Ectopic Umbilical ByWoo-Hyun

Liver in Conjunction With Biliary Atresia: Uncommon Association

Park, Soon-Ok Choi, Sang-Sook Lee, and Judson G. Randolph Taegu,

Korea and Washington,

@The case of a neonate with an ectopic liver in the umbilicus in conjunction with biliary atresia in the liver proper and an ectopic pancreas in the jejunum is reported. Following excision of the ectopic umbilical liver and Kasai type 1 hepatic portoenterostomy, bile fistula originating in the anterior inferior area of the right lobe of the liver was a complication, and it was successfully treated by construction of hepaticojejunostomy. No similar case has been reported in English language literature. It is of particular interest that the pathological features of the ectopic liver and the liver proper are quite similar. Copyright o 1991 by W-B. Saunders Company INDEX WORDS: Ectopic liver, umbilical; topic pancreas, jejunal.

biliary atresia;

ec-

A

LTHOUGH UNCOMMON, ectopic liver tissue has been found in various sites including gallbladder, adrenal gland, spleen, gastrohepatic ligament, umbilicus, esophagus, thorax, and omphalocele. When the liver is found outside the abdominal cavity, it most often forms part of the contents of a diaphragmatic hernia and omphalocele. Ectopic liver in the umbilicus is extremely rare, and only two cases of this anomaly has been reported in the English language literature. This report deals with a neonate who presented with an ectopic liver in the umbilicus in conjunction with biliary atresia in the liver proper and an ectopic pancreas in the jejunum. CASE

REPORT

A 2-day-old Korean girl presented with a fist-sized mass in the fetal end of the umbilical cord. She was born by vaginal delivery on December 12, 1987 after 40 weeks’ gestation and weighed 3,240 g. The mother was a healthy 29-year-old woman (gravida 3, para 3) who has three healthychildren. The antenatal history was unremarkable. The umbilical mass looked like a medium sized omphalocele at a first glance and measured 7.5 x 6.0 x 3.0 cm; the external surface was greenish-yellow and rubbery firm (Fig 1). Admission laboratory data included: hemoglobin 13.0 g, hematocrit 62.3%, white cell count 22,100 with poly 85%, platelet 189,000, bilirubin (T/D) 16.913.2 mg%, alkaline phosphatase 139.0 U/L, glucose 42 mg%, and calcium 9.1 mg%. On day 3, a circular incision was made along the base of umbilical mass from the skin through the peritoneum and the mass was excised. There was no connection between the mass and the peritoneal cavity. No umbilical defect could be found. The cut surface of the mass showed a well-demarcated, round, brownish and solid lesion, measuring 6.5 cm in diameter (Fig 2). The microscopic examination of the mass showed liver tissue with mild to moderate portal fibrosis with ductular proliferation, heavy infiltrates of mononuclear cell, and cholestasis (Fig 3). She recovered smoothly but remained jaundiced. The workup Journaloffediatricsurgery,

Vol26, No 2 (February), 1991: pp

219-222

DC

for jaundice included the following studies. Direct bilirubin increased to 6.1 mg%, alkaline phosphatase to 400.6 U/L, aspartate aminotransferase (AST) to 114.6 U/L, and alanine amino transferase (ALT) to 74.3 U/L. Hepatitis B, rubella, herpes, and cytomegalovirus were negative by enzymatic immunoassay, and Coombs’ test was negative. Technetium 99m DISIDA hepatobiliary scintigraphy before and after a 5-day course of luminal (5 mg/kg/d) pretreatment showed no excretion of the isotope into the extrahepatic biliary duct and intestinal tract in 24 hours, and HCI grade II of mild hepatocyte dysfunction. Ultrasound demonstrated no visible gallbladder and no evidence of the intrahepatic and extrahepatic biliary ductal dilatation. On day 28, the abdomen was entered through a generous right subcostal skin incision. With the right and left triangular ligaments divided, the liver was rotated up and out of the abdomen using the upper edge of the incision as a fulcrum. The liver was enlarged, smooth-surfaced, and dark brownish, and there was no gross cirrhosis (Fig 4). No evidence of connection between the liver and umbilicus could be detected. The gallbladder looked small and fibrotic and it had a small lumen through which a cholangiogram was taken using a no. 6 feeding tube. The cholangiogram showed excretion of dye into the duodenum but no demonstration of the proximal bile duct and intrahepatic biliary tree (Fig 5). The distal common duct looked like a fibrous band and the proximal extrahepatic duct was replaced by fibrotic tissue. With frozen-section of a piece of the liver tissue, Kasai type 1 portoenterostomy was performed with the excision of a small ectopic pancreas in the upper jejunum. On day 35, the abdomen was reentered because of a bile leak through a point of the incision. A pin-point bile leak was noted in the anterior inferior area of the right lobe of the liver, which was suture-ligated. The microscopic examination of the wedge-shaped liver showed significant portal fibrosis with increased ductular proliferation, mild infiltrates of mononuclear cells, and cholestasis, which were compatible with those of the biliary atresia (Fig 6). The microscopic finding of the fibrotic tissue of the porta hepatis showed microscopic bile ductules with 0.1 cm being the largest diameter. Two weeks following hepatic portoenterostomy, %Tc DISIDA scan showed good excretion of the isotope into the intestinal tract. On day 58, the patient made an uneventful recovery with decreased direct bilirubin to 3.5 mg% and was discharged. Since day 96, she was jaundice-free with bilirubin (T/D) 0.8/0.2 mg%. alkaline phosphatase 438.7 U/L, AST34.1 U/L, and ALT 41.5 U/L.

From the Division of Pediatric Surgery, Department of Anatomic Pathologv Keimyung University School of Medicine and Dongsan Medical Center, Taegu, Korea, and Children> Hospital National Medical Center, Washington, DC. Presented at the 4th Annual Meeting of the Korean Association of Pediatric Surgeons, Seoul, Korea, June 24, 1988. Address reprint requests to Woo-Hyun Park, MD, Division of Pediatric Surgery, Keimyung University, Dongsan Medical Center, I94 Dongsan Dong, Taegu 700-310, Korea. Copyright o 1991 by W.B. Saunders Company 0022-3468l91l2602-0028$03.00/0 219

220

PARK ET AL

Fig 1.

Ectopic umbilical liver.

On day 188, she was rehospitalized because of recurrent episodes of bile leak through the incision, and reexploration showed a fistulous tract originating in the anterior superior area of the right lobe of the liver that seemed to be the same site as previous bile leak. Cholangiogram was taken by placing a no. 6 feeding tube into the fistula, and it demonstrated a well-developed intrahepatic biliary tree and prompt excretion of dye into the jejunum (Fig 7). A hepaticojejunostomy was performed by anastomosing a loop of defunctionalized jejunum to the fistula in the liver. On the 10th postoperative day, she was discharged with good recovery and liver function test showed bilirubin (T/D) 0.9/0.2 mg%, alkaline phosphatase 266.5 U/L, AST 29.0 U/L, and ALT 29.8 U/L. With limited follow-up, she has been doing well and growing normally so far.

Fig 3. Photomicrograph of the ectopic liver shows mild to moderate portal fibrosis with ductular proliferation and heavy infiltrates of mononuclear cells (H&E, original magnification x 100).

omphalocele,’ splenic capsule,’ pancreas, and retroperitoneum. Accessory or anomalous lobes of liver have been reported in the abdomen,1~4~‘0thoracic cavity,11q’3anterior abdominal wa11,14and the omphalocele.15 In comparison, most of the ectopic liver tissue is found separately from the liver proper, but many of the accessory or anomalous lobes are connected to the liver proper by means of a pedicle or mesentery.

DISCUSSION

Ectopic liver tissue has been found in a variety of locations including gallbladder,1~2 adrenal gland: gastrohepatic ligament,4 umbilicus,5.6 esophagus,’ sac of

Fig 2. Cut surface of the ectopic liver shows a well-demarceted, round, brownish, solid lesion, M-cm in diameter.

Fig 4. The liver wlth biliary atrasia fat age 23 days). No evidence of connection between the liver and the umbilicus was noted. The gallbladder is very small, and the extrehepatic biliary duct is replaced by fibrotic tissue.

ECTOPIC UMBILICAL

LIVER AND BILIARY ATRESIA

Fig 7. Cholangiogram through the fistula in the anterior inferior surface of the right lobe demonstrates well-visualized intrahepatic biliary tree and prompt excretion of dye into the jejunum.

Fig 5. The gallbladder cholangiogram shows excretion of dye into the duodenum, but no demonstration of the proximal bile duct.

Only two cases of ectopic liver in the umbilicus were found in the English language literature. The first one, reported by Nora and Carr: was a newborn infant with a 7 x 7.5 cm ectopic liver tissue connected

Fig 6. Photomicrograph of the liver proper shows dense portal fibrosis with increased ductular proliferation and mild infiltrates of mononuclear cells (H&E, original magnification x100).

to the liver proper by blood vessels. The second was reported by Shaw and Pierog,6 and was a newborn infant with a 2.0-cm, pendunculated umbilical mass as a focus of infection. Although the majority of cases with an ectopic liver or accessory liver are found incidentally during operation or postmortem examination, and it can give rise to symptoms that vary from location to location. Pujari and Dedhare4 reported an accessory lobe attached by a mesentery to the gastrohepatic ligament, which presented with recurrent abdominal pain by torsion, and the cited six symptomatic cases. Jimenez and Hayward’ described an isolated ectopic liver that caused an esophageal obstruction. Shaw and Pierog6 reported a small ectopic liver in the umbilicus as a focus of infection. The development of ectopic liver tissue appears to be caused by entrapment of the nests of cells in the region of the foregut following closure of diaphragm or umbilical ring. Occasionally, however, the ectopic liver nodules are isolated and the biliary system drains into other organs’ or are without any apparent drainage system.’ The present case most likely resulted from entrapment of a portion of liver by closure of the umbilical ring. We are not sure whether or not the fistula opening in the anterior inferior area of the right lobe is evidence of a biliary duct connected to the ectopic liver. According to the operative finding of the Iiver

222

PARK ET AL

proper mentioned previously, the fistula in the liver is unlikely to be the evidence of connection. Ectopic liver tissue or accessory liver tissue may show the same pathologic features as the liver proper, ie, cirrhosis’ and metastatic tumor.3”6 The case presented herein appears to belong to this category because the pathological pictures of both the ectopic

liver and the liver proper are very similar. The minor difference is that the findings of the liver proper are more advanced than those of the ectopic liver in terms of ductal proliferation and portal fibrosis. This case strongly reemphasizes that factors such as cirrhosis, metastatic tumor, and viral infection act on liver tissue regardless of its location.

REFERENCES 1. Cullen T: Acceory lobes of the liver. Arch Surg 11:718-764, 1925 2. Lieberman MK: Cirrhosis in ectopic liver tissue. Arch Path01 821443~446,1966 3. Tsuchida Y, Yokomori K, Saito S, et al: Stage IV-S neuroblastoma involving the liver and ectopic liver: Report of an unusual case. Cancer 53:1609-1611,1984 4. Pujari BD, Dedhare SG: Symptomatic accessory lobe of liver with a review of the literature. Postgrad Med J 52:234-236,1976 5. Nora E, Carr EE: Umbilical accessory liver. Am J Obstet Gynecol52:330-335,1946 6. Shaw A, Pierog S: Ectopic liver in the umbilicus: An usual focus of infection in a newborn infant. Pediatrics 44:448-450, 1969 7. Jimenez AR, Hayward RH: Ectopic liver: A cause of esophageal obstruction. Ann Thorac Surg 12:300-304,197l 8. Fock C: Ectopic liver in omphalocele. Acta Pediatr 52:288292,1963

9. Heid GJ, Von Haam E: Hepatic heterotopy in the splenic capsule. Arch Pathol46:377-379,1948 10. Watson JR, Lee RE: Accessory lobe of liver with infarction. Arch Surg 88:490-493,1964 11. Kaufman SA, Madoff IM: Intrathoracic accessory lobe of the liver. Ann Intern Med 53:403-407,196O 12. Desvignes G, Mary H, Levasseur P, et al: A propos de deux observations d’heterotopies hepatiques intrathoraciques. Ann Chir Thorac Cardiovasc 14:177-180,1975 13. Hansbrough ET, Lipin RJ: Intrathoracic accessory lobe of the liver. Ann Surg 145:564-567,1956 14. Johnston G: Accessory lobe of liver presenting through a congenital deficiency of anterior abdominal wall. Arch Dis Child 40:541-544,1965 15. Kleinhaus S, Kaufer N, Boley S: Partial hepatectomy in omphalocele repair. Surgery 64:484-486,1968 16. Garber M: Accessory liver containing metastatic tumour. Virchows Arch Path01 Anat 385:361-364,198O

Ectopic umbilical liver in conjunction with biliary atresia: uncommon association.

The case of a neonate with an ectopic liver in the umbilicus in conjunction with biliary atresia in the liver proper and an ectopic pancreas in the je...
3MB Sizes 0 Downloads 0 Views