Extranodal Rosai-Dorfman Disease of the Orbit: Clinical Features of 8 Cases.

PURPOSE: To report on the presentation, radiography, histology, and treatment of 8 cases of extranodal Rosai-Dorfman disease involving the orbit. METHODS: Multicenter retrospective case series. RESULTS: Five males and 3 females had a median age of 10 years (range 2-78 years). Presenting signs and symptoms included proptosis, periorbital pain, palpable mass, blepharoptosis, decreased vision, diplopia, impaired extraocular motility, and afferent pupillary defect. Four patients had bilateral orbital disease, while 4 had unilateral disease. Six cases were extraconal, 1 was intraconal, and 1 was both intra- and extra-conal. Four cases had only extranodal disease without lymphadenopathy (3 of which had localized orbital disease). Diagnosis was confirmed by exam, orbital, and/or systemic radiography, and biopsy in all cases. Treatment strategies included excision or debulking, systemic corticosteroids, chemotherapy, radiotherapy, observation or a combination thereof. At last follow up, 4 patients were disease free, while 4 had residual improved disease. CONCLUSIONS: Rosai-Dorfman disease of the orbit is a rare clinical entity. Purely extranodal disease is rare, with isolated orbital disease being exceedingly rare. This study is unique in that 4 of 8 patients had strictly isolated extranodal disease of the orbit. A large majority of the cases had disease in the extraconal space, contrasting with previous reports. In addition, lacrimal gland disease, particularly bilateral involvement, was prominent in the current study. Although there is no consensus on treatment, surgical excision should be attempted if plausible in symptomatic patients especially if the orbit represents a localized site of disease.

Atypical idiopathic orbital inflammation in a young girl.

A 7-year-old girl presented with chronic right periocular pain worst in abduction, edema, and proptosis with radiographic evidence of right medial rectus myositis. After a negative workup, she was diagnosed and treated for idiopathic orbital inflammation (IOI) with a brisk response to oral steroids. She returned 8 months later with left periocular pain worst in abduction, proptosis, and radiographic evidence of left lateral rectus myositis. She was treated for recurrent and alternating IOI with intravenous (IV) glucocorticoids with resolution. One year later, she recurred with similar left orbital signs and symptoms and was found to have a left inferior oblique myositis on MRI. She was treated with oral steroids and methotrexate with clinical resolution. Ten months later, she presented with left periocular pain worst on adduction and was found to have left medial rectus myositis on MRI. She was treated with IV steroids, IV immunoglobulin, and rituximab with resolution of all symptoms. She is currently maintained on methotrexate and oral glucocorticoids. The atypical pediatric case of IOI over a 4-year period is described in this study.

Early nasal continuous positive airway pressure and necrotizing enterocolitis in preterm infants.

BACKGROUND: The use of early nasal continuous positive airway pressure (ENCPAP) as the mode of initial respiratory support for very low birth weight (VLBW) infants has been increasing. The impact of CPAP and oxygen on gut mucosa and perfusion in premature infants is not known. The relation between ENCPAP and necrotizing enterocolitis (NEC) has not been adequately addressed. OBJECTIVE: To evaluate if the use of an individualized respiratory management strategy encouraging the use of ENCPAP is associated with an increased risk of NEC, and to determine risk factors for NEC in premature infants supported by CPAP. METHODS: A retrospective analysis was conducted on VLBW infants (birth weight < 1500 g) managed at 2 institutions that use an ENCPAP respiratory management strategy for premature infants. Data on the use of oxygen and mode of ventilatory support were collected during the first 3 days of life. Diagnosis of NEC was used as the dependent variable in a logistic regression model. Birth weight, gender, prenatal steroid use, mode of respiratory support (CPAP versus ventilator) and fraction of inspired oxygen, umbilical artery catheter placement, partial pressure of oxygen, patent ductus arteriosus, early sepsis, hospital, and delivery room management (ENCPAP versus initial intubation) were controlled for in the model. RESULTS: Data on 343 premature infants were collected for this study. Mean birth weight was 999 +/- 289 g and gestational age was 28 +/- 2.6 weeks. The majority of patients were managed with ENCPAP, with only 13% of patients intubated in the delivery room. The overall incidence of NEC was 7% (n = 24). The exposure to ENCPAP did not increase the risk for NEC compared with the use of a ventilator. CONCLUSIONS. The risk of NEC in VLBW premature infants was not increased by the use of ENCPAP. Initial respiratory support with ENCPAP seems to be a safe alternative to routine intubation and mechanical ventilation in premature infants.