Large vestibular aqueduct syndrome and endolymphatic hydrops: two presentations of a common primary inner-ear dysfunction?

OBJECTIVE: To present the theory that large vestibular aqueduct syndrome (i.e. the recognised existence of an enlarged vestibular aqueduct with progressive sensorineural hearing loss) and endolymphatic hydrops are due to a common primary dysfunction of inner-ear fluid homeostasis. METHOD: Case report and review of the world literature concerning large vestibular aqueduct syndrome and endolymphatic hydrops. RESULTS: We report a family in which one sibling suffered from large vestibular aqueduct syndrome while the other had classic Ménière's disease. This suggests that large vestibular aqueduct syndrome and endolymphatic hydrops, in some cases, may be due to a common primary dysfunction of inner-ear fluid homeostasis. CONCLUSION: To our knowledge, this is the first report in the world literature to postulate that variation in the relative compliance of inner-ear membranes could be the factor that determines the manifestation of the disorder as either endolymphatic hydrops or large vestibular aqueduct syndrome.

Klebsiella rhinoscleromatis of the membranous nasal septum.

Rhinoscleroma is a chronic, infectious, granulomatous disease that may present with mass lesions in the respiratory tract anywhere from the nose to the trachea. The nose is involved in 95-100 per cent of cases. There are three stages of the disease: catarrhal-atrophic, granulomatous (also known as hypertrophic) and sclerotic. The diagnosis is made either by positive Klebsiella rhinoscleromatis culture or from the classic histological findings of Mikulicz cells and transformed plasma cells with Russell bodies. Rhinoscleroma is endemic to areas of Africa, South-East Asia, Mexico, Central and South America, and Central and Eastern Europe, and it has been associated with low socioeconomic status. In the past, rhinoscleroma was infrequent in the US population but, with current trends in migration, the incidence of rhinoscleroma may be on the rise. There is often a delay in diagnosis in non-endemic areas such as the US due to unfamiliarity with the disease, the stage-dependent clinical and histological manifestations of disease, and the fact that only 50-60 per cent of cultures are positive for K rhinoscleromatis. Such late diagnosis leads to increased morbidity in the form of nasal and airway obstruction and nasal deformity from erosive processes. Rhinoscleroma is difficult to cure and prone to recur. Currently, the recommended treatment consists of a combination of surgical debridement and long-term antibiotic therapy. We present a case report of a culture-positive diagnosis of rhinoscleroma, and we review the existing literature.

Microvascular transplantation and replantation of the rabbit submandibular gland.

BACKGROUND: Xerostomia is a devastating complication of radiation therapy. Previous research has demonstrated that submandibular glands may be removed from the neck and transplanted using microvascular techniques, with good gland survival. However, microvascular transplantation and replantation has never been attempted on a composite tissue such as a salivary gland. OBJECTIVE: To evaluate the ability of a rabbit submandibular gland to undergo 2 successive microvascular transplantations. SUBJECTS AND DESIGN: Study rabbits underwent a midline neck incision with dissection of a submandibular gland to its arterial and venous pedicle. Microvascular techniques were then used to transplant the gland to the femoral system of the right groin. The incisions were reopened later under surgical conditions. The transferred gland was examined for survival and patency of its artery and vein. Healthy glands were dissected and transferred to a suitable artery and vein within the neck, where they were again reanastamosed using microvascular surgical techniques. After additional time, the gland was again examined for survival and pedicle patency, then removed and evaluated for histopathological evidence of survival. RESULTS: Surgical technique evolved during the course of this work to avoid encountered pitfalls. After refining the technique, we have determined that the rabbit submandibular gland is able to withstand successive microvascular transplantation and replantation with good likelihood of long-term survival, according to histopathological criteria. CONCLUSIONS: The rabbit submandibular gland is able to undergo microvascular transplantation and replantation with evidence of long-term survivability and preserved function. The body's natural response to surgery and tissue transplantation makes replantation a technical challenge; however, methods delineated herein alleviate many of the potential pitfalls. Extending these results to humans, patients who are to undergo radiation therapy could have a disease-free gland removed from the neck, transferred outside of the field of radiation, and then returned to the neck at the completion of radiation therapy. This may enable them to maintain salivary gland function and maintain oral cavity function and comfort.

Microvascular transplantation of the rat submandibular gland.

Xerostomia results from salivary gland irradiation during treatment of head and neck malignancies. In addition to having difficulty with speech and swallowing, these patients experience loss of taste, dental caries, and chronic fungal infections. The paired submandibular glands provide 70 percent of the normal salivary flow and are difficult to shield during radiation therapy. Another sicca condition, xerophthalmia, may result from facial nerve injury or other medical disorders and results in pain, corneal ulceration, and possible vision loss. Treatment options for xerostomia are limited, and management of xerophthalmia usually focuses on the eyelids, rather than the fundamental problem of inadequate secretory protection. In this study, a rat model for submandibular gland microvascular transplantation was developed to assess the feasibility of salivary tissue transfer. Sixteen rats underwent submandibular gland transplantation from the neck to the groin. Fourteen of these rats underwent microvascular anastomosis of the vascular pedicle. Ten glands were assessed for viability at 4 days after transplantation, and four glands were examined after 7, 10, 14, or 21 days. By gross and histologic examination, 93 percent of transplanted glands showed expected long-term viability after at least 4 postoperative days. Microvascular techniques were shown to be applicable to the transplantation of submandibular gland salivary tissue. This has not previously been shown in a rat model. It is possible that submandibular glands could be transplanted to the eye for treatment of xerophthalmia and out of the neck during irradiation of the head and neck, with subsequent replantation after treatment as a means of preventing permanent xerostomia.

An anatomical approach to glabellar rhytids.

OBJECTIVE: To identify surface landmarks that can serve as reference points to the underlying musculature in the treatment of glabellar rhytids. METHODS: Fifty cadaver hemibrows were dissected to assess the location, disposition, and relationships of the brow muscles, along with their variations at each of several consistent locations. Particular attention was paid to the corrugator supercilii, frontal belly of the frontalis, and procerus muscles. CONCLUSIONS: The information gained here may be applied to the pharmacological or surgical treatment of glabellar rhytids. Knowledge of the frequent location of the muscles involved, relative to easily identifiable surface landmarks, allows a more precise approach.

Endoscopic harvest of the gracilis muscle flap.

Endoscopic technique is becoming increasing prevalent in plastic surgery and is being explored for use in tissue harvest for microneurovascular transplantation. To advance the art truly, endoscopic technique must provide similar success and complication rates, operative time, and ease of performance as the traditional "open" surgical harvest. We report the successful microvascular transplantation in 6 patients of gracilis muscles harvested with endoscopic guidance. Dissection of the primary pedicle was done through a small proximal incision, and subsequent dissection was accomplished with an endoscope through the primary incision and a small distal incision. This method provides excellent success of transfer and decreased morbidity compared with the open technique. Specifically, the resulting scar from the endoscopic technique is considerably smaller, with just a 5-cm proximal and a 1.5-cm distal scar. The technique is accomplished with standard endoscopic equipment and is learned rapidly. We believe the improved aesthetic outcome makes endoscopic gracilis harvest a preferred technique.

Contemporary presentation and management of a spectrum of mastoid abscesses.

BACKGROUND: The incidence of complications resulting from suppurative otitis media has significantly decreased since the introduction of antibiotics. At the start of the 20th century 50% of all cases of otitis media developed a coalescent mastoiditis. By 1959, the incidence had fallen to 0.4%. Recent studies suggest a current incidence of only 0.24%. Additionally, during the time of Friedrich Bezold (1824-1908), 20% of patients with mastoiditis developed subperiosteal abscess. Interestingly, this has incidence increased; today nearly 50% of patients diagnosed with coalescent mastoiditis have subperiosteal abscess. OBJECTIVE: To review the contemporary presentation, diagnosis, and management of a spectrum of mastoid abscesses. DESIGN: Retrospective case series. SETTING: Hospitals associated with the Department of Otolaryngology/Head and Neck Surgery at the University of California, San Francisco. PATIENTS: Three patients with mastoid abscesses are reported. One patient displayed "classic" Bezold's abscess, with pus escaping the mastoid near the incisura digastrica and tracking along the digastric and sternocleidomastoid muscles into the neck. The second and third patients exhibited temporoparietal swelling secondary to mastoid abscess eroding the root of the zygomatic process, a complication noted by Bezold in 1908 as occurring "in only very rare cases." RESULTS AND CONCLUSIONS: Since only one third of patients show pathologic tympanic membrane changes, and since complaints of otalgia, fever, and tenderness are inconstant, subperiosteal mastoid abscess is frequently a delayed diagnosis. The clinical presentation, pathogenesis, and routes of abscess spread are presented with photographic and radiographic illustration. Medical and surgical management is reviewed, and methods for accurate diagnosis are emphasized.

Monoamniotic and pseudomonoamniotic twins: sonographic diagnosis, detection of cord entanglement, and obstetric management.

OBJECTIVE: To assess accuracy of detecting cord entanglement in monoamniotic twins, and to describe perinatal outcomes with aggressive obstetric management. METHODS: Seven nonconjoined monoamniotic twin pregnancies and one pseudomonoamniotic twin pregnancy were diagnosed sonographically and evaluated with serial scans and cardiotocography. In the absence of other indications, patients were delivered by elective cesarean on demonstration of lung maturity at or beyond 32 weeks' gestation. RESULTS: Cord entanglement was diagnosed correctly in four pregnancies, missed in one, and excluded correctly in three. Four pregnancies were delivered after demonstration of pulmonary maturity, three because of premature rupture of membranes or uncontrollable preterm labor, and one because of fetal heart rate abnormality during tocolysis for preterm labor. The mean gestational age at delivery was 33.2 +/- 1.6 weeks, with birth weight 2011 +/- 262 g; all neonates were live-born. Newborn stays averaged 12.0 +/- 5.8 days for the eight neonates delivered electively. CONCLUSION: Monoamniotic twin pregnancies and cord entanglement in such twins were diagnosed reliably by ultrasound. Abnormal tracings prompting cesarean delivery occurred in two of the five pregnancies with cord entanglement. Amniocentesis reflected pulmonary maturity of both twins in all pregnancies so assessed, and delivery after 32 weeks' gestation, with lung maturity, resulted in good perinatal outcomes. Statistical validity of these findings is limited by our small sample size.