Faculty Bibliography

Sialorrhea (drooling) is most commonly seen in children with cerebral palsy or mental retardation. Surgical procedures for the control of sialorrhea include salivary gland excision, parasympathetic nerve section, and salivary duct ligation and/or rerouting. Eighteen children between the ages of 5 and 17 years underwent bilateral submandibular gland excision and rerouting of Stensen's duct (Wilkie procedure). All children had severe drooling associated with cerebral palsy or mental retardation. Follow-up at 7 years showed satisfactory control of sialorrhea in 16 of 18 patients (89%). There was one major complication: xerostomia. Our results indicate that submandibular gland excision together with parotid duct retropositioning provides effective control of sialorrhea in most cases. Unfavorable head and mandibular posturing seemed to cause persistent sialorrhea in one case

Although spontaneous recovery of denervated facial muscles has been anecdotally recorded in the clinical setting, it has never been fully documented. The establishment of anastomoses between the terminal trigeminal and facial nerves provides a possible explanation of this phenomenon. Mechanisms of myoneurotization have also been described, by which regenerating branches of severed peripheral motor nerves directly reach motor end plates of denervated muscles, with variable recovery of function. A case demonstrating unequivocal clinical evidence of trigeminal-facial cross-innervation is presented, and the pertinent literature is reviewed as it applies to the mechanisms of this phenomenon

Osteogenesis imperfecta type I is a mild, dominantly inherited, connective tissue disorder characterized by bone fragility. Mutations in type I collagen account for all known cases. In Mov-13 mice, integration of a murine retrovirus within the first intron of the alpha 1(I) collagen gene results in a null allele blocked at the level of transcription. This study demonstrates that mutant mice heterozygous for the null allele are a model of osteogenesis imperfecta type I. A defect in type I collagen production is associated with dominant-acting morphological and functional defects in mineralized and nonmineralized connective tissue and with progressive hearing loss. The model provides an opportunity to investigate the effect of a reduced amount of type I collagen on the structure and integrity of extracellular matrix. It also may represent a system in which therapeutic strategies to strengthen connective tissue can be developed.

The speech perception abilities of 15 patients were measured preoperatively using hearing aids and postoperatively using the Nucleus 22-channel cochlear implant over a period of 1, 2, or 3 years. Analysis of mean data revealed that, although the greatest amount of improvement in speech perception scores occurred between the preoperative and 3-month poststimulation evaluation, there was also significant improvement in perception of segmental features and open-set speech recognition over the 3-year time period. When individual patient data were examined, however, it was clear that these improvements were due, in large part, to the performance of a subset of patients who had measurable open-set speech recognition abilities at the time of their 3-month, poststimulation evaluation. Subjects who used the processing scheme that included coding of F1 showed significantly more improvement over time than subjects who used the original F0F2 processing scheme exclusively. It was concluded that open-set speech recognition ability at 3 months is an important prognostic indicator of continued improvement in speech perception abilities over time

Although neurosurgeons have traditionally preferred intracranial repair for the management of cerebrospinal fluid (CSF) fistulas, this approach is associated with the complications of a craniotomy, anosmia, and a high incidence of recurrent fistulas. Extracranial repair, on the other hand, produces no central nervous system morbidity, preserves olfaction, and is associated with a low incidence of recurrence. Although there have been several reports of extracranial repair of CSF fistulas by otorhinolaryngologists, this approach has received scant mention in the neurosurgical literature. We report here our experience with 37 patients with CSF rhinorrhea or otorrhea who underwent extracranial repair. The etiology of the fistula was postoperative in 22, traumatic in 6, and spontaneous in 9. The fistulas were repaired using one of four techniques: external ethmoid-sphenoid in 18 patients, transmastoid in 9, transseptosphenoid in 7, and osteoplastic frontal sinusotomy in 3. In 32 of the 37 patients (86%) the fistulas were successfully repaired with the initial procedure. Of the 5 patients requiring a second operation, the fistula was successfully closed in 4 for an overall success rate of 97%. Complications were few and consisted of a transient facial paresis in a patient undergoing transmastoid repair and one death from meningitis. The authors conclude that because of low morbidity and mortality and a high success rate in closing fistulas, extracranial repair is the preferred technique for the operative management of CSF rhinorrhea and otorrhea.

We describe a case of fibrous histiocytoma of the trachea diagnosed in a 17-year-old female who presented with symptoms of 'asthma'. Management included rigid bronchoscopy with biopsy and debulking of this obstructing tumor, later excised with partial tracheal resection. Although tracheal tumors are quite rare in children, the majority (6/9) of reported cases of fibrous histiocytoma of the trachea have been described in the pediatric age group. The possibility of a tracheal neoplasm as a cause of wheezing, stridor or hemoptysis in children should be recognized. Control of the airway without tracheotomy may facilitate surgical cure via tracheal resection in such cases.

Reconstruction of the tumor-ablated patient remains one of the most challenging problems for the head-and-neck surgeon. Various methods have been described, including alloplastic reconstruction, free vascularized flaps, osteomyocutaneous flaps, and allogeneic reconstruction with particulate marrow grafts. Sixteen patients who have undergone homograft mandibular replacement at the Yale New Haven Hospital are described. Eighty-one percent of these grafts were successful in restoring form and function to a high degree of patient satisfaction. Eleven of the sixteen patients had radiation therapy after initial tumor surgery; 91% of these grafts were successful

Reconstruction of columellar defects continues to be a surgical challenge. We present the results of two child-abuse patients who were successfully treated with a technique in which medially based bilateral horizontal nasolabial flaps were used to reconstruct total columellar loss. Our technique provides adequate tissue with minimal cosmetic deformity. It also obviates the need for multiple staged procedures. We recommend the use of this method for repair of either partial or total columellar defects secondary to trauma or infection.