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BACKGROUND: Plexiform neurofibromatosis is a feature of von Recklinghausen's disease. Head and neck lesions may produce varying degrees of cosmetic and functional deformity. However, life-threatening hemorrhage into facial plexiform neurofibromas has not been previously reported. METHOD: We report two patients with von Recklinghausen's disease who experienced massive hemorrhage into facial neurofibromas, one following a blunt injury and the other without a known initiating event. RESULTS: Conservative management did not stop the hemorrhage into facial neurofibromas in either instance. Surgical exploration was mandated for hemorrhage control and evacuation of blood clots. Hemostasis was attained by a combination of hypotensive anesthesia and chromic catgut suture ligatures. CONCLUSIONS: These case reports demonstrate a potentially lethal complication in patients with facial plexiform neurofibroma. Where a competent and experienced interventional neuroradiologist is not available, surgical exploration should be undertaken to control bleeding.

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True hemimandibular hyperplasia is an uncommon maxillofacial deformity. Patients with this affliction present clinically with varying degrees of asymmetry characterized by an increase in ramus height, a rotated facial appearance with kinking at the mandibular symphysis, and prominence of the lower border of the mandible. In the advanced form, maxillary and mandibular alveolar bone overgrowth result in a compensatory canting of the occlusal plane and a significant functional malocclusion requiring bimaxillary surgical correction. Nine patients with the above condition were treated with various surgical methods over a 9-year period. The clinical results were satisfying and the long-term results showed good postoperative stability. The pathology of hemimandibular hyperplasia is described and the preexisting nomenclature further defined.

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In this report we describe three very different and interesting complications of the Le Fort I osteotomy. The first case is of a unilateral third nerve palsy developing as a result of a carotid-cavernous fistula and false aneurysm of the internal carotid artery. The second complication to be reported is total unilateral blindness. The third case is one of total avulsion of a lateral segment of the palate in a patient with a complete bilateral cleft of the primary and secondary palates. All complications were the result of a Le Fort I osteotomy performed for correction of midface hypoplasia secondary to congenital cleft lip and palate. The literature is reviewed, and the pathomechanics of each injury are hypothesized.

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A retrospective review of 146 cases with Le Fort I osteotomy performed between 1981 and 1990 is presented. A complication of 6.1% was recorded. These complications include intraoperative hemorrhage, trigeminal nerve injury, oronasal fistula and dental injury. Although the Le Fort I osteotomy has proven to be relatively safe, improvements in technique will further reduce the incidence and the severity of complications.

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Polyotia is an extremely rare congenital anomaly of the external ear. Only four case reports are documented in the literature. A 13-month-old child with a mirror-image duplication of the left auricle was operated on in a one-stage corrective procedure. The duplicated portion of the affected ear was positioned anteriorly and measured approximately two-thirds the size of the posterior, normally oriented portion. There were two fully developed conchae and one fully developed and one partially developed anthelix and lobule. The entire complex had a single external auditory meatus and no tragus. There were no other congenital anomalies, and the child was developmentally normal. The technique for surgical correction is described, and a review of the literature is presented.

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Deficiency of the free border of the lip is frequently encountered in secondary cleft lip deformities. The free border of the lip consists of the orbicularis oris marginalis muscle and its overlying subcutaneous tissue, vermilion, and mucosa. The pathology may involve any or all of these components. When there is an absolute shortage of subcutaneous tissue or orbicularis oris marginalis muscle, extra tissue from another source must be used for augmentation. A temporoparietal fascial graft is a simple and reliable method to increase the fullness of the free border of the lip. The tissue is soft in consistency and displays minimal resorption. Most important, more complicated procedures such as an Abbé flap or tongue flap may be avoided. From July of 1988 until June of 1992, the temporoparietal fascial graft was used successfully to correct deficiencies of the free border of the lip in 20 patients with cleft lip deformities. There were two graft exposures that healed with conservative treatment. The procedure is a useful method of augmenting both lateral lip and central lip deficiencies in secondary and tertiary cleft lip deformities.

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Topical application of putrescine, a transglutaminase inhibitor, for 3 days directly to rat skin wounds produced a significant average decrease of 48 percent in wound breaking strength in test animals from 8 pairs studied between day 5 and day 10 after wounding. No external or systemic toxic effects of putrescine were seen with localized topical application of 50 mM putrescine for 3 days in any of the test rats (n = 12), and no systemic toxicity was seen in rabbits (n = 4) after topical exposure to 50 mM putrescine for 3 weeks. Quantitation of tritiated fucose incorporation in rat wound explants from 10 pairs of rats revealed that a significant overall decrease in radiolabeled glycoprotein production of 23 percent occurred when putrescine was present; in addition, the fraction of tritiated glycoprotein which was soluble in buffer was significantly increased, while that in the buffer-insoluble fraction decreased. This study suggests that putrescine inhibits tissue transglutaminase-mediated cross-linking of fucoprotein in the extracellular wound matrix and supports a role for this process in the generation of incisional wound strength.

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