Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) Syndrome in an 8-Year-Old Girl with Ganglioneuroma and SARS-COV-2 infection: A Case Report and Literature Review

Message:
Article Type:
Case Report (دارای رتبه معتبر)
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 SARS-COV-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.

Language:
English
Published:
Iranian Journal of Blood and Cancer, Volume:13 Issue: 4, Dec 2021
Pages:
144 to 147
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