A rare case of dandy-walker syndrome with bilateral choanal atresia: a case report

Abstract

Author(s): Akram Hemmatipour, Maryam Nikbina, Zahra Safari Dehkohneh, Ali Hatami, Khadijeh Asadi Chahar Rah-e Gashin

Introduction: Dandy-Walker syndrome (DWS) is a congenital neurological disorder that is characterized by a triad that includes complete or partial agenesis of the cerebellar vermis, torcular herophili, and so on. Choanal atresia is the obstruction or narrowing of the posterior nasal cavity. This disease may be congenital or acquired. Methods: Considering the referral of the patient to Al-Hadi hospital in Shoushtar, the required data were collected through examination, tests, and documents in the medical records. Results: The patient was a boy born at the 39th week of gestation via cesarean section because the ultrasound findings showed dilatation and enlargement of the fourth ventricle along with hydrocephalus. The patient was admitted with diagnosis of DWS with bilateral choanal atresia, the absence of testis descent and the possibility of cardiac anomalies of hydrocephalus and macrocephaly with respiratory distress. The patient received Ampicillin, Dopamine, Fentanyl, and Midazolam based on the physician's order. After three days, the patient had cardiac arrest, resuscitation was not successful, and the patient died eventually. Conclusion: This syndrome can be diagnosed with advanced imaging techniques, so it is better to perfume routine pregnancy care, especially imaging measures, with more sensitivity to prevent the birth of babies with this abnormality.

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