplasia with secondary cholesteatoma. The initial presentation was unilateral conductive hearing loss. A hard external canal tumor contributing to canal stenosis and a near-absent tympanic membrane were found. Canaloplasty and type I tympanoplasty were performed, but the symptoms recurred after 5 years. She received canal wall down tympanomastoidectomy with ossciculoplasty at the second time, and secondary cholesteatoma in the middle ear was diagnosed. Fifteen years later, left otorrhea recurred again and transcanal endoscopic surgery was performed for middle ear clearance. Currently, revision surgeries provide a stable auditory condition, but her monostotic temporal fibrous dysplasia is still in place.

[The clinico-neuropsychological aspects of arteriovenous malformations of the hippocampus].

Science.gov (United States)

Buklina, S B; Filatov, Iu M; Eliava, Sh Sh

1998-01-01

A comprehensive examination was made in 39 patients with arteriovenous malformations (AVM) of hippocampus. Prior to and following surgery, all the patients underwent neuropsychological study after A. R. Luriia (1962). Mnestic disorder was found to be the most common abnormality in patients with AVM at this site. Before surgery, they were detected in 34 of 39 patients, 11 of them having severe memory disorders with the traits of the Korsakoff's syndrome. These patients were found to have mixed posthemorrhagic lesion of the hippocampus, other portions of the temporal lobe and periventricular structures. Twenty nine patients were operated on, 14 of them had progressive mnestic disorder of the modally nonspecific type irrespective the side operated on. There were no postoperative Korsakoff's syndromes. There was no progression in memory defects in patients after surgery on the brain drastically changed after hemorrhage or removal of minor malformations. Before hemorrhage, epileptic paroxysms were observed in 2 of the 39 patients only in the presence of massive AVM obligatorily involving the temporal cortex. Following surgery, there were no new epileptic paroxysms and changes in the emotional status and motivations in the patients. Thus, the hippocampal formation is involved in the primary mechanisms of fixation, retention, reproduction of a memory trace. The participation of many structures of the brain is required to form an emotional status, motivation, and clinical manifestations of epileptic activity.

Freely-available, true-color volume rendering software and cryohistology data sets for virtual exploration of the temporal bone anatomy.

Science.gov (United States)

Kahrs, Lüder Alexander; Labadie, Robert Frederick

2013-01-01

Cadaveric dissection of temporal bone anatomy is not always possible or feasible in certain educational environments. Volume rendering using CT and/or MRI helps understanding spatial relationships, but they suffer in nonrealistic depictions especially regarding color of anatomical structures. Freely available, nonstained histological data sets and software which are able to render such data sets in realistic color could overcome this limitation and be a very effective teaching tool. With recent availability of specialized public-domain software, volume rendering of true-color, histological data sets is now possible. We present both feasibility as well as step-by-step instructions to allow processing of publicly available data sets (Visible Female Human and Visible Ear) into easily navigable 3-dimensional models using free software. Example renderings are shown to demonstrate the utility of these free methods in virtual exploration of the complex anatomy of the temporal bone. After exploring the data sets, the Visible Ear appears more natural than the Visible Human. We provide directions for an easy-to-use, open-source software in conjunction with freely available histological data sets. This work facilitates self-education of spatial relationships of anatomical structures inside the human temporal bone as well as it allows exploration of surgical approaches prior to cadaveric testing and/or clinical implementation. Copyright © 2013 S. Karger AG, Basel.

Genetics Home Reference: Dandy-Walker malformation

Science.gov (United States)

... Twitter Home Health Conditions Dandy-Walker malformation Dandy-Walker malformation Printable PDF Open All Close All Enable ... to view the expand/collapse boxes. Description Dandy-Walker malformation affects brain development, primarily development of the ...

Neonatal Death Dwarfism in a Girl with Distinctive Bone Dysplasia Compatible with Grebe Chondrodysplasia: Analysis by CT Scan-based Phenotype

OpenAIRE

Ali Al Kaissi; Farid Ben Chehida; Rudolf Ganger; Franz Grill

2014-01-01

We report on a female fetus noted to have severe malformative type of skeletal dysplasia on ultrasonography done at 35 weeks gestation. The girl died shortly after birth. Clinical examination showed a fetus with severe dwarfism, extensive long and short bones, and bone deficiencies associated with multiple dislocations. Computed tomography (CT) scan-based phenotype showed a complex constellation of malformations consistent with the diagnosis of Grebe syndrome. Parents being first cousins (con...

RISK FACTORS IN CHILD CONGENITAL MALFORMATIONS

OpenAIRE

Alina-Costina LUCA; Mirabela SUBOTNICU

2015-01-01

Congenital heart malformations are among the most common congenital malformations. Congenital heart malformations occur due to genetic and environmental factors during embryonic morphogenesis period of the heart. About 25% of these malformations are severe, requiring intervention immediately after birth or in infancy. Abnormalities of structure and function of the heart and great vessels are the consequence of teratogenic factors occurring between day 19 and 45 of gestation. (Yagel et al...

A clinical and experimental overview of sirenomelia: insight into the mechanisms of congenital limb malformations.

Science.gov (United States)

Garrido-Allepuz, Carlos; Haro, Endika; González-Lamuño, Domingo; Martínez-Frías, María Luisa; Bertocchini, Federica; Ros, Maria A

2011-05-01

Sirenomelia, also known as sirenomelia sequence, is a severe malformation of the lower body characterized by fusion of the legs and a variable combination of visceral abnormalities. The causes of this malformation remain unknown, although the discovery that it can have a genetic basis in mice represents an important step towards the understanding of its pathogenesis. Sirenomelia occurs in mice lacking Cyp26a1, an enzyme that degrades retinoic acid (RA), and in mice that develop with reduced bone morphogenetic protein (Bmp) signaling in the caudal embryonic region. The phenotypes of these mutant mice suggest that sirenomelia in humans is associated with an excess of RA signaling and a deficit in Bmp signaling in the caudal body. Clinical studies of sirenomelia have given rise to two main pathogenic hypotheses. The first hypothesis, based on the aberrant abdominal and umbilical vascular pattern of affected individuals, postulates a primary vascular defect that leaves the caudal part of the embryo hypoperfused. The second hypothesis, based on the overall malformation of the caudal body, postulates a primary defect in the generation of the mesoderm. This review gathers experimental and clinical information on sirenomelia together with the necessary background to understand how deviations from normal development of the caudal part of the embryo might lead to this multisystemic malformation.

Cardiac and vascular malformations

International Nuclear Information System (INIS)

Ley, S.; Ley-Zaporozhan, J.

2015-01-01

Malformations of the heart and great vessels show a high degree of variation. There are numerous variants and defects with only few clinical manifestations and are only detected by chance, such as a persistent left superior vena cava or a partial anomalous pulmonary venous connection. Other cardiovascular malformations are manifested directly after birth and need prompt mostly surgical interventions. At this point in time echocardiography is the diagnostic modality of choice for morphological and functional characterization of malformations. Additional imaging using computed tomography (CT) or magnetic resonance imaging (MRI) is only required in a minority of cases. If so, the small anatomical structures, the physiological tachycardia and tachypnea are a challenge for imaging modalities and strategies. This review article presents the most frequent vascular, cardiac and complex cardiovascular malformations independent of the first line diagnostic imaging modality. (orig.) [de

CT and MRI findings of temporal bone anomaly in patients with tinnitus

Energy Technology Data Exchange (ETDEWEB)

Bing, Wang; Junfang, Xian; Zhenchang, Wang; Zhaohui, Liu [Department of Radiology, Beijing Tongren Hospital, Capital Medical University (China)

2011-03-15

Objective: To study high resolution CT (HRCT) and MRI findings of temporal hone anomaly in patients with tinnitus and identify the optimal examination method in the detection of the anomaly. Methods: The HRCT and MRI data were analyzed retrospectively in 1015 patients including 145 patients with pulsatile tinnitus (PT) and 870 patients with nonpulsatile tinnitus (NPT). The positive rates of HRCT and MRI in the identification of temporal bone anomaly were analyzed and the efficiency of various examination methods was compared in revealing the anomaly. Data were tested by Chi-square test analysis. Results: Among 1015 patients, anomaly was seen in 767 cases (75.57%). High jugular bulb was found in 414 patients, accounting for 40.79%. Sigmoid sinus anomaly was detected in 387 patients (38.13%), while otitis media was found in 148 cases (14.58%), and low middle cranial fossa in 70 cases (6.90%). The positive rate of HRCT in the detection of high jugular bulb was 54.89% (365/665), which was significantly higher than those of other methods (P<0.05). The positive rate of enhanced HRCT in showing sigmoid sinus anomaly was 73.68% (56/76), which was significantly higher than those of other methods (P<0.05). Sigmoid sinus anomaly was the most frequent finding in patients with PT, accounting for 66.21% (96/145). The incidence of sigmoid sinus anomaly was higher in PT than in NPT (291/870, 33.45%; χ{sup 2}=56.537, P<0.01). The fast imaging employing steady-state acquisition (FIESTA) sequence was the best examination method in displaying the vessel within the internal auditory canal (42/42,100%). Conclusions: High jugular bulb and sigmoid sinus anomaly were the most frequent abnormal findings of temporal bone in patients with tinnitus. Enhanced HRCT was the choice of modality in patients with PT. Plain HRCT was recommended for NPT. FIESTA sequence was the best in the evaluation of the vessel within the internal auditory canal. (authors)

Chiari malformations: diagnosis, treatments and failures.

Science.gov (United States)

Abd-El-Barr, M M; Strong, C I; Groff, M W

2014-12-01

Chiari malformations refer to abnormalities of the hindbrain originally described by the Austrian pathologist Hans Chiari in the early 1890s. These malformations range from herniation of the cerebellar tonsils through the foramen magnum to complete agenesis of the cerebellum. In this review, we review the different classification schemes of Chiari malformations. We discuss the different signs and symptoms that the two most common malformations present with and diagnostic criteria. We next discuss current treatment paradigms, including the new measure of possible in utero surgery to help decrease the incidence of Chiari type II malformations. There is also a small discussion of treatment failures and salvage procedures in these difficult cases. Chiari malformations are a difficult clinical entity to treat. As more is learned about the genetic and environmental factors relating to their characteristics, it will be interesting if we are able to predict which treatments are better suited for different patients. Similarly, with the evolution of in utero techniques especially for Chiari II malformations, it will be interesting to see if the incidence and practice of treating these difficult patients will change.

3D-CT of the temporal bone area with high-speed processing

International Nuclear Information System (INIS)

Hattori, Taku

1994-01-01

Three-dimentional (3D)-CT was introduced to represent abnormal findings in the temporal bone area utilizing a SOMATOM DRH CT scanner with accessory 3D reconstruction software and an exclusive high-speed 3D processing system, VOXEL FLINGER. In a patient with eosinophilic granuloma, a defect in the squamous part of the temporal bone was demonstrated suggesting exposure of the dura mater during surgery. In a patient with a normal ear, well-developed mastoid cavity, a part of the handle and the head of the malleus, the incudomalleal joint, the short limb, body and a part of the long limb of the incus and the round window niche were demonstrated. In a case of chronic otitis media, poorly developed mastoid cavity and a possible defect of the tip of the long limb of the incus were demonstrated, in contrast to the patient with the normal ear. 3D-CT yields objective and solid images which are useful for diagnosis, treatment planning and explanation of the pathology to patients and their family. To obtain convincing 3D images, physicians themselves have to choose exact rotation angles. It is not adequate to reconstruct original CT data using a CT computer with accessory 3D software whose processing capability is not good enough for this purpose. The conclusion is as follows: 1) it is necessary and effective to transfer original CT data into the memory of the exclusive high-speed 3D processing system and 2) process the data by the voxel memory method to establish a clinically valuable 3D-CT imaging system. (author)

Accuracy of linear drilling in temporal bone using drill press system for minimally invasive cochlear implantation.

Science.gov (United States)

Dillon, Neal P; Balachandran, Ramya; Labadie, Robert F

2016-03-01

A minimally invasive approach for cochlear implantation involves drilling a narrow linear path through the temporal bone from the skull surface directly to the cochlea for insertion of the electrode array without the need for an invasive mastoidectomy. Potential drill positioning errors must be accounted for to predict the effectiveness and safety of the procedure. The drilling accuracy of a system used for this procedure was evaluated in bone surrogate material under a range of clinically relevant parameters. Additional experiments were performed to isolate the error at various points along the path to better understand why deflections occur. An experimental setup to precisely position the drill press over a target was used. Custom bone surrogate test blocks were manufactured to resemble the mastoid region of the temporal bone. The drilling error was measured by creating divots in plastic sheets before and after drilling and using a microscope to localize the divots. The drilling error was within the tolerance needed to avoid vital structures and ensure accurate placement of the electrode; however, some parameter sets yielded errors that may impact the effectiveness of the procedure when combined with other error sources. The error increases when the lateral stage of the path terminates in an air cell and when the guide bushings are positioned further from the skull surface. At contact points due to air cells along the trajectory, higher errors were found for impact angles of [Formula: see text] and higher as well as longer cantilevered drill lengths. The results of these experiments can be used to define more accurate and safe drill trajectories for this minimally invasive surgical procedure.

Megalencephaly, polymicrogyria and ribbon-like band heterotopia: A new cortical malformation.

Science.gov (United States)

Kobayashi, Yu; Magara, Shinichi; Okazaki, Kenichi; Komatsubara, Takao; Saitsu, Hirotomo; Matsumoto, Naomichi; Kato, Mitsuhiro; Tohyama, Jun

2016-11-01

Megalencephalic polymicrogyria syndromes include megalencephaly-capillary malformation and megalencephaly-polymicrogyria-polydactyly-hydrocephalus. Recent genetic studies have identified that genes in the PI3K-AKT pathway are involved in the pathogenesis of these disorders. Herein, we report a patient who presented with developmental delay, epilepsy and peculiar neuroimaging findings of megalencephaly, polymicrogyria, and symmetrical band heterotopia in the periventricular region. The heterotopias exhibited inhomogeneous signals with undulatory mixtures of gray and white matter, resembling ribbon-like heterotopia, with a predominance in the temporal to occipital regions. These neuroradiological findings were not consistent with those in known megalencephalic polymicrogyria syndromes. No genetic abnormality was identified through whole-exome sequencing. The neuroimaging findings of this patient may represent a novel cortical malformation involving megalencephaly with polymicrogyria and ribbon-like band heterotopia. Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Congenital heart defects and extracardiac malformations.

Science.gov (United States)

Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

2013-06-01

To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

Computed tomography of the temporal bone and orbit

International Nuclear Information System (INIS)

Zonneveld, F.W.

1987-01-01

The basis for this dissertation is the combination of the best set of high-resolution CT scanning parameters, on the one hand, and the technique of scanning perpendicular to the tissue interface, or parallel to an elongated anatomical structure (direct multiplanar CT technique) on the other. Although this technique yields better visualization of a number of anatomical details, the problem remains that the radiologist is as yet unfamiliar with these alternative cross-sectional planes. For this reason, a technique for cryosectioning fresh frozen specimens was selected and improved to create cross-sectional images that can be correlated with the direct multiplanar CT scans. The selection of special scan planes, the positioning, preparation and examination of the patient, and the CT and correlative anatomy are discussed separately for the temporal bone and the orbit. A few clinical applications are discussed. In the orbit, the value of high-resolution CT is demonstrated in the establishment of the relationship between space-occupying lesions and the optic nerve, and in the management of fractures of the orbital floor. 548 refs.; 253 figs.; 24 tabs

Initial results of a new generation dual source CT system using only an in-plane comb filter for ultra-high resolution temporal bone imaging.

Science.gov (United States)

Meyer, Mathias; Haubenreisser, Holger; Raupach, Rainer; Schmidt, Bernhard; Lietzmann, Florian; Leidecker, Christianne; Allmendinger, Thomas; Flohr, Thomas; Schad, Lothar R; Schoenberg, Stefan O; Henzler, Thomas

2015-01-01

To prospectively evaluate radiation dose and image quality of a third generation dual-source CT (DSCT) without z-axis filter behind the patient for temporal bone CT. Forty-five patients were either examined on a first, second, or third generation DSCT in an ultra-high-resolution (UHR) temporal bone-imaging mode. On the third generation DSCT system, the tighter focal spot of 0.2 mm(2) removes the necessity for an additional z-axis-filter, leading to an improved z-axis radiation dose efficiency. Images of 0.4 mm were reconstructed using standard filtered-back-projection or iterative reconstruction (IR) technique for previous generations of DSCT and a novel IR algorithm for the third generation DSCT. Radiation dose and image quality were compared between the three DSCT systems. The statistically significantly highest subjective and objective image quality was evaluated for the third generation DSCT when compared to the first or second generation DSCT systems (all p generation examination as compared to the first and second generation DSCT. Temporal bone imaging without z-axis-UHR-filter and a novel third generation IR algorithm allows for significantly higher image quality while lowering effective dose when compared to the first two generations of DSCTs. • Omitting the z-axis-filter allows a reduction in radiation dose of 50% • A smaller focal spot of 0.2 mm (2) significantly improves spatial resolution • Ultra-high-resolution temporal-bone-CT helps to gain diagnostic information of the middle/inner ear.

High-resolution T1-weighted 3D real IR imaging of the temporal bone using triple-dose contrast material

Energy Technology Data Exchange (ETDEWEB)

Naganawa, Shinji; Koshikawa, Tokiko; Nakamura, Tatsuya; Fukatsu, Hiroshi; Ishigaki, Takeo [Department of Radiology, Nagoya University School of Medicine, 65 Tsurumai-cho, Shouwa-ku, 466-8550, Nagoya (Japan); Aoki, Ikuo [Medical System Company, Toshiba Corporation, Tokyo (Japan)

2003-12-01

The small structures in the temporal bone are surrounded by bone and air. The objectives of this study were (a) to compare contrast-enhanced T1-weighted images acquired by fast spin-echo-based three-dimensional real inversion recovery (3D rIR) against those acquired by gradient echo-based 3D SPGR in the visualization of the enhancement of small structures in the temporal bone, and (b) to determine whether either 3D rIR or 3D SPGR is useful for visualizing enhancement of the cochlear lymph fluid. Seven healthy men (age range 27-46 years) volunteered to participate in this study. All MR imaging was performed using a dedicated bilateral quadrature surface phased-array coil for temporal bone imaging at 1.5 T (Visart EX, Toshiba, Tokyo, Japan). The 3D rIR images (TR/TE/TI: 1800 ms/10 ms/500 ms) and flow-compensated 3D SPGR images (TR/TE/FA: 23 ms/10 ms/25 ) were obtained with a reconstructed voxel size of 0.6 x 0.7 x 0.8 mm{sup 3}. Images were acquired before and 1, 90, 180, and 270 min after the administration of triple-dose Gd-DTPA-BMA (0.3 mmol/kg). In post-contrast MR images, the degree of enhancement of the cochlear aqueduct, endolymphatic sac, subarcuate artery, geniculate ganglion of the facial nerve, and cochlear lymph fluid space was assessed by two radiologists. The degree of enhancement was scored as follows: 0 (no enhancement); 1 (slight enhancement); 2 (intermediate between 1 and 3); and 3 (enhancement similar to that of vessels). Enhancement scores for the endolymphatic sac, subarcuate artery, and geniculate ganglion were higher in 3D rIR than in 3D SPGR. Washout of enhancement in the endolymphatic sac appeared to be delayed compared with that in the subarcuate artery, suggesting that the enhancement in the endolymphatic sac may have been due in part to non-vascular tissue enhancement. Enhancement of the cochlear lymph space was not observed in any of the subjects in 3D rIR and 3D SPGR. The 3D rIR sequence may be more sensitive than the 3D SPGR sequence in

Multicentric osteolysis with nodulosis and arthropathy (MONA) with cardiac malformation, mimicking polyarticular juvenile idiopathic arthritis

DEFF Research Database (Denmark)

Castberg, Filip Christian; Kjaergaard, Susanne; Mosig, Rebecca A

2013-01-01

six also had congenital cardiac malformations. Despite treatment attempts of our patient with methotrexate, eternacept and prednisolone, serial X-ray studies documented continuous severe bone degeneration. Conclusion: The case documents the natural history of MONA and establishes a link between MMP2...... deficiency and heart development, and given the recurring cardiac association, we suggest that all MONA patients be examined for possible cardiac defects....

Prevalence of radiographic semicircular canal dehiscence in very young children: an evaluation using high-resolution computed tomography of the temporal bones

International Nuclear Information System (INIS)

Hagiwara, Mari; Fatterpekar, Girish; Shaikh, Jamil A.; Fang, Yixin; Roehm, Pamela C.

2012-01-01

Previous studies suggest that semicircular canal dehiscences (SCDs) have a developmental origin. We hypothesized that if SCDs originate during development, incidence of radiographic SCDs in young children will be higher than in adults. Thirty-four temporal bone HRCTs of children younger than 2 years and 40 temporal bone HRCTs of patients older than 18 years were reformatted and re-evaluated for presence of SCD or canal thinning. Results were compared with indications for HRCT and clinical information. SCDs were detected in 27.3% of children younger than 2 years of age (superior, 13.8%; posterior, 20%) and in 3% of adults (P < 0.004). Of children with one radiographic dehiscence, 55.6% had multiple and 44% had bilateral SCDs on HRCT. No lateral canal SCDs were present. Thinning of bone overlying the semicircular canals was found in 44% of children younger than 2 years and 2.5% of adults (P < 0.0001). SCDs are more common on HRCTs of very young children. This supports the hypothesis that SCDs originate from discontinuation of bone deposition/maturation. However, SCDs on imaging do not necessarily correlate with canal dehiscence syndrome and should therefore be interpreted carefully. (orig.)

Prevalence of radiographic semicircular canal dehiscence in very young children: an evaluation using high-resolution computed tomography of the temporal bones

Energy Technology Data Exchange (ETDEWEB)

Hagiwara, Mari; Fatterpekar, Girish [New York University School of Medicine, Department of Radiology, New York, NY (United States); Shaikh, Jamil A. [New York University School of Medicine, Department of Otolaryngology, New York, NY (United States); Fang, Yixin [New York University School of Medicine, Department of Otolaryngology, New York, NY (United States); New York University School of Medicine, Division of Biostatistics, Department of Environmental Medicine, New York, NY (United States); Roehm, Pamela C. [New York University School of Medicine, Department of Otolaryngology, New York, NY (United States); New York University School of Medicine, Department of Otolaryngology, Division of Otology/Neurotology, New York, NY (United States)

2012-12-15

Previous studies suggest that semicircular canal dehiscences (SCDs) have a developmental origin. We hypothesized that if SCDs originate during development, incidence of radiographic SCDs in young children will be higher than in adults. Thirty-four temporal bone HRCTs of children younger than 2 years and 40 temporal bone HRCTs of patients older than 18 years were reformatted and re-evaluated for presence of SCD or canal thinning. Results were compared with indications for HRCT and clinical information. SCDs were detected in 27.3% of children younger than 2 years of age (superior, 13.8%; posterior, 20%) and in 3% of adults (P < 0.004). Of children with one radiographic dehiscence, 55.6% had multiple and 44% had bilateral SCDs on HRCT. No lateral canal SCDs were present. Thinning of bone overlying the semicircular canals was found in 44% of children younger than 2 years and 2.5% of adults (P < 0.0001). SCDs are more common on HRCTs of very young children. This supports the hypothesis that SCDs originate from discontinuation of bone deposition/maturation. However, SCDs on imaging do not necessarily correlate with canal dehiscence syndrome and should therefore be interpreted carefully. (orig.)

Activity patterns in malformed fetuses.

Science.gov (United States)

Rayburn, W F; Barr, M

1982-04-15

Knowledge of a malformed fetus before the onset of labor would assist the physician in preparing the expectant parents, managing the timing and method of delivery, and preparing for the immediate care of a salvageable infant. This 3-year prospective investigation compared the activity patterns of fetuses who were later found to have major malformation with those of fetuses who had no apparent defects. Fetal motion over prolonged periods was determined by daily charting of fetal movement by the mother. Although not a reliable predictor for all malformations, evidence of fetal inactivity was found to be more common (p less than 0.0001) among fetuses with anomalies (16 of 58 cases, 28%) than among those with no defects (39 of 1,098 cases, 4%). All malformations associated with fetal inactivity were strongly suspected ultrasonographically and included hydrocephalus, gastroschisis, nonimmune hydrops, bilateral renal agenesis, and bilateral dislocation of the hips. Documentation of fetal inactivity is helpful in recognizing certain major malformations and constitutes grounds for more detailed study by ultrasonography.

A clinical and experimental overview of sirenomelia: insight into the mechanisms of congenital limb malformations

Directory of Open Access Journals (Sweden)

Carlos Garrido-Allepuz

2011-05-01

Full Text Available Sirenomelia, also known as sirenomelia sequence, is a severe malformation of the lower body characterized by fusion of the legs and a variable combination of visceral abnormalities. The causes of this malformation remain unknown, although the discovery that it can have a genetic basis in mice represents an important step towards the understanding of its pathogenesis. Sirenomelia occurs in mice lacking Cyp26a1, an enzyme that degrades retinoic acid (RA, and in mice that develop with reduced bone morphogenetic protein (Bmp signaling in the caudal embryonic region. The phenotypes of these mutant mice suggest that sirenomelia in humans is associated with an excess of RA signaling and a deficit in Bmp signaling in the caudal body. Clinical studies of sirenomelia have given rise to two main pathogenic hypotheses. The first hypothesis, based on the aberrant abdominal and umbilical vascular pattern of affected individuals, postulates a primary vascular defect that leaves the caudal part of the embryo hypoperfused. The second hypothesis, based on the overall malformation of the caudal body, postulates a primary defect in the generation of the mesoderm. This review gathers experimental and clinical information on sirenomelia together with the necessary background to understand how deviations from normal development of the caudal part of the embryo might lead to this multisystemic malformation.

Expression of Gla proteins during fish skeletal development

OpenAIRE

Gavaia, Paulo J.

2006-01-01

Senegal sole skeletal development; Skeletal malformations; Skeletal malformation in mediterranean species; Senegal sole skeletal deformities; Zebra fish as model system: skeletal development; Identification of bone cells / skeletal development; Spatial - temporal pattern of bgp expression; Single cell resolution: localization of bgp mRNA; Single cell resolution: Immunolocalization of Bgp; Single cell resolution: localization of mgp mRNA; Single cell resolution: Immunolocalization of Mgp; An i...

Characteristics and treatment of cavernous malformations

International Nuclear Information System (INIS)

Takagi, Kenji; Ishida, Mitsuaki; Okabe, Hidetoshi; Nozaki, Kazuhiko

2013-01-01

Cavernous malformations are clusters of dilated sinusoidal channels with thin walls devoid of elastin and smooth muscle. They have no intervening brain tissue. They occur both in sporadic and familial forms. The genes responsible for cavernous malformations have been identified. Recent reports show that mutations of these responsible genes are involved not only in familial but also in sporadic forms. Germline and somatic mutations may occur before cavernous malformations develop (two-hit mechanism). Two patterns, with mulberry-like and hematoma-like appearances, are seen intraoperatively, and from histological findings, mulberry-like appearance may change into hematoma-like one by intralesional hemorrhage. Cavernous malformation treatments include open surgery and radiosurgery. Open surgery is usually chosen for the treatment of symptomatic hemorrhagic cavernous malformations because post-radiosurgical annual bleeding risks at the early phase seem to be higher than those of open surgery. If open surgery has a high risk because of lesion location, radiosurgery becomes an effective alternative treatment. Brainstem cavernous malformations have high annual bleeding and re-bleeding incidence, so if the lesion is accessible with low risk, open surgery is recommended. (author)

Headache in children with Chiari I malformation.

Science.gov (United States)

Toldo, Irene; Tangari, Marta; Mardari, Rodica; Perissinotto, Egle; Sartori, Stefano; Gatta, Michela; Calderone, Milena; Battistella, Pier Antonio

2014-05-01

Headache is the most common symptom of Chiari 1 malformation, a condition characterized by the herniation of cerebellar tonsils through the foramen magnum. However, the headache pattern of cases with Chiari 1 malformations is not well defined in the literature, especially in children. The aim of this retrospective chart review was to evaluate the frequency and the characteristics of headache in children with Chiari 1 malformation at initial evaluation and during follow up. Forty-five cases with tonsillar ectopia were selected among 9947 cases under 18 years of age who underwent neuroimaging between 2002 and 2010. A semistructured clinical interview (mean follow-up: 5.2 years) was conducted. Headache was classified according to the second edition of the International Classification of Headache Disorders. Possible associations between clinical picture, in particular headache pattern, but also other signs and symptoms attributable to Chiari 1 malformation, and the extent of tonsillar ectopia were found for 3 different groups: those with borderline (headache, and 9/33 (27%) of those patients (5 with mild and 4 with severe tonsillar ectopia) reported headache attributed to Chiari 1 malformation. In our studied pediatric population, the most common symptom for cases diagnosed with Chiari 1 malformation was headache, and headache attributed to Chiari 1 malformation was the most common headache pattern in patients with Chiari 1 malformation. The presence of headache attributed to Chiari 1 malformation along with 3 other signs or symptoms of Chiari 1 malformation were highly predictive of severe tonsillar ectopia. © 2014 American Headache Society.

Cerebral malformations without antenatal diagnosis

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Girard, Nadine J. [Diagnostic Neuroradiology, Hopital Timone, Marseille (France)

2010-06-15

Cerebral malformations are usually described following the different steps in development. Disorders of neurulation (dysraphisms), or diverticulation (holoprosencephalies and posterior fossa cysts), and total commissural agenesis are usually diagnosed in utero. In contrast, disorders of histogenesis (proliferation-differentiation, migration, organization) are usually discovered in infants and children. The principal clinical symptoms that may be a clue to cerebral malformation include congenital hemiparesis, epilepsy and mental or psychomotor retardation. MRI is the imaging method of choice to assess cerebral malformations. (orig.)

Presbycusis: a human temporal bone study of individuals with flat audiometric patterns of hearing loss using a new method to quantify stria vascularis volume.

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Nelson, Erik G; Hinojosa, Raul

2003-10-01

The purpose of this study was to determine the prevalence of stria vascularis atrophy in individuals with presbycusis and flat audiometric patterns of hearing loss. Individuals with presbycusis have historically been categorized by the shape of their audiograms, and flat audiometric thresholds have been reported to be associated with atrophy of the stria vascularis. Stria vascularis volume was not measured in these studies. Retrospective case review. Archival human temporal bones from individuals with presbycusis were selected on the basis of strict audiometric criteria for flat audiometric thresholds. Six temporal bones that met these criteria were identified and compared with 10 temporal bones in individuals with normal hearing. A unique quantitative method was developed to measure the stria vascularis volume in these temporal bones. The hair cell and spiral ganglion cell populations also were quantitatively evaluated. Only one of the six individuals with presbycusis and flat audiometric thresholds had significant atrophy of the stria vascularis. This individual with stria vascularis atrophy also had reduced inner hair cell, outer hair cell, and ganglion cell populations. Three of the individuals with presbycusis had spiral ganglion cell loss, three individuals had inner hair cell loss, and all six individuals had outer hair cell loss. The results of this investigation suggest that individuals with presbycusis and flat audiometric patterns of hearing loss infrequently have stria vascularis atrophy. Outer hair cell loss alone or in combination with inner hair cell or ganglion cell loss may be the cause of flat audiometric thresholds in individuals with presbycusis.

Hypoglossal-facial nerve "side"-to-side neurorrhaphy for facial paralysis resulting from closed temporal bone fractures.

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Su, Diya; Li, Dezhi; Wang, Shiwei; Qiao, Hui; Li, Ping; Wang, Binbin; Wan, Hong; Schumacher, Michael; Liu, Song

2018-06-06

Closed temporal bone fractures due to cranial trauma often result in facial nerve injury, frequently inducing incomplete facial paralysis. Conventional hypoglossal-facial nerve end-to-end neurorrhaphy may not be suitable for these injuries because sacrifice of the lesioned facial nerve for neurorrhaphy destroys the remnant axons and/or potential spontaneous innervation. we modified the classical method by hypoglossal-facial nerve "side"-to-side neurorrhaphy using an interpositional predegenerated nerve graft to treat these injuries. Five patients who experienced facial paralysis resulting from closed temporal bone fractures due to cranial trauma were treated with the "side"-to-side neurorrhaphy. An additional 4 patients did not receive the neurorrhaphy and served as controls. Before treatment, all patients had suffered House-Brackmann (H-B) grade V or VI facial paralysis for a mean of 5 months. During the 12-30 months of follow-up period, no further detectable deficits were observed, but an improvement in facial nerve function was evidenced over time in the 5 neurorrhaphy-treated patients. At the end of follow-up, the improved facial function reached H-B grade II in 3, grade III in 1 and grade IV in 1 of the 5 patients, consistent with the electrophysiological examinations. In the control group, two patients showed slightly spontaneous innervation with facial function improved from H-B grade VI to V, and the other patients remained unchanged at H-B grade V or VI. We concluded that the hypoglossal-facial nerve "side"-to-side neurorrhaphy can preserve the injured facial nerve and is suitable for treating significant incomplete facial paralysis resulting from closed temporal bone fractures, providing an evident beneficial effect. Moreover, this treatment may be performed earlier after the onset of facial paralysis in order to reduce the unfavorable changes to the injured facial nerve and atrophy of its target muscles due to long-term denervation and allow axonal

Imaging of the cloacal malformation

International Nuclear Information System (INIS)

Jaramillo, D.; Lebowitz, R.L.; Hendren, W.H.

1989-01-01

Sixty-two newborns with the cloacal malformation were evaluated to define variation in cloacal configuration, associated anomalies, and optimal approach to imaging. Cloacal malformations were classified according to level of urethrovaginal confluence (47 low, 15 high); level of rectal communication (47 vaginal, seven cloacal, eight other); and cloacal appearance (34 urethral, 28 vaginal, 10 transistional). Lower urinary abnormalities included reflux, ureteral ectopia, bladder diverticula, bladder duplication (five), and urachal remnants (five). Uterovaginal abnormalities included duplication and partial agenesis. Anomalies were common in the upper urinary tract, spine (sacral agenesis, 20), and bony pelvis (pubic diastasis, 10). Gastrointestinal, cardiovascular, neurologic, and limb (seven) malformations were seen. Fluoroscopically monitored opacification of the cloacal components and voiding cystourethrogram were essential. Although sonography of the malformation was of limited usefulness, MR imaging seemed to be useful for evaluation of the spinal cord and pelvic musculature

Temporal bone paragangliomas: 15 years experience

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Mehmet Düzlü

Full Text Available Abstract Introduction Temporal bone paragangliomas (TBPs are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. Objective In this paper we aim to present our clinical experience with TBPs and to review literature data. Methods The patients who were operated for tympanomastoid paraganglioma (TMP or tympanojugular paraganglioma (TJP in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. Results There were 18 (52.9% cases with TMPs and 16 (47.1% cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3 ± 11.7 (range 25-71 years. The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4% and 10 (62.5% cases for TMPs and TJPs, respectively. Five patients (31.2% with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months. Conclusion In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age.

Temporal bone paragangliomas: 15 years experience.

Science.gov (United States)

Düzlü, Mehmet; Tutar, Hakan; Karamert, Recep; Karaloğlu, Furkan; Şahin, Muammer Melih; Göcek, Mehmet; Uğur, Mehmet Birol; Göksu, Nebil

2016-12-08

Temporal bone paragangliomas (TBPs) are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. In this paper we aim to present our clinical experience with TBPs and to review literature data. The patients who were operated for tympanomastoid paraganglioma (TMP) or tympanojugular paraganglioma (TJP) in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. There were 18 (52.9%) cases with TMPs and 16 (47.1%) cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3± 11.7 (range 25-71 years). The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4%) and 10 (62.5%) cases for TMPs and TJPs, respectively. Five patients (31.2%) with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months). In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

High-output cardiac failure secondary to multiple vascular malformations in the liver: case report

International Nuclear Information System (INIS)

Spaner, S.; Demeter, S.; Lien, D.; Shapiro, J.; McCarthy, M.; Raymond, G.

2001-01-01

High-output cardiac failure is associated with several systemic illnesses, including hyperthyroidism, thiamine deficiency, severe anemia, multiple myeloma, Paget's disease of bone and Osler-Weber-Rendu syndrome. We present an unusual case of a woman with high-output cardiac failure as a result of multiple arteriovenous fistulas in the liver, most likely representing an unusual variant of Osler-Weber-Rendu syndrome (i.e., no other telangiectasias or a family history of vascular malformations was demonstrated). (author)

Differentiating levels of surgical experience on a virtual reality temporal bone simulator.

Science.gov (United States)

Zhao, Yi C; Kennedy, Gregor; Hall, Richard; O'Leary, Stephen

2010-11-01

Virtual reality simulation is increasingly being incorporated into surgical training and may have a role in temporal bone surgical education. Here we test whether metrics generated by a virtual reality surgical simulation can differentiate between three levels of experience, namely novices, otolaryngology residents, and experienced qualified surgeons. Cohort study. Royal Victorian Eye and Ear Hospital. Twenty-seven participants were recruited. There were 12 experts, six residents, and nine novices. After orientation, participants were asked to perform a modified radical mastoidectomy on the simulator. Comparisons of time taken, injury to structures, and forces exerted were made between the groups to determine which specific metrics would discriminate experience levels. Experts completed the simulated task in significantly shorter time than the other two groups (experts 22 minutes, residents 36 minutes, and novices 46 minutes; P = 0.001). Novices exerted significantly higher average forces when dissecting close to vital structures compared with experts (0.24 Newton [N] vs 0.13 N, P = 0.002). Novices were also more likely to injure structures such as dura compared to experts (23 injuries vs 3 injuries, P = 0.001). Compared with residents, the experts modulated their force between initial cortex dissection and dissection close to vital structures. Using the combination of these metrics, we were able to correctly classify the participants' level of experience 90 percent of the time. This preliminary study shows that measurements of performance obtained from within a virtual reality simulator can differentiate between levels of users' experience. These results suggest that simulator training may have a role in temporal bone training beyond foundational training. Copyright © 2010 American Academy of Otolaryngology–Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.

Magnetic resonance imaging in temporal lobe epilepsy. Usefulness for the etiological diagnosis of temporal lobe epilepsy

International Nuclear Information System (INIS)

Mohamed, A.; Lueders, H.O.

2000-01-01

With improvement in magnetic resonance (MR) imaging techniques, the ability to identify lesions responsible for temporal lobe epilepsy has increased. MR imaging has also enabled the in vivo diagnosis of hippocampal sclerosis. Brain tumors are responsible for 2-4% of epilepsies in adult population and 10-20% of medically intractable epilepsy. The sensitivity of MR imaging in the diagnosis of tumors and other lesions of the temporal lobe (vascular malformations, etc.) is around 90%. Both hippocampal sclerosis and other temporal lobe lesions are amenable to surgical therapy with excellent postsurgical seizure outcome. In this article, we characterize and underline distinguishing features of the different pathological entities. We also suggest an approach to reviewing the MR images of an epileptic patient. (author)

High strength, biodegradable and cytocompatible alpha tricalcium phosphate-iron composites for temporal reduction of bone fractures.

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Montufar, E B; Casas-Luna, M; Horynová, M; Tkachenko, S; Fohlerová, Z; Diaz-de-la-Torre, S; Dvořák, K; Čelko, L; Kaiser, J

2018-04-01

In this work alpha tricalcium phosphate (α-TCP)/iron (Fe) composites were developed as a new family of biodegradable, load-bearing and cytocompatible materials. The composites with composition from pure ceramic to pure metallic samples were consolidated by pulsed electric current assisted sintering to minimise processing time and temperature while improving their mechanical performance. The mechanical strength of the composites was increased and controlled with the Fe content, passing from brittle to ductile failure. In particular, the addition of 25 vol% of Fe produced a ceramic matrix composite with elastic modulus much closer to cortical bone than that of titanium or biodegradable magnesium alloys and specific compressive strength above that of stainless steel, chromium-cobalt alloys and pure titanium, currently used in clinic for internal fracture fixation. All the composites studied exhibited higher degradation rate than their individual components, presenting values around 200 μm/year, but also their compressive strength did not show a significant reduction in the period required for bone fracture consolidation. Composites showed preferential degradation of α-TCP areas rather than β-TCP areas, suggesting that α-TCP can produce composites with higher degradation rate. The composites were cytocompatible both in indirect and direct contact with bone cells. Osteoblast-like cells attached and spread on the surface of the composites, presenting proliferation rate similar to cells on tissue culture-grade polystyrene and they showed alkaline phosphatase activity. Therefore, this new family of composites is a potential alternative to produce implants for temporal reduction of bone fractures. Biodegradable alpha-tricalcium phosphate/iron (α-TCP/Fe) composites are promising candidates for the fabrication of temporal osteosynthesis devices. Similar to biodegradable metals, these composites can avoid implant removal after bone fracture healing, particularly in

Mastoiditis and facial paralysis as initial manifestations of temporal bone systemic diseases - the significance of the histopathological examination.

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Maniu, Alma Aurelia; Harabagiu, Oana; Damian, Laura Otilia; Ştefănescu, Eugen HoraŢiu; FănuŢă, Bogdan Marius; Cătană, Andreea; Mogoantă, Carmen Aurelia

2016-01-01

Several systemic diseases, including granulomatous and infectious processes, tumors, bone disorders, collagen-vascular and other autoimmune diseases may involve the middle ear and temporal bone. These diseases are difficult to diagnose when symptoms mimic acute otomastoiditis. The present report describes our experience with three such cases initially misdiagnosed. Their predominating symptoms were otological with mastoiditis, hearing loss, and subsequently facial nerve palsy. The cases were considered an emergency and the patients underwent tympanomastoidectomy, under the suspicion of otitis media with cholesteatoma, in order to remove a possible abscess and to decompress the facial nerve. The common features were the presence of severe granulation tissue filling the mastoid cavity and middle ear during surgery, without cholesteatoma. The definitive diagnoses was made by means of biopsy of the granulation tissue from the middle ear, revealing granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) in one case, middle ear tuberculosis and diffuse large B-cell lymphoma respectively. After specific associated therapy facial nerve functions improved, and atypical inflammatory states of the ear resolved. As a group, systemic diseases of the middle ear and temporal bone are uncommon, but aggressive lesions. After analyzing these cases and reviewing the literature, we would like to stress upon the importance of microscopic examination of the affected tissue, required for an accurate diagnosis and effective treatment.

Genetics Home Reference: cerebral cavernous malformation

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... Additional NIH Resources (1 link) National Institute of Neurological Disorders and Stroke Educational Resources (7 links) Boston Children's Hospital: Cavernous Malformations Disease InfoSearch: Cerebral Cavernous Malformation ...

Recurrent Streptococcus Pneumoniae Meningitis in a Child with Split Hand and Foot Malformation and Undiagnosed Mondini Dysplasia.

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Katarzyna, Mazur-Melewska; Jarosław, Szydłowski; Katarzyna, Jończyk-Potoczna; Wojciech, Służewski; Magdalena, Figlerowicz

Recurrent bacterial meningitis is a life-threatening infection of the central nervous system that is mostly connected with anatomical abnormalities of the skull, chronic parameningeal infections and immunodeficiencies. It's rarely seen, but when it occurs an extensive investigation should be carried out to discover the responsible factor, so that further episodes can be prevented. We report on a child with split hand and foot (SHFM), confirmed incorrect karyotype 46, XY, t(7:12)(q21.2;q21.3) and a fourth episode of fulminant meningitis caused by penicillin-resistant Streptococcus pneumoniae . After a broad evaluation of factors predisposing to recurrent meningitis, the undiagnosed malformation of his inner and middle ears - Mondini dysplasia was found. We suggest examining all children with SHFM for hearing impairment before they develop recurrent meningitis. The time when the radiological procedure for searching for inner ear dysplasia should be performed could be a controversial issue: before or after the first episode of meningitis. From the epidemiological point of view, high-resolution computer tomography scanning of the temporal bones should be considered after the diagnosis of deafness in a child with SHFM related to 7q21 deletion.

Inner ear anatomy in Waardenburg syndrome: radiological assessment and comparison with normative data.

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Kontorinis, Georgios; Goetz, Friedrich; Lanfermann, Heinrich; Luytenski, Stefan; Giesemann, Anja M

2014-08-01

As patients with Waardenburg syndrome (WS) represent potential candidates for cochlear implantation, their inner ear anatomy is of high significance. There is an ongoing debate whether WS is related to any inner ear dysplasias. Our objective was to evaluate radiologically the inner ear anatomy in patients with WS and identify any temporal bone malformations. A retrospective case review was carried out in a tertiary, referral center. The high resolution computed tomography (HRCT) scans of the temporal bone from 20 patients (40 ears) with WS who were managed for deafness in a tertiary referral center from 1995 to 2012 were retrospectively examined. Measurements of 15 different inner ear dimensions, involving the cochlea, the vestibule, the semicircular canals and the internal auditory meatus, as well as measurements of the vestibular aqueduct, were performed independently by two neuroradiologists. Finally, we compared the results from the WS group with a control group consisting of 50 normal hearing subjects (100 ears) and with previously reported normative values. Inner ear malformations were not found in any of the patients with WS. All measured inner ear dimensions were within the normative values compiled by our study group as well as by others. Inner ear malformations are not characteristic for all types of WS; however, certain rare subtypes might be related to inner ear deformities. Normative cochleovestibular dimensions that can help in assessing the temporal bone anatomy are provided. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Insertion forces and intracochlear trauma in temporal bone specimens implanted with a straight atraumatic electrode array.

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Mirsalehi, Marjan; Rau, Thomas S; Harbach, Lenka; Hügl, Silke; Mohebbi, Saleh; Lenarz, Thomas; Majdani, Omid

2017-05-01

The aim of the study was to evaluate insertion forces during manual insertion of a straight atraumatic electrode in human temporal bones, and post-implantation histologic evaluation of the samples to determine whether violation of intracochlear structures is related to insertion forces. In order to minimize intracochlear trauma and preserve residual hearing during cochlear implantation, knowledge of the insertion forces is necessary. Ten fresh frozen human temporal bones were prepared with canal wall down mastoidectomy. All samples were mounted on a one-axis force sensor. Insertion of a 16-mm straight atraumatic electrode was performed from different angles to induce "traumatic" insertion. Histologic evaluation was performed in order to evaluate intracochlear trauma. In 4 of 10 samples, dislocation of the electrode into scala vestibuli was observed. The mean insertion force for all 10 procedures was 0.003 ± 0.005 N. Insertion forces measured around the site of dislocation to scala vestibuli in 3 of 4 samples were significantly higher than insertion forces at the same location of the cochleae measured in samples without trauma (p straight atraumatic electrode is lower than reported by other studies using longer electrodes. Based on our study, insertion forces leading to basilar membrane trauma may be lower than the previously reported direct rupture forces.

Direct radiocarbon dating and DNA analysis of the Darra-i-Kur (Afghanistan) human temporal bone.

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Douka, Katerina; Slon, Viviane; Stringer, Chris; Potts, Richard; Hübner, Alexander; Meyer, Matthias; Spoor, Fred; Pääbo, Svante; Higham, Tom

2017-06-01

The temporal bone discovered in the 1960s from the Darra-i-Kur cave in Afghanistan is often cited as one of the very few Pleistocene human fossils from Central Asia. Here we report the first direct radiocarbon date for the specimen and the genetic analyses of DNA extracted and sequenced from two areas of the bone. The new radiocarbon determination places the find to ∼4500 cal BP (∼2500 BCE) contradicting an assumed Palaeolithic age of ∼30,000 years, as originally suggested. The DNA retrieved from the specimen originates from a male individual who carried mitochondrial DNA of the modern human type. The petrous part yielded more endogenous ancient DNA molecules than the squamous part of the same bone. Molecular dating of the Darra-i-Kur mitochondrial DNA sequence corroborates the radiocarbon date and suggests that the specimen is younger than previously thought. Taken together, the results consolidate the fact that the human bone is not associated with the Pleistocene-age deposits of Darra-i-Kur; instead it is intrusive, possibly re-deposited from upper levels dating to much later periods (Neolithic). Despite its Holocene age, the Darra-i-Kur specimen is, so far, the first and only ancient human from Afghanistan whose DNA has been sequenced. Copyright © 2017 Elsevier Ltd. All rights reserved.

Associated malformations among infants with anophthalmia and microphthalmia.

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Stoll, Claude; Dott, Beatrice; Alembik, Yves; Roth, Marie-Paule

2012-03-01

Infants with anophthalmia and microphthalmia frequently have other associated congenital anomalies. The reported frequency and types of associated malformations vary among different studies. The purpose of this investigation was to assess the frequency and types of associated malformations among infants with anophthalmia and microphthalmia in a geographically well defined population from 1979 to 2004 of 346,831 consecutive births. Of the 87 infants with anophthalmia and microphthalmia born during this period (prevalence at birth, 2.5 per 10,000), 90% had associated malformations. Infants with associated malformation were divided into recognizable conditions (22 infants [25%] with chromosomal and 15 infants [17%] with nonchromosomal conditions), and nonrecognizable conditions (41 infants [47%] with multiple malformations). Trisomies 13 and 18 were the most frequent chromosomal abnormalities. Amniotic bands sequence, CHARGE syndrome, Meckel-Gruber syndrome, and VACTERL association were most often present in recognizable nonchromosomal conditions. Malformations in the musculoskeletal, cardiovascular, and central nervous systems were the most common other anomalies in infants with multiple malformations and nonrecognizable conditions. The frequency of associated malformations in infants with anophthalmia or microphthalmia emphasizes the need for a thorough investigation of these infants. Routine screening for other malformations-especially musculoskeletal, cardiac, and central nervous system anomalies-may need to be considered in infants with anophthalmia or microphthalmia, and referral of these infants for genetics evaluation and counseling seems warranted. Copyright © 2012 Wiley Periodicals, Inc.

Rare malformation of glans penis: arteriovenous malformation.

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Akin, Y; Sarac, M; Yucel, S

2013-01-01

Pediatric glans penis malformations, especially arteriovenous malformations (AVM), are very rare. Herein, we report two rare cases. A 14-year-old boy attended our outpatient clinic with chief complaints of purple swelling and rapidly growing lesion on the glans penis. The lesion was excised surgically after physical and radiological evaluations. Pathology reported AVM and the patient is being followed up. The second case is a 2-year-old boy who was admitted with a big lesion involving glans penis and genital area that has been present since birth. In physical and radiological evaluations, lesion on the glans penis was pulsatile. Parents of the patient did not want any surgery and patient has been in follow-up. Diagnosis of the vascular lesions on glans penis is very easy by physical and radiological examinations today. Long-term follow-up is very important for AVM. Clinicians must make a careful effort to document new glans lesions in the pediatric population and decrease anxiety in the parents of affected children.

Radiation dose optimization in pediatric temporal bone computed tomography: influence of tube tension on image contrast and image quality

International Nuclear Information System (INIS)

Nauer, Claude Bertrand; Zubler, Christoph; Weisstanner, Christian; Stieger, Christof; Senn, Pascal; Arnold, Andreas

2012-01-01

The purpose of this experimental study was to investigate the effect of tube tension reduction on image contrast and image quality in pediatric temporal bone computed tomography (CT). Seven lamb heads with infant-equivalent sizes were scanned repeatedly, using four tube tensions from 140 to 80 kV while the CT-Dose Index (CTDI) was held constant. Scanning was repeated with four CTDI values from 30 to 3 mGy. Image contrast was calculated for the middle ear as the Hounsfield unit (HU) difference between bone and air and for the inner ear as the HU difference between bone and fluid. The influence of tube tension on high-contrast detail delineation was evaluated using a phantom. The subjective image quality of eight middle and inner ear structures was assessed using a 4-point scale (scores 1-2 = insufficient; scores 3-4 = sufficient). Middle and inner ear contrast showed a near linear increase with tube tension reduction (r = -0.94/-0.88) and was highest at 80 kV. Tube tension had no influence on spatial resolution. Subjective image quality analysis showed significantly better scoring at lower tube tensions, with highest image quality at 80 kV. However, image quality improvement was most relevant for low-dose scans. Image contrast in the temporal bone is significantly higher at low tube tensions, leading to a better subjective image quality. Highest contrast and best quality were found at 80 kV. This image quality improvement might be utilized to further reduce the radiation dose in pediatric low-dose CT protocols. (orig.)

Mucous retention cyst of temporal bone: a mimic of cholesteatoma on DW-MRI.

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Karandikar, Amit; Goh, Julian; Loke, Siu Cheng; Yeo, Seng Beng; Tan, Tiong Yong

2013-01-01

Non-EPI DW imaging is increasingly being used as a sensitive sequence in detecting cholesteatomas especially if CT findings are not confirmatory. Cholesteatoma appears as a hyperintense focus on DWI. We present two cases of mucous retention cysts in the mastoid temporal bone/middle ear cavity, which present as hyperintense on non-EPI DWI and potentially may mimic cholesteatomas. Differentiating between the two conditions is important, as surgery can be avoided in mucous retention cysts. We have also discussed ways to differentiate between these two conditions on MRI. To our knowledge, this entity is not reported previously. © 2013 Elsevier Inc. All rights reserved.

Diagnosis and surgical treatment of a Chiari I-like malformation in an African lion (Panthera leo).

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McCain, Stephanie; Souza, Marcy; Ramsay, Ed; Schumacher, Juergen; Hecht, Silke; Thomas, William

2008-09-01

A 13-mo-old intact male African lion (Panthera leo) presented with a 3-mo history of lethargy, ventral flexion of the neck, abnormal vocalization, and ataxia. Hemogram and serum biochemistries were within normal limits except for the presence of hypokalemia (2.7 mEq/L) and hypochloridemia (108 mEq/L). When no improvement was noted with oral potassium gluconate supplementation, a computed tomography scan of the brain and skull was performed, and no abnormalities were noted. However, magnetic resonance imaging detected occipital bone thickening, crowding of the caudal cranial fossa with cerebellar compression and herniation, and cervical syringohydromyelia, which was consistent with a Chiari I-like malformation. Foramen magnum decompression was performed to relieve the compression of the cerebellum. The animal recovered well with subsequent resolution of clinical signs. Hypovitaminosis A has been proposed previously as the underlying etiology for this malformation in lions with similar clinical presentations. This lion's serum and liver vitamin A concentrations were low (100 ng/ml and 25.31 microg/g, respectively) compared to concentrations reported for domestic carnivores and support hypovitaminosis A as the underlying cause of this animal's Chiari I-like malformation.

Pattern and outcome of gross congenital malformations at birth ...

African Journals Online (AJOL)

Background: Congenital malformation(s) do occur in newborns and are thought to be often responsible for a significant proportion of perinatal morbidity and mortality worldwide. Objective: This prospective study was designed to determine the pattern and outcome of congenital malformation(s) among newborn deliveries ...

Importance of bony analysis for interpreting ear CT scans: part three; ORL - tomodensitometrie de l'oreille. Interet de l'analyse osseuse dans l'interpretation des scanners de l'oreille (troisieme partie)

Energy Technology Data Exchange (ETDEWEB)

Serhal, M.; Dordea, M.; Cymbalista, M. [Hopital de Montfermeil, Service de Radiologie, 93 - Montfermeil (France); Halimi, P. [Hopital Europeen Georges-Pompidou, Service de Radiologie, 75 - Paris (France); Iffenecker, C. [Clinique Radiologique, 62 - Boulogne sur Mer (France); Bensimon, J.L

2003-02-01

The accurate description of bony changes in ear CT scans has a great diagnostic and therapeutic impact. The third part shows the way to analyze bone remodeling when CT scan is performed for tumors in the vicinity of the temporal bone, for intra temporal lesions of the facial nerve and for external auditory canal malformations. It demonstrates how bony analysis should be included in postoperative report of ear CT scan. The importance of bony signs in tumors and pseudo tumors of the inner ear are outlined. (authors)

Neonatal Death Dwarfism in a Girl with Distinctive Bone Dysplasia Compatible with Grebe Chondrodysplasia: Analysis by CT Scan-based Phenotype.

Science.gov (United States)

Al Kaissi, Ali; Chehida, Farid Ben; Ganger, Rudolf; Grill, Franz

2014-01-01

We report on a female fetus noted to have severe malformative type of skeletal dysplasia on ultrasonography done at 35 weeks gestation. The girl died shortly after birth. Clinical examination showed a fetus with severe dwarfism, extensive long and short bones, and bone deficiencies associated with multiple dislocations. Computed tomography (CT) scan-based phenotype showed a complex constellation of malformations consistent with the diagnosis of Grebe syndrome. Parents being first cousins (consanguineous marriage) strongly suggests autosomal recessive pattern of inheritance. To our knowledge, this is the first report of neonatal death dwarfism of Grebe syndrome analyzed by CT scan-based phenotype.

The shortened cochlea: its classification and histopathologic features.

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Zheng, Yiqing; Schachern, Patricia A; Cureoglu, Sebahattin; Mutlu, Cemil; Dijalilian, Hamid; Paparella, Michael M

2002-03-15

The term 'Mondini dysplasia' has been used to describe virtually any congenital abnormality of the osseous labyrinth resulting in confusion and seemingly contradictory observations and conclusions about this type of deformity. The purpose of this study is to histopathologically classify and describe temporal bones whose cochleas have less than 2.5 turns. Of the 1800 temporal bones in our collection, 21 from 12 cases were found to have cochleas with less than 2.5 cochlear turns. Ages ranged from stillborn to 50 years. Temporal bones were harvested at autopsy, processed and embedded in celloidin. Sections were cut at a thickness of 20 microm and every 10th section stained with hematoxylin-eosin and examined using light microscopy. The number of turns, length of cochlea, integrity of cochlear base, length of modiolus, abnormalities of the semicircular canals and vestibule, enlargement of the vestibular aqueduct and middle ears were documented. Twenty-one temporal bones from age-matched patients without cochlear deformities were used as controls for modiolar length measurements. Malformation of the shortened cochlea was histopathologically classified into three groups as follows: (1) Common cavity, cochlear dysplasia (one ear)--severe dysplasia of the cochlea without a complete basal turn; (2) Mondini dysplasia (11 ears)--1.5 cochlear turns, a complete basal turn, an incomplete or absent interscalar septum and a complete bone at the base of the modiolus; and (3) Mondini-like dysplasia type A (five ears)--2 turns to the cochlea including a complete basal turn and complete bone at the base of the modiolus; and type B (four ears)--1.5-2 turns to the cochlea, hypoplasia of or a missing bone at the base of the modiolus (either with or without a communication between the internal auditory canal and the cochlea) and a complete basal turn. The range of congenital malformations in short cochlea is highly variable. Fundamental to the accurate evaluation of a labyrinthine anomaly

Facial nerve paralysis associated with temporal bone masses.

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Nishijima, Hironobu; Kondo, Kenji; Kagoya, Ryoji; Iwamura, Hitoshi; Yasuhara, Kazuo; Yamasoba, Tatsuya

2017-10-01

To investigate the clinical and electrophysiological features of facial nerve paralysis (FNP) due to benign temporal bone masses (TBMs) and elucidate its differences as compared with Bell's palsy. FNP assessed by the House-Brackmann (HB) grading system and by electroneurography (ENoG) were compared retrospectively. We reviewed 914 patient records and identified 31 patients with FNP due to benign TBMs. Moderate FNP (HB Grades II-IV) was dominant for facial nerve schwannoma (FNS) (n=15), whereas severe FNP (Grades V and VI) was dominant for cholesteatomas (n=8) and hemangiomas (n=3). The average ENoG value was 19.8% for FNS, 15.6% for cholesteatoma, and 0% for hemangioma. Analysis of the correlation between HB grade and ENoG value for FNP due to TBMs and Bell's palsy revealed that given the same ENoG value, the corresponding HB grade was better for FNS, followed by cholesteatoma, and worst in Bell's palsy. Facial nerve damage caused by benign TBMs could depend on the underlying pathology. Facial movement and ENoG values did not correlate when comparing TBMs and Bell's palsy. When the HB grade is found to be unexpectedly better than the ENoG value, TBMs should be included in the differential diagnosis. Copyright © 2017 Elsevier B.V. All rights reserved.

Study of placenta of children born with congenital malformations.

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Stoll, Claude; Alembik, Yves; Dott, Béatrice; Roth, Marie-Paule

2003-01-01

The malformations in this study were observed in a series of 279,642 consecutive births of known outcome registered in our Registry of congenital anomalies. For each case, more than 50 factors included in the registration forms were studied. One of the factors studied was the placenta. For each malformed child, a control was chosen. Cases with maternal known factors impairing placenta function, i.e. vasculopathy and diabetes, were excluded. In each category of malformations studied, the malformed children were divided into isolated and non-isolated (multiple malformed) cases. The weight of placenta of isolated cases was not lower than the weight of placenta of the controls. In contrast, the weight of placenta of the cases with non-isolated malformations was lower than the weight of placenta of the controls and of the isolated cases, for all categories of malformations but gastroschisis and omphalocele. The mean weights at birth of the cases with multiple malformations were also lower than those of the controls. The human placenta discounts a principal functional part, the maternal blood in the intervillous space. Congenital malformations may interact with this function.

Brain Malformations

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Most brain malformations begin long before a baby is born. Something damages the developing nervous system or causes it ... medicines, infections, or radiation during pregnancy interferes with brain development. Parts of the brain may be missing, ...

Initial results of a new generation dual source CT system using only an in-plane comb filter for ultra-high resolution temporal bone imaging

International Nuclear Information System (INIS)

Meyer, Mathias; Haubenreisser, Holger; Schoenberg, Stefan O.; Henzler, Thomas; Raupach, Rainer; Schmidt, Bernhard; Leidecker, Christianne; Allmendinger, Thomas; Flohr, Thomas; Lietzmann, Florian; Schad, Lothar R.

2015-01-01

To prospectively evaluate radiation dose and image quality of a third generation dual-source CT (DSCT) without z-axis filter behind the patient for temporal bone CT. Forty-five patients were either examined on a first, second, or third generation DSCT in an ultra-high-resolution (UHR) temporal bone-imaging mode. On the third generation DSCT system, the tighter focal spot of 0.2 mm 2 removesthe necessity for an additional z-axis-filter, leading to an improved z-axis radiation dose efficiency. Images of 0.4 mm were reconstructed using standard filtered-back-projection or iterative reconstruction (IR) technique for previous generations of DSCT and a novel IR algorithm for the third generation DSCT. Radiation dose and image quality were compared between the three DSCT systems. The statistically significantly highest subjective and objective image quality was evaluated for the third generation DSCT when compared to the first or second generation DSCT systems (all p < 0.05). Total effective dose was 63 %/39 % lower for the third generation examination as compared to the first and second generation DSCT. Temporal bone imaging without z-axis-UHR-filter and a novel third generation IR algorithm allows for significantly higher image quality while lowering effective dose when compared to the first two generations of DSCTs. (orig.)

Multislice spiral computed tomography imaging in congenital inner ear malformations.

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Ma, Hui; Han, Ping; Liang, Bo; Tian, Zhi-liang; Lei, Zi-qiao; Kong, Wei-jia; Feng, Gan-sheng

2008-01-01

The purpose of this study is to evaluate the usefulness of multislice spiral computed tomography (CT) in the diagnosis of congenital inner ear malformations. Forty-four patients with sensorineural hearing loss were examined on a Somatom Sensation 16 (Siemens) CT scanner. The 3-dimensional reconstructions and multiplanar reformation (MPR) were performed using the volume-rendering technique (VRT) on the workstation. Of the 44 patients examined for this study, 25 patients were found to be normal and 19 patients (36 ears) were diagnosed with congenital inner ear malformations. Of the malformations, the axial, MPR, and VRT images can all display the site and degree in 33 of the ears. Volume-rendering technique images were superior to the axial images in displaying the malformations in 3 ears with small lateral semicircular canal malformations. The common malformations were Michel deformity (1 ear), common cavity deformity (3 ears), incomplete partition I (3 ears), incomplete partition II (Mondini deformity) (5 ears), vestibular and semicircular canal malformations (14 ears), enlarged vestibular aqueduct (16 ears, 6 of which had other malformations), and internal auditory canal malformation (8 ears, all accompanied by other malformations). Multislice spiral CT allows a comprehensively assessment of various congenital inner ear malformations through high-quality MPR and VRT reconstructions. Volume-rendering technique images can display the site and degree of the malformation 3-dimensionally and intuitionisticly. This is very useful to the cochlear implantation.

Spatial and temporal changes of subchondral bone proceed to articular cartilage degeneration in rats subjected to knee immobilization.

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Xu, Lei; Li, Zhe; Lei, Lei; Zhou, Yue-Zhu; Deng, Song-Yun; He, Yong-Bin; Ni, Guo-Xin

2016-03-01

This study was aimed to investigate the spatial and temporal changes of subchondral bone and its overlying articular cartilage in rats following knee immobilization. A total of 36 male Wistar rats (11-13 months old) were assigned randomly and evenly into 3 groups. For each group, knee joints in 6 rats were immobilized unilaterally for 1, 4, or 8 weeks, respectively, while the remaining rats were allowed free activity and served as external control groups. For each animal, femurs at both sides were dissected after sacrificed. The distal part of femur was examined by micro-CT. Subsequently, femoral condyles were collected for further histological observation and analysis. For articular cartilage, significant changes were observed only at 4 and 8 weeks of immobilization. The thickness of articular cartilage and chondrocytes numbers decreased with time. However, significant changes in subchondral bone were defined by micro-CT following immobilization in a time-dependent manner. Immobilization led to a thinner and more porous subchondral bone plate, as well as a reduction in trabecular thickness and separation with a more rod-like architecture. Changes in subchondral bone occurred earlier than in articular cartilage. More importantly, immobilization-induced changes in subchondral bone may contribute, at least partially, to changes in its overlying articular cartilage. © 2016 Wiley Periodicals, Inc.

Radiation dose optimization in pediatric temporal bone computed tomography: influence of tube tension on image contrast and image quality.

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Nauer, Claude Bertrand; Zubler, Christoph; Weisstanner, Christian; Stieger, Christof; Senn, Pascal; Arnold, Andreas

2012-03-01

The purpose of this experimental study was to investigate the effect of tube tension reduction on image contrast and image quality in pediatric temporal bone computed tomography (CT). Seven lamb heads with infant-equivalent sizes were scanned repeatedly, using four tube tensions from 140 to 80 kV while the CT-Dose Index (CTDI) was held constant. Scanning was repeated with four CTDI values from 30 to 3 mGy. Image contrast was calculated for the middle ear as the Hounsfield unit (HU) difference between bone and air and for the inner ear as the HU difference between bone and fluid. The influence of tube tension on high-contrast detail delineation was evaluated using a phantom. The subjective image quality of eight middle and inner ear structures was assessed using a 4-point scale (scores 1-2 = insufficient; scores 3-4 = sufficient). Middle and inner ear contrast showed a near linear increase with tube tension reduction (r = -0.94/-0.88) and was highest at 80 kV. Tube tension had no influence on spatial resolution. Subjective image quality analysis showed significantly better scoring at lower tube tensions, with highest image quality at 80 kV. However, image quality improvement was most relevant for low-dose scans. Image contrast in the temporal bone is significantly higher at low tube tensions, leading to a better subjective image quality. Highest contrast and best quality were found at 80 kV. This image quality improvement might be utilized to further reduce the radiation dose in pediatric low-dose CT protocols.

Syringomyelia associated with Chiari malformation in children

International Nuclear Information System (INIS)

Sakamoto, Hiroaki; Kitano, Shouhei; Nishikawa, Misao; Yasui, Toshihiro; Fujitani, Ken; Hakuba, Akira; Nakanishi, Naruhiko

1997-01-01

Among 28 patients with myelomeningocele (MMC group), the myelomeningocele in all patients was repaired shortly after birth, and a shunt was implanted for the associated hydrocephalus in 18 patients. MRI of the group of 28 indicated 20 were afflicted with Chiari II malformation, and the remaining 8 by Chiari I malformation. Among 8 patients lacking myelomeningocele (non-MMC group), seven demonstrated a large syrinx at the cervical and cervico-thoracic level; only one had a syrinx extending from the cervical level down to the lumbar level. None of these patients had hydrocephalus. Surgical decompression to improve cerebrospinal fluid (CSF) flow at the major cistern improved neurological signs in 7 patients. MRI indicated 4 patients were afflicted with Chiari I malformation, and the remaining 4 with Chiari II malformation. In the MMC group, the initial development of the syrinx at the lumbar level may be the result of a combination of occlusion of the caudal end of the central canal brought about by repair of the myelomeningocele and CSF flow into the hydromyelic cavity via the patent proximal portion of the central canal. In the non-MMC group, the syringomyelia may be considered an early onset type of syringomyelia associated with adult type Chiari malformation because the location of the syrinx was quite similar to that found in adult type Chiari malformation, and decompressive surgery was quite effective. In the non-MMC group, turbulence of the CSF now at the major cistern caused by the herniated cerebellum plays an important role in the enlargement of the syringomyelia. To offer greater appropriate management of pediatric Chiari malformation accompanied by syringomyelia, the malformation should be classified not by degree of the herniated brain tissue but by its association with neural tube defect (myelomeningocele). (K.H.)

Chiari I malformation associated with turner syndrome

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Kamble Jayaprakash Harsha

2017-01-01

Full Text Available Turner syndrome (TS is a rare genetic disease due to the absence of one X chromosome. Patients with TS have more subtle neurological/neuropsychiatric problems, while headache is an uncommon clinical presentation which needs attention. We report a 12-year-old child presenting with typical cough headache. Her magnetic resonance imaging revealed Chiari I malformation associated with TS. To the best of our knowledge, Chiari I malformation associated with TS is not described in literature. We report the first case of TS associated with Chiari I malformation. Interestingly, Chiari I malformation is also associated with Noonan's syndrome, which is a close morphological mimicker of TS, raising the possibility of sharing similar pathogenesis in both conditions.

Human malformations induced by environmental noxae

International Nuclear Information System (INIS)

Hecker, W.C.; Angerpointner, T.A.

1980-01-01

The paper reviews congenital malformations in humans and presents possible causes. 60% of all malformations are a result of environmental and other factors; i.e. not hereditary or caused by a disease of the mother. The teratogenic effects of ionizing radiation, drugs, alcohol, polyvinyl chloride and trichlorophenol are discussed as well as the effect of the mother's working in certain fields, e.g. clinical laboratories or printing offices; in the latter case the teratogenic noxae are still unknown. Efficient research requires centralized storage of all data on children born with malformations and on the mother's health situation during pregnancy, and the legislator is asked to do so while observing the law on data protection. Foundation of a German Institute of Teratology is recommended. In order to intensify research, it is suggested to set up groups or departments for research on malformations in some major paediatric hospitals. (MG) [de

Fever in pregnancy and the risk of congenital malformations

DEFF Research Database (Denmark)

Sass, L.; Urhoj, S. K.; Kjærgaard, J.

2017-01-01

fetal malformations or death. Fever during pregnancy, especially during embryogenesis, has also been associated with congenital malformations in human offspring. The purpose of this large cohort study of clinically recognized pregnancies was to investigate whether fever during first trimester...... was associated with an increased risk of congenital malformations in the offspring. Methods: The Danish National Birth Cohort is a population-based cohort of 100,418 pregnant women and their offspring recruited in 1996 to 2002. Information on fever during pregnancy was collected prospectively by means of two....... Congenital malformations within the first three and a half years of life were categorized according to EUROCAT's classification criteria. Logistic regression models were used to estimate the associations between fever in first trimester and overall congenital malformations and congenital malformations...

Genetics Home Reference: megalencephaly-capillary malformation syndrome

Science.gov (United States)

... Additional NIH Resources (1 link) National Institute of Neurological Disorders and Stroke: Megalencephaly Educational Resources (5 links) Boston Children's Hospital: Capillary Malformation Cincinnati Children's Hospital: Capillary Malformations ...

Arteriovenous Malformations

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Arteriovenous malformations (AVMs) are defects in your vascular system. The vascular system includes arteries, veins, and capillaries. Arteries carry blood away from the heart to other organs; veins carry blood back to the heart. Capillaries connect the arteries and veins. An ...

The 'temporal effect' in hominids: Reinvestigating the nature of support for a chimp-human clade in bone morphology.

Science.gov (United States)

Pearson, Alannah; Groves, Colin; Cardini, Andrea

2015-11-01

In 2004, an analysis by Lockwood and colleagues of hard-tissue morphology, using geometric morphometrics on the temporal bone, succeeded in recovering the correct phylogeny of living hominids without resorting to potentially problematic methods for transforming continuous shape variables into meristic characters. That work has increased hope that by using modern analytical methods and phylogenetically informative anatomical data we might one day be able to accurately infer the relationships of hominins, including the closest extinct relatives of modern humans. In the present study, using 3D virtually generated models of the hominid temporal bone and a larger suite of geometric morphometric and comparative techniques, we have re-examined the evidence for a Pan-Homo clade. Despite differences in samples, as well as the type of raw data, the effect of measurement error (and especially landmark digitization by a different operator), but also a broader perspective brought in by our diverse set of approaches, our reanalysis largely supports Lockwood and colleagues' original results. However, by focusing not only mainly on shape (as in the original 2004 analysis) but also on size and 'size-corrected' (non-allometric) shape, we demonstrate that the strong phylogenetic signal in the temporal bone is largely related to similarities in size. Thus, with this study, we are not suggesting the use of a single 'character', such as size, for phylogenetic inference, but we do challenge the common view that shape, with its highly complex and multivariate nature, is necessarily more phylogenetically informative than size and that actually size and size-related shape variation (i.e., allometry) confound phylogenetic inference based on morphology. This perspective may in fact be less generalizable than often believed. Thus, while we confirm the original findings by Lockwood et al., we provide a deep reinterpretation of their nature and potential implications for hominid phylogenetics

Spontaneous ileal perforation complicating low anorectal malformation

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TiJesuni Olatunji

2015-01-01

Full Text Available Anorectal malformation is a common anomaly in neonates. Although colorectal perforations have been reported as a complication, ileal perforation is rarely encountered. This is a report of a 2-day-old boy presenting with a low anorectal malformation, complicated with ileal perforation, necessitating laparotomy and ileal repair. Anoplasty was done for the low anomaly. Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication.

Congenital malformations of the skull and meninges.

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Kanev, Paul M

2007-02-01

The surgery and management of children who have congenital malformations of the skull and meninges require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. The high definition of three-dimensional CT and MRI allows precise surgery planning of reconstruction and management of associated malformations. The reconstruction of meningoencephaloceles and craniosynostosis are challenging procedures that transform the child's appearance. The embryology, clinical presentation, and surgical management of these malformations are reviewed.

Neonatal Death Dwarfism in a Girl with Distinctive Bone Dysplasia Compatible with Grebe Chondrodysplasia: Analysis by CT Scan-based Phenotype

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Ali Al Kaissi

2014-01-01

Full Text Available We report on a female fetus noted to have severe malformative type of skeletal dysplasia on ultrasonography done at 35 weeks gestation. The girl died shortly after birth. Clinical examination showed a fetus with severe dwarfism, extensive long and short bones, and bone deficiencies associated with multiple dislocations. Computed tomography (CT scan-based phenotype showed a complex constellation of malformations consistent with the diagnosis of Grebe syndrome. Parents being first cousins (consanguineous marriage strongly suggests autosomal recessive pattern of inheritance. To our knowledge, this is the first report of neonatal death dwarfism of Grebe syndrome analyzed by CT scan-based phenotype.

Facial vascular malformations in children

International Nuclear Information System (INIS)

Brunelle, F.O.; Lallemand, D.; Chaumont, P.; Teillac, D.; Manach, Y.

1988-01-01

The authors present their experience with conventional and digital angiography of vascular malformations of the head and neck in children. 22 hemangioendotheliomas, 8 venous angiomas, and 3 arteriovenous fistula were studied. 22 patients were embolised. DSA offers many advantages during the diagnostic as well as during the therapeutic phase of angiography. Embolization appears to have a major role in treatment of such vascular malformations. (orig.)

Sincipital Encephaloceles: A Study of Associated Brain Malformations

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Shashidhar Vedavyas Achar

2016-01-01

Full Text Available Objective: The aim of this study was to evaluate the associated intracranial malformations in patients with sincipital encephaloceles. Materials and Methods: A hospital-based cross-sectional study was conducted over 8 years from June 2007 to May 2015 on 28 patients. The patients were evaluated by either computed tomography or magnetic resonance imaging whichever was feasible. Encephaloceles were described with respect to their types, contents, and extensions. A note was made on the associated malformations with sincipital encephaloceles. Results: Fifty percent of the patients presented before the age of 3 years and both the sexes were affected equally. Nasofrontal encephalocele was the most common type seen in 13 patients (46.4%, and corpus callosal agenesis (12 patients was the most common associated malformation. Other malformations noted were arachnoid cyst (10 patients, hydrocephalus (7 patients, and agyria-pachygyria complex (2 patients. Conclusion: Capital Brain malformations are frequently encountered in children with sincipital encephaloceles. Detail radiological evaluation is necessary to plan treatment and also to prognosticate such rare malformations.

Prognostic factors of congenital diaphragmatic hernia accompanied by cardiovascular malformation.

Science.gov (United States)

Takahashi, Shigehiro; Sago, Haruhiko; Kanamori, Yutaka; Hayakawa, Masahiro; Okuyama, Hiroomi; Inamura, Noboru; Fujino, Yuji; Usui, Noriaki; Taguchi, Tomoaki

2013-08-01

Congenital diaphragmatic hernia is associated with cardiovascular malformation. Many prognostic factors have been identified for isolated congenital diaphragmatic hernia; however, reports of concurrent congenital diaphragmatic hernia and cardiovascular malformation in infants are limited. This study evaluated congenital diaphragmatic hernia associated with cardiovascular malformation in infants. Factors associated with prognosis for patients were also identified. This retrospective cohort study was based on a Japanese survey of congenital diaphragmatic hernia patients between 2006 and 2010. Frequency and outcome of cardiovascular malformation among infants with congenital diaphragmatic hernia were examined. Severity of congenital diaphragmatic hernia and cardiovascular malformation were compared as predictors of mortality and morbidity. Cardiovascular malformation was identified in 76 (12.3%) of 614 infants with congenital diaphragmatic hernia. Mild cardiovascular malformation was detected in 19 (33.9%) and severe cardiovascular malformation in 37 (66.1%). Their overall survival rate at discharge was 46.4%, and the survival rate without morbidity was 23.2%. Mortality and morbidity at discharge were more strongly associated with severity of cardiovascular malformation (adjusted OR 7.69, 95%CI 1.96-30.27; adjusted OR 7.93, 95%CI 1.76-35.79, respectively) than with severity of congenital diaphragmatic hernia. The prognosis for infants with both congenital diaphragmatic hernia and cardiovascular malformation remains poor. Severity of cardiovascular malformation is a more important predictive factor for mortality and morbidity than severity of congenital diaphragmatic hernia. © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.

TEMPORAL MODELING OF DNA DEGRADATION IN BONE REMAINS

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Andrei Stefan

2012-06-01

Full Text Available The aim of this study is to follow the changes that occur, in time, at DNA level and to establish an efficient and reliable protocol for ancestral DNA extraction from bones found in archaeological sites. To test whether the protocol is efficient and capable of yielding good quality DNA, extraction was first performed on fresh bones. The material consists of fresh pig (Sus scrofa and cow (Bos taurus bones that were grounded by using a drill operating at low speed. The bone powder was then incubated in lysis buffer in the presence of proteinase K. DNA isolation and purification were done by using the phenol:chloroform protocol and DNA was precipitated with absolute ethanol stored at -20oC. The extractions were carried out once every month for a total of four extractions

Angiographic findings of congenital vascular malformation in soft tissue

International Nuclear Information System (INIS)

Choi, Dae Seob; Park, Jae Hyung; Han, Joon Koo; Chung, Jin Wook; Moon, Woo Kyung; Han, Man Chung

1994-01-01

We evaluated the clinical, plain radiographic, and angiographic findings of congenital vascular malformation of the soft tissue. Retrospective analysis was performed in 36 patients. Pathological diagnosis was done in 25 patients by surgery and the others were clinically and angiographically diagnosed. On the basis of angiographic findings, we classified the lesions to three groups as arteriovenous malformation (AVM), hemangioma, and venous malformation. In pathologically proven 25 cases, we compared the angiographic diagnosis with the pathologic diagnosis. By angiographic classification, AVM was 13 cases, hemangioma 16 cases, and venous malformation 7 cases. The locations of the lesions were upper extremities in 14 cases, lower extremities in 20 cases, both extremities in 1 case, and back in 1 case. Clinical findings were bruit and thrill in 13 cases(12 AVMs,1 hemangioma) and varicosities in 16 cases(11 AVMs, 3 hemangiomas and 2 venous malformations). The varicosities in AVM were pulsating nature, but not in hemangioma and venous malformation. The concordance rate of the angiographic and pathologic diagnosis was 100%(6/6) in AVM, 71%(10/14) in hemangioma and 60% (3/5) in venous malformation. We think that angiography is an essential study for accurate diagnosis and appropriate treatment of congenital vascular malformation

MR imaging of anorectal malformations and associated anomalies

International Nuclear Information System (INIS)

Nievelstein, R.A.J.; Valk, J.; Vos, A.

1998-01-01

Congenital anorectal malformations are found in many forms, and are frequently associated with other anomalies, especially of the spinal cord, spine, and urogenital system. Decisions concerning initial management of children with anorectal malformations can be made only after accurate determination of (a) the level and type of malformation, (b) the type of fistula, (c) the developmental state of the sphincter muscle complex, and (d) the presence of associated anomalies. Magnetic resonance imaging has proven to be the only modality to answer all these crucial questions, and has contributed to a better insight in the morphology and pathogenesis of such complex congenital malformations. (orig.)

Endometriosis and uterine malformations: infertility may increase severity of endometriosis.

Science.gov (United States)

Boujenah, Jeremy; Salakos, Eleonora; Pinto, Mélodie; Shore, Joanna; Sifer, Christophe; Poncelet, Christophe; Bricou, Alexandre

2017-06-01

The aim of our study was to compare the stage and severity of endometriosis in fertile and infertile women with congenital uterine malformations. We performed an observational study from September 2007 to December 2015 in a tertiary care university hospital and assisted reproductive technology center. A total of 52 patients with surgically proven uterine malformations were included. We compared 41 infertile patients with uterine malformations with 11 fertile patients with uterine malformation. The main outcome was the stage, score and type of endometriosis in regard to infertility and class of uterine malformation. The rate of endometriosis did not differ between the two groups (43.9 vs. 36.4%). The mean revised American Fertility Society score was higher in infertile patients with uterine malformations (19.02 vs. 6, p endometriosis (43.9 vs. 37.5%). Endometrioma and deep infiltrating endometriosis were associated with uterine malformations in infertile women, respectively 14.6 and 0%. No difference in the characteristics of endometriosis was found regarding the class of malformation. The association of uterine malformations and infertility may increase the severity of endometriosis and raise the issue of their diagnosis and management. © 2016 Nordic Federation of Societies of Obstetrics and Gynecology.

Transcatheter arterial embolization for congenital renal arteriovenous malformation

International Nuclear Information System (INIS)

Zhou Jun; Hu Tingyang; Yuan Jianhua; Yu Wenqiang

2008-01-01

Objective: To evaluate the effectiveness of transcatheter arterial embolization for congenital renal arteriovenous malformation. Methods: Seven cases of congenital renal arteriovenous malformation causing gross hematuria were retrospectively studied. All of 7 cases were demonstrated by means of angiography and then the catheter was placed superselectively into the involved arterial end of the malformation undertaking embolization with gelfoam, dehydrated ethanol, coils, etc. Results: All the malformations of the 7 cases were successfully embolized with stoppage of gross hematuria within 24 hours. No serious complications occurred except lumbago, fever, gastrointestinal reaction for one week. There was no recurrence of haematuria and the renal function was also normal in all cases during the follow-up for 36 to 98 months. Conclusions: Transcatheter renal arterial angiography and embolization are the important and effective management for the diagnosis and treatment of congenital renal arteriovenous malformation. (authors)

Nerve canals at the fundus of the internal auditory canal on high-resolution temporal bone CT

International Nuclear Information System (INIS)

Ji, Yoon Ha; Youn, Eun Kyung; Kim, Seung Chul

2001-01-01

To identify and evaluate the normal anatomy of nerve canals in the fundus of the internal auditory canal which can be visualized on high-resolution temporal bone CT. We retrospectively reviewed high-resolution (1 mm thickness and interval contiguous scan) temporal bone CT images of 253 ears in 150 patients who had not suffered trauma or undergone surgery. Those with a history of uncomplicated inflammatory disease were included, but those with symptoms of vertigo, sensorineural hearing loss, or facial nerve palsy were excluded. Three radiologists determined the detectability and location of canals for the labyrinthine segment of the facial, superior vestibular and cochlear nerve, and the saccular branch and posterior ampullary nerve of the inferior vestibular nerve. Five bony canals in the fundus of the internal auditory canal were identified as nerve canals. Four canals were identified on axial CT images in 100% of cases; the so-called singular canal was identified in only 68%. On coronal CT images, canals for the labyrinthine segment of the facial and superior vestibular nerve were seen in 100% of cases, but those for the cochlear nerve, the saccular branch of the inferior vestibular nerve, and the singular canal were seen in 90.1%, 87.4% and 78% of cases, respectiveIy. In all detectable cases, the canal for the labyrinthine segment of the facial nerve was revealed as one which traversed anterolateralIy, from the anterosuperior portion of the fundus of the internal auditory canal. The canal for the cochlear nerve was located just below that for the labyrinthine segment of the facial nerve, while that canal for the superior vestibular nerve was seen at the posterior aspect of these two canals. The canal for the saccular branch of the inferior vestibular nerve was located just below the canal for the superior vestibular nerve, and that for the posterior ampullary nerve, the so-called singular canal, ran laterally or posteolateralIy from the posteroinferior aspect of

Nerve canals at the fundus of the internal auditory canal on high-resolution temporal bone CT

Energy Technology Data Exchange (ETDEWEB)

Ji, Yoon Ha; Youn, Eun Kyung; Kim, Seung Chul [Sungkyunkwan Univ., School of Medicine, Seoul (Korea, Republic of)

2001-12-01

To identify and evaluate the normal anatomy of nerve canals in the fundus of the internal auditory canal which can be visualized on high-resolution temporal bone CT. We retrospectively reviewed high-resolution (1 mm thickness and interval contiguous scan) temporal bone CT images of 253 ears in 150 patients who had not suffered trauma or undergone surgery. Those with a history of uncomplicated inflammatory disease were included, but those with symptoms of vertigo, sensorineural hearing loss, or facial nerve palsy were excluded. Three radiologists determined the detectability and location of canals for the labyrinthine segment of the facial, superior vestibular and cochlear nerve, and the saccular branch and posterior ampullary nerve of the inferior vestibular nerve. Five bony canals in the fundus of the internal auditory canal were identified as nerve canals. Four canals were identified on axial CT images in 100% of cases; the so-called singular canal was identified in only 68%. On coronal CT images, canals for the labyrinthine segment of the facial and superior vestibular nerve were seen in 100% of cases, but those for the cochlear nerve, the saccular branch of the inferior vestibular nerve, and the singular canal were seen in 90.1%, 87.4% and 78% of cases, respectiveIy. In all detectable cases, the canal for the labyrinthine segment of the facial nerve was revealed as one which traversed anterolateralIy, from the anterosuperior portion of the fundus of the internal auditory canal. The canal for the cochlear nerve was located just below that for the labyrinthine segment of the facial nerve, while that canal for the superior vestibular nerve was seen at the posterior aspect of these two canals. The canal for the saccular branch of the inferior vestibular nerve was located just below the canal for the superior vestibular nerve, and that for the posterior ampullary nerve, the so-called singular canal, ran laterally or posteolateralIy from the posteroinferior aspect of

Multiple Bony Injuries on Bone Scan in a Case of Unsuspected Child Abuse

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Ya-Wen Chuang

2017-01-01

Full Text Available This case is described of an eleven-month-old infant with lower limbs swelling and the left elbow skeletal malformation following a fall. The radionuclide bone scan was performed to exclude bone infection or congenital skeletal anomaly. The images unexpectedly showed multiple increased radioactive foci throughout the whole body. It was a strong probability of child abuse. All lesions are readily apparent on the following plain film radiographs and MRI.

Maternal smoking in pregnancy and risk for congenital malformations

DEFF Research Database (Denmark)

Leite, Mimmi; Albieri, Vanna; Kjaer, Susanne K.

2014-01-01

and registered in the Danish Medical Birth Register containing detailed information on smoking during pregnancy and congenital malformations. METHODS: Associations [odds ratios (OR) with 95% CI] between maternal smoking and risk for various groups of congenital malformations, investigated using the generalized.......e. when two or more malformations are diagnosed in a child) (odds ratio 1.06, 95% confidence interval 1.01-1.10) and various main groups of congenital malformations including the cardiovascular system (odds ratio 1.13, 95% confidence interval 1.07-1.19), the respiratory system (odds ratio 1.25, 95......% confidence interval 1.11-1.41), the digestive system (odds ratio 1.15, 95% confidence interval 1.07-1.24) and oral clefts (odds ratio 1.29, 95% confidence interval 1.14-1.46), as well as for some specific congenital malformations including cardiac septal defects, malformations of the pulmonary and tricuspid...

[The supportive outcome of periodontal non-surgical therapy to severe chronic periodontitis accompanied with malformed lingual groove in lateral incisor for 6 years: a case report].

Science.gov (United States)

Li, Zheng; Kang, Jun

2011-06-01

To track the initiating and developing process of one case diagnosed as chronic periodontitis accompanied with malformed lingual groove in maxillary lateral incisor and report the long-term prognosis to the periodontal conservative and supportive therapy. The patient was diagnosed with mild chronic periodontitis 6 years ago and accepted routine periodontal scaling and root planning (SRP) plus supportive periodontal therapy (SPT) one time. Two years later the periodontal condition deteriorated by deep pockets in molars and severe bone destruction around the maxillary lateral incisor with malformed lingual groove. After SRP in sites which pocket depth more than 4mm plus root canal therapy and lingual groove plasty of maxillary right lateral incisor, the SPT regularly proceeded at 3rd, 6th and 12th month. At present the whole periodontal tissue was healthy, the bone lesion around maxillary lateral incisor recovered well, the tooth had no mobile, the cosmetic effect andtooth function was in good state, and the patient was very satisfied.

Update on neuroimaging phenotypes of mid-hindbrain malformations

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Jissendi-Tchofo, Patrice [University Hospital of Lille (CHRU), Department of Neuroradiology, MRI 3T Research, Plateforme Imagerie du vivant, IMPRT-IFR 114, Lille-Cedex (France); CHU Saint-Pierre, Radiology Department, Pediatric Neuroradiology Section, Brussels (Belgium); Severino, Mariasavina [Istituto Giannina Gaslini, Neuroradiology Unit, Genoa (Italy); Nguema-Edzang, Beatrice; Toure, Cisse; Soto Ares, Gustavo [University Hospital of Lille (CHRU), Department of Neuroradiology, MRI 3T Research, Plateforme Imagerie du vivant, IMPRT-IFR 114, Lille-Cedex (France); Barkovich, Anthony James [University of California, Neuroradiology Section, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States)

2014-10-23

Neuroimaging techniques including structural magnetic resonance imaging (MRI) and functional positron emission tomography (PET) are useful in categorizing various midbrain-hindbrain (MHB) malformations, both in allowing diagnosis and in helping to understand the developmental processes that were disturbed. Brain imaging phenotypes of numerous malformations are characteristic features that help in guiding the genetic testing in case of direct neuroimaging-genotype correlation or, at least, to differentiate among MHB malformations entities. The present review aims to provide the reader with an update of the use of neuroimaging applications in the fine analysis of MHB malformations, using a comprehensive, recently proposed developmental and genetic classification. We have performed an extensive systematic review of the literature, from the embryology main steps of MHB development through the malformations entities, with regard to their molecular and genetic basis, conventional MRI features, and other neuroimaging characteristics. We discuss disorders in which imaging features are distinctive and how these features reflect the structural and functional impairment of the brain. Recognition of specific MRI phenotypes, including advanced imaging features, is useful to recognize the MHB malformation entities, to suggest genetic investigations, and, eventually, to monitor the disease outcome after supportive therapies. (orig.)

Congenital malformations of the external and middle ear

International Nuclear Information System (INIS)

Koesling, S.; Omenzetter, M.; Bartel-Friedrich, S.

2009-01-01

With the focus on imaging, this paper gives a summarized view of the present knowledge on fields, which are necessary to know for a profound understanding of congenital malformations of the external and middle ear. Typical and less typical combinations of malformed parts of the ear can be derived from the embryogenesis. Clinical signs and audiometric findings lead to diagnosis in congenital aural atresia. Isolated middle ear malformations can be clinically mixed up especially with otosclerosis and tympanosclerosis. Imaging is needed for exact morphological information. In malformations of the external and middle ear, CT is the imaging modality of choice. Requirements on CT-technique as well as radiological findings including classification and pre-surgical rating are described. Morphological CT-correlates of congenital malformations and their differential diagnoses are enlisted and illustrated. The impact of CT-results on therapy is explained and actual therapeutic concepts are briefly presented

Congenital Vascular Malformation

Science.gov (United States)

... also be effective for small, localized birthmarks (port wine stains). Patients with a rare venous malformation (Kleppel–Trenaunay Syndrome) of the limbs, frequently benefit from elastic garments and bandages used for com- ...

Interventional treatment of pulmonary arteriovenous malformations

DEFF Research Database (Denmark)

Andersen, Poul Erik; Kjeldsen, Anette Drøhse

2010-01-01

Pulmonary arteriovenous malformations (PAVM) are congenital vascular communications in the lungs. They act as right to left shunts so that the blood running through these malformations is not oxygenated or filtered. These patients are typically hypoxaemic with exercise intolerance and are at high...... risk of paradoxical emboli to the brain and other organs. These malformations are most commonly seen in hereditary haemorrhagic telangiectasia (HHT) (Mb. Osler-Weber-Rendu syndrome). Nowadays, the generally accepted treatment strategy of first choice is embolization of the afferent arteries...... the functional level. Embolization is a well-established method of treating PAVM, with a significant effect on oxygenation of the blood. Screening for PAVM in patients at risk is recommended, especially in patients with HHT....

Eye malformations in Cameroonian children: a clinical survey

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Eballé AO

2012-10-01

Full Text Available André Omgbwa Eballé,1,2 Augustin Ellong,3 Godefroy Koki,3 Ngoune Chantal Nanfack,3 Viola Andin Dohvoma,3 Côme Ebana Mvogo2,31Yaoundé Gynaecology, Obstetrics and Pediatrics Hospital, Yaoundé, Cameroon; 2Faculty of Medicine and Pharmaceutical Sciences, University of Douala, Cameroon; 3Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, CameroonSummary: The aim of this work was to describe the clinical aspects of eye malformations observed at the ophthalmology unit of the Yaoundé Gynaecology, Obstetrics and Pediatrics Hospital.Patients and methods: We carried out a retrospective study of all malformations of the eye and its adnexae observed among children aged 0–5 years who were seen at the ophthalmology unit from January 2003 to December 2009.Results: Out of the 2254 children who were examined, 150 (6.65% presented eye malformations. The mean age was 14.40 ± 4 months. Eye malformations were diagnosed in 71.66% of cases during the first year of life. The most frequent malformations were congenital lacrimal duct obstruction (66.66%, congenital cataract (10.9%, congenital glaucoma (10.9%, microphthalmos (5.03%, and congenital ptosis (3.77%.Conclusion: Eye malformations among children can lead to visual impairment and are a cause for discomfort to children and parents. Therefore, systematic postnatal screening is recommended to enable early management.Keywords: malformations, ophthalmology, child, Cameroon

Ethanol sclerotherapy of peripheral venous malformations

Energy Technology Data Exchange (ETDEWEB)

Rimon, U. E-mail: rimonu@sheba.health.gov.il; Garniek, A.; Galili, Y.; Golan, G.; Bensaid, P.; Morag, B

2004-12-01

Background: venous malformations are congenital lesions that can cause pain, decreased range of movement, compression on adjacent structures, bleeding, consumptive coagulopathy and cosmetic deformity. Sclerotherapy alone or combined with surgical excision is the accepted treatment in symptomatic malformations after failed treatment attempts with tailored compression garments. Objectives: to report our experience with percutaneous sclerotherapy of peripheral venous malformations with ethanol 96%. Patients and methods: 41 sclerotherapy sessions were performed on 21 patients, aged 4-46 years, 15 females and 6 males. Fourteen patients were treated for painful extremity lesions, while five others with face and neck lesions and two with giant chest malformations had treatment for esthetic reasons. All patients had a pre-procedure magnetic resonance imaging (MRI) study. In all patients, 96% ethanol was used as the sclerosant by direct injection using general anesthesia. A minimum of 1-year clinical follow-up was performed. Follow-up imaging studies were performed if clinically indicated. Results: 17 patients showed complete or partial symptomatic improvement after one to nine therapeutic sessions. Four patients with lower extremity lesions continue to suffer from pain and they are considered as a treatment failure. Complications were encountered in five patients, including acute pulmonary hypertension with cardiovascular collapse, pulmonary embolus, skin ulcers (two) and skin blisters. All patients fully recovered. Conclusion: sclerotherapy with 96% ethanol for venous malformations was found to be effective for symptomatic improvement, but serious complications can occur.

Ethanol sclerotherapy of peripheral venous malformations

International Nuclear Information System (INIS)

Rimon, U.; Garniek, A.; Galili, Y.; Golan, G.; Bensaid, P.; Morag, B.

2004-01-01

Background: venous malformations are congenital lesions that can cause pain, decreased range of movement, compression on adjacent structures, bleeding, consumptive coagulopathy and cosmetic deformity. Sclerotherapy alone or combined with surgical excision is the accepted treatment in symptomatic malformations after failed treatment attempts with tailored compression garments. Objectives: to report our experience with percutaneous sclerotherapy of peripheral venous malformations with ethanol 96%. Patients and methods: 41 sclerotherapy sessions were performed on 21 patients, aged 4-46 years, 15 females and 6 males. Fourteen patients were treated for painful extremity lesions, while five others with face and neck lesions and two with giant chest malformations had treatment for esthetic reasons. All patients had a pre-procedure magnetic resonance imaging (MRI) study. In all patients, 96% ethanol was used as the sclerosant by direct injection using general anesthesia. A minimum of 1-year clinical follow-up was performed. Follow-up imaging studies were performed if clinically indicated. Results: 17 patients showed complete or partial symptomatic improvement after one to nine therapeutic sessions. Four patients with lower extremity lesions continue to suffer from pain and they are considered as a treatment failure. Complications were encountered in five patients, including acute pulmonary hypertension with cardiovascular collapse, pulmonary embolus, skin ulcers (two) and skin blisters. All patients fully recovered. Conclusion: sclerotherapy with 96% ethanol for venous malformations was found to be effective for symptomatic improvement, but serious complications can occur

Magnetic resonance imaging (MRI) of congenital cardiovascular malformations

International Nuclear Information System (INIS)

Sakakibara, Makoto; Kobayashi, Shirou; Imai, Hitoshi; Watanabe, Shigeru; Masuda, Yoshiaki; Inagaki, Yoshiaki; Morita, Huminori; Uematsu, Sadao; Arimizu, Noboru

1986-01-01

In order to determine the value of MRI in diagnosing congenital cardiovascular malformations, MR Images were obtained in 25 adult patients with congenital cardiovascular malformations. Gated MRI detected all of 13 atrial septal defects, and all of 4 ventricular septal defects, but ungated MRI detected none of 3 atrial septal defects. Other congenital cardiovascular malformations (2 with Ebstein's disease, 1 with Fallot's pentalogy, and 1 with Pulmonary stenosis) were well visualized. Vascular malformations (1 with Patent ducts arteriosus, 1 with Supravalvelar aortic stenosis, 1 with Coarctation of Aorta, 1 with Right Aortic Arch) were well visualized in all of 7 patients by ungated MRI. MRI was a valuable noninvasive method of diagnosing congenital heart disease. (author)

Congenital skeletal malformations induced by maternal ingestion of Conium maculatum (poison hemlock) in newborn pigs.

Science.gov (United States)

Panter, K E; Keeler, R F; Buck, W B

1985-10-01

Skeletal malformations were induced in newborn pigs from gilts fed Conium maculatum seed or plant during gestation days 43 through 53 and 51 through 61. The teratogenic effects in groups dosed during gestation days 43 through 53 were more severe than those in groups dosed during the later period, with many newborn pigs showing arthrogryposis and twisted and malaligned bones in the limbs and with 1 pig showing scoliosis and deformity of the thoracic cage. The pigs born to gilts given C maculatum during gestation days 51 through 61 had excessive flexure primarily in the carpal joints, without scoliosis or bone malalignment in the limbs. The teratogenicity of poison hemlock depends on the alkaloid concentration and content. Based on the data presented, we speculate that gamma-coniceine is the teratogenic alkaloid in the poison hemlock fed to the gilts.

Consequence of reduced necrotic bone elastic modulus in a Perthes' hip

DEFF Research Database (Denmark)

Salmingo, Remel A.; Skytte, Tina Lercke; Mikkelsen, Lars Pilgaard

Introduction Perthes is a destructive hip joint disorder characterized as a malformation of the femoral head which affects young children. Several studies have shown the change of mechanical properties of the femoral head in Perthes’ disease. However, the consequence of the changes in bone...... mechanical properties in a Perthes’ hip is not well established. Due to the material differences, changes in bone mechanical properties might lead to localization of stress and deformation. Thus, the objective of this study was to investigate the effects of reduced elastic modulus of necrotic bone...... weight) was applied on the top of the femoral head. The distal part of the femur was fixed. The same Poisson’s ratio 0.3 was set for the femoral and necrotic bone. The elastic modulus (E) of femoral bone was 500 MPa. To investigate the effects of reduced elastic modulus, the necrotic bone E was reduced...

Presbycusis: a human temporal bone study of individuals with downward sloping audiometric patterns of hearing loss and review of the literature.

Science.gov (United States)

Nelson, Erik G; Hinojosa, Raul

2006-09-01

The purpose of this retrospective case review was to identify patterns of cochlear element degeneration in individuals with presbycusis exhibiting downward sloping audiometric patterns of hearing loss and to correlate these findings with those reported in the literature to clarify conflicting concepts regarding the association between hearing loss and morphologic abnormalities. Archival human temporal bones from individuals with presbycusis were selected on the basis of strict audiometric criteria for downward-sloping audiometric thresholds. Twenty-one temporal bones that met these criteria were identified and compared with 10 temporal bones from individuals with normal hearing. The stria vascularis volumes, spiral ganglion cell populations, inner hair cells, and outer hair cells were quantitatively evaluated. The relationship between the severity of hearing loss and the degeneration of cochlear elements was analyzed using univariate linear regression models. Outer hair cell loss and ganglion cell loss was observed in all individuals with presbycusis. Inner hair cell loss was observed in 18 of the 21 individuals with presbycusis and stria vascularis loss was observed in 10 of the 21 individuals with presbycusis. The extent of degeneration of all four of the cochlear elements evaluated was highly associated with the severity of hearing loss based on audiometric thresholds at 8,000 Hz and the pure-tone average at 500, 1,000, and 2,000 Hz. The extent of ganglion cell degeneration was associated with the slope of the audiogram. Individuals with downward-sloping audiometric patterns of presbycusis exhibit degeneration of the stria vascularis, spiral ganglion cells, inner hair cells, and outer hair cells that is associated with the severity of hearing loss. This association has not been previously reported in studies that did not use quantitative methodologies for evaluating the cochlear elements and strict audiometric criteria for selecting cases.

RNA analysis of inner ear cells from formalin fixed paraffin embedded (FFPE) archival human temporal bone section using laser microdissection--a technical report.

Science.gov (United States)

Kimura, Yurika; Kubo, Sachiho; Koda, Hiroko; Shigemoto, Kazuhiro; Sawabe, Motoji; Kitamura, Ken

2013-08-01

Molecular analysis using archival human inner ear specimens is challenging because of the anatomical complexity, long-term fixation, and decalcification. However, this method may provide great benefit for elucidation of otological diseases. Here, we extracted mRNA for RT-PCR from tissues dissected from archival FFPE human inner ears by laser microdissection. Three human temporal bones obtained at autopsy were fixed in formalin, decalcified by EDTA, and embedded in paraffin. The samples were isolated into spiral ligaments, outer hair cells, spiral ganglion cells, and stria vascularis by laser microdissection. RNA was extracted and heat-treated in 10 mM citrate buffer to remove the formalin-derived modification. To identify the sites where COCH and SLC26A5 mRNA were expressed, semi-nested RT-PCR was performed. We also examined how long COCH mRNA could be amplified by semi-nested RT-PCR in archival temporal bone. COCH was expressed in the spiral ligament and stria vascularis. However, SLC26A5 was expressed only in outer hair cells. The maximum base length of COCH mRNA amplified by RT-PCR was 98 bp in 1 case and 123 bp in 2 cases. We detected COCH and SLC26A5 mRNA in specific structures and cells of the inner ear from archival human temporal bone. Our innovative method using laser microdissection and semi-nested RT-PCR should advance future RNA study of human inner ear diseases. Copyright © 2013 Elsevier B.V. All rights reserved.

Arteriovenous malformation of face

Directory of Open Access Journals (Sweden)

Ashok Kumar

2017-01-01

Full Text Available Arteriovenous malformations (AVMs are rare congenital vascular malformations accounting only 1.5% of all vascular anomalies with 50% occurrence in the oral and maxillofacial region. It usually results from birth defects of the vasculature. A literature search revealed only few case reports of AVMs in the facial region. Lack of meticulous diagnosis, scarcity of knowledge, and paucity of literature can result in their exsanguinations leading to fatal hemorrhagic incidents after various dental procedures such as tooth extraction, surgical intervention, puncture wound, or blunt injury in involved area. The present case describes the accidental diagnosis of asymptomatic high-flow AVMs in the facial region of pediatric patient reported primarily for the treatment of periapical abscess. This case report is unique because although there was no history of bleeding episodes, thorough examination and investigations diagnosed it as high-flow vascular malformation. It is important for the dental practitioner to be aware of AVM which may be present in the head and neck region that can produce fatal bleeding episodes during various dental procedures. Proper diagnosis of AVMs through complete history, precise clinical examination, and advanced imaging modalities can help in preventing serious life-threatening complications.

The presentation and management of complex female genital malformations.

Science.gov (United States)

Acién, Pedro; Acién, Maribel

2016-01-01

Common uterine anomalies are important owing to their impact on fertility, and complex mesonephric anomalies and certain Müllerian malformations are particularly important because they cause serious clinical symptoms and affect woman's quality of life, in addition to creating fertility problems. In these cases of complex female genital tract malformations, a correct diagnosis is essential to avoid inappropriate and/or unnecessary surgery. Therefore, acquiring and applying the appropriate embryological knowledge, management and therapy is a challenge for gynaecologists. Here, we considered complex malformations to be obstructive anomalies and/or those associated with cloacal and urogenital sinus anomalies, urinary and/or extragenital anomalies, or other clinical implications or symptoms creating a difficult differential diagnosis. A diligent and comprehensive search of PubMed and Scopus was performed for all studies published from 1 January 2011 to 15 April 2015 (then updated up to September 2015) using the following search terms: 'management' in combination with either 'female genital malformations' or 'female genital tract anomalies' or 'Müllerian anomalies'. The MeSH terms 'renal agenesis', 'hydrocolpos', 'obstructed hemivagina' 'cervicovaginal agenesis or atresia', 'vaginal agenesis or atresia', 'Herlyn-Werner-Wunderlich syndrome', 'uterine duplication' and 'cloacal anomalies' were also used to compile a list of all publications containing these terms since 2011. The basic embryological considerations for understanding female genitourinary malformations were also revealed. Based on our experience and the updated literature review, we studied the definition and classification of the complex malformations, and we analysed the clinical presentation and different therapeutic strategies for each anomaly, including the embryological and clinical classification of female genitourinary malformations. From 755 search retrieved references, 230 articles were analysed and

Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging

Energy Technology Data Exchange (ETDEWEB)

Hedlund, Gary [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States)

2006-07-15

The newborn, infant, or young child who presents with a midline frontonasal mass often poses a diagnostic challenge to the clinician. The most pressing issue is whether the mass extends intracranially. The development of the frontonasal region or anterior neuropore is complex. Aberrant embryogenesis leads to three main types of anomalies: nasal dermal sinus, anterior cephalocele, and nasal glioma. Understanding the developmental anatomy of the anterior neuropore and postnatal maturation will serve the radiologist well when it comes to imaging frontonasal masses. Pitfalls particularly common to CT imaging interpretation include the evolving ossification of the frontal, nasal and ethmoid bones in the first year of life, morphology and size of the foramen cecum, and the natural intumescence of the anterior nasal septum. Determination of the presence of a connection between the frontonasal mass and the anterior cranial fossae is crucial in the imaging assessment and clinical management. In the case of the nasal dermal sinus, failure to appreciate the intracranial components of the malformation can lead to fatal meningitis. MR imaging is the modality of choice for assessing the pediatric frontonasal region. Its advantages include multiplanar imaging, distinguishing the interface among cartilage, bone, brain and fluid, diffusion imaging to detect epidermoid tumors, and the capacity to evaluate the brain for associated cerebral anomalies. (orig.)

Arteriovenous malformation of the mandible

International Nuclear Information System (INIS)

Kim, Dong Youn; Lee, Kang Sook; Choi, Karp Shik

1995-01-01

The arteriovenous malformation, an uncommon lesion, is a direct communication between an artery and a vein that bypasses the intervening capillary bed. The authors experienced two cases of arteriovenous malformation in 17-year-old and 18-year-old female patients who had suffered from mandibular pain and gingival bleeding. As a result of careful analysis of clinical and angiographic radiological examinations, we diagnosed them as arteriovenous malformations. The following result were obtained; 1. Main clinical symptoms were dull pain and gingival bleeding on the mandibular body area and redness, pain on palpitation in the lesional sites were also observed. 2. Radiographically, well-defined radiolucent lesions with multiple osteolytic defects were observed. In radionuclide scan, increased uptakes of radioisotope were observed in the lesional sites. 3. Increased venous shunt supplied by the facial, lingual and inferior alveolar arteries was observed in angiography. After embolotherapy was performed, no recurrence was reported during 3 to 6 months follow up.

MR findings of congenital anorectal malformation

Energy Technology Data Exchange (ETDEWEB)

Kim, Yoo Kyung; Kim, Hyae Young; Kwag, Hyon Joo; Chung, Eun Chul; Lee, Jung Sik; Suh, Jeong Soo [Ewha Womens University, medical College, Seoul (Korea, Republic of)

1995-05-15

To assess the usefulness of MRI in preoperative diagnosis of congenital anorectal malformation. MR findings of 11 cases with surgically proved anorectal malformations were retrospectively reviewed and compared with operative findings, according to the level of atresia, the development of sphincter muscle, fistula and associated anomalies of other organs. Four of 11 cases were low type of anorectal atresia, 3 cases were intermediate type, and 3 cases were high type. There was one case of Currarino triad with low type of anorectal stenosis. MRI demonstrated the levels of atresia correctly in all cases and revealed fistulas in all high type of anomalies. Degrees of the development of the sphincter muscles were good in all cases of low types and fair in a case of intermediate type and an anorectal stenosis, whereas the development was poor in 2 cases of intermediate type and all 4 cases of high type. The associated anomalies in anorectal malformation were renal agenesis, congenital hip dysplasia and sacral defect with presacral teratoma in Currarino triad. MRI was a simple and useful study to confirm the level of atresia, fistula and associated anomalies in the diagnosis of the congenital anorectal malformation.

MR findings of congenital anorectal malformation

International Nuclear Information System (INIS)

Kim, Yoo Kyung; Kim, Hyae Young; Kwag, Hyon Joo; Chung, Eun Chul; Lee, Jung Sik; Suh, Jeong Soo

1995-01-01

To assess the usefulness of MRI in preoperative diagnosis of congenital anorectal malformation. MR findings of 11 cases with surgically proved anorectal malformations were retrospectively reviewed and compared with operative findings, according to the level of atresia, the development of sphincter muscle, fistula and associated anomalies of other organs. Four of 11 cases were low type of anorectal atresia, 3 cases were intermediate type, and 3 cases were high type. There was one case of Currarino triad with low type of anorectal stenosis. MRI demonstrated the levels of atresia correctly in all cases and revealed fistulas in all high type of anomalies. Degrees of the development of the sphincter muscles were good in all cases of low types and fair in a case of intermediate type and an anorectal stenosis, whereas the development was poor in 2 cases of intermediate type and all 4 cases of high type. The associated anomalies in anorectal malformation were renal agenesis, congenital hip dysplasia and sacral defect with presacral teratoma in Currarino triad. MRI was a simple and useful study to confirm the level of atresia, fistula and associated anomalies in the diagnosis of the congenital anorectal malformation

Cranial CT signs of the Chiari II malformation

International Nuclear Information System (INIS)

Naidich, T.P.; Pudlowski, R.M.

1980-01-01

Serial CT scans of 32 patients with proved Chiari II malformations and 19 patients with hydrocephalus and meningomyelocele (presumed to have Chiari II malformation) were reviewed and compared with CT scans from 30 patients with non-chiari aqueductal stenosis to develop criteria for identifying the Chiari II malformation and for differentiating it from other forms of hydrocephalus. Correlation with post-mortem specimens of Chiari II brains provided a pathologic basis for the CT signs observed. (orig.) [de

A case of pancreatic AV malformation in an elderly man.

Science.gov (United States)

Gupta, Vipin; Kedia, Saurabh; Sonika, Ujjwal; Madhusudhan, Kumble Seetharama; Pal, Sujoy; Garg, Pramod

2018-06-01

A 60-year-old man presented with recurrent abdominal pain and weight loss for 6 months. Abdominal imaging showed a large vascular lesion in the head and neck of pancreas suggestive of arteriovenous malformation (AV malformation). Endoscopic ultrasound was done which showed features of AV malformation with no evidence of pancreatic malignancy. Surgery was planned for definitive treatment of malformation. Digital subtraction angiography with angioembolization was done prior to surgery to reduce vascularity of the lesion. He recovered after a pylorus preserving pancreaticoduodenectomy. Histopathology of the resected specimen confirmed the pancreatic AV malformation. There has been no recurrence at 2 years of follow-up.

Pattern of Gross Congenital Malformations in a Tertiary Referral Hospital in Northeast India.

Science.gov (United States)

Baruah, Jenita; Kusre, Giriraj; Bora, Reeta

2015-10-01

To explore the prevalence of structural congenital malformations among newborns and study some of the fetal and maternal characteristics of the malformed babies in North east India where such systematic study on congenital malformation has not been undertaken before. A cross sectional study was undertaken from May 2010 through Feb 2013 for estimation of gross congenital malformations among live birth and stillbirth children born in Assam Medical College. All live births were clinically examined for detection of gross congenital malformations and autopsy was carried out on still births and neonates dying within 24 h of birth to detect gross congenital malformations in the internal organs. All malformations were classified as per ICD 10 classification. The mothers of the newborns with congenital malformations were interviewed in a predesigned, pretested proforma. The variables included maternal age, antenatal registration, antenatal history of drug intake, consanguinity and previous history of malformations. Statistical analysis was done using chi square test. A total 18,192 births including live births and still births were examined and 206 cases of structural malformations were observed. Prevalence of congenital malformations was 1.2 % of the total live births. Distribution of malformation was predominant among males than in females (60.67 vs. 37.37 %; p malformation of the cardiovascular system was found to be very low. Malformations among stillbirths and newborns born to unregistered mothers were significantly more. Percentage of malformations in babies born to mothers of more than 30 y of age was higher than other age groups (2.2 %). Occurrence of malformations in low birth weight babies were significantly more (p malformations was 1.2 % of the total live births. Musculoskeletal system was the most common system involved. Congenital malformations were significantly associated with sex of the new born, registration of the mother and birth weight of the

Fetal chromosome abnormalities and congenital malformations: an ...

African Journals Online (AJOL)

The results also showed that Multiple congenital anomalies (MCA) represented among 42.2%, congenital malformation of CNS represents 26.6%, congenital malformation of the skeletal system 20%, congenital polycystic kidney 8.8% and pyloric stenosis in 2.2%. Among the 21 women with abnormal karyotype of amniotic ...

Congenital malformations among newborns in Kenya | Muga ...

African Journals Online (AJOL)

Therefore, a study was conducted to determine the patterns and incidence of congenital malformations at birth in newborns in Kenya and thereby analyze associated predisposing factors in their mothers. This single cross-sectional ... followed by malformations of the central nervous system (28.6%). Polydactyl was the most ...

Abernethy malformation: a case report

Directory of Open Access Journals (Sweden)

Pathak Ashish

2012-05-01

Full Text Available Abstract Background Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. It is commonly associated with multiple congenital anomalies. We present a case of Abernethy malformation, without associated congenital anomalies from India. Case presentation A 5-year-old female child presented with short history of jaundice. A provisional diagnosis of acute viral hepatitis was made in view of clinical presentation and local endemicity of viral hepatitis A. Persistence of jaundice on follow up after 4 weeks led to detailed investigations. Ultrasound and doppler study of abdomen revealed drainage of portal vein into inferior vena cava. CT angiography was performed which confirmed the diagnosis of Type 1 b Abernethy malformation without associated major anomalies. We discuss the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder. Conclusion The treatment of the patients with congenital porto-systemic shunts depends on the site of the shunt, associated congenital anomalies and the extent of liver damage but the prognosis depends on the complications irrespective of anatomical type. However, the extent of associated abnormalities should not deter paediatricians to refer patients for treatment. Whenever possible closure of the shunt should be advised for cure or to prevent complications. Only symptomatic type I patients with absence of possibility to close the shunt may require liver transplant. Long-term follow-up is indicated for all patients.

Suboccipital neuropathy after bone conduction device placement

NARCIS (Netherlands)

Faber, H.T.; Ru, J.A. de

2013-01-01

OBJECTIVE: To describe the clinical characteristics of a 70-year-old female with occipital neuropathy following bone conduction device surgery. DESCRIPTION: A 65-year-old woman underwent bone conduction device placement surgery on the left temporal bone. Postoperatively she progressively developed

Contribution of Rare Copy Number Variants to Isolated Human Malformations

Science.gov (United States)

Serra-Juhé, Clara; Rodríguez-Santiago, Benjamín; Cuscó, Ivon; Vendrell, Teresa; Camats, Núria; Torán, Núria; Pérez-Jurado, Luis A.

2012-01-01

Background Congenital malformations are present in approximately 2–3% of liveborn babies and 20% of stillborn fetuses. The mechanisms underlying the majority of sporadic and isolated congenital malformations are poorly understood, although it is hypothesized that the accumulation of rare genetic, genomic and epigenetic variants converge to deregulate developmental networks. Methodology/Principal Findings We selected samples from 95 fetuses with congenital malformations not ascribed to a specific syndrome (68 with isolated malformations, 27 with multiple malformations). Karyotyping and Multiplex Ligation-dependent Probe Amplification (MLPA) discarded recurrent genomic and cytogenetic rearrangements. DNA extracted from the affected tissue (46%) or from lung or liver (54%) was analyzed by molecular karyotyping. Validations and inheritance were obtained by MLPA. We identified 22 rare copy number variants (CNV) [>100 kb, either absent (n = 7) or very uncommon (n = 15, malformations (21%), including 11 deletions and 11 duplications. One of the 9 tested rearrangements was de novo while the remaining were inherited from a healthy parent. The highest frequency was observed in fetuses with heart hypoplasia (8/17, 62.5%), with two events previously related with the phenotype. Double events hitting candidate genes were detected in two samples with brain malformations. Globally, the burden of deletions was significantly higher in fetuses with malformations compared to controls. Conclusions/Significance Our data reveal a significant contribution of rare deletion-type CNV, mostly inherited but also de novo, to human congenital malformations, especially heart hypoplasia, and reinforce the hypothesis of a multifactorial etiology in most cases. PMID:23056206

CT-Scans of Cochlear Implant Patients with Characteristics of Pendred Syndrome

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Sebastian Roesch

2013-12-01

Full Text Available Background: Sensorineural hearing loss (SNHL in newborns is estimated with an incidence around 1:10,000 per year and is divided into syndromic and non-syndromic forms. In case of present retrocochlear function‚ cochlear implantation allows speech and cognitive development in affected children, comparable to that of normal hearing children. Pathogenesis of SNHL remains unclear in many cases. Imaging of the temporal bone, such as computed tomography (CT and magnetic resonance imaging (MRI, can reveal conspicuous findings, e.g. enlarged vestibular aqueduct (EVA and Mondini malformation (MM of the cochlea. These malformations can be a clinical sign for Pendred syndrome. Methods: We screened CT scans of 75 cochlear implant patients for EVA and MM. Results: Six patients were observed to have either EVA alone (n=3, or MM alone (n=2, or a combination of both (n=1. Further malformations of the temporal bone could be found within the whole group, as well. Conclusion: Our results confirm the general opinion on EVA and MM, being commonly found in patients with SNHL. A possible association with Pendred syndrome needs to be confirmed by genetic investigations with search for mutations in the SLC26A4 gene and further clinical tests, such as Perchlorate test for surveillance of thyroid function.

Perinatal outcome of obvious congenital malformation as seen at the ...

African Journals Online (AJOL)

Gastrointestinal, central nervous and musculo-skeletal system malformations were the commonest seen with individual incidences of 3.9, 3.5 and 2.1 per 1000 total births, respectively. Unclassified congenital malformations had the highest case fatality while central nervous system malformations constituted the commonest ...

Common congenital malformations of the brain

International Nuclear Information System (INIS)

Naidich, T.P.; Zimmerman, R.A.

1987-01-01

In nearly all cases, congenital malformations are characterized most easily by their anatomic features and are best images with T1-weighted short TR/short TE pulse sequences. T2-weighted, long TR/long TE images are used primarily for the phakomatoses that are commonly associated with brain tumors. This chapter reviews the features of the most common congenital malformations and illustrates their typical magnetic resonance imaging (MRI) appearance

Endoscopic transnasal odontoidectomy to treat basilar invagination with congenital osseous malformations

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YU Yong

2012-08-01

Full Text Available Objective To introduce the surgical techniques of image-guided endoscopic transnasal odontoidectomy to treat basilar invagination with congenital osseous malformations and describe several advantages compared to the traditional transoral procedure. Methods From September 2009 to February 2010, two cases with basilar invagination, of which the etiology was congenital osseous malformations, underwent endoscopic transnasal odontoidectomy. Case 2 also received occipitocervical fixation and bone fusion during the same surgical episode to ensure stability. The clinical symptoms of the two cases were evaluated by using the Japanese Orthopaedic Association (JOA score for the evaluation of cervical myelopathy. Results Both patients were extubated after recovery from anesthesia and allowed oral food intake the next day. Cerebrospinal fluid rhinorrhea was found in the second case and cured by continuous lumber drainage of cerebrospinal fluid. No infection was noted. The average follow?up time was more than 24 months. Remarkable neurological recovery was observed at postoperation in both patients. The JOA scores elevated from preoperative 12 and 8 to postoperative 17 and 15. Conclusion The endoscopic transnasal odontoidectomy is a more minimally invasive approach for anterior decompression of cervicomedullary with basilar invagination. The advantages over the standard transoral odontoidectomy include visualization improvement, elimination of risk of tongue swelling and teeth damaging, alleviation of prolonged intubation, reduction of need for enteral tube feeding, and less risk of affecting phonation.

Surgical experience with arteriovenous malformations of the brain

International Nuclear Information System (INIS)

Stein, B.M.

1984-01-01

Personal experience with the treatment of 200 arteriovenous malformations of the brain seen during the past decade is presented. One hundred and twenty of these patients underwent surgery, with a mortality of 1,7%. The morbidity rate of 10% includes cases of hemianopia after occipital malformations had been removed. Significant morbidity, including aphasia, hemiparesis and hemisensory loss, occurred in under 10% of cases. Computerized tomography plays an important role in determining the relationships of the arteriovenous malformations to associated structures such as the ventricular system

Congenital Malformations in Neonates after irradiation of Rats During Pregnancy

International Nuclear Information System (INIS)

Abdel-Gawad, I.I.; Mohammad, M.H.M.

2000-01-01

Radiation is considered a teratogen during the whole period of embryonic development and fetal growth. However, the time of gestation at which irradiation takes place will affect the type of congenital malformation Induced. A study was carried out to observe various forms of congenital malformations induced after irradiation of pregnant rats to 1,2 and 3 Gy on the 9 th , 12 th and 15 th days of gestation. Various types of congenital malformations were observed in the neonates of irradiated animals as compared to controls. Most of the malformations were observed in neonates of animals irradiated with 2 and 3 Gy on the 12 th and 15 th days of gestation. This confirms that developmental anomalies occur mostly during the period of organ development. Other periods of gestation are less vulnerable to, induction of malformation after irradiation. Some representative photographs of the malformations induced such as penguin shape, absence of tail, low set ears, growth retardation and others are illustrated in the text

Congenital spinal malformations; Kongenitale spinale Malformationen

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Ertl-Wagner, B.B.; Reiser, M.F. [Klinikum Grosshadern, Ludwig-Maximilians-Univ. Muenchen (Germany). Inst. fuer Klinische Radiologie

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. (orig.) [German] Kongenitale spinale Malformationen stellen eine komplexe Gruppe an Stoerungen dar, deren Genese sich am einfachsten aus der Embryologie heraus erklaeren laesst. Bei der klinisch-radiologischen Begutachtung ist zunaechst ihre korrekte Klassifikation im Rahmen der Erstdiagnose wichtig. Im weiteren Verlauf ist es jedoch zudem entscheidend, moegliche Komplikationen wie beispielsweise eine Hydromyelie oder ein Wiederanheften des Myelons nach Operation einer Spina bifida aperta zu erkennen. Zudem sollte bei der Diagnosestellung einer kongenitalen spinalen Malformation immer auch auf assoziierte Fehlbildungen, wie z.B. die Diastematomyelie oder das intraspinale Lipom bei der Spina bifida aperta, sowie auf eine moegliche syndromale Einordnung wie beispielsweise beim OEIS-oder VACTERL-Syndrom geachtet werden. (orig.)

Therapies for neonates with congenital malformations admitted to a neonatal unit

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Maria Vera Lúcia Moreira Leitão Cardoso

2015-03-01

Full Text Available The aim of this study was to characterize the treatments applied to newborns with congenital malformation hospitalized in a neonatal unit and to identify whether there is an association among the treatments used and the type of malformation. A descriptive, prospective and quantitative study was developed in a public institution in Fortaleza, Ceará, Brazil. Data were collected using the medical records of 30 neonates with congenital malformations. The incidence of malformations was higher among females, regardless of the mother’s age, gestational age or weight at birth; malformations of the central nervous and musculoskeletal systems prevailed. The treatments used varied according to the clinical evolution of the neonate. The data collected did not present statistical significance when associated with the variable of congenital malformation and the treatments used (p>0.05. The treatments are not directly related to the type of malformation, but to the clinical condition of the neonate.

Vascular malformations in pediatrics; Vaskulaere Erkrankungen bei Neugeborenen, Saeuglingen und Kindern

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Reith, W. [Abteilung fuer Neuroradiologie, Radiologische Universitaetsklinik Homburg/Saar (Germany); Abteilung fuer Neuroradiologie, Radiologische Universitaetsklinik, 66421, Homburg/Saar (Germany); Shamdeen, M.G. [Kinderklinik, Universitaetsklinikum Homburg/Saar (Germany)

2003-11-01

Vascular malformations are the cause of nearly all non-traumatic intracranial hemorrhage in children beyond the neonatal stage. Therefore, any child presenting with spontaneous intracranial hemorrhage should be evaluated for child abuse and for vascular malformations. Intracerebral malformations of the cerebral vasculature include vein of Galen malformations, arteriovenous malformation (AVM), cavernomas, dural arteriovenous fistulas, venous anomalies (DVA), and capillary teleangiectasies. Although a few familial vascular malformation have been reported, the majority are sporadic. Clinical symptoms, diagnostic and therapeutic options are discussed. (orig.) [German] Vaskulaere Malformationen sind die haeufigste Ursache einer intrakraniellen Blutung im Saeuglings- und Kindesalter. Deswegen sollte jedes Kind mit einer intrakraniellen Blutung auf eine vaskulaere Malformation und Kindesmisshandlung untersucht werden. Intrazerebrale Gefaessmalformationen beinhalten Vena-Galeni-Anomalien, arteriovenoese Malformationen (AVM), Kavernome, durale arteriovenoese Fisteln, kapillaere Teleangiektasien und venoese Anlagevarianten (DVA). Obwohl einige familiaere Gefaessmalformationen beschrieben sind, tritt die ueberwiegende Mehrzahl spontan auf. Die Klinik, Diagnostik und therapeutischen Optionen werden diskutiert. (orig.)

[Diagnosis and management of Mondini malformation].

Science.gov (United States)

Yang, W; Fang, Y; Yang, S

1997-02-01

Owing to the development of imaging technology and audiology, some of the sensorineural hearing loss cases that were previcusly considered to be of unknown cause have been found to be inner ear malformation. Five cases of Mondini malformation are reviewed in this paper. CSF otorhinorrhea occurred in four cases, Klippel-Feil syndrome in three, and concurrent otosclerosis in one patient. In discussion, the authors point out that: 1) CT scanning is an supplement to audiologic tests for such patients; 2) perilymph fistula in Mondini malformation is often found at the oval window and its vicinity, the foot plate and the round window; 3) the fistula can be treated by plugging with fascial tissue via tympanoplastic approach with endaural incision. The mucosa around the fistula should be stripped away and the plugging tissue should be of dumb-bell shape.

First report of microcephaly-capillary malformations syndrome in ...

African Journals Online (AJOL)

Background: Microcephaly-capillary malformation (MIC-CAP) syndrome is a newly described autosomal recessive syndrome characterized by microcephaly, multiple cutaneous capillary malformations, intractable epilepsy and profound developmental delay. We present the first description of MIC-CAP syndrome in Russia.

Anaesthetic management of a child with massive extracranial arteriovenous malformation

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Faisal Shamim

2012-01-01

Full Text Available Vascular tumors affect the head and neck commonly but arteriovenous malformations are rare. Vascular malformations are often present at birth and grow with the patient, usually only becoming significant later in childhood. Embolization has been the mainstay of treatment in massive and complex arteriovenous malformations. We present a case of massive extracranial arteriovenous malformation in a 7-year-old boy causing significant workload on right heart and respiratory distress. The management of angioembolization under general anaesthesia and anaesthetic concerns are presented.

Lamb congenital lymphatic malformation - a case report

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Neria Santos

2014-01-01

Full Text Available Lymphatic malformations have been rarely reported in literature either in humans or in animals. However, in recent years, concern about these malformations in humans has increased. A five-month-old Rasa Aragonesa male lamb was received at the Ovine Clinical Service of the Veterinary Faculty of Zaragoza, Spain, with a history of cervical protuberances coming from birth. The lamb showed three round swollen lumps (5–12 cm in diameter parallel to the trachea on the left side of the neck. Clinical examination, haematology, ultrasonography, fluid examination and histopathology were performed. No abnormalities were found in blood samples and ultrasound confirmed a multicystic lesion with internal separations. Histological evaluation of the tissue revealed dilated lymphatic vessels and channels in the dermis and hypodermis; some lymphatic vessels were filled with amorphous proteinaceous material and occasional lymphocytes and macrophages. These protuberances were diagnosed as congenital lymphatic malformations. Most of the gross and microscopical lesions were very similar to those described in humans. To the authors’ knowledge, this is the first time that a congenital lymphatic malformation is described in sheep.

Genetic causes of congenital brain malformations in epilepsy patients

DEFF Research Database (Denmark)

Møller, Rikke Steensbjerre

2008-01-01

The search for genetic causes of congenital brain malformations, severe epilepsy and mental retardation plays an important role in neuropediatrics and neurology. Disclosure of the aetiology of the intellectual disabilities, seizures and the underlying brain malformation may be of psychological va...... genes for developmental brain defects. The overall aim of the present study has been to identify new candidate genes or predisposing factors involved in congenital brain malformations in epilepsy patients.......The search for genetic causes of congenital brain malformations, severe epilepsy and mental retardation plays an important role in neuropediatrics and neurology. Disclosure of the aetiology of the intellectual disabilities, seizures and the underlying brain malformation may be of psychological...... value for the family, and it is essential for proper genetic counselling. The human brain is one of the most complex structures known, and probably many of the 25.000- 30.000 genes that comprise the human genome are involved in its development, which means that thousands of genes could be candidate...

The use of 3D computer graphics in the diagnosis and treatment of spinal vascular malformations.

Science.gov (United States)

Takai, Keisuke; Kin, Taichi; Oyama, Hiroshi; Iijima, Akira; Shojima, Masaaki; Nishido, Hajime; Saito, Nobuhito

2011-12-01

Digital subtraction (DS) angiography is the gold standard for diagnosing spinal vascular malformations. Recently, multidetectorrow spiral CT and contrast-enhanced MR angiography have been introduced as screening examinations before DS angiography. These methods, however, do not always determine the accurate location of an arteriovenous shunt because the resulting images lack information about the spinal cord or the dura mater. Between April 2009 and December 2010, 13 patients underwent imaging evaluations for spinal vascular malformations at the authors' university hospital. This group included 8 patients with spinal dural arteriovenous fistulas (AVFs), 3 with perimedullary AVFs, and 2 with intramedullary arteriovenous malformations. Using data from these patients, the authors attempted to develop 3D computer graphics (CG) based upon the fusion of 3D rotational angiography and postmyelographic CT. They subsequently verified the accuracy of this imaging method. Ten of these 13 patients underwent surgical treatment for their lesions (11 AVFs), and for these 11 lesions the authors compared the diagnoses obtained using 3D CG with those obtained using conventional DS angiography. In all 13 cases, 3D CG images of the spinal lesions were successfully developed using the patients' actual data. Four (36%) of 11 AVFs were correctly identified using DS angiography, whereas 10 (91%) were correctly identified using 3D CG. Results from 3D CG of spinal AVFs corresponded well with operative findings, and 3D CG was significantly better than conventional DS angiography at predicting AVF location (p = 0.024, Fisher exact test). To the authors' knowledge, this is the first reported case series in which 3D CG of spinal vascular malformations was used to provide simultaneous, stereoscopic visualization of the spinal vascular system, spinal cord, dura mater, and bone. The 3D CG method provides precise visual images for the diagnosis and treatment of these lesions.

Mortality among infants with congenital malformations, New York State, 1983 to 1988.

Science.gov (United States)

Druschel, C; Hughes, J P; Olsen, C

1996-01-01

OBJECTIVE. The authors examined first-year mortality and risk factors for mortality among infants with major congenital malformations. METHODS. Infants with major congenital malformations born from 1983 to 1988 were identified from a statewide population-based congenital malformations registry. Variables analyzed included year of birth, birth weight, gestational age, infant sex, number of malformations, number of organ systems involved, level of care of the birth hospital, maternal age, maternal education, and maternal ethnicity. RESULTS. Infants with major malformations had a risk of death 6.3 times higher than the general population of live births. The risk declined from 6.5 in 1983 to 5.9 in 1988. Birth weight and number of malformations were the strongest risk factors. The likelihood of survival was similar for white and black infants. CONCLUSIONS. Being born with a malformation outweighs most of the other risks for infant mortality. Children with congenital malformations had higher cause-specific mortality for all causes except injury. PMID:8711105

Anatomy-Specific Virtual Reality Simulation in Temporal Bone Dissection: Perceived Utility and Impact on Surgeon Confidence.

Science.gov (United States)

Locketz, Garrett D; Lui, Justin T; Chan, Sonny; Salisbury, Kenneth; Dort, Joseph C; Youngblood, Patricia; Blevins, Nikolas H

2017-06-01

Objective To evaluate the effect of anatomy-specific virtual reality (VR) surgical rehearsal on surgeon confidence and temporal bone dissection performance. Study Design Prospective pre- and poststudy of a novel virtual surgical rehearsal platform. Setting Academic otolaryngology-head and neck surgery residency training programs. Subjects and Methods Sixteen otolaryngology-head and neck surgery residents from 2 North American training institutions were recruited. Surveys were administered to assess subjects' baseline confidence in performing 12 subtasks of cortical mastoidectomy with facial recess. A cadaver temporal bone was randomly assigned to each subject. Cadaver specimens were scanned with a clinical computed tomography protocol, allowing the creation of anatomy-specific models for use in a VR surgical rehearsal platform. Subjects then rehearsed a virtual mastoidectomy on data sets derived from their specimens. Surgical confidence surveys were administered again. Subjects then dissected assigned cadaver specimens, which were blindly graded with a modified Welling scale. A final survey assessed the perceived utility of rehearsal on dissection performance. Results Of 16 subjects, 14 (87.5%) reported a significant increase in overall confidence after conducting an anatomy-specific VR rehearsal. A significant correlation existed between perceived utility of rehearsal and confidence improvement. The effect of rehearsal on confidence was dependent on trainee experience and the inherent difficulty of the surgical subtask. Postrehearsal confidence correlated strongly with graded dissection performance. Subjects rated anatomy-specific rehearsal as having a moderate to high contribution to their dissection performance. Conclusion Anatomy-specific virtual rehearsal improves surgeon confidence in performing mastoid dissection, dependent on surgeon experience and task difficulty. The subjective confidence gained through rehearsal correlates positively with subsequent

Detecting congenital malformations - Lessons learned from the Mpepu study, Botswana.

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Gbolahan Ajibola

Full Text Available A large and increasing number of HIV-infected women are conceiving on antiretroviral treatment (ART. While most antiretrovirals are considered safe in pregnancy, monitoring for rare pregnancy and infant adverse outcomes is warranted.We conducted a retrospective secondary analysis nested within a clinical trial of infant cotrimoxazole vs. placebo prophylaxis in Botswana (the Mpepu Study. Infants were examined at birth, and at least every 3 months through 18 months of age. Abnormal physical findings and diagnostic testing revealing malformations were documented. Post hoc, a geneticist classified all reported malformations based on available documentation. Structural malformations with surgical, medical or cosmetic importance were classified as major malformations. We present a descriptive analysis of identified malformations.Between 2011 and 2014, 2,933 HIV-infected women who enrolled in the Mpepu study delivered 2,971 live-born infants. Study staff conducted 2,944 (99% newborn exams. One thousand eighty-eight (38% women were taking ART at conception; 1,147 (40% started ART during pregnancy; 442 (15% received zidovudine monotherapy; and 223 (7% received no antiretroviral during pregnancy. Of 33 reported anomalies, 25 (76% met congenital malformations criteria, 10 (30% were classified as major malformations, 4 (40% of which were identified after the birth exam.Our results highlight the importance of staff training on identification of congenital malformations, programmatic monitoring beyond the birth examination and the value of geneticist involvement in the malformations classification process in resource-limited settings. These elements will be important to fully define antiretroviral drug safety in pregnancy.Surveillance systems for monitoring the safety of antiretroviral use during pregnancy among HIV-infected women in resource-limited setting are lacking. The World Health Organization's published programmatic recommendations for such

Arteriovenous Malformation of the Pancreas

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Alexandros Charalabopoulos

2011-01-01

Full Text Available Pancreatic arteriovenous malformation (PAVM is a very rare and mostly congenital lesion, with less than 80 cases described in the English-published literature. It is defined as a tumorous vascular abnormality that is constructed between an anomalous bypass anastomosis of the arterial and venous networks within the pancreas. It represents about 5% of all arteriovenous malformations found in the gastrointestinal tract. Herein, we present a 64-year-old patient with symptomatic PAVM involving the body and tail of the organ, which was successfully treated by transcatheter arterial embolization. The disease spectrum and review of the literature are also presented.

Chondroblastoma of the sphenoid bone

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Patrocíni, Tomas Gomes

2008-12-01

Full Text Available Introduction: Chondroblastoma is an uncommon cartilaginous benign neoplasm, highly destructive, which specifically appears in the epiphysis of long bones in young patients. Its occurrence is extremely rare in the cranial base, normally occurring in the temporal bone. Objective: To describe a rare case in a patient presenting with a sphenoid bone chondroblastoma that invaded the middle cranial cavity, submitted to a successful surgical resection, without recurrence after 2 years. Case Report: W.J.S, 37 years old, male, forwarded to the otorhinolaryngology service with persistent and strong otalgia for 3 months. He had normal otoscopy and without visible tumorations. The computerized tomography confirmed tumor mass in the left infra-temporal cavity, invading the middle cranial cavity. The biopsy suggested giant cells tumor. After wide resection by frontal approach via orbitozygomatic osteotomy. During the surgery, we confirmed tomographic statements and didn't find temporal bone involvement. The histopathological exam confirmed chondroblastoma. After 18 months after the surgery, he doesn't present with complaints, without motor, sensitive deficits or of cranial nerves and without recurrence tomographic signals. Conclusion: The importance of differential diagnosis of chondroblastoma is remarkable in the cranial base lesions and its therapeutic approach, whose objective must always be the major possible resection with the maximum function conservation.

Clinical and Genetic Studies in Inherited Cardiovascular Malformations

NARCIS (Netherlands)

I.M.B.H. van de Laar (Ingrid)

2012-01-01

textabstractCardiovascular malformations comprise a broad spectrum of anomalies of the heart and blood vessels, including congenital heart malformations (CHM) and aortic aneurysms, the two main topics of this thesis. These conditions lead to significant morbidity and mortality both in infancy and

Congenital pseudoarthrosis associated with venous malformation

International Nuclear Information System (INIS)

Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N.; Hamamy, H.; Al-Hadidi, S.

2007-01-01

Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

Congenital pseudoarthrosis associated with venous malformation

Energy Technology Data Exchange (ETDEWEB)

Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N. [Jordan University Hospital, Radiology Department, P.O. Box 340621, Amman (Jordan); Hamamy, H. [Endocrinology and Genetics, National Center for Diabetes, Amman (Jordan); Al-Hadidi, S. [Jordan University Hospital, Departments of Orthopedics, Amman (Jordan)

2007-06-15

Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

Angiotensin-Converting Enzyme Inhibitors and the Risk of Congenital Malformations.

Science.gov (United States)

Bateman, Brian T; Patorno, Elisabetta; Desai, Rishi J; Seely, Ellen W; Mogun, Helen; Dejene, Sara Z; Fischer, Michael A; Friedman, Alexander M; Hernandez-Diaz, Sonia; Huybrechts, Krista F

2017-01-01

To examine the association between first-trimester angiotensin-converting enzyme (ACE) inhibitor exposure and the risk of overall major congenital, cardiac, and central nervous system malformations. We used a cohort of completed pregnancies linked to liveborn neonates derived from Medicaid claims from 2000 to 2010. We examined the risk of malformations associated with first-trimester exposure to an ACE inhibitor. Propensity score-based methods were used to control for potential confounders including maternal demographics, medical conditions, exposure to other medications, and measures of health care utilization. The cohort included 1,333,624 pregnancies, of which 4,107 (0.31%) were exposed to ACE inhibitors during the first trimester. The prevalence of overall malformations in the ACE inhibitor-exposed pregnancies was 5.9% compared with 3.3% in the unexposed (unadjusted relative risk, 1.82; 95% confidence interval [CI] 1.61-2.06), of cardiac malformations was 3.4% compared with 1.2% (relative risk 2.95, 95% CI 2.50-3.47), and of central nervous system malformations was 0.27% compared with 0.18% (relative risk 1.46, 95% CI 0.81-2.64). After restricting the cohort to pregnancies complicated by chronic hypertension (both exposed and unexposed) and accounting for other confounding factors, there was no significant increase in the risk of any of the outcomes assessed. Relative risks associated with first-trimester ACE inhibitor exposure were 0.89 (95% CI 0.75-1.06) for overall malformations, 0.95 (95% CI 0.75-1.21) for cardiac malformations, and 0.54 (95% CI 0.26-1.11) for CNS malformations. After accounting for confounders, among women with hypertension, exposure to ACE inhibitors during the first trimester was not associated with an increased risk of major congenital malformations.

Loss of col8a1a Function during Zebrafish Embryogenesis Results in Congenital Vertebral Malformations

Science.gov (United States)

Gray, Ryan S.; Wilm, Thomas; Smith, Jeff; Bagnat, Michel; Dale, Rodney M.; Topczewski, Jacek; Johnson, Stephen L.; Solnica-Krezel, Lilianna

2014-01-01

Congenital vertebral malformations (CVM) occur in 1 in 1,000 live births and in many cases can cause spinal deformities, such as scoliosis, and result in disability and distress of affected individuals. Many severe forms of the disease, such as spondylocostal dystostosis, are recessive monogenic traits affecting somitogenesis, however the etiologies of the majority of CVM cases remain undetermined. Here we demonstrate that morphological defects of the notochord in zebrafish can generate congenital-type spine defects. We characterize three recessive zebrafish leviathan/col8a1a mutant alleles (m531, vu41, vu105) that disrupt collagen type VIII alpha1a (col8a1a), and cause folding of the embryonic notochord and consequently adult vertebral column malformations. Furthermore, we provide evidence that a transient loss of col8a1a function or inhibition of Lysyl oxidases with drugs during embryogenesis was sufficient to generate vertebral fusions and scoliosis in the adult spine. Using periodic imaging of individual zebrafish, we correlate focal notochord defects of the embryo with vertebral malformations (VM) in the adult. Finally, we show that bends and kinks in the notochord can lead to aberrant apposition of osteoblasts normally confined to well-segmented areas of the developing vertebral bodies. Our results afford a novel mechanism for the formation of VM, independent of defects of somitogenesis, resulting from aberrant bone deposition at regions of misshapen notochord tissue. PMID:24333517

Congenital malformations in infants born after in vitro fertilization in Sweden.

Science.gov (United States)

Källén, Bengt; Finnström, Orvar; Lindam, Anna; Nilsson, Emma; Nygren, Karl-Gösta; Otterblad, Petra Olausson

2010-03-01

The risk for congenital malformations is increased in infants born after in vitro fertilization (IVF). Some specific malformations appear to be more affected than others. The presence of congenital malformations in 15,570 infants born after IVF with an embryo transfer between April 1, 2001, and the end of 2006 were compared with all infants born in Sweden during 2001 to 2007 (n = 689,157). Risk estimates were made after adjusting for year of birth, maternal age, parity, smoking, and body mass index. The risks of specific malformations were compared with data from a previous study (1982 to March 31, 2001) of 16,280 infants born after IVF. Different IVF methods were compared to respect to malformation risk. Increased risks of a similar magnitude were found for most cardiovascular malformations and limb reduction defects for both study periods. For neural tube defects, cardiac septal defects, and esophageal atresia, there was still an increased risk, but it was lower during the second than during the first period. For small bowel atresia, anal atresia, and hypospadias, the risk increase observed during the first study period had disappeared during the second period. An increased risk was seen for some syndromes that have been associated with imprinting errors. No difference in malformation risk according to IVF method was apparent. A slightly increased risk for congenital malformations after IVF persists. A decreasing risk is seen for some specific malformations, either true or the result of multiple testing. Copyright 2010 Wiley-Liss, Inc.

Temporal trends in obesity, osteoporosis treatment, bone mineral density, and fracture rates: a population-based historical cohort study.

Science.gov (United States)

Leslie, William D; Lix, Lisa M; Yogendran, Marina S; Morin, Suzanne N; Metge, Colleen J; Majumdar, Sumit R

2014-04-01

Diverging international trends in fracture rates have been observed, with most reports showing that fracture rates have stabilized or decreased in North American and many European populations. We studied two complementary population-based historical cohorts from the Province of Manitoba, Canada (1996-2006) to determine whether declining osteoporotic fracture rates in Canada are attributable to trends in obesity, osteoporosis treatment, or bone mineral density (BMD). The Population Fracture Registry included women aged 50 years and older with major osteoporotic fractures, and was used to assess impact of changes in osteoporosis treatment. The BMD Registry included all women aged 50 years and older undergoing BMD tests, and was used to assess impact of changes in obesity and BMD. Model-based estimates of temporal changes in fracture rates (Fracture Registry) were calculated. Temporal changes in obesity and BMD and their association with fracture rates (BMD Registry) were estimated. In the Fracture Registry (n=27,341), fracture rates declined 1.6% per year (95% confidence interval [CI], 1.3% to 2.0%). Although osteoporosis treatment increased from 5.6% to 17.4%, the decline in fractures was independent of osteoporosis treatment. In the BMD Registry (n=36,587), obesity increased from 12.7% to 27.4%. Femoral neck BMD increased 0.52% per year and lumbar spine BMD increased 0.32% per year after covariate adjustment (pobesity or osteoporosis treatment. © 2014 American Society for Bone and Mineral Research.

Atypical febrile seizures, mesial temporal lobe epilepsy, and dual pathology.

Science.gov (United States)

Sanon, Nathalie T; Desgent, Sébastien; Carmant, Lionel

2012-01-01

Febrile seizures occurring in the neonatal period, especially when prolonged, are thought to be involved in the later development of mesial temporal lobe epilepsy (mTLE) in children. The presence of an often undetected, underlying cortical malformation has also been reported to be implicated in the epileptogenesis process following febrile seizures. This paper highlights some of the various animal models of febrile seizures and of cortical malformation and portrays a two-hit model that efficiently mimics these two insults and leads to spontaneous recurrent seizures in adult rats. Potential mechanisms are further proposed to explain how these two insults may each, or together, contribute to network hyperexcitability and epileptogenesis. Finally the clinical relevance of the two-hit model is briefly discussed in light of a therapeutic and preventive approach to mTLE.

Decolonizing Straight Temporality Through Genre Trouble in Edwidge Danticat's The Farming of Bones

Directory of Open Access Journals (Sweden)

Eliana de Souza Ávila

2014-12-01

Full Text Available http://dx.doi.org/10.5007/2175-8026.2014n67p21 Framing genre trouble (McKenzie 2006 as a decolonial methodology, this paper considers the relevance of Edwidge Danticat’s The Farming of Bones (1998 for reading migrant texts against the grain of straight temporality which sustains the coloniality of power (Lugones 2007. Scrutinizing historiographic suppression, Danticat’s migrant text interrupts the chrononormative portrayal of the Trujillo genocide of Haitian workers in the Dominican Republic as a reality pertaining to an obsolete past and to the geocultural margins alone. Read in the aftermath of the testimonio controversy, it may thus decenter the ongoing deflection of attention from Rigoberta Menchú’s impact on the geocultural structures that sanction ongoing military intervention and genocide by refocusing on historiography as a terrain of relentless decolonial contestation rather than prescriptive narrative closure.

Congenital cystic lung malformations

International Nuclear Information System (INIS)

Stoever, B.; Scheer, I.; Bassir, C.; Chaoui, R.; Henrich, W.; Schwabe, M.; Wauer, R.

2006-01-01

Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

Meningitis after cochlear implantation in Mondini malformation.

Science.gov (United States)

Page, E L; Eby, T L

1997-01-01

Although the potential for CSF leakage and subsequent meningitis after cochlear implantation in the malformed cochlea has been recognized, this complication has not been previously reported. We report a case of CSF otorhinorrhea and meningitis after minor head trauma developing 2 years after cochlear implantation in a child with Mondini malformation. Leakage of CSF was identified from the cochleostomy around the electrode of the implant, and this leak was sealed with a temporalis fascia and muscle plug. Although this complication appears to be rare, care must be taken to seal the cochleostomy in children with inner ear malformations at the initial surgery, and any episode of meningitis after surgery must be thoroughly investigated to rule out CSF leakage from the labyrinth.

Mondini-like malformation mimicking otosclerosis and superior semicircular canal dehiscence.

Science.gov (United States)

Karlberg, M; Annertz, M; Magnusson, M

2006-05-01

In 2003, it was reported that superior semicircular canal dehiscence can mimic otosclerosis because of low-frequency bone conduction hearing gain and dissipation of air-conducted acoustic energy through the dehiscence. We report the case of a 17-year-old girl with left-sided combined hearing loss thought to be due to otosclerosis. Bone conduction thresholds were -10 dB at 250 and 500 Hz and she had a 40 dB air-bone gap at 250 Hz. When a tuning fork was placed at her ankle she heard it in her left ear. Acoustic reflexes and vestibular evoked myogenic potentials could be elicited bilaterally. Imaging of the temporal bones showed no otosclerosis, superior semicircular canal dehiscence or large vestibular aqueduct, but a left-sided, Mondini-like dysplasia of the cochlea with a modiolar deficiency could be seen. Mondini-like cochlear dysplasia should be added to the causes of inner-ear conductive hearing loss.

Mondini dysplasia and congenital cytomegalovirus infection.

Science.gov (United States)

Bauman, N M; Kirby-Keyser, L J; Dolan, K D; Wexler, D; Gantz, B J; McCabe, B F; Bale, J F

1994-01-01

We report a case of bilateral temporal bone anomalies in a child with symptomatic congenital cytomegalovirus infection and severe, bilateral sensorineural hearing loss identified at 3 months of age. High-resolution temporal bone computed tomography (HRCT) revealed bilateral findings of a short, malformed cochlea lacking an interscalar septum, a short and wide internal auditory canal, and an enlarged vestibular aqueduct, features diagnostic of bilateral Mondini dysplasia. To determine the importance of this observation, we completed HRCT in five additional children between 7 months and 9 years of age who had evidence of symptomatic congenital cytomegalovirus infection. One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplasia with a small cochlea lacking an interscalar septum, an abnormal vestibule, and a large cochlear aqueduct. Of the remaining four children, hearing thresholds ranged from normal to profoundly decreased, but their HRCT scans were normal to visual inspection. When inner ear dimensions of these temporal bones were compared with norms established by Pappas and coworkers, however, seven of the eight ears had short cochleas and narrow lateral semicircular canals, and three ears had short or narrow vestibules. These results indicate that congenital cytomegalovirus infection may cause anomalies or growth disturbances of the temporal bone.

Gross congenital malformation at birth in a government hospital.

Science.gov (United States)

Sachdeva, Sandeep; Nanda, Smiti; Bhalla, Kapil; Sachdeva, Ruchi

2014-01-01

A hospital-based cross-sectional study was undertaken to determine proportion of gross congenital malformation (GCMF) occurring at intramural births. Rate of GCMF was found to be 16.4/1000 consecutive singleton births (>28 weeks) with three leading malformation as anencephaly (44.68%), talipes equinovarus (17.02%) and meningomyelocele (10.63%). Higher risk of malformed births were noticed amongst un-booked (2.07%) in-comparison to booked (1.01%) mothers; women with low level of education (up to 8 years [2.14%] vs. at least 9 years of schooling [0.82%]); gravida status of at least 3 (2.69%) followed by 1 (1.43%) and 2 (1.0%) respectively; pre-term (5.13%) vs. term (0.66%); cesarean section (4.36%) versus vaginal delivery (0.62%). Mortality was significantly higher among congenitally malformed (17.35%) than normal (0.34%) newborns. With-in study limitation, emergence of neural tube defect as the single largest category of congenital malformation indicates maternal malnutrition (especially folic acid) that needs appropriate attention and management.

Insertion characteristics and placement of the Mid-Scala electrode array in human temporal bones using detailed cone beam computed tomography.

Science.gov (United States)

Dietz, Aarno; Gazibegovic, Dzemal; Tervaniemi, Jyrki; Vartiainen, Veli-Matti; Löppönen, Heikki

2016-12-01

The aim of this study was to evaluate the insertion results and placement of the new Advanced Bionics HiFocus Mid-Scala (HFms) electrode array, inserted through the round window membrane, in eight fresh human temporal bones using cone beam computed tomography (CBCT). Pre- and post-insertion CBCT scans were registered to create a 3D reconstruction of the cochlea with the array inserted. With an image fusion technique both the bony edges of the cochlea and the electrode array in situ could accurately be determined, thus enabling to identify the exact position of the electrode array within the scala tympani. Vertical and horizontal scalar location was measured at four points along the cochlea base at an angular insertion depth of 90°, 180° and 270° and at electrode 16, the most basal electrode. Smooth insertion through the round window membrane was possible in all temporal bones. The imaging results showed that there were no dislocations from the scala tympani into the scala vestibule. The HFms electrode was positioned in the middle of the scala along the whole electrode array in three out of the eight bones and in 62 % of the individual locations measured along the base of the cochlea. In only one cochlea a close proximity of the electrode with the basilar membrane was observed, indicating possible contact with the basilar membrane. The results and assessments presented in this study appear to be highly accurate. Although a further validation including histopathology is needed, the image fusion technique described in this study represents currently the most accurate method for intracochlear electrode assessment obtainable with CBCT.

Fetal MRI clues to diagnose cloacal malformations

Energy Technology Data Exchange (ETDEWEB)

Calvo-Garcia, Maria A.; Kline-Fath, Beth M.; Patel, Manish N.; Kraus, Steven [Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States); Levitt, Marc A.; Pena, Alberto [Cincinnati Children' s Hospital Medical Center, Colorectal Center for Children, Pediatric Surgery, Cincinnati, OH (United States); Lim, Foong-Yen; Crombleholme, Timothy M. [Cincinnati Children' s Hospital Medical Center, Fetal Care Center of Cincinnati, Pediatric Surgery, Cincinnati, OH (United States); Linam, Leann E. [Arkansas Children' s Hospital, Department of Radiology, Little Rock, AR (United States)

2011-09-15

Prenatal US detection of cloacal malformations is challenging and rarely confirms this diagnosis. To define the prenatal MRI findings in cloacal malformations. We performed a retrospective study of patients with cloacal malformations who had pre- and post-natal assessment at our institution. Fetal MRI was obtained in six singleton pregnancies between 26 and 32 weeks of gestation. Imaging analysis was focused on the distal bowel, the urinary system and the genital tract and compared with postnatal clinical, radiological and surgical diagnoses. The distal bowel was dilated and did not extend below the bladder in five fetuses. They had a long common cloacal channel (3.5-6 cm) and a rectum located over the bladder base. Only one fetus with a posterior cloacal variant had a normal rectum. Three fetuses had increased T2 signal in the bowel and two increased T1/decreased T2 signal bladder content. All had renal anomalies, four had abnormal bladders and two had hydrocolpos. Assessment of the anorectal signal and pelvic anatomy during the third trimester helps to detect cloacal malformations in the fetus. The specificity for this diagnosis was highly increased when bowel fluid or bladder meconium content was identified. (orig.)

Developing effective automated feedback in temporal bone surgery simulation.

Science.gov (United States)

Wijewickrema, Sudanthi; Piromchai, Patorn; Zhou, Yun; Ioannou, Ioanna; Bailey, James; Kennedy, Gregor; O'Leary, Stephen

2015-06-01

We aim to test the effectiveness, accuracy, and usefulness of an automated feedback system in facilitating skill acquisition in virtual reality surgery. We evaluate the performance of the feedback system through a randomized controlled trial of 24 students allocated to feedback and nonfeedback groups. The feedback system was based on the Melbourne University temporal bone surgery simulator. The study was conducted at the simulation laboratory of the Royal Victorian Eye and Ear Hospital, Melbourne. The study participants were medical students from the University of Melbourne, who were asked to perform virtual cortical mastoidectomy on the simulator. The extent to which the drilling behavior of the feedback and nonfeedback groups differed was used to evaluate the effectiveness of the system. Its accuracy was determined through a postexperiment observational assessment of recordings made during the experiment by an expert surgeon. Its usability was evaluated using students' self-reports of their impressions of the system. A Friedman's test showed that there was a significant improvement in the drilling performance of the feedback group, χ(2)(1) = 14.450, P feedback (when trainee behavior was detected) 88.6% of the time and appropriate feedback (accurate advice) 84.2% of the time. Participants' opinions about the usefulness of the system were highly positive. The automated feedback system was observed to be effective in improving surgical technique, and the provided feedback was found to be accurate and useful. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

Neuroradiological evaluation of dorsal cyst malformations

International Nuclear Information System (INIS)

Utsunomiya, Hidetsuna; Hayashi, Takashi; Hashimoto, Takeo; Matsuishi, Toyojiro; Okudera, Toshio.

1988-01-01

We discussed six cases with dorsal cyst malformations listing their neuroradiological observations and proposed to differentiate between the holosphere and hemisphere as defined by Yokota (1984). The cases were divided into holospheric and hemispheric groups depending on the continuity of their frontal lobe midlines. Cases 1, 2 and 3 were placed in the holospheric group because of their unseparated frontal lobe sbeneath the partially formed anterior interhemispheric fissures. Cases 4, 5 and 6 were grouped in the hemisphere due to the completion of the interhemispheric fissures. There has been a tendency in recent years for most cases of cerebral malformations having an endogenous dorsal cyst with monoventricular configuration to be diagnosed as holoprosencephaly. However, we believe that only patients who have a dorsal cyst in the holospheric brain should be included, and the others in the hemispheric brain, which is capable of completing hemispheric cleavage, should not. Therefore, we emphasize the importance of correctly identifying the holospheric state in the dorsal cyst malformations for diagnosing holoprosencephaly. (author)

Complex arteriovenous malformation - a case report

International Nuclear Information System (INIS)

Sirakov, S.; Penkov, M.; Marinov, M.; Kamenov, B.

2014-01-01

AVMs are composed of a network of channels interposed between feeding arteries and draining veins, without any direct shunt. Two different anatomic types of nidus may be more or less differentiated: The most frequent clinical presentations of brain AVMs are hemorrhage, seizure, chronic headache, and focal deficits not related to hemorrhage. We show a case of 27 years old female came to the hospital in heavy condition with subarachnoid hemorrhage Fisher 4. She had a history of 4 surgical operation of brain AVM in the last 10 years, without significant reduction of the malformation. After discussion of multidisciplinary team, of neuroradiologist and neurosurgeon was decided to be perform endovascular embolization, because of the better outcome for the patient. We performed endovascular treatment of the AVM with achieving subtotal embolization of malformation (90%). The patient recovery completely after 22 days and went home. For the next 18 months follow up there is no data of accidents for the patient. Key words: Arteriovenous Malformation. Subarachnoid hemorrhage. Endovascular treatment. Embolization

A rare association of rectal and genitourinary duplication and anorectal malformation

Institute of Scientific and Technical Information of China (English)

王俊; 施诚仁; 余世耀; 吴燕; 徐长辉

2003-01-01

@@ It is very rare to see multiple malformations occurring in both the urogenital and digestive systems in a case of congenital anorectal malformation. In this particular care, an imperforated anus occurred with other multiple malformations, including a double kidney, urethral duplication and rectal duplication, etc.

Clinico-roentgenological atlas of congenital malformations in human limbs

International Nuclear Information System (INIS)

Luzina, E.V.; Shakirov, Eh.A.

1990-01-01

The objective of the present atlas is to familiarize a wide range of physicians with localizations of congenital malformations in human limbs which are little studied clinically and roentgenologically. The atlas illustrates different variants of malformations of upper and lower limbs systematized by nosological principle; multiple and some, rarely occuring system deformations of the skeleton. Malformation features are described and their names are presented in compliance with the international classification taking into account the vocabulary of medical terms. 102 refs.; 121 figs

Fever in pregnancy and the risk of congenital malformations

DEFF Research Database (Denmark)

Sass, L; Urhoj, S K; Kjærgaard, J

2017-01-01

Background: In a variety of animal species, hyperthermia in pregnancy has been recognized as teratogenic. Hyperthermia interferes with protein synthesis via heat-shock proteins, which can entail membrane disruption, cell death, vascular disruption, and placental infarction. This can induce severe....... Congenital malformations within the first three and a half years of life were categorized according to EUROCAT's classification criteria. Logistic regression models were used to estimate the associations between fever in first trimester and overall congenital malformations and congenital malformations...

Dandy-Walker malformation | Hamid | Egyptian Journal of Medical ...

African Journals Online (AJOL)

Dandy-Walker malformation is a rare congenital malformation and involves the cerebellum and fourth ventricle. The condition is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. A large number of concomitant problems may be ...

Infertility, infertility treatment, and congenital malformations: Danish national birth cohort

Science.gov (United States)

Zhu, Jin Liang; Basso, Olga; Obel, Carsten; Bille, Camilla; Olsen, Jørn

2006-01-01

Objectives To examine whether infertile couples (with a time to pregnancy of > 12 months), who conceive naturally or after treatment, give birth to children with an increased prevalence of congenital malformations. Design Longitudinal study. Setting Danish national birth cohort. Participants Three groups of liveborn children and their mothers: 50 897 singletons and 1366 twins born of fertile couples (time to pregnancy ≤ 12 months), 5764 singletons and 100 twins born of infertile couples who conceived naturally (time to pregnancy > 12 months), and 4588 singletons and 1690 twins born after infertility treatment. Main outcome measures Prevalence of congenital malformations determined from hospital discharge diagnoses. Results Compared with singletons born of fertile couples, singletons born of infertile couples who conceived naturally or after treatment had a higher prevalence of congenital malformations—hazard ratios 1.20 (95% confidence interval 1.07 to 1.35) and 1.39 (1.23 to 1.57). The overall prevalence of congenital malformations increased with increasing time to pregnancy. When the analysis was restricted to singletons born of infertile couples, babies born after treatment had an increased prevalence of genital organ malformations (hazard ratio 2.32, 1.24 to 4.35) compared with babies conceived naturally. No significant differences existed in the overall prevalence of congenital malformations among twins. Conclusions Hormonal treatment for infertility may be related to the occurrence of malformations of genital organs, but our results suggest that the reported increased prevalence of congenital malformations seen in singletons born after assisted reproductive technology is partly due to the underlying infertility or its determinants. The association between untreated infertility and congenital malformations warrants further examination. PMID:16893903

Congenital Malformations in River Buffalo (Bubalus bubalis)

Science.gov (United States)

Albarella, Sara; Ciotola, Francesca; D’Anza, Emanuele; Coletta, Angelo; Zicarelli, Luigi; Peretti, Vincenzo

2017-01-01

Simple Summary Congenital malformations (due to genetic causes) represent a hidden danger for animal production, above all when genetic selection is undertaken for production improvements. These malformations are responsible for economic losses either because they reduce the productivity of the farm, or because their spread in the population would decrease the total productivity of that species/breed. River buffalo is a species of increasing interest all over the world for its production abilities, as proved by the buffalo genome project and the genetic selection plans that are currently performed in different countries. The aim of this review is to provide a general view of different models of congenital malformations in buffalo and their world distribution. This would be useful either for those who performed buffalo genetic selection or for researchers in genetic diseases, which would be an advantage to their studies with respect to the knowledge of gene mutations and interactions in this species. Abstract The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis) world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital

Direct Puncture Embolization of Scalp Arteriovenous Malformation in a Patient with Severe Hemophilia A: A Case Report

International Nuclear Information System (INIS)

Lee, Kyung Mi; Kim, Eui Jong; Park, Bong Jin; Kim, Keon Ha

2011-01-01

We present a case of scalp arteriovenous malformation (AVM) in a patient with severe hemophilia A. The 22-year-old man presented with a pulsatile right parietal scalp mass. Digital subtraction angiography revealed an AVM in the right parietal scalp, supplied by superficial temporal and occipital arteries that drained into multiple venous structures. We successfully performed direct puncture embolization followed by surgical resection of the scalp AVM in conjunction with supplemental infusion of coagulation factor VIII before, during and after the embolization and the operation.

Direct Puncture Embolization of Scalp Arteriovenous Malformation in a Patient with Severe Hemophilia A: A Case Report

Energy Technology Data Exchange (ETDEWEB)

Lee, Kyung Mi; Kim, Eui Jong [Dept. of Radiology, Kyung Hee University Hospital, Kyung Hee University Graduate School of Medicine, Seoul (Korea, Republic of); Park, Bong Jin [Dept. of Neurosurgery, Kyung Hee University Hospital, Kyung Hee University Graduate School of Medicine, Seoul (Korea, Republic of); Kim, Keon Ha [Dept. of Radiology, Samsug Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

2011-09-15

We present a case of scalp arteriovenous malformation (AVM) in a patient with severe hemophilia A. The 22-year-old man presented with a pulsatile right parietal scalp mass. Digital subtraction angiography revealed an AVM in the right parietal scalp, supplied by superficial temporal and occipital arteries that drained into multiple venous structures. We successfully performed direct puncture embolization followed by surgical resection of the scalp AVM in conjunction with supplemental infusion of coagulation factor VIII before, during and after the embolization and the operation.

Lymphatic malformations: a proposed management algorithm.

LENUS (Irish Health Repository)

Oosthuizen, J C

2012-02-01

OBJECTIVE: The aim of this study was to develop a management algorithm for cervicofacial lymphatic malformations, based on the authors\\' experience in managing these lesions as well as current literature on the subject. STUDY DESIGN AND METHODS: A retrospective medical record review of all the patients treated for lymphatic malformations at our institution during a 10-year period (1998-2008) was performed. DATA COLLECTED: age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms, treatment modality used, complications and results achieved. RESULTS: 14 patients were identified. Eight (57%) male and six (43%) female. There was an equal distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical excision and laser therapy. In 5 cases (36%) a combination of these were used. CONCLUSION: Historically surgical excision has been the management option of choice for lymphatic malformations. However due to the morbidity and high complication rate associated this is increasingly being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant promise. Based on experience in managing these lesions as well as current literature the authors of this paper have developed an algorithm for the management of cervicofacial lymphatic malformations.

Chondrosarcoma of the temporal bone. Diagnosis and treatment of 13 cases and review of the literature

International Nuclear Information System (INIS)

Coltrera, M.D.; Googe, P.B.; Harrist, T.J.; Hyams, V.J.; Schiller, A.L.; Goodman, M.L.

1986-01-01

Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with multiple sclerosis, glomus jugulare tumors, meningioma, and chordomas. The cranial nerve palsies frequently observed with the tumors are related to the anatomic locations of the tumors. Thirteen patients with this entity are presented and the eleven other cases in the literature are reviewed. Histologically the tumors are low grade and exhibit myxoid features. The myxoid features must be differentiated from chordoma and chondroid chordoma. The tumor locations preclude surgical excision and conventional radiation therapy can cause unacceptable neurologic sequelae. Proton beam therapy has been effective in short-term results and appears capable of avoiding serious neurologic side effects

Prevalence of Congenital Malformations

Directory of Open Access Journals (Sweden)

Akhavan Karbasi Sedighah

2009-05-01

Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

Analysis of Lethality and Malformations During Zebrafish (Danio rerio) Development.

Science.gov (United States)

Raghunath, Azhwar; Perumal, Ekambaram

2018-01-01

The versatility offered by zebrafish (Danio rerio) makes it a powerful and an attractive vertebrate model in developmental toxicity and teratogenicity assays. Apart from the newly introduced chemicals as drugs, xenobiotics also induce abnormal developmental abnormalities and congenital malformations in living organisms. Over the recent decades, zebrafish embryo/larva has emerged as a potential tool to test teratogenicity potential of these chemicals. Zebrafish responds to compounds as mammals do as they share similarities in their development, metabolism, physiology, and signaling pathways with that of mammals. The methodology used by the different scientists varies enormously in the zebrafish embryotoxicity test. In this chapter, we present methods to assess lethality and malformations during zebrafish development. We propose two major malformations scoring systems: binomial and relative morphological scoring systems to assess the malformations in zebrafish embryos/larvae. Based on the scoring of the malformations, the test compound can be classified as a teratogen or a nonteratogen and its teratogenic potential is evaluated.

[Diagnostic significance of multi-slice computed tomography imaging in congenital inner ear malformations].

Science.gov (United States)

Ma, Hui; Han, Ping; Liang, Bo; Liu, Fang; Tian, Zhi-Liang; Lei, Zi-Qiao; Li, You-Lin; Kong, Wei-Jia

2005-04-01

To evaluate the feasibility and usability of multi-slice computed tomography (MSCT) in congenital inner ear malformations. Fourty-four patients with sensorineural hearing loss (SNHL) were examined by a Somatom Sensation 16 (siemens, Germany) CT scanner with following parameters: 120 kV, 100 mAs, 0.75 mm collimation, 1 mm reconstruction increment, a pitch factor of 1 and a field of view of 100 mm. The axial images of interested ears were reconstructed with 0.1 mm reconstruction increment, and a field of view of 50 mm. The 3D reconstructions were done with volume rendering technique (VRT) on the workstation (3D Virtuoso and Wizard,siemens). Twenty-five patients were normal and 19 patients (36 ears) were congenital inner ear malformations among 44 patients scanned with MSCT. Of the malformations, all the axial, MPR and VRT images can display the site and degree in 33 ears. VRT images were superior to the axial images in displaying the malformations in 3 ears with the small lateral semicircular canal malformations. The malformations were Michel deformity (1 ear), common cavity deformity (3 ears), incomplete partition I (3 ears), incomplete partition II (Mondini deformity, 5 ears), vestibular and semicircular canal malformations( 14 ears), vestibular aqueduct dilate( 16 ears, of which 6 ears accompanied by other malformations), the internal auditory canal malformation(8 ears, all accompanied by other malformations). MSCT allows a comprehensively assessing various congenital ear malformations through high quality MPR and VRT reconstructions. VRT images can display the site and degree of the malformations three-dimensionally and intuitionisticly. It is very useful to the cochlear implantation.

ECOLOGICALLY DETERMINED MALFORMATIONS IN CHILDREN IN THE ULYANOVSK REGION

Directory of Open Access Journals (Sweden)

Elizaveta Grigoryevna Panchenko

2018-03-01

Full Text Available Currently particularly relevant is the interaction between ecology and people. The study had been carried to examine the correlation of congenital malformations from the residence. The analysis showed that increasing concentrations of heavy metals (lead, cadmium, and chromium in soil and air in some districts of the Ulyanovsk region correlates with a large number of congenital malformations in children, in contrast to those areas, where their content does not excees MPC, which allows to consider imbalance of trace elements as a possible factor in the development of congenital malformations.

Evaluation of magnetic resonance imaging of Arnold-Chiari malformation

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Yoshino, Kimihiro; Suga, Masakazu; Takemoto, Motohisa

1987-06-01

We evaluated the usefulness of magnetic resonance imaging (MRI) in the diagnosis of the Arnold-Chiari malformation. The patient, a 52-year-old man, complained of dizziness on walking. He initially refused to undergone myelography, but 4 months later, underwent MRI test, which lead to the diagnosis of Arnold-Chiari malformation (I type). Not all patients with symptoms of Arnold-Chiari malformation, syringomyelia, syringobulbia and cervical spinal tumor, undergone myelography, which is an invasive technique, therefore MRI should be the first examination for the patients with disorders involving the craniocervical junction.

MR imaging evaluation of congenital malformation of the spine

International Nuclear Information System (INIS)

Byrd, S.E.; Radkowski, M.A.; McLone, D.G.; Storrs, B.B.

1988-01-01

One hundred fifty children with congenital malformations of the spine were studied with MR imaging, US, and water-soluble myelography with CT. The malformations encountered included myelomeningoceles and Chiari II malformations, diastematomyelia, hydromyelia, arachnoid cyst, dermal sinus and dermoid, spinal lipomas and teratomas, tight filum terminale, anterior sacral meningoceles, lipomyelomeningoceles, myelocystocele, and severe scoliosis. MR imaging was the most effective modality in delineating these abnormalities. At times, real-time US and water-soluble CT myelography were required as adjuncts to MR imaging in delineating subtle spinal cord tethering, arachnoid cyst, diastematomyelia, and severe scoliosis

MRI findings of intracranial cavernous malformations

International Nuclear Information System (INIS)

Han, Byoung Hee; Kim, Dong Ik; Cho, Yong Kuk

1995-01-01

To analyze the variable MRI features and clinical significance of intracranial cavernous malformations. Forty patients(mean age 35.4) with cavernous malformation were evaluated by MRI. Eleven patients were surgically confirmed. Cavernous malformations were divided into four categories on the basis of the MR imaging characteristics, especially on T2-weighted image. Type I lesion was defined as an extralesional subacute hemorrhage outside the low signal rim, type II as an intralesional hemorrhage surrounded by low signal rim, type III lesion as an intralesional thrombosis with variegated central core surrounded by low signal rim, and type IV lesion as a focal old hemorrhagic core with small low signal intensity. Type IV was further divided into IVa and IVb, whether the lesion has small iso-or hypersignal central core (IVa) or not (IVb). Follow-up MRI was evaluated in 12 patients who were managed conservatively. Follow-up intervals ranged from 2 weeks to 29 months (mean 6 months). Total 80 lesions were detected in 40 patients. Multiple lesions were noted in 10 patients. The topography of the cavernous malformations was supratentorial in 75% and infratentorial in 23%. There were 10 lesions in type I, 15 in type II, 21 in type III, 14 in type IVa, and 20 in type IVb. Type I lesions mainly showed mass effect and edema. Type III lesions showed minimal contrast enhancements in 7 lesions on delayed images. Type II lesions showed the characteristics of both type I and type III lesions. On follow up images, decrease in size in 5, change of type in 7, rebleeding in 2 and no change in 12 lesions were demonstrated. Hemorrhage, edema and mass effect were combined in the cases of rebleeding. On follow-up study, the estimated risk of bleeding was 32.3%/person-year and 13.7%/lesion-year. Cavernous malformations show as variable appearance, on MR imaging suggesting variable stages of evolution. The MR morphologic classification and evaluation of secondary findings are helpful to

Effect of Gastrointestinal Malformations on the Outcomes of Patients With Congenital Heart Disease.

Science.gov (United States)

Mery, Carlos M; De León, Luis E; Rodriguez, J Rubén; Nieto, R Michael; Zhang, Wei; Adachi, Iki; Heinle, Jeffrey S; Kane, Lauren C; McKenzie, E Dean; Fraser, Charles D

2017-11-01

The goal of this study was to assess the effect of associated gastrointestinal malformations (GI) on the outcomes of patients undergoing congenital heart operations. Neonates and infants with thoracic (esophageal atresia, tracheoesophageal fistula) and abdominal (duodenal stenosis/atresia, imperforate anus, Hirschsprung disease) GI malformations undergoing congenital heart operations between 1995 and 2015 were included. Two control groups were created, one for each group. Patients were matched by diagnosis, procedure, history of prematurity, presence of genetic syndrome, and a propensity score including weight and year of operation. The cohort included 383 patients: 52 (14%) with thoracic GI malformations and 98 (25%) thoracic GI controls, 80 (21%) with abdominal GI malformations and 153 (40%) abdominal GI controls. Median follow-up was 6 years (range, 16 days to 20 years). Patients with thoracic GI malformations had longer length of stay (p malformations and controls. Patients with thoracic GI malformations have worse perioperative outcomes than controls, but their long-term survival does not seem to be significantly different. Abdominal GI malformations do not have a significant effect on outcomes. The presence of GI malformations should likely not preclude patients from undergoing congenital heart operations, but careful family counseling is necessary, especially for thoracic GI malformations. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Temporal mechanically-induced signaling events in bone and dorsal root ganglion neurons after in vivo bone loading.

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Jason A Bleedorn

Full Text Available Mechanical signals play an integral role in the regulation of bone mass and functional adaptation to bone loading. The osteocyte has long been considered the principle mechanosensory cell type in bone, although recent evidence suggests the sensory nervous system may play a role in mechanosensing. The specific signaling pathways responsible for functional adaptation of the skeleton through modeling and remodeling are not clearly defined. In vitro studies suggest involvement of intracellular signaling through mitogen-activated protein kinase (MAPK, phosphatidylinositol 3-kinase (PI3K/protein kinase B (Akt, and mammalian target of rapamycin (mTOR. However, anabolic signaling responses to bone loading using a whole animal in vivo model have not been studied in detail. Therefore, we examined mechanically-induced signaling events at five time points from 0 to 24 hours after loading using the rat in vivo ulna end-loading model. Western blot analysis of bone for MAPK's, PI3K/Akt, and mTOR signaling, and quantitative reverse transcription polymerase chain reaction (qRT-PCR to estimate gene expression of calcitonin gene-related protein alpha (CGRP-α, brain-derived neurotrophic factor (BDNF, nerve growth factor (NGF, c-jun, and c-fos in dorsal root ganglion (DRG of the brachial intumescence were performed. There was a significant increase in signaling through MAPK's including extracellular signal-related kinase (ERK and c-Jun N-terminal kinase (JNK in loaded limbs at 15 minutes after mechanical loading. Ulna loading did not significantly influence expression of the genes of interest in DRG neurons. Bone signaling and DRG gene expression from the loaded and contralateral limbs was correlated (SR>0.40, P<0.05. However, bone signaling did not correlate with expression of the genes of interest in DRG neurons. These results suggest that signaling through the MAPK pathway may be involved in load-induced bone formation in vivo. Further characterization of the

Imaging of head and neck venous malformations

International Nuclear Information System (INIS)

Flis, Christine M.; Connor, Stephen E.

2005-01-01

Venous malformations (VMs) are non proliferative lesions that consist of dysplastic venous channels. The aim of imaging is to characterise the lesion and define its anatomic extent. We will describe the plain film, ultrasound (US) (including colour and duplex Doppler), computed tomography (CT), magnetic resonance imaging (MRI), conventional angiographic and direct phlebographic appearances of venous malformations. They will be illustrated at a number of head and neck locations, including orbit, oral cavity, superficial and deep facial space, supraglottic and intramuscular. An understanding of the classification of such vascular anomalies is required to define the correct therapeutic procedure to employ. Image-guided sclerotherapy alone or in combination with surgery is now the first line treatment option in many cases of head and neck venous malformations, so the radiologist is now an integral part of the multidisciplinary management team. (orig.)

NPHP4 Variants Are Associated With Pleiotropic Heart Malformations

NARCIS (Netherlands)

French, Vanessa M.; van de Laar, Ingrid M. B. H.; Wessels, Marja W.; Rohe, Christan; Roos-Hesselink, Jolien W.; Wang, Guangliang; Frohn-Mulder, Ingrid M. E.; Severijnen, Lies-Anne; de Graaf, Bianca M.; Schot, Rachel; Breedveld, Guido; Mientjes, Edwin; van Tienhoven, Marianne; Jadot, Elodie; Jiang, Zhengxin; Verkerk, Annemieke; Swagemakers, Sigrid; Venselaar, Hanka; Rahimi, Zohreh; Najmabadi, Hossein; Meijers-Heijboer, Hanne; de Graaff, Esther; Helbing, Wim A.; Willemsen, Rob; Devriendt, Koen; Belmont, John W.; Oostra, Ben A.; Amack, Jeffrey D.; Bertoli-Avella, Aida M.

2012-01-01

Rationale: Congenital heart malformations are a major cause of morbidity and mortality, especially in young children. Failure to establish normal left-right (L-R) asymmetry often results in cardiovascular malformations and other laterality defects of visceral organs. Objective: To identify genetic

NPHP4 variants are associated with pleiotropic heart malformations.

NARCIS (Netherlands)

French, V.M.; Laar, I.M. van de; Wessels, M.W.; Rohe, C.; Roos-Hesselink, J.W.; Wang, G.; Frohn-Mulder, I.M.; Severijnen, L.A.; Graaf, B.M. de; Schot, R.; Breedveld, G.; Mientjes, E.; Tienhoven, M. van; Jadot, E.; Jiang, Z.; Verkerk, A.; Swagemakers, S.; Venselaar, H.; Rahimi, Z.; Najmabadi, H.; Meijers-Heijboer, H.; Graaff, E. de; Helbing, W.A.; Willemsen, R.; Devriendt, K.; Belmont, J.W.; Oostra, B.A.; Amack, J.D.; Bertoli-Avella, A.M.

2012-01-01

RATIONALE: Congenital heart malformations are a major cause of morbidity and mortality, especially in young children. Failure to establish normal left-right (L-R) asymmetry often results in cardiovascular malformations and other laterality defects of visceral organs. OBJECTIVE: To identify genetic

Morbidity rate due to renal malformations in patients from Las Tunas province

Directory of Open Access Journals (Sweden)

Silvio Laffita Estévez

2015-09-01

Full Text Available Background: The urogenital congenital malformations represent the first place within the genetic malformations in the province of Las Tunas.Objective: To characterize the renal congenital malformations in pediatric patients of the herein mentioned province, from 2010 to 2014.Methods: A descriptive study was carried out with 283 paediatric patients with renal and urinary tract congenital malformation, in the province and period herein stated. The variables included: sex, clinical manifestations, more frequent malformations, which of them needed surgical treatment, the positive results of the radiological studies, and the malformations that developed into chronic renal failure. The data were analyzed according to descriptive statistics.Results: The male sex represented a 63,96 %. A 57,95 % of the children were asymptomatic. Within the congenital malformations ectasia prevailed with a 49,47 %. 100 % of the cases with a compromise of the ureteovesiccal union, ureterocele and shell of the posterior urethra, 78 % of the patients with stenosis of the ureteropyelic union and 4,76 % of the patients with vesicoureteric reflux needed surgical treatment. The radiological studies were highly positive. 1,41 % of the patients developed chronic renal failure.Conclusions: Most of the patients with renal and urinary tract congenital malformations were diagnosed before the 5 years of age. Only four patients developed chronic renal failure.

Antidepressant exposure during early pregnancy and congenital malformations

DEFF Research Database (Denmark)

Pedersen, Lars Henning

are reassuring, however, an association with heart malformations has been suggested for e.g. paroxetine. A potential biological explanation will be reviewed. The potential teratogenic potential of antidepressants needs to be balanced against the obvious problems associated with under-treated maternal depression......Pharmacological treatment of pregnant women with depression is hampered by concerns for the developing fetus. The presentation will summarize existing knowledge on the potential association between antidepressants and congenital malformations, elaborate on the scientific background, and discuss...... the clinical significance. Most information on malformations in humans is derived from epidemiological studies. The strengths and limitations of the different designs need careful consideration, including issues of confounding by indication, recall bias, and power. For most antidepressants existing data...

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)

The petrous bone

DEFF Research Database (Denmark)

Jørkov, Marie Louise Schjellerup; Heinemeier, Jan; Lynnerup, Niels

2009-01-01

Intraskeletal variation in the composition of carbon (delta(13)C) and nitrogen (delta(15)N) stable isotopes measured in collagen is tested from various human bones and dentine. Samples were taken from the femur, rib, and petrous part of the temporal bone from well-preserved skeletons of both adults...... (n = 34) and subadults (n = 24). Additional samples of dentine from the root of 1st molars were taken from 16 individuals. The skeletal material is from a medieval cemetery (AD 1200-1573) in Holbaek, Denmark. Our results indicate that the petrous bone has an isotopic signal that differs significantly...... from that of femur and rib within the single skeleton (P bone and the 1st molar. The intraskeletal variation may reflect differences...

[Epidemiological analysis of selected congenital limb malformations in Hengyang].

Science.gov (United States)

Li, Na-Na; Yuan, Yu-Mei; Liu, Yong; Dai, Li; Deng, Chang-Fei; Nie, Xing-Hui; Zheng, Xiang-Chi; Hu, Yan-Zhen; Liu, Yun-Rong

2013-07-01

To describe the epidemiological characteristics of selected congenital limb malformations (CLM) in newborns of Hengyang. During the period of 2008-2010, cluster sampling survey was adopted to investigate the congenital limb malformations of neonates born to women resident in Hengyang, including Nanyue District, Zhuhui District, Changning City and Hengshan County. Each newborn was examined for the screening of CLM after birth. Limb malformations were grouped into the isolated (ILM) and the syndromic (SLM) form, depending on associated malformations of the affected. Prevalence rates, CLM spectrum and clinical manifestations were analyzed. A total of 170 CLM cases were identified among 52,307 newborns during the study period, resulting overall rate of 32.50/10(4). The rates for isolated and syndromic CLM were 28.29 and 4.21 per 10 000 births respectively. The rates for polydactyly, congenital talipes equinovarus, syndactyly and limb reduction defects were 13.00/10(4), 9.56/10(4), 5.16/10(4) and 3.63/10(4), respectively. No significant difference in rates of overall CLM or specified CLM was observed across urban-rural, gender and maternal age groups. Of the cases affected by polydactyly, syndactyly and limb reduction defects, malformation involved upper limbs, lower limbs and the both accounted for 68.14%, 14.16% and 17.70%. Preterm birth, low birth-weight, still birth and neonatal death were observed more frequently in syndromic cases than in isolated patients. The high CLM prevalence rate and fatality rate in Hengyang suggest that effective measures should be taken to prevent malformations and to improve survival of the affected.

Posterior fossa malformations: main features and limits in prenatal diagnosis

Energy Technology Data Exchange (ETDEWEB)

Garel, Catherine [Hopital d' Enfants Armand-Trousseau, Department of Radiology, Paris (France)

2010-06-15

Posterior fossa (PF) malformations are commonly observed during prenatal screening. Their understanding requires knowledge of the main steps of PF development and knowledge of normal patterns in US and MR imaging. The vast majority of PF malformations can be strongly suspected by acquiring a midline sagittal slice and a transverse slice and by systematically scrutinizing the elements of the PF: cerebellar vermis, hemispheres, brainstem, fourth ventricle, PF fluid spaces and tentorium. Analysis of cerebellar echogenicity and biometry is also useful. This review explains how to approach the diagnosis of the main PF malformations by performing these two slices and answering six key questions about the elements of the PF. The main imaging characteristics of PF malformations are also reviewed. (orig.)

Urogenital tract anomalies in children with congenital anorectal malformation

NARCIS (Netherlands)

J.W. Hoekstra

1991-01-01

textabstractThe term 'imperforate anus' covers a variety of congenital anorectal malformations ranging in severity from anal stenosis to cloacal exstrophy. The clinical picture of the anorectal malformation has been known for thousands of years, during which many attempts have been made to find

About kinetics of paramagnetic radiation malformations in beryllium ceramics

International Nuclear Information System (INIS)

Polyakov, A.I.; Ryabinkin, Yu.A.; Zashkvara, O.V.; Bitenbaev, M.I.; Petukhov, Yu.V.

1999-01-01

This paper [1] specifies that γ-radiation of the beryllium-oxide-based ceramics results in development of paramagnetic radiation malformations emerging the ESR spectrum in form of doublet with the splitting rate of oestrasid Δ∼1.6 and g-factor of 2.008. This report presents evaluation outcomes of dependence of paramagnetic radiation malformations concentration in beryllium ceramics on gamma-radiation dose ( 60 Co) within the range of 0-100 Mrad. Total paramagnetic parameters of beryllium ceramics in the range 0-100 Mrad of gamma-radiation dose varied slightly, and were specified by the first type of paramagnetic radiation malformations

Congenital bronchopulmonary foregut malformations: concepts and controversies

International Nuclear Information System (INIS)

Newman, Beverley

2006-01-01

This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions are discussed in more detail, particularly regarding controversial issues in classification, understanding, imaging and management. (orig.)

Congenital bronchopulmonary foregut malformations: concepts and controversies

Energy Technology Data Exchange (ETDEWEB)

Newman, Beverley [University of Pittsburgh School of Medicine and Children' s Hospital of Pittsburgh, Pittsburgh, PA (United States)

2006-08-15

This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions are discussed in more detail, particularly regarding controversial issues in classification, understanding, imaging and management. (orig.)

Effects of Co60 gamma radiation on Biomphalaria glabrata (Say, 1818) Embryo. II. Malformations

International Nuclear Information System (INIS)

Okazaki, K.; Kawano, T.

1990-01-01

The morphogenetic effects of ionizing radiation were investigated in Biomphalaria glabrata embryos irradiated in the cleavage, blastula, gastrula, young trochophore and trochophore stages with 5 to 25 Gy doses of 60 CO gamma radiation. The number of malformed embryos rapidly increased with increasing radiation dose, reaching a maximum between 5th to 8th day after irradiation in all stages analyzed. Susceptibility to malformation induction was higher the younger than the age of the irradiated embryo. However, for the cleavage stage the frequency of malformed embryos was inversely proportional to radiation dose for the same radiation dose. Several types of morphogenetic malformations were obtained, among then cephalic malformations, exogastrula, shell malformations and embryos with everted stomodeum, unspecific malformations being the most frequent. The results show that the types of malformation induced by radiation probably are not radiation-specific and do not depend on the dose applied [pt

Classification of Cortical Brain Malformations

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J Gordon Millichap

2008-03-01

Full Text Available Clinical, radiological, and genetic classifications of 113 cases of malformations of cortical development (MCD were evaluated at the Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands.

Angiographic treatment of the arteriovenous malformation occurred after caesarean section

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Selim Büyükkurt

2009-03-01

Full Text Available INTRODUCTION: Uterine arteriovenous malformations are classified as acquired or congenital. Caesarean section which is performed more frequently on nowadays, uterine curettage and other uterine surgeries are the most common causes of the acquired arteriovenous malformations. CASE: Twenty-two years old woman delivered her second child by caesarean section, due to history of caesarean section, was admitted with complaint of profuse vaginal bleeding 15 days after the surgery. She demonstrated the clinical features of the hypovolemic shock. At first she treated with four units of red blood suspension, two units of fresh frozen plasma and rapid infusion of the fluids. On endometrial curettage only blood and coagulum were obtained. The bleeding could only be controlled by the internal pressure of the 18 F Foley catheter. Doppler analyze of the uterus revealed a vascular malformation signifying a turbulent flow pattern with low pressure and high flow rate. A unilateral uterine artery embolization was performed her and her menstruel cycles are resumed at the fifth month of the puerperium. DISCUSSION: The clinical presentation of the uterine arteriovenous malformations depends on the localization and the dimensions of the malformation. The uterine arteriovenous malformation should be kept in mind in cases of late occurrence vaginal bleeding unresponsive to the uterotonic medications, especially in a woman with previous history of uterine damage, such as curettage or caesarean section.

Screening for pulmonary arteriovenous malformations

DEFF Research Database (Denmark)

Oxhøj, H; Kjeldsen, A D; Nielsen, G

2000-01-01

Pulmonary arteriovenous malformations (PAVM) cause right-to-left shunt and imply risk of paradoxical embolism and cerebral abscess. These complications can be prevented by appropriate treatment. Detection of PAVMs is therefore important, so simple and reliable screening methods are needed...... for this purpose. The aim of this investigation was to compare pulse oximetry and contrast echocardiography as screening tools for detection of pulmonary arteriovenous malformations. Eighty-five hereditary haemorrhagic telangiectasia (HHT) patients and first-degree relatives identified in a comprehensive study...... echocardiography with intravenous injection of echo contrast was performed in all subjects. Outcome measures were oxygen saturation change >2% units on changing body position and echo contrast observed in the left-sided heart chambers. Positive contrast echocardiography indicating the presence of PAVM was found...

Multiple congenital skeletal malformations in a lamb associated with ...

African Journals Online (AJOL)

Other malformations included patella absence, resulting in bowing of both fore and hind limbs with poorly developed muscles associated with these skeletal structure. Dystocia was believed to be a result of fetal monstrosity resulting in abnormal posture. The cause of the congenital malformations was not obvious ...

Variation and diversity in Homo erectus: a 3D geometric morphometric analysis of the temporal bone.

Science.gov (United States)

Terhune, Claire E; Kimbel, William H; Lockwood, Charles A

2007-07-01

Although the level of taxonomic diversity within the fossil hominin species Homo erectus (sensu lato) is continually debated, there have been relatively few studies aiming to quantify the morphology of this species. Instead, most researchers have relied on qualitative descriptions or the evaluation of nonmetric characters, which in many cases display continuous variation. Also, only a few studies have used quantitative data to formally test hypotheses regarding the taxonomic composition of the "erectus" hypodigm. Despite these previous analyses, however, and perhaps in part due to these varied approaches for assessing variation within specimens typically referred to H. erectus (sensu lato) and the general lack of rigorous statistical testing of how variation within this taxon is partitioned, there is currently little consensus regarding whether this group is a single species, or whether it should instead be split into separate temporal or geographically delimited taxa. In order to evaluate possible explanations for variation within H. erectus, we tested the general hypothesis that variation within the temporal bone morphology of H. erectus is consistent with that of a single species, using great apes and humans as comparative taxa. Eighteen three-dimensional (3D) landmarks of the temporal bone were digitized on a total of 520 extant and fossil hominid crania. Landmarks were registered by Generalized Procrustes Analysis, and Procrustes distances were calculated for comparisons of individuals within and between the extant taxa. Distances between fossil specimens and between a priori groupings of fossils were then compared to the distances calculated within the extant taxa to assess the variation within the H. erectus sample relative to that of known species, subspecies, and populations. Results of these analyses indicate that shape variation within the entire H. erectus sample is generally higher than extant hominid intraspecific variation, and putative H. ergaster

Percutaneous Sclerotherapy of Congenital Slow-Flow Vascular Malformations of the Orbit

Energy Technology Data Exchange (ETDEWEB)

Chiramel, George Koshy, E-mail: gkchiramel@gmail.com; Keshava, Shyamkumar Nidugala, E-mail: aparna-shyam@yahoo.com; Moses, Vinu, E-mail: vinu@cmcvellore.ac.in; Mammen, Suraj, E-mail: surajmammen77@gmail.com [Christian Medical College, Department of Radiology (India); David, Sarada, E-mail: saradadavid@gmail.com [Christian Medical College, Department of Ophthalmology (India); Sen, Sudipta, E-mail: paedsur@cmcvellore.ac.in [Christian Medical College, Department of Pediatric Surgery (India)

2015-04-15

PurposeThis manuscript describes the clinical features, imaging findings, treatment details, and short-term outcomes of a series of congenital slow-flow vascular malformations.MethodsThis was a prospective study of congenital slow-flow vascular malformations involving the orbital region treated at a single institution with percutaneous sclerotherapy.ResultsTen patients presented during the study period, comprising eight venous malformations, one lymphatic malformation, and one veno-lymphatic malformation. Nine patients underwent percutaneous sclerotherapy under digital subtraction angiography guidance, of which three developed marked rise in intraocular pressure requiring lateral canthotomy. The treatments were performed in the presence of an ophthalmologist who measured the intraorbital pressure during and after the procedure. On follow-up, some of the patients required repeat sessions of sclerotherapy. All patients had improvement of symptoms on follow up after the procedure.ConclusionCongenital slow-flow vascular malformations of the orbital region are rare lesions that should be treated using a multidisciplinary approach. Monitoring of the intraorbital pressure is required both during and after the procedure to decide about the need for lateral canthotomy to reduce the transiently increased intraorbital pressure.

Percutaneous Sclerotherapy of Congenital Slow-Flow Vascular Malformations of the Orbit

International Nuclear Information System (INIS)

Chiramel, George Koshy; Keshava, Shyamkumar Nidugala; Moses, Vinu; Mammen, Suraj; David, Sarada; Sen, Sudipta

2015-01-01

PurposeThis manuscript describes the clinical features, imaging findings, treatment details, and short-term outcomes of a series of congenital slow-flow vascular malformations.MethodsThis was a prospective study of congenital slow-flow vascular malformations involving the orbital region treated at a single institution with percutaneous sclerotherapy.ResultsTen patients presented during the study period, comprising eight venous malformations, one lymphatic malformation, and one veno-lymphatic malformation. Nine patients underwent percutaneous sclerotherapy under digital subtraction angiography guidance, of which three developed marked rise in intraocular pressure requiring lateral canthotomy. The treatments were performed in the presence of an ophthalmologist who measured the intraorbital pressure during and after the procedure. On follow-up, some of the patients required repeat sessions of sclerotherapy. All patients had improvement of symptoms on follow up after the procedure.ConclusionCongenital slow-flow vascular malformations of the orbital region are rare lesions that should be treated using a multidisciplinary approach. Monitoring of the intraorbital pressure is required both during and after the procedure to decide about the need for lateral canthotomy to reduce the transiently increased intraorbital pressure