Dandy-Walker syndrome with bilateral choanal atresia: A case report

Raghad K Alsalamah; Mazyad M Alenezi; Fahad Alsaab

doi:10.1016/j.ijscr.2021.106702

Dandy-Walker syndrome with bilateral choanal atresia: A case report

Int J Surg Case Rep. 2022 Jan:90:106702. doi: 10.1016/j.ijscr.2021.106702. Epub 2021 Dec 20.

Authors

Raghad K Alsalamah¹, Mazyad M Alenezi², Fahad Alsaab³

Affiliations

¹ College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia. Electronic address: Raghad.ks@gmail.com.
² Department of Otolaryngology Head and Neck Surgery, College of Medicine, Qassim University, Qassim, Saudi Arabia.
³ Department of Pediatric Surgery, Department of Otolaryngology Head and Neck Surgery, Ministry of the National Guard - Health Affairs, King Abdullah International Medical Research Center, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.

Abstract

Introduction: Dandy-Walker syndrome is a rare congenital brain malformation characterized by cerebellar vermis agenesis or hypoplasia, cystic dilatation of the fourth ventricle and a large posterior fossa causing upward displacement of tentorium and torcula. In this paper, we present a case of bilateral choanal atresia with Dandy-Walker Syndrome in a female newborn.

Case presentation: We present a case of a female patient who was born at 38th weeks of gestation via emergency cesarean section due to non-reassuring cardiotocography and abnormal antenatal ultrasounds findings. The imaging revealed the presence of Dandy-walker malformation. The patient presented with cyanosis and respiratory distress. Bedside flexible nasoendoscopy revealed bilateral choanal atresia which is confirmed by computed tomography of sinuses. Endoscopic bilateral choanal atresia repair was performed. On postoperative follow up, nasal endoscopy showed bilateral intact flap and patent neochoana.

Discussion and conclusion: Dandy-Walker syndrome is a congenital disorder that can be diagnosed prenatally. The syndrome is associated with multiple anomalies. However, there are few published reports of bilateral choanal atresia in Dandy-Walker Syndrome. Bilateral choanal atresia is considered a life-threatening condition in newborns that requires early surgical intervention.

Keywords: Bilateral choanal atresia; Case report; Dandy-Walker syndrome; Neonates; Otorhinolaryngology.