Choledochal Malformations in the Netherlands.

Eijnden, M.H.A. van den (2015) Choledochal Malformations in the Netherlands. thesis, Medicine.

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Abstract

Introduction; A choledochal malformation (CM) is a rare biliary entity with an incidence of 1:100.000 live births in the Western world. Due to the standardized ultrasound screening of all pregnancies in Western countries, up to 15% of the patients with CM will be diagnosed antenatally. Symptoms originate from complications associated with bile stasis in the CM. A CM is considered to be a premalignant condition and all aberrant bile duct epithelium should be excised. The most optimal surgical approach of these patients, via laparotomy or laparoscopy, and when to perform surgical intervention in the asymptomatic, antenatally diagnosed neonate, remains a matter of debate. Aims of study are to describe early and late complications in patients operated on CM in the Netherlands. Subsequently possible predictive factors for early and late complications such as surgical technique and timing of surgical intervention, especially in the asymptomatic neonate, will be identified. Methods; The only option to achieve adequate numbers for a research purpose is to create a nationwide database; the Netherlands Study on CHoledochal Cyst/malformation (NeSCHoC). Using the NeSCHoC all Dutch patients who underwent surgical intervention <18years of age for CM and those with antenatal abnormalities leading to postnatal confirmation were identified. Age, symptoms, biochemical investigation, surgical details and short and long term complications were noted retrospectively and analyzed. The association between surgical technique, age/weight at the time of surgery and the development of short and long term complications were tested. Short and long term complications were defined as complications occurring before or after 30 days postoperatively respectively. Results; Between January 1989 and December 2014, 96 paediatric patients underwent surgical intervention for CM. Laparoscopic resection was performed in 12 patients (12%). Short term complications occurred in 22 patients (23%), long term complications occurred in 17 patients (18%). Mortality was 3% (3 patients), with 1 death due to postoperative complications. There were no risk factors for the occurrence of short term complications. Risk factors for the occurrence of long term complications were laparoscopic technique (odds ratio 4.2) and surgical intervention before 1 year of age (odds ratio 7.5). Three out of 13 (23%) initially asymptomatic, antenatally diagnosed children progressed to CM-related symptoms at the ages of 0.7, 2.1 and 21.5 months. There were no differences in liver enzyme tests between patients who progressed to symptoms and those who did not, although the difference in gamma-glutamyl transpeptidase (GGT) levels at time of postnatal confirmation of diagnosis appeared different. Four patients (4/13, 31%) developed short term complications and three patients (3/13, 23%) developed long term complications. Conclusion; Morbidity after surgery for CM is significant. Short term complications occur in 23% of cases, long term complications in 18%. Long term complications are associated with the laparoscopic resection and surgical intervention <1 year of age. In the asymptomatic, antenatal diagnosed patients surgical intervention can be delayed until the age of at least 12 months/weight>10 kilograms when symptoms do not occur before the age of 6 months. GGT seems a promising marker for the detection of asymptomatic biliary stasis.

Item Type:	Thesis (Thesis)
Supervisor name:	Hulscher, Dr. J.B.F. M.D. and Kleine, Dr. R.H. de M.D.
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:38
Last Modified:	25 Jun 2020 10:38
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/16

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