How airway venous malformations differ from airway infantile hemangiomas.

OBJECTIVE: To compare airway infantile hemangiomas (IHs) and venous malformations (VMs) clinically, radiographically, endoscopically, and histologically. DESIGN: Retrospective cohort study. SETTING: Tertiary care pediatric hospital. PATIENTS: The study included patients seen in the Vascular Anomaly Clinic, Seattle Children's Hospital, Seattle, Washington, between 2001 and 2008. METHODS: All patients with airway vascular anomalies were identified by searching the Vascular Anomaly Quality Improvement Database and hospital discharge data. The data, which were analyzed with descriptive statistics and the Fisher exact test, included presenting age, sex, presenting signs, lesion site, and radiographic, endoscopic, and histologic findings.. RESULTS: Seventeen patients with airway lesions were identified, 6 with VMs and 11 with IHs. Patients with VMs presented at a mean (SD) age of 11.3 (13.7) months (age range, 3-39 months), while those with IHs presented at 3 (1.8) months of age (age range, 1-6 months) (P = .03). The patients with IHs were predominantly female (9 of 11 [81%]), while no sex difference was noted among the patients with VMs (3 of 6 [50%]). All patients with IHs presented with stridor and cutaneous lesions, whereas patients with VMs more often presented with hemoptysis or dysphagia (P = .001). Computed tomographic angiograms demonstrated enhancing endolaryngeal lesions in all IHs, while VMs enhanced poorly. Endoscopically, IHs were transglottic, while VMs were postcricoid or epiglottic (P < .001). Histologically, immunostained lesions showed submucosal lobules of capillaries lined by GLUT-1 (glucose transporter isoform 1)-positive endothelium in IHs, whereas VMs consisted of loosely organized venous channels that lacked GLUT-1 staining. CONCLUSION: Patients with airway IHs and VMs differ in presenting age and signs, sex, airway lesion location, enhancement on computed tomographic angiograms, and histologic appearance.

Nasopharyngeal airway for management of airway obstruction in infants with micrognathia.

OBJECTIVE: Describe airway management using nasopharyngeal airway in infants. DESIGN: Retrospective case series (1996 to 2006). SETTING: Tertiary pediatric hospital. PATIENTS, PARTICIPANTS: The craniofacial database of Seattle Children's Hospital was searched to identify patients with one of the following diagnoses: micrognathia, secondary cleft palate, branchial arch anomalies, Pierre Robin sequence (PRS), or velocardiofacial syndrome. Thirty-five (10.9%) of the 320 infants born between January 1, 1996, and March 31, 2006, identified using the criteria listed above were managed with nasopharyngeal airway (NPA) during infancy. INTERVENTIONS: Use of NPA. MAIN OUTCOME MEASURE: Summary statistics describing the distribution of the infants' demographic characteristics, duration, and timing of their NPA placement, need for tracheotomy, feeding interventions, and death. RESULTS: Of the 35 patients included in this case series, 60% (21) were male. Eighteen (51.4%) patients had the diagnosis of PRS, 13 (37.1%) had secondary cleft palate and other craniofacial anomalies, and four (11.4%) had branchial anomalies and micrognathia (nonsyndromic or syndromic). Thirty-one children (88.6%) were born at term. Mean and median age at initial NPA placement was 3.2 and 1.3 weeks, respectively; median duration of NPA was 8.0 weeks. Nine children received tracheotomies. Feeding tubes were required in 85.7% of patients. Two children died; however, neither death was attributed to airway obstruction or the use of NPA. CONCLUSIONS: NPA is one option in the management of patients with craniofacial anomalies and airway obstruction. The majority of nonsyndromic PRS patients treated with NPA during infancy did not require airway intervention beyond NPA.

Facial nerve rerouting in skull base surgery.

Facial nerve rerouting techniques were developed to facilitate re-section of extensive tumors occupying the skull base. Facial nerve rerouting has its own limitations and risks, requiring microsurgical expertise, additional surgical time, and often some degree of facial nerve paresis. This article presents different degrees of anterior and posterior facial nerve rerouting, techniques of facial nerve rerouting, and a comprehensive review of outcomes. It then reviews anatomic and functional preservation of the facial nerve in acoustic neuroma resection, technical aspects of facial nerve dissection, intracranial facial nerve repair options, and outcomes for successful acoustic neuroma surgery.

Metastatic melanoma in a patient with Clouston syndrome successfully treated with isolated hyperthermic limb perfusion.

BACKGROUND: Clouston syndrome is an autosomal dominant disorder characterized by nail dystrophy, partial or total alopecia, and hyperkeratosis of the palms and soles. OBJECTIVE: Although a variety of unusual cutaneous manifestations have been described, the incidence of melanoma in this population is unknown. METHODS: This article reports a case of in-transit metastatic subungual melanoma in a patient with Clouston syndrome successfully treated with hyperthermic limb perfusion with melphalan. RESULTS: Six months postperfusion, the patient is doing well, with resolution of the extremity erythema and edema. Followup ultrasonography revealed reduction in size of the eight subcutaneous nodules, with the largest measuring 3.3 cm in maximum diameter, representing nearly a 50% reduction in tumor volume postperfusion. CONCLUSION: Although melanoma has also been associated with some forms of ectodermal dysplasia, such as ectrodactyly--ectodermal dysplasia--clefting (EEC) syndrome, the incidence of melanoma in patients with Clouston syndrome is unknown. Thus far, to our knowledge, this is the first case report of melanoma arising in a patient with Clouston syndrome.

The Hunsaker Mon-Jet ventilation tube for microlaryngeal surgery: optimal laryngeal exposure.

Methods of delivering and monitoring anesthesia during microlaryngeal surgery are constantly evolving. In 1994, Hunsaker and colleagues introduced a laser-safe subglottic Mon-Jet ventilation tube, which has the ability to periodically measure end-tidal carbon dioxide levels. We conducted a retrospective review of 84 consecutive patients who had undergone microlaryngeal procedures with the aid of the Hunsaker Mon-Jet tube. Study parameters included the length of anesthetic induction and recovery times, the duration of surgery, the degree of surgical access to the larynx, and the incidence of anesthetic and surgical complications. We found that anesthetic induction and recovery times with the use of the Mon-Jet tube were comparable to those seen with standard endotracheal intubation. We also observed an apparent reduction in surgical time and a consistent subjective improvement in surgical visualization and access. The complication rate was acceptable, airway control was adequate, and use of the Mon-Jet tube was safe in all patients. We conclude that the Mon-Jet tube is a safe and effective subglottic jet ventilation system and that it has distinct advantages over other methods for both the surgeon and the anesthesiologist.