Bony syngnathia, vertebral segmentation defect, coloboma, microcephaly and mental retardation: confirmation of Dobrow syndrome and review of syndromal syngnathias

Congenital bony fusion of the maxilla and mandible is a rare condition. Two classifications were previously proposed dealing exclusively with craniofacial malformations. Most of the reported cases to date represent either aglossia-aclactylia or hemifacial microsomia syndromes. We report a young girl with bony syngnathia associated with multiple defects (severe microcephaly, coloboma, vertebral segmentation defects), growth and mental delay. This patient is very similar to the patient described by Dobrow in 1983 and confirms the existence of this extremely rare disorder. (C) 2004 Lippincott Williams Wilkins

Radical debridement and omental transposition for post sternotomy mediastinitis

OBJECTIVE: Reported mortality for postoperative mediastinitis treated by debridement alone can reach 40%. The authors' experience with radical debridement and omental transposition is reviewed. METHODS: Between May 1990 and August 1996, 14 patients with untractable mediastinitis had a transfer of the greater omentum: 11 after coronary artery bypass grafting (CABG) (6 bilateral internal thoracic arteries ITA grafts), one after a heart transplant, one after an aortic valve replacement and CABG, and one after a repair of the aortic isthmus related to a motor vehicle accident. The mean age was 63 +/- 8 years. Infection was proven in all patients by cultures of intraoperative specimens. Two patients had such a large sternal defect that no primary closure could be attempted. The remaining 12 patients had a mean of 1.4 +/- 0.7 previous debridement. Five patients had a total sternectomy. After radical debridement, the omentum was transferred over the entirety of the wound and covered with a meshed thin skin graft. All patients had a minimum of 4 weeks of i.v. antibiotic therapy. RESULTS: There was no operative death. Apart from one focal necrosis and one traumatic dehiscence of the omentum, there was no hospital complication. Sepsis was controlled in all patients. The median hospital stay was 31 days (range 20-154 days). At a median follow-up of 20 months (range: 6-44 months), there were two late deaths: one sudden and unexpected death and one after a re-do CABG. The remaining patients had resumed their previous activities. One patient had developed an incisional hernia and another underwent further surgery for cosmetic reasons. CONCLUSION: Radical debridement and omental transposition may achieve a cure for postoperative mediastinitis with good mid-term results

Six families with van der Woude and/or popliteal pterygium syndrome: all with a mutation in the IRF6 gene

Van der Woude syndrome (VWS, OMIM #119300) is a dominantly inherited developmental disorder characterised by pits and/or sinuses of the lower lip, cleft lip with or without cleft palate (CL/P), isolated cleft palate (CP), bifid uvula (BU), and hypodontia (H).1–3 Cleft lip deformity is established during the first 6 weeks of life due to failure of fusion of maxillary and medial nasal processes or to incomplete mesodermal ingrowth into the processes. Palatal clefts result from failure of fusion of the palatal shelves that normally change from a vertical to horizontal position and fuse during the sixth to ninth weeks of gestation

The versatility of the sub-mental flap: a case report.

The sub-mental flap has been used in four elderly patients (mean age: 83 years) for reconstruction of defects after oncological resection: three had basal cell carcinoma (cheek, temporal region and fronto-temporal region). One had a squamous cell carcinoma of the hard palate. We believe that the latter example is original and present it in this article. This case shows that the sub-mental flap in addition to its intrinsic qualities is a reliable flap which may be useful in difficult repairs. It can be used to repair wide palatal fistula which occurs after oncological resections