Heterotopic neuroglial tissue causing airway obstruction in the newborn.

BACKGROUND: Heterotopic neuroglial (brain) tissue is a rare cause of airway obstruction in newborns. Fewer than 30 cases have been reported in the English literature. Brain heterotopias can mimic more common congenital anomalies of the head and neck. OBJECTIVE: To review our experience in the diagnosis and treatment of children with heterotopic pharyngeal neuroglial tissue. DESIGN: Case series. SETTING: Tertiary care children's hospital. PATIENTS: Four newborns with airway obstruction caused by heterotopic neuroglial tissue. RESULTS: All patients were infants (3 full-term girls and a 32 weeks' gestation boy) who had airway obstruction in the newborn period. All patients underwent preoperative computed tomography and magnetic resonance imaging, which revealed a heterogeneous mass involving the pharynx, neck, and parapharyngeal space. Bony deformities of the skull base and mandible were present in all patients, although intracranial connection was absent. Multiple surgical procedures were performed in all 4 patients. Tracheotomy was performed in 2 patients, gastrostomy tube placement was required in 3, and a nasopharyngeal tube was used in 1. Combined cervicofacial and transoral approaches were used for resection, preserving vital structures. Histopathologic evaluation revealed mature glial tissue and choroid plexus-like structures. CONCLUSIONS: Heterotopic neuroglial tissue must be considered in the differential diagnosis of airway obstruction in the newborn. Management is surgical resection, with attention to vital structures and function-analogous to surgery for lymphangioma. Multiple surgical procedures might be necessary in the treatment of these patients.

Paranasal sinus development and choanal atresia.

BACKGROUND: Although the determinates of paranasal sinus development and sinusitis are not well defined, a candidate factor is blockage of the choana. HYPOTHESIS: Maxillary sinuses ipsilateral to unilateral choanal atresia are comparatively small and have more evidence of sinusitis than do the contralateral sinuses. DESIGN: Retrospective. SETTING: Children's hospitals. PATIENTS: Sixteen nonsyndomic children with isolated unilateral congenital choanal atresia. MAIN OUTCOME MEASURES: Determination of maxillary sinus volumes and mucoperiosteal thickening on preoperative computed tomograms. RESULTS: Maxillary sinuses ipsilateral to unilateral choanal atresia have slightly larger volumes than, and mucoperiosteal thickening that is similar to, the contralateral sinuses. CONCLUSION: These data suggest that maxillary sinus development and sinusitis are independent of posterior nasal ventilation and drainage.

Gender and intra-observer agreement about laryngoscopy of papilloma.

BACKGROUND: The gender of the observer may bias data. OBJECTIVE: Compare the intra-observer agreements of male and female pediatric otolaryngologists about videotaped images of laryngeal papilloma. DESIGN: Five male and six female pediatric otolaryngologists independently viewed videotapes of ten children undergoing treatment for laryngeal papilloma. Each of 12 anatomic sites was categorized as disease present, absent, or indeterminate. Each observer estimated the percent overall airway obstruction. 5-24 weeks later, each observer repeated his/her assessments. RESULTS: The mean intra-observer agreements for both male and female pediatric otolaryngologists were good, and identical (kappa 0.63; proportion of positive agreement 0.82; proportion of negative agreement 0.72). Females more frequently categorized a site as indeterminate. Males more frequently categorized a site oppositely on repeat assessment. The males' indeterminate/opposite ratio was less than that of the females' (P = 0.03). Intra-observer estimates of overall airway obstruction have wide variability: for male pediatric otolaryngologists, differences exceeding 30% are significant; for females, 40%. CONCLUSION: Male and female pediatric otolaryngologists had equally good and identical intra-observer kappa scores and proportions of positive and negative agreement. However, males used the indeterminate category less than did the females, and males more often gave an opposite categorization at the second viewing session. Estimates of overall airway obstruction have much intra-observer variability.

Resuscitation of the newborn with airway compromise.

Respiratory problems in the newborn period are a diagnostic and therapeutic challenge. The goal of expeditious airway establishment may not be straightforward. Familiarity with features of the normal and abnormal infant airway anatomy, physiology, as well as intubation equipment and alternatives to intubation is essential. A systemic approach provides clues that are helpful in the successful management of the neonate in respiratory distress.

Atypical presentations of actinomycosis.

BACKGROUND: Actinomycotic infections of the cervicofacial region are uncommon. Most major medical centers report approximately one case per year. Presenting clinical manifestations are confusing because they often mimic other disease processes. Diagnosis may be difficult due to a general lack of familiarity with the disease and the fastidious nature of the organism in culture. The cervicofacial manifestations of actinomycosis are varied, and a high index of suspicion is required to make an accurate and timely diagnosis. METHODS: Retrospective chart review with the presentation of four unusual cases of actinomycosis were performed. RESULTS: Two patients were initially seen with dysphagia from a tongue base mass. The third patient was initially seen with a 3-week history of worsening hoarseness and stridor. Examination revealed an ulcerative lesion of the left hemilarynx and pyriform sinus. All three patients were thought to have a neoplastic process. Diagnosis was made on histologic examination of a tissue biopsy. The fourth patient was initially seen with a buccal space mass that was draining externally. Culture of the purulent drainage revealed Actinomyces. In all four cases, symptoms resolved after appropriate antimicrobial therapy. CONCLUSIONS: Actinomycosis of the head and neck, although rare, is an important entity to the otolaryngologist. A confusing clinical presentation combined with the fastidious nature of the organism make for a difficult diagnosis. A high index of suspicion is required to make an accurate diagnosis and institute the appropriate antibiotic therapy.

Multicentric infantile myofibromatosis.

Infantile myofibromatosis (IM) is a rare tumor of infancy and childhood, typically presenting as a firm, nodular mass involving soft tissue, bone or viscera. Approximately one-third of cases involve the head and neck. These tumors can be solitary or multicentric. Biopsy reveals tumor cells that resemble myofibroblasts. Spontaneous regression may occur. A high degree of suspicion is necessary to differentiate this entity from other more aggressive processes histiocytosis, fibrosarcoma, rhabdomyosarcoma. We describe the case of a male infant with multicentric myofibromatosis, presenting with multiple thoraco-abdominal subcutaneous nodules and lytic mass lesions of the temporal bone and calvarium. The characteristic clinical, radiologic and histopathologic features of this process are reviewed along with diagnostic and therapeutic options.