The role of episcleral venous pressure in glaucoma associated with Sturge-Weber syndrome.

PURPOSE: To evaluate the role of episcleral venous pressure (EVP) in the pathogenesis of glaucoma associated with Sturge-Weber syndrome (SWS). METHODS: EVPs were determined prospectively using an episcleral venomanometer in 22 eyes of 11 patients aged 8-18 years with SWS with or without glaucoma. Pressure measurements in the glaucomatous eyes of patients with SWS were compared to those of patients with facial port wine marks but no glaucoma and to the contralateral uninvolved eye in both groups. RESULTS: EVP in eyes with glaucoma (mean, 20.9 mm Hg) was significantly higher (P < 0.01) than EVP in contralateral uninvolved eyes (mean, 9.6 mm Hg). In patients with unilateral port wine mark and no glaucoma, EVP was normal for ipsilateral and contralateral eyes (mean, 8.6 mm Hg and 9.6 mm Hg, respectively). CONCLUSIONS: Our data support the hypothesis that elevated EVP plays an important role in eyes with SWS glaucoma.

An apparent duplication of the mouth in a patient with midline dysraphism and a teratoid polyp: A 15-year postsurgical follow-up.

True median cleft of the upper and lower lip occurs very rarely. Complete or partial facial duplication is an extremely rare malformation. Approximately 26 cases have been reported since 1900, spanning a wide spectrum of clinical severity. Most cases appear to share a number of features, including cleft palate, duplication of the tongue, orbital hypertelorism and macrostomia. An unusual patient with midline clefts of the upper and lower lips with an apparent duplication of the mouth and a teratoid polyp, with fusion of the maxilla and mandible, is reported. The early management and surgical intervention is discussed. Furthermore, postoperative outcomes are reviewed as part of a 15-year postsurgical follow-up.

Angular dermoid cysts in the endoscopic era: retrospective analysis of aesthetic results using the direct, classic method.

The concept of the endoscopic approach was recently introduced for the removal of angular dermoid cysts. Therefore, it was considered necessary to evaluate the conventional direct incisional approach for angular dermoid cyst excision, with respect to long-term aesthetic results and complications. The need to change from the direct excisional technique to the endoscopic approach in hair-bearing areas was investigated. During a 25-year period, 95 children were surgically treated by a single surgeon (H.G.T.) for removal of angular dermoid cysts. Only 22 patients (23 percent) were available for reassessment. The follow-up periods after the excisional procedure were 1 to 12 years. Assessments were performed by a 15-member team of assessors, who scored the aesthetic results of the scars with comparative slides. In addition, a questionnaire was mailed to each family, to document the family members' perceptions of the aesthetic appearance of the scar. Twenty-eight families (29 percent) responded. The complications were determined through the medical records department. For 19 of 22 patients (86 percent), the scar was scored by 85 percent of the assessors as excellent or good. No assessor stated that a scar was unacceptable. In the family questionnaires, 26 of 28 families (93 percent) reported an excellent scar and two (7 percent) reported a fair scar. No family stated that the scar was unacceptable. The operative notes for the 95 patients revealed that only two cysts had ruptured during the surgical procedure (2 percent) and only one infection had occurred (1 percent). No other major complications were reported. It is concluded that the direct method for dermoid cyst excision is an excellent approach, with a low complication rate and a very high aesthetic success rate, when performed through a supra-eyebrow or infra-eyebrow incision.

Johanson-Blizzard syndrome: A challenge in nasal reconstruction.

Johanson-Blizzard syndrome is rare and involves multiple congenital anomalies, including bilateral absence or deficiency of the nasal alae, giving the affected infant a characteristic appearance. Two cases are described that illustrate the difficulties of nasal reconstruction in young children. Surgical options and the timing of intervention are discussed.

Le syndrome de Johanson-Blizzard est rare. Il comporte des anomalies congénitales multiples, y compris une absence bilatérale ou une anomalie des ailes du nez, qui donne à l'enfant atteint une apparence caractéristique. Deux cas sont décrits qui illustrent les difficultés de la reconstruction nasale chez les jeunes enfants. Sont examinés les possibilités chirurgicales et le moment de prévoir l'intervention.