Author/Authors :
Reppucci, Diana Division of Respiratory Medicine - Department of Pediatrics - The Hospital for Sick Children, Toronto, Canada , Medin, Debra Division of Respiratory Medicine - Department of Pediatrics - The Hospital for Sick Children, Toronto, Canada , Al-Saleh, Suhail Division of Respiratory Medicine - Department of Pediatrics - The Hospital for Sick Children, Toronto, Canada , Jane Smith, Mary Janeway Children’s Health and Rehabilitation Centre, St. John’s, Canada , Barter, Jill Janeway Children’s Health and Rehabilitation Centre, St. John’s, Canada , Amin, Reshma Division of Respiratory Medicine - Department of Pediatrics - The Hospital for Sick Children, Toronto, Canada
Abstract :
We present a case of a 15-month-old boy with Cornelia de Lange Syndrome (NIPBL gene mutation). On a PSG, central sleep
apnea (central apnea-hypopnea index of 19/hour) and nocturnal hypoventilation (transcutaneous CO2 > 50 mmHg for 53% of the
night) were found. A positive pressure initiation study was aborted because the patient developed a serious adverse reaction. The
differential diagnosis included a skin fragility condition versus an allergic contact dermatitis to the interface; this could be from the
povidone-iodine solution used to clean the NiPPV interface or from the plastic of the interface itself. A skin biopsy was performed
which was normal. The reaction was likely secondary to an allergic contact dermatitis from the povidone-iodine solution used to
clean the NiPPV interface. The patient is currently tolerating NiPPV.
