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Journal of Pediatric Surgery (2012) 47, 1449–1452

Portal teratoma causing obstructive jaundice in children:a rarityDeepak Bagga a, Bibekanand Jindal b,⁎, Bikash Kumar Naredi b,Devendra Kumar Yadav a, Samir Kant Acharya a, Rajiv Mahato a, Kusum Gupta c

aDepartment of Pediatric Surgery, Safdarjung Hospital and associated Vardhaman Mahavir Medical College, New Delhi,India – 110029bDepartment of Pediatric Surgery, JIPMER, Pondicherry – 605006cDepartment of Pathology, Safdarjung Hospital, New Delhi, India

Received 13 October 2011; revised 28 March 2012; accepted 16 April 2012

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Key words:Portal teratoma;Hepatoduodenal ligamentteratoma;Obstructive jaundice inchildren

Abstract Teratoma in children is a common entity, usually occurring both in gonadal and extragonadalsites. Common extragonadal sites for teratoma in children are the sacrococcygeal region, retro-peritoneum, and mediastinum. Various unusual extragonadal sites have been reported. However,teratoma in the hepatoduodenal ligament is a very rare occurrence. We herein report a case of a matureteratoma in the hepatoduodenal ligament in an 11-year-old child presenting with obstructive jaundicealong with its surgical management and review of the literature.© 2012 Elsevier Inc. All rights reserved.

Obstructive biliary disease in the pediatric age group isnot uncommon and includes instances of biliary atresia,inspissated bile plug syndrome, choledochal cyst ininfancy, and choledochal cyst, calculus disease of thegallbladder, or extrahepatic biliary duct in older children[1,2]. Because of the anatomical relationship, any massarising in and around the hepatoduodenal ligament canproduce obstructive jaundice. Benign hepatic stricture andother extraductal pathology such as lymph node enlarge-ment secondary to tuberculosis or neoplasm (lymphoma,rhabdomyosarcoma of the extrahepatic biliary tree),hydatid cyst, duodenal duplication, and cystic lesionsnear the biliopancreatic junction are less common causes

⁎ Corresponding author. Present affiliation: Department of Pediatricurgery, JIPMER, Pondicherry – 605006.E-mail address: drvivekjindal@rediffmail.com (B. Jindal).

022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved.oi:10.1016/j.jpedsurg.2012.04.016

presenting with obstructive jaundice in children [2–4].Biliary ascariasis presenting as obstructive jaundice is yetanother though rare cause, prevalent in Northern India [5].

Teratomas arise from the totipotent primordial germinalcells and contain elements from all 3 primary germ celllayers. Depending on the levels of maturity of tumortissue, they are classified histologically as mature,immature, or malignant teratoma containing one of themalignant germ cell elements. It occurs commonly ininfancy and in the prepubertal age group and can occur inany part of the body [6]. Teratoma in the hepatoduodenalligament is a very rare entity, and only 7 such casespresenting with obstructive jaundice or portal hypertensionsyndrome have been reported in the literature [1,4,7–10].We describe a case of mature cystic teratoma at thehepatoduodenal ligament causing obstructive jaundice inan 11-year-old child.

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1. Case report

An 11-year-old girl presented with jaundice and aprogressively enlarging upper abdominal mass of 3months duration. On physical examination, she was ictericwith normal vital signs and no pallor. She had abdominaldistension with hepatomegaly extending 5.0 cm below theright costal margin. A tense 7- × 6-cm cystic mass waspalpated separately in the right upper quadrant. Laboratoryinvestigation revealed hemoglobin within normal limits(10 gm%) and elevated liver function tests (serumbilirubin, 19.4 g/dL [direct bil, 11.3]; aspartate amino-transferase, 94 IU; alanine aminotransferase, 61 IU; serumalkaline phosphatase, 48 IU; prothrombin time, 16/13)with presence of urobilirubinogen in urine. The serumalpha-fetoprotein level was normal.

Abdominal ultrasound showed a cystic heterogeneousmass with predominantly hyperechoic floating contents. Thelesion compressed the common bile duct (CBD) with diffusedilatation of the left and right hepatic ducts. Intrahepaticbiliary radicals were also dilated in addition to gallbladderdistension. Computed tomography (CT scan of the abdomen[Fig. 1]) showed a well-defined, 9.3- × 9.9-cm, round to ovalnonenhancing cystic mass in the hepatoduodenal ligament.The mass had heterogeneous internal contents with softtissue, fat, and calcific density components. The lesionproduced obstruction of the distal CBD with proximaldilatation including diffuse dilatation of the intrahepaticbiliary radicles and displaced adjacent structures includingstomach, bowel loops, portal vein, and hepatic artery. Therewas no obvious enhancement of the lesion after contrast

Fig. 1 Computed tomographic scan of the abdomen (coronalimage) showing a large nonenhancing cystic mass in the region ofthe porta with heterogenous internal contents comprising soft tissue,fat, and calcifications. The presence of distended gallbladder,diffuse intrahepatic biliary radicle dilatation, and hepatic duct (rightand left) dilatation were also noted.

injection. The liver appeared diffusely enlarged withnormal parenchymal density. The gallbladder was dis-tended with normal wall thickness. Ultrasound and contrastenhanced computed tomography (CECT) were clearlysuggestive of a teratomatous lesion causing compressionof the extrahepatic biliary tract; hence, magnetic resonancecholangiopancreatography and hepatobiliary scintigraphywere not obtained because these investigations would nothave changed the management.

At laparotomy, the liver was enlarged but of normalconsistency, and a large cyst was found in the portalregion. The distal CBD was stretched acutely over the cystwith massive dilatation of hepatic ducts and intensepericystic inflammation. The cyst contained hair andcheeselike soft tissue material that was bile stainedsuggesting fistulization with the CBD. On opening theCBD, the ductal communication with the cyst was notedwith hair emanating from the fistulous opening. Thegallbladder and the cyst were inflamed and thickened withadhesions to surrounding structures, and this precluded alllayer excision of the cyst. The cyst was excised along withthe gallbladder and the distal CBD incorporating thefistulized portion. Portal vein, hepatic artery, and inferiorvena cava were incorporated in the posterior wall of thecyst. Extirpation of the cyst removing the inner cyst liningand leaving the outer cyst wall in situ (Lilly technique) inthis area was performed to avoid damage to these posteriorstructures. There was an inadvertent injury to the portalvein, which was repaired appropriately. The commonhepatic duct margin was freshened, and bilioentericcontinuity was established by hepaticoduodenostomy. Thepostoperative course was uneventful.

The resected specimen was sent for histopathologicexamination, which was consistent with a mature cysticteratoma. The liver biopsy showed normal histology with mildcongestive changes. Postoperatively, the direct serum bilirubinlevel fell to 3 gm% by 2 weeks and was within normal rangeafter 2 months. On follow-up at 2 years, the patient is doingwell with normal bilirubin level and a serum alpha-fetoproteinlevel that was within normal range, and the abdominalultrasound revealed no local recurrence at the porta hepatis.

2. Discussion

Teratoma can occur in any part of the body. In children, itoccurs mostly in the sacrococcygeal region, gonads, retro-peritoneum, mediastinum, testes, and central nervoussystem. Rare cases of these tumors occurring in thegastrointestinal tract, liver, nasal sinuses, cervix, and thyroidhave been reported [6]. Teratoma in the hepatoduodenalligament is very rare, and we could find only 7 reported caseshitherto after an extensive literature search [1,4,7–10]. Mostof the teratomas in children are benign in nature, withexcision being the preferred treatment of choice.

Fig. 2 Operative photograph showing a large teratomatous mass (1) in the hepatoduodenal ligament, stretched CBD (2) over the mass withfistulization of CBD; hairs (3 and 4) could be seen emanating from the fistula and the dilated proximal common hepatic duct (5).

1451Portal teratoma causing obstructive jaundice in children

Reviewing the literature, we found that teratomas in thehepatoduodenal ligament (portal teratoma) typically presentin infancy [8,9] and occur in females more frequently thanmales. The common modes of presentation were abdominaldistension, pain, and jaundice. The serum alpha-fetoproteinlevel is usually within normal range [8,9] for age.Ultrasound and CT scan are radiographic studies of choicefor these lesions and frequently show fatty components andcalcifications. Excision of benign teratomas is usuallyadequate treatment unless the tumor has invaded localstructures. Occasionally, a Whipple procedure (for sus-pected malignancy of the CBD) and ductocystostomy incombination with cystojejunostomy for a teratoma arisingfrom anomalous CBD have been described in the literature[4,7]. Of the previously reported cases, most of theteratomas in the hepatoduodenal ligament were benign(mature teratoma) except for 1 described as an endodermalsinus tumor [4]. The long-term prognosis is usuallyfavorable unless the tumor has malignant or immaturecomponents [9]. The 2 reported fatal cases were caused bymalignancy and portal hypertension [4,10]. In our case, thecystic teratoma was located in the hepatoduodenal ligamentdistorting the gallbladder and CBD associated with intensepericystic inflammation. The hepatic ducts were massivelydilated with fistulization of the CBD into the cyst, and haircould be seen emanating from the fistula into the CBD(Fig. 2). The contents of the cyst were heavily bile stained.This prompted us to excise the gallbladder and CBD alongwith the cyst. However, because the portal vein and hepaticartery were incorporated into the posterior wall of the cyst, itprecluded complete excision of the cyst. There was an

inadvertent injury to a tributary of the portal vein despiteleaving part of the posterior cyst wall. Attempts at completeexcision in this area may have resulted in serious injury toimportant structures in its vicinity. Hepaticoduodenostomywas fashioned to restore bilioenteric continuity, which hasfunctioned well in the 2-year follow-up.

Although teratomas in the portal region are very rare, itshould be considered in the differential diagnosis ofchildren presenting with obstructive jaundice with a cysticmass in the right upper quadrant. Apart from the rarity ofthis lesion presenting with obstructive jaundice, fistulizationof the cyst with the CBD with hair in its lumen noticedintraoperatively was most interesting. Although simpleexcision is the treatment of choice when feasible, our casemandated a treatment quite akin to that of an inflamedcholedochal cyst. The lesion was successfully managedwith a satisfactory outcome.

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