title: Cranial nerve VI palsy as an initial presentation of necrotizing sarcoid granulomatosis in a 14-year-old female: case report and literature review.
abstract: OBJECTIVE
  We describe a case of pediatric necrotizing sarcoid granulomatosis (NSG) presenting with right cranial nerve VI palsy and multiple lung nodules, successfully treated with corticosteroids.
  
  
  METHODS
  This is a descriptive case report of one patient with review of the literature.
  
  
  RESULTS
  A 14-year-old Caucasian female presented with complaints of pain on inspiration and dyspnea on exertion, as well as diplopia that was worse with right gaze. The patient presented to our emergency department with persistent diplopia and was found to have stable right cranial nerve VI palsy. CTA showed multiple pulmonary nodules. Despite continued extensive multispecialty work-up, the patient׳s cranial nerve VI palsy had not resolved, thus tissue confirmation via lung biopsy was performed. Pathologic diagnosis revealed necrotizing sarcoid granulomatosis. The patient was subsequently started on intravenous corticosteroids, which led to the rapid resolution of her presenting symptoms.
  
  
  CONCLUSIONS
  Necrotizing sarcoid granulomatosis is a multisystem organ disease that is rare in children. Pathology commonly reveals epithelioid noncaseating granuloma and granulomatous vasculitis with necrosis. We report an unusual presentation involving sixth nerve palsy in a 14-year-old girl. Diagnosis was determined and confirmed by histopathology of a pulmonary nodule biopsy. This is the first case to our knowledge of NSG presenting with cranial nerve palsy in a pediatric patient.
authors.affiliation: Department of Pediatrics/Medical Education, Connecticut Children׳s Medical Center, Medical Education, University of Connecticut, 4H, 282 Washington St, Hartford, CT 06106. Electronic address: mdoss@connecticutchildrens.org.
authors.forename: Mona
authors.initials: M
authors.lastname: Doss
authors.affiliation: Department of Pathology, Hartford Hospital, Hartford, CT.
authors.forename: Ronald
authors.initials: R
authors.lastname: Araneta
authors.affiliation: Department of Pathology, Hartford Hospital, Hartford, CT.
authors.forename: Mary
authors.initials: M
authors.lastname: Fiel-Gan
authors.affiliation: Department of Pediatric Rheumatology, Connecticut Children׳s Medical Center, University of Connecticut, Hartford, CT.
authors.forename: Barbara
authors.initials: B
authors.lastname: Edelheit
mesh_terms: D020434:Abducens Nerve Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001706:Biopsy; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D055613:Multiple Pulmonary Nodules / Q000473:pathology; D017565:Sarcoidosis, Pulmonary / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones
keywords: Cranial nerve; Necrotizing sarcoid granulomatosis (NSG); Pediatric; Pulmonary nodules