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DOI: 10.1055/s-0044-1791813
Choledochal Cyst and Right Congenital Diaphragmatic Hernia: When to Intervene?
Funding None.Abstract
Patients with congenital diaphragmatic hernia (CDH) can present with other congenital anomalies, but an associated choledochal cyst (CC) has rarely been described. The simultaneous diagnosis of both anomalies complicates patient management. There is no consensus on the ideal timing for CC excision. Unrepaired CC is associated with risks of developing biliary sludge, choledocholithiasis, and cholangitis. After a CDH repair with mesh, secondary bacterial translocation caused by a delayed CC repair could lead to mesh superinfection. Conversely, early CC surgical management could cause mesh displacement and lead to CDH recurrence, requiring reintervention. We present the rare case of a CC occurring in a neonate with a prenatally diagnosed right CDH. One month after an uncomplicated CDH repair with mesh, while the patient was still hospitalized for pulmonary hypertension, she developed progressive cholestasis and acholic stools. Investigations revealed a nonpreviously suspected CC. Conservative treatment was attempted, but CC perforation with secondary biliary peritonitis occurred. Open CC excision with a Roux-en-Y hepaticojejunostomy was therefore performed on day of life (DOL) 41. Having suffered no short-term surgical complications, the patient was discharged on DOL 83 because of prolonged ventilatory support due to pulmonary hypertension. Now 12 months after surgery, she is doing well with normal liver function tests and imaging studies. In summary, CC should be considered in the differential diagnosis of progressive cholestasis in patients with CDH. Surgical repair of a symptomatic CC should not be delayed even in the presence of mesh given the risks of CC complications.
Publication History
Received: 07 May 2024
Accepted: 17 September 2024
Article published online:
28 October 2024
© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
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References
- 1 Kirby E, Keijzer R. Congenital diaphragmatic hernia: current management strategies from antenatal diagnosis to long-term follow-up. Pediatr Surg Int 2020; 36 (04) 415-429
- 2 Mah VK, Zamakhshary M, Mah DY. et al. Absolute vs relative improvements in congenital diaphragmatic hernia survival: what happened to “hidden mortality.”. J Pediatr Surg 2009; 44 (05) 877-882
- 3 Chatterjee D, Ing RJ, Gien J. Update on congenital diaphragmatic hernia. Anesth Analg 2020; 131 (03) 808-821
- 4 Puligandla PS, Skarsgard ED, Offringa M. et al; Canadian Congenital Diaphragmatic Hernia Collaborative. Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline. CMAJ 2018; 190 (04) E103-E112
- 5 Fauza DO, Wilson JM. Congenital diaphragmatic hernia and associated anomalies: their incidence, identification, and impact on prognosis. J Pediatr Surg 1994; 29 (08) 1113-1117
- 6 Krebs D, Herman RS, Blewett C. Newborn male presented with congenital diaphragmatic hernia and choledochal cyst: a case report. J Pediatr Surg Case Rep 2015; 3: 179-181
- 7 Cazares J, Koga H, Yamataka A. Choledochal cyst. Pediatr Surg Int 2023; 39 (01) 209
- 8 de Vries JS, de Vries S, Aronson DC. et al. Choledochal cysts: age of presentation, symptoms, and late complications related to Todani's classification. J Pediatr Surg 2002; 37 (11) 1568-1573
- 9 Pan S, Li W, Chen H, Lu C. The timing of minimally invasive surgery for prenatally diagnosed choledochal cysts. BMC Pediatr 2024; 24 (01) 250
- 10 Ho IG, Ihn K, Jeon HJ, Lee DE, Han SJ. Optimal timing of surgery for prenatally diagnosed choledochal cysts. Front Pediatr 2023; 11: 1308667
- 11 Kowalski A, Kowalewski G, Kaliciński P. et al. Choledochal cyst excision in infants: a retrospective study. Children (Basel) 2023; 10 (02) 10
- 12 Holder AM, Klaassens M, Tibboel D, de Klein A, Lee B, Scott DA. Genetic factors in congenital diaphragmatic hernia. Am J Hum Genet 2007; 80 (05) 825-845
- 13 Impellizzeri P, Nascimben F, Di Fabrizio D. et al. Pathogenesis of congenital malformations: possible role of oxidative stress. Am J Perinatol 2022; 39 (08) 816-823
- 14 De Leon N, Tse WH, Ameis D, Keijzer R. Embryology and anatomy of congenital diaphragmatic hernia. Semin Pediatr Surg 2022; 31 (06) 151229
- 15 Ando H. Embryology of the biliary tract. Dig Surg 2010; 27 (02) 87-89
- 16 Kim SH, Cho YH, Kim HY. Perforated choledochal cyst: its clinical implications in pediatric patient. Pediatr Gastroenterol Hepatol Nutr 2020; 23 (03) 259-265
- 17 Soares KC, Goldstein SD, Ghaseb MA, Kamel I, Hackam DJ, Pawlik TM. Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int 2017; 33 (06) 637-650
- 18 Rygl M, Pycha K, Stranak Z. et al. Congenital diaphragmatic hernia: onset of respiratory distress and size of the defect: analysis of the outcome in 104 neonates. Pediatr Surg Int 2007; 23 (01) 27-31
- 19 Heiwegen K, de Blaauw I, Botden SMBI. A systematic review and meta-analysis of surgical morbidity of primary versus patch repaired congenital diaphragmatic hernia patients. Sci Rep 2021; 11 (01) 12661
- 20 Flohr SJ, Land SD, Hedrick HL. et al. Synthetic patch infection after congenital diaphragmatic hernia repair: a case series. J Pediatr Surg Case Rep 2023; 90: 102583