Teratoma Arising from Hepato Duodenal Ligament in the Newborn with Transection of Portal Vein, Hepatic Artery and Common Bile Duct: A Surgical Challenge.

A 7-day-old neonate presented with a large intra-abdominal mass adherent to the hilum of the liver encasing the portal triad. During excision, the portal vein, hepatic artery, and common bile duct were injured. The repair was done promptly and needed massive blood transfusion. Histopathology revealed immature teratoma Grade III. Survival in neonate following total transection of portal triad is rare and has not been reported.

INTRODUCTION

Teratomas are the most common tumors in infancy. Although the risk of malignancy is small, it increases substantially in those diagnosed later in infancy. It is extremely rare to present in the hilum of the liver extending on to the hepatoduodenal ligament. Surgery carries risk of injury to vital structures in porta hepatis. Injury to portal vein and hepatic artery carry significant mortality and morbidity. This child weighing 2.7 kg needed massive transfusion of nearly 350 ml of blood during the repair of the injured portal triad. Although the liver parameters showed increase in liver enzymes in the immediate postoperative period the liver function returned to normal.

CASE REPORT

A 7-day-old baby boy diagnosed antenatally with a large intra-abdominal mass was investigated postnataly with ultrasound and magnetic resonance imaging. It showed a large abdominal mass probably arising from the mesentery [Figure 1]. On admission, the baby had gross abdominal distension with dilated veins over the abdomen. Clinically, it was a large lobulated mass occupying the whole abdomen except right lumbar and iliac fossa. The alpha fetoprotein was elevated to 20,000 units and the beta human chorionic gonadotropin was 1.2 units and other routine investigations were normal.

Figure 1

Large intra-abdominal teratoma with magnetic resonance imaging picture showing variegated appearance

Intraoperatively, a large tumor was adherent to the under surface of the liver and to the hilum, stretching the hepatoduodenal ligament [Figure 2a]. Intraoperatively, the vascular pedicle comprising of portal vein, hepatic artery and the common bile duct got injured. It was repaired with the help of a vascular team. The portal vein was sutured with eight zero prolene. The hepatic artery was stented with 1 mm coronary stent and sutured with interrupted eight zero prolene A choledochoduodenostomy was done. The warm ischemic time was 45 min. The child needed massive transfusion and nearly 350 ml of blood was transfused during the procedure.

Figure 2

(a) Large tumor attached to the hilum of the liver. (b) Histopathology with features of immature teratoma

The child was electively ventilated for 3 days. The liver enzymes were elevated in the immediate postoperative period. Liver protective agent N acetyl cystine at the dose of 100 mg daily was infused for 6 days with supportive measures. The child recovered completely and the liver enzymes decreased and the alpha fetoprotein level dropped to 40 units. The child was discharged on the 12th postoperative day with Ursodeoxycholic acid. At the time of discharge, the serum conjugated bilirubin was 3 mg and indirect bilirubin was 3.4 mg. Review after 6 months showed that the liver functions normalized with serum bilirubin of 1.2 mg with conjugated fraction of 0.5 mg. The serum albumin was normal at 3.6 g.

The tumor cut surface showed predominantly solid areas with variegated appearance, focal areas of calcifications with a yellowish nodule. Histologically, the neoplasm was composed of skin with adnexal structures, fat, cartilage, bone, muscle, intestinal tissue, salivary gland tissue, and neural elements. Areas of immature intraepithelial elements were also seen which accounts for more than 3 low power microscopic fields. Liver parenchyma was not seen in sections studied. The impression was that of an immature teratoma Grade III [Figure 2b].

DISCUSSION

Teratomas are the most frequent perinatal neoplasms. In the neonate, it commonly occurs in sacrococcygeal region. The other areas are palate, neck, mediastinum and in the abdomen, it can happen in the stomach, ovary and commonly in the retroperitoneum.[1] In this child, the tumor was arising in the region of the hilum of the liver extending on to the capsule of the left lobe of the liver from which it could not be separated. Teratoma arising from hepatoduodenal ligament producing portal hypertension has been described.[2] Twenty-seven children with giant liver tumors involving the hepatic hilum have been described with one teratoma and the rest being malignant tumors.[3] However, these are primarily liver tumors extending on to hilum of liver. In this child, histologically, the liver parenchyma was not seen in the tumor. It is probable that the teratoma was arising within the peritoneum covering the portal triad extending to the hilum encasing the portal vein, hepatic artery, and the common bile duct.

It was very challenging to remove such a large tumor in a 7-day-old infant in whom the portal vein, hepatic artery, and common bile duct were transected and repaired. Traumatic injuries to the portal vein are rare but devastating. The mortality rate in adults for portal vein injury ranges from 50% to 70%. A recent study of injuries to portal vein and hepatic artery has highlighted the higher mortality rates associated with combination injuries involving multiple components, especially those that include portal vein injury.[3] In another study, 66% of deaths occurred in the operating room, primarily from exsanguinations; 18% of deaths occurred within 48 h of injury from refractory shock, coagulopathy, or cardiac arrest.[4] The child needed a massive transfusion of nearly 350 ml of blood. In a study of risk factors for perioperative mortality and transfusion in sacrococcygeal teratoma resections, high mortality associated with infants who had transfusion volumes of 240 ml/kg or greater was stressed.[5] Transfusion-associated hyperkalemic cardiac arrest in pediatric patients and co morbidities such as hypocalcemia, acidemia and hypotension have been stressed in neonates receiving massive transfusion.[6] N acetyl cysteine was used in the immediate postoperative period as a cytoprotective agent to the liver which had warm ischemia time of nearly 45 min.

The case is presented for its rarity in the location of teratoma and survival following repair of total transection of the portal vein, hepatic artery, and common bile duct. Such case in a neonate has not been reported so far.

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Conflicts of interest

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