Author(s)
Jenna Devare, MD
Mark Halverson, MD
Denise M. Adams, MD
Luv R. Javia, MD
Affiliation(s)
Children's Hospital of Philadelphia;
Abstract:
Introduction: Kaposiform lymphangiomatosis (KLA) is a rare, aggressive complex lymphatic anomaly resulting from clusters of spindled lymphatic endothelial cells referred to as “kaposiform” that are accompanied by malformed lymphatic channels. Patients commonly present with thoracic involvement causing pleural or pericardial effusions. Overall survival rate has increased from 34% to 79% likely due to newer therapies; mortality is typically due to cardiorespiratory failure or coagulopathy. The otolaryngologic manifestations of KLA have not been well described, and temporal bone involvement has not previously been reported.
Objective: To describe the otologic radiologic manifestations of KLA in the temporal bone and provide considerations for management in this population.
Case Description: A 5-year old boy with KLA and identified NRAS mutation presented for consideration of adenotonsillectomy due to concern for KLA involvement of the tonsils. On exam, he was found to have a clear left middle ear effusion with an accompanying moderately severe rising to mild conductive hearing loss on audiogram. On the day of adenotonsillectomy, he underwent a left myringotomy complicated by persistent clear otorrhea concerning for cerebrospinal fluid (CSF) leak. The fluid was collected for beta-2 transferrin testing, and the leak was controlled with gelfoam packing of the external auditory canal.
Results: Beta-2 transferrin testing was positive for CSF. Noncontrast computed tomography of the temporal bones showed extensive multifocal osseous erosion and rarefaction of the left temporal bone including the middle cranial fossa floor. MR imaging including cisternogram was subsequently performed, showing multiple areas of CSF leak including the left cavernous sinus, left petrous bone and middle ear, and left carotid canal extending to the neck. The patient was recommended to obtain pneumococcal vaccines for meningitis prophylaxis.
Discussion: Our radiologic findings represent a new otologic manifestation in this patient population. CSF leak may be temporized following limited myringotomy, however definitive surgical management may not be feasible given the extent of disease.