Department of Anesthesiology, Regina Margherita Hospital, Torino, Italy
Case Report
Successful Combination Therapy for Pulmonary Hypertension in Incontinentia Pigmenti: Case Report and Review of the Literature
Author(s): Mattia Zanin*, Enrico Bonaveglio, Martina Scanu, Annalisa Longobardo, Stefania Iannandrea, Elisa Catalano, Fortunato Ciliberto F, Sergio Grassitelli, Francesca Fusco, Mirela Bojan and Simona Quaglia
Pulmonary Arterial Hypertension (PAH) is a well-known but very rare and severe complication of Incontinentia Pigmenti
(IP). Only few cases are reported in literature, most of them fatal. Recently, some reports described better
outcome with a more aggressive treatment combining multiple pulmonary vasodilators and immunomodulatory
drugs.
We present the case of a three months old female diagnosed with IP and severe PAH refractory to inhaled nitric
oxyde, admitted in cardio-pediatric ICU for mixed cardiogenic and hemorrhagic shock and treated with a combination
of high dose iNO, sildenafil, iloprost, TNF-alpha inhibitor and steroids. Following this treatment, PAP dropped
from iso-systemic to half systemic, and PVRI halved as well. After an episode of septic shock and a hospital acquired
pneumopathy from Pneumocystis jirovecii, the baby was discharged home in g.. View More»
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