Transnasal endoscopic approach to the sella turcica.

The transseptal/transsphenoidal approach to the pituitary gland has been the most commonly used approach for resection of pituitary adenomas for the last 50 years. This procedure has a low morbidity and provides direct midline access to the sella and pituitary gland. Recent advancements in endoscopic surgery, however, suggest that a lower morbidity approach to the sella would be possible via transnasal endoscopic route. Prior reports have confirmed effectiveness of this approach to the pituitary gland and we report here an early series of endoscopic transnasal pituitary surgery from our institution. We report seven cases of transnasal endoscopic pituitary surgery. Our technique consists of endoscopic exposure of the sphenoid ostium unilaterally, excision of the posterior septum anterior to the rostrum of the sphenoid sinus with resection of the sphenoid rostrum for bilateral exposure of the sphenoid sinus. A specially designed nasal speculum is positioned to displace the posterior septum and lateralize the middle turbinates, permitting direct midline exposure of the sphenoid sinus and sella. We have progressively modified the technique over the seven cases that we present and will discuss our specific instrumentation, indications, and technique for this procedure. We have encountered one cerebrospinal fluid leak in this series. Patient satisfaction has been high and hospitalization is less than with the conventional transseptal approach, averaging 1 day. Our impression is that the transnasal endoscopic approach to pituitary adenomas is a safe technique with reduced morbidity permitting shortened hospital stay.

Angiosarcomas of the head and neck: clinical and pathologic characteristics.

Between 1974 and 1992, 32 patients with pathologically diagnosed angiosarcoma of the head and neck were evaluated at our institution. The primary treatment group consisted of 24 patients who had the initial diagnosis made or confirmed at our institution, and the other 8 patients formed the salvage group. There were 23 men and 9 women. The median age in the primary treatment group was 63 years (range 18 to 91 years). The overall median survival among the primary group patients was 4.8 years, and the 3-year survival was estimated to be 57% (95% confidence interval 39% to 84%). The median follow-up was 2.1 years (range 83 days to 9.7 years). Patients who had tumors less than 7.0 cm in diameter and tumors with invasion only to the subcutaneous tissues had better overall survival and longer time to first adverse event. Diploid DNA content was a significant favorable prognostic factor for time to first adverse event. Mitotic activity was of borderline significance with both end points. Patients who had tumors of less than 1.5 cm were treated successfully with surgery alone. Patients treated with combined surgery and radiotherapy also tended to do better. Because most patients in whom regional recurrences developed had tumors larger than 7.0 cm, we conclude that patients with tumors of this size may benefit from regional neck node dissection at the time of primary excision or from elective neck irradiation.

Early speech results with the Provox prosthesis after laryngectomy.

OBJECTIVES: To assess our initial success using the Provox (Atos Medical AB, Horby, Sweden, and Entermad BV, Woerden, the Netherlands) tracheoesophageal speaking valve as the primary prosthesis to rehabilitate patients after total laryngectomy and to compare our success and complication rates with those previously reported. DESIGN: A retrospective analysis of all the patients who had a Provox prosthesis placed from November 1994 to November 1995. PATIENTS AND METHODS: Medical charts were reviewed for success or failure of the prosthesis as determined by the quality of speech and the utility of the device. In addition, complications, whether the patient had a pharyngeal myotomy and/or radiation, and the reasons and timing for replacement were assessed. Twenty-one patients had a Provox prosthesis placed either primarily at the time of their total laryngectomy (n = 8), delayed (n = 5), or as a replacement for another type of prosthesis (n = 8). Follow-up ranged from 2 to 17 months. RESULTS: The initial success rate was 84%, and the long-term success rate was 74%. Of note, 3 patients had substantially improved speech and none were worse when changed from another type of prosthesis. The mean interval to prosthesis change was 166 days. Reasons for failure included infection, radiation fibrosis, manual incoordination, cerebrovascular accident, and combination of total laryngectomy and total glossectomy. The most common complication (in 2 patients) was retraction of the prosthesis into the esophagus that was successfully managed by replacement with a longer device. CONCLUSION: Our early results confirm the effectiveness, longevity, and safety of the Provox prosthesis for speech rehabilitation following total laryngectomy.

Mucin mRNA expression in normal and vasomotor inferior turbinates.

Mucins are the major glycoprotein component of respiratory tract secretions. Little is known about their expression in the upper respiratory tract. In order to define this expression, in situ hybridization was performed on 19 normal and 4 vasomotor rhinitis (VMR) inferior turbinates to identify mucin mRNA. MUC1, MUC2, MUC4, MUC5AC, MUC5B, and MUC7 were expressed in both the normal and VMR turbinates. MUC4 and MUC5AC were the most highly expressed mucins. MUC1, MUC2, MUC4, and MUC5AC were expressed mainly by the epithelial border, whereas MUC5B and MUC7 were expressed by the submucosal glands. MUC1 and MUC4 exhibited a diffuse expression by multiple cell types along the mucosal border, whereas MUC2 and MUC5AC expression appeared to be limited to a subpopulation of epithelial cells, most likely goblet cells. Although MUC1, MUC4, and MUC5AC showed sporadic submucosal glandular expression, MUC5B and MUC7 appeared to be the predominant submucosal gland mucins in the inferior turbinates. MUC3 and MUC6 expression, which have been found primarily in the gastric mucosa, were not seen in any of the inferior turbinate samples examined. The only difference seen between normal and VMR turbinates was a slight decrease in MUC1 expression in the VMR group. The variety of mucins expressed and the diversity of their expression patterns may have significance in terms of the rheologic and particle clearance properties of nasal secretions. Understanding the expression patterns in normal turbinates will serve as the foundation for further study of these mucins in disease states.

Parathyromatosis: a rare yet important cause of persistent or recurrent hyperparathyroidism.

BACKGROUND: Parathyromatosis is described by as small nodules of hyperfunctioning parathyroid tissue scattered in the soft tissues of the neck and/or mediastinum. Seeding of hypercellular parathyroid glands during surgical excision and overgrowth of parathyroid rests left behind during ontogenesis are the most likely causes of this rare phenomenon. To characterize the clinical presentation and management of this rare condition, we report on a patient with uremia and persistent hyperparathyroidism occurring after total parathyroidectomy. METHODS: The records of one man with parathyromatosis treated from 1989 to 1993 were reviewed. In addition, a review of the literature was undertaken. RESULTS: Findings at the patient's final operation (a median sternotomy) included multiple nodules of hyperplastic parathyroid tissue scattered throughout the thymus. The characteristics of this histologically benign tissue are consistent throughout various case reports in the literature. CONCLUSIONS: This case presentation shows the physiologic significance of parathyromatosis, particularly in the setting of kidney failure. The importance of meticulous handling of potentially hyperplastic parathyroid glands and routine cervical thymectomy among this subset of patients is emphasized.

Molecular analysis of mutations in a patient with purine nucleoside phosphorylase deficiency.

Purine nucleoside phosphorylase (PNP) deficiency is an inherited autosomal recessive disorder resulting in severe combined immunodeficiency. The purpose of this study was to determine the molecular defects responsible for PNP deficiency in one such patient. The patient's PNP cDNA was amplified by PCR and sequenced. Point mutations leading to amino acid substitutions were found in both alleles. One point mutation led to a Ser-to-Gly substitution at amino acid 51 and was common to both alleles. In addition, an Asp-to-Gly substitution at amino acid 128 and an Arg-to-Pro substitution at amino acid 234 were found in the maternal and paternal alleles, respectively. In order to prove that these mutations were responsible for the disease state, each of the three mutations was constructed separately by site-directed mutagenesis of the normal PNP cDNA, and each was transiently expressed in COS cells. Lysates from cells transfected with the allele carrying the substitution at amino acid 51 retained both function and immunoreactivity. Lysates from cells transfected with PNP alleles carrying a substitution at either amino acid 128 or amino acid 234 contained immunoreactive material but had no detectable human PNP activity. In summary, molecular analysis of this patient identified point mutations within the PNP gene which are responsible for the enzyme deficiency.