Otolaryngologic manifestations of Rosai-Dorfman Disease.

PURPOSE: To describe an unusual head and neck occurrence of Rosai-Dorfman Disease (RDD) and to review the otolaryngologic manifestations of this rare entity. METHODS: A case presentation with review of the literature describing Rosai-Dorfman Disease and its head and neck involvement. SETTING: A tertiary care, urban children's hospital. RESULTS: This is the first description, to the best of our knowledge, of RDD [Sinus Histiocytosis with Massive Lymphadenopathy (SHML)] involving bilateral external auditory canals and middle ear in a 12 year old patient previously diagnosed with 'asthma' and hearing loss. This patient also had extensive involvement of the tracheobronchial tree. Representative clinical, radiographic and histological findings are presented. Its etiology, diagnosis and management are also reviewed. CONCLUSION: This is the first reported case of middle ear and external auditory canal involvement of RDD in a patient with hearing loss and associated tracheobronchial lesions. RDD, although rare, may be considered in the differential diagnosis of unusual histiocytic lesions of the external auditory canal, especially with findings of similar or comparable lesions involving the respiratory tract. Confirmation is with identification of emperipolesis and appropriate immunohistochemical staining (S-100 positive, CD-68 positive and CD-1a negative). Intervention is recommended in cases where there is increased risk of mortality, as in severe obstruction of the tracheobronchial tree. Otherwise, since these lesions are self-limiting, the patients can be observed closely.

Unilateral conductive hearing loss secondary to a high jugular bulb in a pediatric patient.

A high jugular bulb is not an uncommon otologic anomaly. It may be noted as an incidental finding on physical exam, middle ear surgery, or computed tomography of the temporal bones. Frequently the patient is asymptomatic, but a high jugular bulb can occasionally cause tinnitus or conductive hearing loss. The case of a seven-year-old black male with unilateral conductive hearing loss secondary to a high jugular bulb is presented. The diagnosis, differential diagnosis, and management of a conductive hearing loss associated with a high jugular bulb are discussed.

Pediatric tracheotomy discharge teaching: a comprehensive checklist format.

Discharge planning for a child undergoing a tracheotomy is a complex process. In 1989, a multidisciplinary team at Children's Hospital of Michigan developed specific discharge criteria in a checklist format to address all facets of home care for these patients. We present and discuss the checklist. A survey of user satisfaction with the checklist demonstrated that 80% of parents and care-givers felt well-prepared by this format at the time their child was discharged from the hospital with a new tracheotomy. We conclude that the protocol allows for comprehensive and efficient discharge teaching of parents and care-givers for children with new tracheotomies.

The incidence of gastroesophageal reflux in recurrent croup.

The incidence of gastroesophageal reflux in recurrent croup was evaluated by a retrospective analysis of patients at the Children's Hospital of Michigan from 1986 to 1991. Sixty-six patients required hospitalization for recurrent croup during this 6-year period. Of the patients evaluated, 47% with recurrent croup had an additional diagnosis established of gastroesophageal reflux. In patients with three or more hospitalizations for croup, there was a 63% association with gastroesophageal reflux. Compared to all patients with recurrent croup, the patients with gastroesophageal reflux tended to be younger and had a shorter interval between episodes of croup. A prospective series of six cases of recurrent croup was seen in consultation. The diagnostic procedures involving lipid-laden macrophage quantitation, endoscopy, and gastroesophageal reflux scintiscans were utilized to establish the diagnosis of gastroesophageal reflux with tracheal aspiration. The results of these studies and the follow-up is discussed.

The enlarged vestibular aqueduct syndrome (EVA syndrome).

The presentation to the Department of Pediatric Otolaryngology at the Children's Hospital of Michigan of a series of patients with sensorineural hearing loss and enlargement of the vestibular aqueduct prompted exploratory tympanotomy in three patients (two unilateral and one bilateral), for a total of four ears. These explorations were prompted by progression and/or fluctuation of hearing levels. The discovery of abnormal round windows in all four ears with a post-traumatic fistula present in one ear suggested the presence of a new association. A previously undescribed association of an enlarged vestibular aqueduct, sensorineural hearing loss and round window abnormality with potential fistula formation was identified. A review of the anatomy and physiology, literature review, and a prospective analysis with discussion of eight patients with enlarged vestibular aqueduct syndrome evaluated and treated at Children's Hospital of Michigan, is presented. We conclude that all children with sensorineural hearing loss should undergo extensive evaluation to determine etiology, including radiographic studies of the temporal bone. Further, the presence of an enlarged vestibular aqueduct should prompt the otolaryngologist to consider the presence of a round window abnormality and the potential for predisposition to perilymph fistula.

An analysis of the inferior based tracheal flap for pediatric tracheotomy.

In the past, various tracheotomy incisions have been used at the Children's Hospital of Michigan with the occurrence of complications related to accidental decannulation and immediate recannulation. Since that time the inferior based tracheal cartilage flap has been used to minimize early complications. A retrospective study of 126 pediatric tracheotomies performed at the Children's Hospital of Michigan from June 1986 to January 1991 was reviewed. Only tracheotomies performed by a staff otolaryngologist utilizing the inferior based tracheal cartilage flaps were reviewed. This study includes patients with a 6 month to 5 year follow up. The early complication rate was 4%, while the late was 50%. Stomal granulation tissue was comparatively increased in this series of patients but did not hinder decannulation. We consider the use of the inferior based tracheal cartilage flap in the pediatric population a safe and effective technique without increasing the morbidity of long-term tracheotomy.

Congenital subglottic hemangioma: frequency of symmetric subglottic narrowing on frontal radiographs of the neck.

OBJECTIVE: This study was undertaken to determine how often symmetric subglottic airway narrowing is present in cases of infantile subglottic stenosis and to determine if the radiographic finding has any association with the anatomic location of the hemangioma. MATERIALS AND METHODS: All cases (n = 12) of endoscopically proved subglottic hemangioma from 1976 to 1991 were collected from the records at Children's Hospital of Michigan. In 10 cases, high-kilovoltage magnification studies or frontal radiographs of the neck were available for review by two observers who classified the subglottic narrowing as either symmetric or asymmetric. The radiographic findings in these 10 cases were then compared with the location and extent of the lesion as described endoscopically. RESULTS: In 50% of cases (n = 5), narrowing of the subglottic airway was symmetric. In four of these the hemangioma was either situated on the posterior wall or was circumferential, and in the remaining one an associated marked fibrotic reaction to a lateral wall lesion was present. All other lesions were on the lateral wall, and asymmetric subglottic airway narrowing was consistently shown on radiographs. CONCLUSION: Our results show that subglottic hemangioma often manifests as a symmetric subglottic airway narrowing and that the anatomic location of the hemangioma appears to be associated with the appearance on radiographs.

Postoperative technetium scanning in patients with submandibular duct diversion.

Submandibular duct diversion is a common procedure for refractory sialorrhea in children. The procedure reroutes Wharton's ducts from the floor of the mouth to the tongue base. As the majority of saliva in the resting state is produced by the submandibular glands, rerouting markedly decreases sialorrhea. However, the procedure has been criticized in that diversion may cause fibrosis and stricture of the ducts. The gland would atrophy, and the physiologic functions of saliva would be lost. Glandular function of six patients with cerebral palsy (mean age, 14.7 years) was evaluated by technetium scanning (mean time after surgery, 43 months). Four patients had normal bilateral function; two patients had no function in one gland but normal function in the contralateral gland. We conclude that bilateral submandibular duct diversion maintains long-term function in at least one gland.