Author/Authors :
khanbabaei, ghamartaj shahid beheshti university of medical sciences - mofid children’s hospital - department of pediatric pulmonology, Tehran, Iran , tabatabaei, ahmad shahid beheshti university of medical sciences - mofid children’s hospital - department of pediatric pulmonology, Tehran, Iran , farahbakhsh, nazanin shahid beheshti university of medical sciences - mofid children’s hospital - department of pediatric pulmonology, Tehran, Iran , modarresi, zalfa shahid beheshti university of medical sciences - mofid children’s hospital - department of pediatric pulmonology, Tehran, Iran , poorghasem, matin shahid beheshti university of medical sciences - mofid children’s hospital - department of pediatric pulmonology, Tehran, Iran , tabiei, ebrahim shahid beheshti university of medical sciences - mofid children’s hospital - department of pediatric pulmonology, Tehran, Iran , taghizadeh, abdolhamid shahid beheshti university of medical sciences - mofid children’s hospital - department of pediatric pulmonology, Tehran, Iran , managheb, morteza erfan niayesh hospital, Tehran, Iran , behzad moghadam, hosein erfan niayesh hospital - neonatal and nicu ward, Tehran, Iran , vahidshahi, kourosh shahid beheshti university of medical sciences - school of medicine, modarres teaching hospital, Tehran, Iran , tabatabaei yazdi, morteza shahid beheshti university of medical sciences - pediatric congenital hematologic disorders research center, Tehran, Iran , taheri, hossein shahid beheshti university of medical sciences - student research committee, Tehran, Iran
Abstract :
Background: ROHHAD syndrome (rapid-onset obesity, hypoventilation, hypothalamic dysfunction, and autonomic dysregulation) is an extremely rare disease in children. This article describes the clinical features, laboratory findings, imaging results, and treatment of a case of ROHHAD syndrome with ganglioneuroma. Case Report: An 8-year-old girl in Covid-19 pandemic was admitted to our emergency department due to dyspnea, cyanosis, and respiratory distress. She had a history of close contact with his father (a case of covid 19) prior to hospital admission. Spiral chest CT scan of the patient revealed bilateral ground glass opacities and also a right paravertebral mass. She had a history of rapid onset morbid obesity that had started 2 years ago. Furthermore, she had sleep disturbances and hypoventilation, electrolyte imbalance, polyuria and enuresis, irritability, and impulsivity. She was diagnosed as a case of ROHHAD syndrome based on her clinical manifestations. She was also diagnosed as a case of SARSCOV- 2 infection. The paravertebral mass was resected which pathology was consistent with ganglioneuroma. Conclusion: Because of the high prevalence of morbidity and mortality in ROHHAD syndrome and accompanying neural crest tumors, early diagnosis of this syndrome should be kept in mind. It is essential to pay attention to the cases with rapid onset obesity associated with hypoventilation to prevent severe complications.
Keywords :
ROHHAD syndrome , Rapid , onset obesity , Hypoventilation , Ganglioneuroma , Covid 19
