Congenital extraneural hemangioblastoma in a lamb.

A 1-mo-old Ivesi male lamb was presented with 2 large red masses on the skin of the left ear. The tumors were removed using gentle dissection and submitted for histologic evaluation. The tumors consisted of numerous thin-walled capillaries lined by endothelial cells and nests of stromal cells. Immunohistochemically, the endothelial cells were positive for CD45, and the stromal cells were positive for neuron-specific enolase. GFAP-positive cells were occasionally present within the tumor. Endothelial and stromal cells were negative for S100, CD34, CD31, and factor VIII-related antigen. The tumor had strong gross, microscopic, and immunohistochemical similarities with human extraneural hemangioblastoma.

A staging system for congenital cholesteatoma.

OBJECTIVE: To develop a staging system for congenital cholesteatoma in predicting the likelihood of residual disease. DESIGN: Retrospective analysis of data from a case series, to identify predictors of residual disease. SETTING: Tertiary care pediatric hospital. PARTICIPANTS: Children undergoing surgical removal of congenital cholesteatoma. There were 156 patients, with 160 cholesteatomas; 4 children had bilateral disease. INTERVENTIONS: Each case was scored as to quadrants of the middle ear involved, ossicular involvement, and mastoid extension. MAIN OUTCOME MEASURE: Surgically confirmed residual disease at any time after the initial procedure. RESULTS: Four stages were defined as follows: stage I, disease confined to a single quadrant; stage II, cholesteatoma in multiple quadrants, but without ossicular involvement or mastoid extension; stage III, ossicular involvement without mastoid extension; and stage IV, mastoid disease. There was a strong association between stage and residual disease, ranging from a 13% risk in stage I to 67% in stage IV. CONCLUSIONS: This simple staging system may be particularly useful in standardizing the reporting of congenital cholesteatoma and in adjusting for severity in evaluating outcomes. It also provides information that is useful in counseling parents.

Congenital cholesteatoma of external auditory canal.

A 7-month-old male child is reported with congenital cholesteatoma of the external auditory canal. We describe the clinical features, computed tomography finding and surgical treatment. Congenital cholesteatomas can occur within the temporal bone. There are six places of location: (1) petrous apex, (2) mastoid, (3) middle ear, (4) both middle ear and mastoid, (5) external ear canal and (6) within the squamous portion of the temporal bone. Congenital cholesteatoma of the external auditory canal is rare. Generally, it appears in the canal floor without lesions in the tympanic membrane. Computed tomography completes the study. Treatment consists of excision of the mass.

An unusual presentation of bilateral prominent ears.

Patients with bilateral prominent ears commonly present for surgical correction. The cause of this condition is probably multifactorial and, whilst there is certainly a strong familial component, pathological causes are few. We present a case of bilateral postauricular dermoid cysts giving rise to this condition.

Imaging of congenital anomalies of the temporal bone.

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.

Periauricular cysts and sinuses.

Periauricular cysts, sinuses, and fistulas occur commonly in the pediatric population. They arise from developmental defects of the first branchial cleft and first branchial arch. In most instances the diagnosis and management of these conditions are straightforward, but exceptional presentations sometimes occur. Failure to recognize these unusual cases may result in inadequate treatment and subsequent recurrence, and even if the correct diagnosis is made, surgical management of these lesions may be complicated. A series of 15 cases of periauricular congenital lesions is reviewed, of which three cases illustrating a diagnostic or surgical challenge are presented. The embryology, presentation, and management of these anomalies are discussed. This is one of the largest series of first branchial cleft anomalies reported in the literature, and our paper uniquely discusses first branchial cleft anomalies and preauricular sinuses together, with an emphasis on the surgical management of facial nerve, external ear, and middle ear involvement.

Middle ear teratoma in a newborn.

Teratomas are tumours that rarely exist in children. Their location in head and neck is, however, an unusual finding. A strange case of middle ear congenital teratoma reaching the cervical area is presented here. In the last 20 years it is only the second case to be published in the English literature. The tumour was revealed by the presence of a peripheral congenital facial palsy.

Primary reconstruction of congenital facial lesion defects with tissue expansion.

BACKGROUND: Congenital facial lesions may require surgical intervention to correct deformity or to decrease the potential for malignant degeneration. OBJECTIVE: Hemangiomata and melanocytic and sebaceous nevi are among the most common congenital facial defects. The techniques and outcome of serial tissue expansion in the management of facial lesions were evaluated. METHODS: Seven pediatric patients had congenital facial defects treated with tissue expansion and subsequent excision and reconstruction. RESULTS: All patients had a satisfactory outcome. Complications are reviewed, and in this patient population were minor. CONCLUSIONS: Tissue expansion has a role in surgical management of congenital facial lesions. Staged excision, skin grafting and nonintervention are also options which must be individualized to the case at hand.

Otologic manifestations of the hydantoin syndrome.

The temporal bones of a newborn infant with hydantoin syndrome showed multiple middle ear and inner ear anomalies. There was a constellation of bony and membranous defects involving the oval and round windows, cochlear ducts, cochlear aqueducts, endolymphatic ducts and sacs, and vestibular labyrinths. To the authors' knowledge, supernumerary vestibular sensory epithelial structures and an inner ear epidermoid cyst have not been previously reported. Wide communications between the subarachnoid space and inner ear were of surgical relevance.

Dermoid cyst of the Eustachian tube.

A case of congenital dermoid cyst of the Eustachian tube is reported. Only seven similar cases have been found in our review of the literature. The embryology and usual mode of presentation of these cysts are discussed. Finally, a new surgical approach using a combined retro-auricular and protympanic route is presented.

Cholesteatoma auris congenita arising from microtia.

The external auditory canal opens medial to lateral in embryonal development. It is obvious that a developmental arrest anywhere during this stage would produce an absence or discontinuity of the canal, that is, congenital atretic ear. Whether or not the obliterative tissue has a bony structure, cholesteatoma can be formed behind it. Microtia is usually associated with an atretic meatus, and it may involve the possibility of epithelial cyst or cholesteatoma. The diagnosis can usually be made by the finding of tender swelling in the deformed auricle or its surroundings. On occasions, it may become necessary to distinguish these conditions from secondary infection of an epidermal cyst on the auricular surface. Several recurrent painful swellings on the deformed auricle in spite of conservative treatment indicate the need for routine otologic radiographs.

First branchial cleft anomalies.

A series of nine cases of first branchial cleft anomalies treated at Children's Hospital of Michigan from 1970-1978 is presented. A review of the literature and analysis of these cases reveals that the accepted criteria for classification of these anomalies are confusing and inadequate as they preclude the proper classification of a number of these cases. The embryogenesis of these anomalies is discussed with attention given to their relationship to the facial nerve. A proper classification is suggested.