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Portal teratoma causing obstructive jaundice in children: a rarity
Journal of Pediatric Surgery (2012) 47, 1449–1452
www.elsevier.com/locate/jpedsurg
Portal teratoma causing obstructive jaundice in children: a rarity Deepak Bagga a , Bibekanand Jindal b,⁎, Bikash Kumar Naredi b , Devendra Kumar Yadav a , Samir Kant Acharya a , Rajiv Mahato a , Kusum Gupta c a
Department of Pediatric Surgery, Safdarjung Hospital and associated Vardhaman Mahavir Medical College, New Delhi, India – 110029 b Department of Pediatric Surgery, JIPMER, Pondicherry – 605006 c Department of Pathology, Safdarjung Hospital, New Delhi, India Received 13 October 2011; revised 28 March 2012; accepted 16 April 2012
Key words: Portal teratoma; Hepatoduodenal ligament teratoma; Obstructive jaundice in children
Abstract Teratoma in children is a common entity, usually occurring both in gonadal and extragonadal sites. Common extragonadal sites for teratoma in children are the sacrococcygeal region, retroperitoneum, 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 mature teratoma in the hepatoduodenal ligament in an 11-year-old child presenting with obstructive jaundice along with its surgical management and review of the literature. © 2012 Elsevier Inc. All rights reserved.
Obstructive biliary disease in the pediatric age group is not uncommon and includes instances of biliary atresia, inspissated bile plug syndrome, choledochal cyst in infancy, and choledochal cyst, calculus disease of the gallbladder, or extrahepatic biliary duct in older children [1,2]. Because of the anatomical relationship, any mass arising in and around the hepatoduodenal ligament can produce obstructive jaundice. Benign hepatic stricture and other extraductal pathology such as lymph node enlargement secondary to tuberculosis or neoplasm (lymphoma, rhabdomyosarcoma of the extrahepatic biliary tree), hydatid cyst, duodenal duplication, and cystic lesions near the biliopancreatic junction are less common causes ⁎ Corresponding author. Present affiliation: Department of Pediatric Surgery, JIPMER, Pondicherry – 605006. E-mail address: [email protected] (B. Jindal). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2012.04.016
presenting with obstructive jaundice in children [2–4]. Biliary ascariasis presenting as obstructive jaundice is yet another though rare cause, prevalent in Northern India [5]. Teratomas arise from the totipotent primordial germinal cells and contain elements from all 3 primary germ cell layers. Depending on the levels of maturity of tumor tissue, they are classified histologically as mature, immature, or malignant teratoma containing one of the malignant germ cell elements. It occurs commonly in infancy and in the prepubertal age group and can occur in any part of the body [6]. Teratoma in the hepatoduodenal ligament is a very rare entity, and only 7 such cases presenting with obstructive jaundice or portal hypertension syndrome have been reported in the literature [1,4,7–10]. We describe a case of mature cystic teratoma at the hepatoduodenal ligament causing obstructive jaundice in an 11-year-old child.
1450
1. Case report An 11-year-old girl presented with jaundice and a progressively enlarging upper abdominal mass of 3 months duration. On physical examination, she was icteric with normal vital signs and no pallor. She had abdominal distension with hepatomegaly extending 5.0 cm below the right costal margin. A tense 7- × 6-cm cystic mass was palpated separately in the right upper quadrant. Laboratory investigation revealed hemoglobin within normal limits (10 gm%) and elevated liver function tests (serum bilirubin, 19.4 g/dL [direct bil, 11.3]; aspartate aminotransferase, 94 IU; alanine aminotransferase, 61 IU; serum alkaline phosphatase, 48 IU; prothrombin time, 16/13) with presence of urobilirubinogen in urine. The serum alpha-fetoprotein level was normal. Abdominal ultrasound showed a cystic heterogeneous mass with predominantly hyperechoic floating contents. The lesion compressed the common bile duct (CBD) with diffuse dilatation of the left and right hepatic ducts. Intrahepatic biliary radicals were also dilated in addition to gallbladder distension. Computed tomography (CT scan of the abdomen [Fig. 1]) showed a well-defined, 9.3- × 9.9-cm, round to oval nonenhancing cystic mass in the hepatoduodenal ligament. The mass had heterogeneous internal contents with soft tissue, fat, and calcific density components. The lesion produced obstruction of the distal CBD with proximal dilatation including diffuse dilatation of the intrahepatic biliary radicles and displaced adjacent structures including stomach, bowel loops, portal vein, and hepatic artery. There was no obvious enhancement of the lesion after contrast
D. Bagga et al. injection. The liver appeared diffusely enlarged with normal parenchymal density. The gallbladder was distended with normal wall thickness. Ultrasound and contrast enhanced computed tomography (CECT) were clearly suggestive of a teratomatous lesion causing compression of the extrahepatic biliary tract; hence, magnetic resonance cholangiopancreatography and hepatobiliary scintigraphy were not obtained because these investigations would not have changed the management. At laparotomy, the liver was enlarged but of normal consistency, and a large cyst was found in the portal region. The distal CBD was stretched acutely over the cyst with massive dilatation of hepatic ducts and intense pericystic inflammation. The cyst contained hair and cheeselike soft tissue material that was bile stained suggesting fistulization with the CBD. On opening the CBD, the ductal communication with the cyst was noted with hair emanating from the fistulous opening. The gallbladder and the cyst were inflamed and thickened with adhesions to surrounding structures, and this precluded all layer excision of the cyst. The cyst was excised along with the gallbladder and the distal CBD incorporating the fistulized portion. Portal vein, hepatic artery, and inferior vena cava were incorporated in the posterior wall of the cyst. Extirpation of the cyst removing the inner cyst lining and leaving the outer cyst wall in situ (Lilly technique) in this area was performed to avoid damage to these posterior structures. There was an inadvertent injury to the portal vein, which was repaired appropriately. The common hepatic duct margin was freshened, and bilioenteric continuity was established by hepaticoduodenostomy. The postoperative course was uneventful. The resected specimen was sent for histopathologic examination, which was consistent with a mature cystic teratoma. The liver biopsy showed normal histology with mild congestive changes. Postoperatively, the direct serum bilirubin level fell to 3 gm% by 2 weeks and was within normal range after 2 months. On follow-up at 2 years, the patient is doing well with normal bilirubin level and a serum alpha-fetoprotein level that was within normal range, and the abdominal ultrasound revealed no local recurrence at the porta hepatis.
2. Discussion
Fig. 1 Computed tomographic scan of the abdomen (coronal image) showing a large nonenhancing cystic mass in the region of the porta with heterogenous internal contents comprising soft tissue, fat, and calcifications. The presence of distended gallbladder, diffuse intrahepatic biliary radicle dilatation, and hepatic duct (right and left) dilatation were also noted.
Teratoma can occur in any part of the body. In children, it occurs mostly in the sacrococcygeal region, gonads, retroperitoneum, mediastinum, testes, and central nervous system. Rare cases of these tumors occurring in the gastrointestinal tract, liver, nasal sinuses, cervix, and thyroid have been reported [6]. Teratoma in the hepatoduodenal ligament is very rare, and we could find only 7 reported cases hitherto after an extensive literature search [1,4,7–10]. Most of the teratomas in children are benign in nature, with excision being the preferred treatment of choice.
Portal teratoma causing obstructive jaundice in children
1451
Fig. 2 Operative photograph showing a large teratomatous mass (1) in the hepatoduodenal ligament, stretched CBD (2) over the mass with fistulization of CBD; hairs (3 and 4) could be seen emanating from the fistula and the dilated proximal common hepatic duct (5).
Reviewing the literature, we found that teratomas in the hepatoduodenal ligament (portal teratoma) typically present in infancy [8,9] and occur in females more frequently than males. The common modes of presentation were abdominal distension, pain, and jaundice. The serum alpha-fetoprotein level is usually within normal range [8,9] for age. Ultrasound and CT scan are radiographic studies of choice for these lesions and frequently show fatty components and calcifications. Excision of benign teratomas is usually adequate treatment unless the tumor has invaded local structures. Occasionally, a Whipple procedure (for suspected malignancy of the CBD) and ductocystostomy in combination with cystojejunostomy for a teratoma arising from anomalous CBD have been described in the literature [4,7]. Of the previously reported cases, most of the teratomas in the hepatoduodenal ligament were benign (mature teratoma) except for 1 described as an endodermal sinus tumor [4]. The long-term prognosis is usually favorable unless the tumor has malignant or immature components [9]. The 2 reported fatal cases were caused by malignancy and portal hypertension [4,10]. In our case, the cystic teratoma was located in the hepatoduodenal ligament distorting the gallbladder and CBD associated with intense pericystic inflammation. The hepatic ducts were massively dilated with fistulization of the CBD into the cyst, and hair could 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 along with the cyst. However, because the portal vein and hepatic artery were incorporated into the posterior wall of the cyst, it precluded complete excision of the cyst. There was an
inadvertent injury to a tributary of the portal vein despite leaving part of the posterior cyst wall. Attempts at complete excision in this area may have resulted in serious injury to important structures in its vicinity. Hepaticoduodenostomy was fashioned to restore bilioenteric continuity, which has functioned well in the 2-year follow-up. Although teratomas in the portal region are very rare, it should be considered in the differential diagnosis of children presenting with obstructive jaundice with a cystic mass in the right upper quadrant. Apart from the rarity of this lesion presenting with obstructive jaundice, fistulization of the cyst with the CBD with hair in its lumen noticed intraoperatively was most interesting. Although simple excision is the treatment of choice when feasible, our case mandated a treatment quite akin to that of an inflamed choledochal cyst. The lesion was successfully managed with a satisfactory outcome.
References [1] Frexes M, Neblett 3rd WW, Holcomb Jr GW. Spectrum of biliary diseases in childhood. South Med J 1986;79:1342-9. [2] Otgun I, Karnak I, Haliloglu M, et al. Obstructive jaundice caused by primary choledochal hydatid cyst mimicking radiologically choledochal cyst. J Pediatr Surg 2003;38:256-8. [3] Krishna RP, Lal R, Sikora SS, et al. Unusual causes of extrahepatic biliary obstruction in children: a case series with review of literature. Pediatr Surg Int 2008;24:183-90. [4] Kim WS, Choi BI, Lee YS, et al. Endodermal sinus tumour associated with benign teratoma of the common bile duct. Pediatr Radiol 1993;23: 59-60.
1452 [5] Baba AA, Sera AH, Bhat MA, et al. Management of biliary ascariasis in children living in endemic area. Eur J Pediatr Surg 2010;20:187-90. [6] Billmire DF, Grosfeld JL. Teratoma in childhood: analysis of 142 cases. J Pediatr Surg 1986;21(6):548-51. [7] Demircan BM, Uguralp S, Mutus M, et al. Teratoma arising from the anomalous common bile ducts: a case report. J Pediatr Surg 2004; 39(4):e1-2.
D. Bagga et al. [8] Brown B, Khalil B, Batra G, et al. Mature cystic teratoma arising at the porta hepatis: a diagnostic dilemma. J Pediatr Surg 2008;43(4):e1-3. [9] Etsuji U, Masao E, Fumiko Y. Hepatoduodenal ligament teratoma with hepatic artery running inside. Pediatr Surg Int 2008;24(11): 1239-42. [10] Akimov OV. Hepatoduodenal ligament teratoma followed by hypertensive syndrome of the portal vein. Arkh Patol 1989;51:60-2.
www.elsevier.com/locate/jpedsurg
Portal teratoma causing obstructive jaundice in children: a rarity Deepak Bagga a , Bibekanand Jindal b,⁎, Bikash Kumar Naredi b , Devendra Kumar Yadav a , Samir Kant Acharya a , Rajiv Mahato a , Kusum Gupta c a
Department of Pediatric Surgery, Safdarjung Hospital and associated Vardhaman Mahavir Medical College, New Delhi, India – 110029 b Department of Pediatric Surgery, JIPMER, Pondicherry – 605006 c Department of Pathology, Safdarjung Hospital, New Delhi, India Received 13 October 2011; revised 28 March 2012; accepted 16 April 2012
Key words: Portal teratoma; Hepatoduodenal ligament teratoma; Obstructive jaundice in children
Abstract Teratoma in children is a common entity, usually occurring both in gonadal and extragonadal sites. Common extragonadal sites for teratoma in children are the sacrococcygeal region, retroperitoneum, 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 mature teratoma in the hepatoduodenal ligament in an 11-year-old child presenting with obstructive jaundice along with its surgical management and review of the literature. © 2012 Elsevier Inc. All rights reserved.
Obstructive biliary disease in the pediatric age group is not uncommon and includes instances of biliary atresia, inspissated bile plug syndrome, choledochal cyst in infancy, and choledochal cyst, calculus disease of the gallbladder, or extrahepatic biliary duct in older children [1,2]. Because of the anatomical relationship, any mass arising in and around the hepatoduodenal ligament can produce obstructive jaundice. Benign hepatic stricture and other extraductal pathology such as lymph node enlargement secondary to tuberculosis or neoplasm (lymphoma, rhabdomyosarcoma of the extrahepatic biliary tree), hydatid cyst, duodenal duplication, and cystic lesions near the biliopancreatic junction are less common causes ⁎ Corresponding author. Present affiliation: Department of Pediatric Surgery, JIPMER, Pondicherry – 605006. E-mail address: [email protected] (B. Jindal). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2012.04.016
presenting with obstructive jaundice in children [2–4]. Biliary ascariasis presenting as obstructive jaundice is yet another though rare cause, prevalent in Northern India [5]. Teratomas arise from the totipotent primordial germinal cells and contain elements from all 3 primary germ cell layers. Depending on the levels of maturity of tumor tissue, they are classified histologically as mature, immature, or malignant teratoma containing one of the malignant germ cell elements. It occurs commonly in infancy and in the prepubertal age group and can occur in any part of the body [6]. Teratoma in the hepatoduodenal ligament is a very rare entity, and only 7 such cases presenting with obstructive jaundice or portal hypertension syndrome have been reported in the literature [1,4,7–10]. We describe a case of mature cystic teratoma at the hepatoduodenal ligament causing obstructive jaundice in an 11-year-old child.
1450
1. Case report An 11-year-old girl presented with jaundice and a progressively enlarging upper abdominal mass of 3 months duration. On physical examination, she was icteric with normal vital signs and no pallor. She had abdominal distension with hepatomegaly extending 5.0 cm below the right costal margin. A tense 7- × 6-cm cystic mass was palpated separately in the right upper quadrant. Laboratory investigation revealed hemoglobin within normal limits (10 gm%) and elevated liver function tests (serum bilirubin, 19.4 g/dL [direct bil, 11.3]; aspartate aminotransferase, 94 IU; alanine aminotransferase, 61 IU; serum alkaline phosphatase, 48 IU; prothrombin time, 16/13) with presence of urobilirubinogen in urine. The serum alpha-fetoprotein level was normal. Abdominal ultrasound showed a cystic heterogeneous mass with predominantly hyperechoic floating contents. The lesion compressed the common bile duct (CBD) with diffuse dilatation of the left and right hepatic ducts. Intrahepatic biliary radicals were also dilated in addition to gallbladder distension. Computed tomography (CT scan of the abdomen [Fig. 1]) showed a well-defined, 9.3- × 9.9-cm, round to oval nonenhancing cystic mass in the hepatoduodenal ligament. The mass had heterogeneous internal contents with soft tissue, fat, and calcific density components. The lesion produced obstruction of the distal CBD with proximal dilatation including diffuse dilatation of the intrahepatic biliary radicles and displaced adjacent structures including stomach, bowel loops, portal vein, and hepatic artery. There was no obvious enhancement of the lesion after contrast
D. Bagga et al. injection. The liver appeared diffusely enlarged with normal parenchymal density. The gallbladder was distended with normal wall thickness. Ultrasound and contrast enhanced computed tomography (CECT) were clearly suggestive of a teratomatous lesion causing compression of the extrahepatic biliary tract; hence, magnetic resonance cholangiopancreatography and hepatobiliary scintigraphy were not obtained because these investigations would not have changed the management. At laparotomy, the liver was enlarged but of normal consistency, and a large cyst was found in the portal region. The distal CBD was stretched acutely over the cyst with massive dilatation of hepatic ducts and intense pericystic inflammation. The cyst contained hair and cheeselike soft tissue material that was bile stained suggesting fistulization with the CBD. On opening the CBD, the ductal communication with the cyst was noted with hair emanating from the fistulous opening. The gallbladder and the cyst were inflamed and thickened with adhesions to surrounding structures, and this precluded all layer excision of the cyst. The cyst was excised along with the gallbladder and the distal CBD incorporating the fistulized portion. Portal vein, hepatic artery, and inferior vena cava were incorporated in the posterior wall of the cyst. Extirpation of the cyst removing the inner cyst lining and leaving the outer cyst wall in situ (Lilly technique) in this area was performed to avoid damage to these posterior structures. There was an inadvertent injury to the portal vein, which was repaired appropriately. The common hepatic duct margin was freshened, and bilioenteric continuity was established by hepaticoduodenostomy. The postoperative course was uneventful. The resected specimen was sent for histopathologic examination, which was consistent with a mature cystic teratoma. The liver biopsy showed normal histology with mild congestive changes. Postoperatively, the direct serum bilirubin level fell to 3 gm% by 2 weeks and was within normal range after 2 months. On follow-up at 2 years, the patient is doing well with normal bilirubin level and a serum alpha-fetoprotein level that was within normal range, and the abdominal ultrasound revealed no local recurrence at the porta hepatis.
2. Discussion
Fig. 1 Computed tomographic scan of the abdomen (coronal image) showing a large nonenhancing cystic mass in the region of the porta with heterogenous internal contents comprising soft tissue, fat, and calcifications. The presence of distended gallbladder, diffuse intrahepatic biliary radicle dilatation, and hepatic duct (right and left) dilatation were also noted.
Teratoma can occur in any part of the body. In children, it occurs mostly in the sacrococcygeal region, gonads, retroperitoneum, mediastinum, testes, and central nervous system. Rare cases of these tumors occurring in the gastrointestinal tract, liver, nasal sinuses, cervix, and thyroid have been reported [6]. Teratoma in the hepatoduodenal ligament is very rare, and we could find only 7 reported cases hitherto after an extensive literature search [1,4,7–10]. Most of the teratomas in children are benign in nature, with excision being the preferred treatment of choice.
Portal teratoma causing obstructive jaundice in children
1451
Fig. 2 Operative photograph showing a large teratomatous mass (1) in the hepatoduodenal ligament, stretched CBD (2) over the mass with fistulization of CBD; hairs (3 and 4) could be seen emanating from the fistula and the dilated proximal common hepatic duct (5).
Reviewing the literature, we found that teratomas in the hepatoduodenal ligament (portal teratoma) typically present in infancy [8,9] and occur in females more frequently than males. The common modes of presentation were abdominal distension, pain, and jaundice. The serum alpha-fetoprotein level is usually within normal range [8,9] for age. Ultrasound and CT scan are radiographic studies of choice for these lesions and frequently show fatty components and calcifications. Excision of benign teratomas is usually adequate treatment unless the tumor has invaded local structures. Occasionally, a Whipple procedure (for suspected malignancy of the CBD) and ductocystostomy in combination with cystojejunostomy for a teratoma arising from anomalous CBD have been described in the literature [4,7]. Of the previously reported cases, most of the teratomas in the hepatoduodenal ligament were benign (mature teratoma) except for 1 described as an endodermal sinus tumor [4]. The long-term prognosis is usually favorable unless the tumor has malignant or immature components [9]. The 2 reported fatal cases were caused by malignancy and portal hypertension [4,10]. In our case, the cystic teratoma was located in the hepatoduodenal ligament distorting the gallbladder and CBD associated with intense pericystic inflammation. The hepatic ducts were massively dilated with fistulization of the CBD into the cyst, and hair could 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 along with the cyst. However, because the portal vein and hepatic artery were incorporated into the posterior wall of the cyst, it precluded complete excision of the cyst. There was an
inadvertent injury to a tributary of the portal vein despite leaving part of the posterior cyst wall. Attempts at complete excision in this area may have resulted in serious injury to important structures in its vicinity. Hepaticoduodenostomy was fashioned to restore bilioenteric continuity, which has functioned well in the 2-year follow-up. Although teratomas in the portal region are very rare, it should be considered in the differential diagnosis of children presenting with obstructive jaundice with a cystic mass in the right upper quadrant. Apart from the rarity of this lesion presenting with obstructive jaundice, fistulization of the cyst with the CBD with hair in its lumen noticed intraoperatively was most interesting. Although simple excision is the treatment of choice when feasible, our case mandated a treatment quite akin to that of an inflamed choledochal cyst. The lesion was successfully managed with a satisfactory outcome.
References [1] Frexes M, Neblett 3rd WW, Holcomb Jr GW. Spectrum of biliary diseases in childhood. South Med J 1986;79:1342-9. [2] Otgun I, Karnak I, Haliloglu M, et al. Obstructive jaundice caused by primary choledochal hydatid cyst mimicking radiologically choledochal cyst. J Pediatr Surg 2003;38:256-8. [3] Krishna RP, Lal R, Sikora SS, et al. Unusual causes of extrahepatic biliary obstruction in children: a case series with review of literature. Pediatr Surg Int 2008;24:183-90. [4] Kim WS, Choi BI, Lee YS, et al. Endodermal sinus tumour associated with benign teratoma of the common bile duct. Pediatr Radiol 1993;23: 59-60.
1452 [5] Baba AA, Sera AH, Bhat MA, et al. Management of biliary ascariasis in children living in endemic area. Eur J Pediatr Surg 2010;20:187-90. [6] Billmire DF, Grosfeld JL. Teratoma in childhood: analysis of 142 cases. J Pediatr Surg 1986;21(6):548-51. [7] Demircan BM, Uguralp S, Mutus M, et al. Teratoma arising from the anomalous common bile ducts: a case report. J Pediatr Surg 2004; 39(4):e1-2.
D. Bagga et al. [8] Brown B, Khalil B, Batra G, et al. Mature cystic teratoma arising at the porta hepatis: a diagnostic dilemma. J Pediatr Surg 2008;43(4):e1-3. [9] Etsuji U, Masao E, Fumiko Y. Hepatoduodenal ligament teratoma with hepatic artery running inside. Pediatr Surg Int 2008;24(11): 1239-42. [10] Akimov OV. Hepatoduodenal ligament teratoma followed by hypertensive syndrome of the portal vein. Arkh Patol 1989;51:60-2.