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Biomedical subjects

J M Converse

Publications and source records attributed to J M Converse.

At least 55 records · Page 3Linked to original sources

Unilateral arhinencephaly in goldenhar-gorlin syndrome.

The post-mortem examination of the brain of a 2 1/2-year-old girl with clinical featutes of oculo-auriculo-vertebral dysplasia and hemifacial microsomia (Goldenhar-Gorlin syndrom) revealed a unilateral absence of the olfactory foramina of the lamina cribrosa of the ethmoid bone and ipsilateral absence of olfactory bulb and tract. Other cerebral abnormalities were also present. In this report, unilateral arhinencephaly in this disorder has been documented for the first time and an attempt has been made to correlate various nosological, clinical and pathological aspects of the case with previously reported instances of arhinencephaly. It is concluded that unilateral arhinencephaly occurs in a variety of cranial and facial abnormalities and is not specific for any particular syndrome.

Abnormalities, Multiple↗

Congenital trigeminal neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).

A 2 1/2 year old child with clinical features of Goldenhar-Gorlin syndrome showed diminished pinprick sensation over the right half of the face. After surgery for the cleft lip, the child died. Neuropathological investigations showed agenesis of the right trigeminal nerve and hypoplasia of the right trigeminal brain-stem nuclei. Nosological aspects of the Goldenhar-Gorlin syndrome and previously reported cases of congenital trigeminal anaesthesia in this disorder are discussed. It is suggested that the hypoplasia of the trigeminal nerve is responsible for the diminished facial sensation seen in some patients with this craniofacial syndrome.

Abnormalities, Multiple↗

Correction of ocular hypertelorism.

36 patients with congenital hypertelorism, eight with Crouzon's disease, and three with a posttraumatic deformity, were operated with Tessier's technique with some modifications, i.e. the dura is not separated from the cribriform plate avoiding olfactory damage. The entire procedure for hypertelorism could be carried out in less than 5 h but correction of Crouzon's disease required 6-8 h, and longer in older patients. There was only one intraoperative anesthesic death in a 3-year-old child. One cardiac arrest recovered. Nine staphylococcus infections were superficial and readily treated. There was postoperative cerebral edema in one case and convulsions following surgery in another. Anticonvulsants and steroids were subsequently routinely employed. There were no SF fistulas. Excellent cosmetic results are due primarily to the skill of the plastic surgeons.

Craniofacial Dysostosis↗

Deformities of the midface resulting from malunited orbital and naso-orbital fractures.

In midfacial fractures the crucial area is the upper portion of the midfacial skeleton which lodges the orbits, the interorbital space with its anatomic relationship with the anterior cranial fossa, the lacrimal apparatus, the levator muscle, and the skeletal structures of the nose. Because of the severity of the injuries in a multisystem injured patient, these fractures may be neglected or receive inadequate primary treatment. In some cases the damage is so extensive that primary treatment can only accomplish part of the task. Late reconstruction is necessary, therefore, and is the subject of this paper.

Blepharoptosis↗

Clinical and radiographic variations in hemifacial microsomia.

Variations expressed clinically and radiographically in hemifacial microsomia preclude classifying all the abnormalities as coming from the first and second branchial arches. Anatomic structures arising from the branchial arches are directly involved but the final expression results from the combined impact of the primary anatomic defect and the secondary effects on contiguous structures. The deformities resulting from the latter are probably due to abnormal neuromuscular function.

Abnormalities, Multiple↗