Understanding choledochal malformation

Abstract

Choledochal malformations (also known as choledochal cysts) may be characterised as an abnormal dilatation of the biliary tract, in the absence of any acute obstruction. Most appear to be of congenital origin probably related to distal bile duct stenosis, and almost 15% can now be detected antenatally. Excision and biliary reconstruction using a Roux loop as an open operation is still the standard to compare to. This article discusses recent advances in the understanding of their aetiology and classification together with the place of newer modalites of surgical treatment such as laparoscopic excision and biliary reconstruction. Although these are definitely feasible and safe, care should be taken before dispensing with standard open techniques which have minimal complications and proven long-term benefit.

Diagnostic challenge of large congenital liver cyst in the newborn

“…2 Asymptomatic simple cysts can be monitored on periodic USG, while large SLC in the newborn may cause abdominal distention, pain, jaundice, feeding difficulties and respiratory distress. 6,7 The best treatment of symptomatic SLC is total surgical excision. 7 SIBC have all the characteristics of SLC, but contain bile.…”

“…Electrolyte disturbances cause variable and nonspecific clinical signs, like fever, vomiting, failure to thrive, irritability, polyuria and dehydration. Although PHA may be potentially life-threatening, 6 cases of cardiopulmonary arrest (CPA) have not previously been reported. This was probably because in this critical condition a proper diagnosis was difficult and, more importantly, because resuscitation was unsuccessful.…”

Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland

“…The incidence is substantially higher among Asian populations, approximating 1:1000, and about 80% of CMs are found in females [1,3]. CMs usually present during childhood with jaundice, abdominal pain, or pancreatitis; however, about 20% are diagnosed in adult age [4,5].…”

“…CMs may be shaped cystic or fusiform and they usually involve the extrahepatic biliary tree (types I), although more complex ones extend to intrahepatic ducts (type IV) [4,6]. Types I and IV account for over 90% of all CMs [1,5].…”

3D printing to simulate laparoscopic choledochal surgery

“…The accuracy of 3D printing to recreate anatomy from layer imaging and its application as an educational tool is being increasingly reported [20]. Choledochal cysts can present with great variation, even outside of the broad 4-part classification [21]. In such situations 3D printing could allow more high fidelity, patient specific practice and operative planning prior to surgery, to the benefit of even the most experienced surgeons.…”