Laparoscopic Portoenterostomy (Kasai Operation)

Indications for Workup and Operation

Signs suggestive of BA include jaundice, pale stools, dark urine, and hepatomegaly. Unfortunately, there is no single clinical feature with sufficient sensitivity and specificity to differentiate BA from other causes of neonatal jaundice. Thus neonatal jaundice that persists for more than 2 weeks should be investigated at a tertiary health care facility. Meconium staining may be normal, and feces may be yellow during the neonatal period in more than half of patients, but the urine gradually turns dark brown. Although the liver is diseased, the bilirubin is conjugated, and kernicterus is not usually a concern in BA. Early diagnosis is mandatory, and investigations should not be protracted because treatment will be delayed.

Many protocols have been suggested, but the diagnostic workup should always include ultrasonography, biochemical exclusion of α1-antitrypsin deficiency and cystic fibrosis, liver scintigraphy, and a percutaneous liver biopsy. The histological appearance of the liver is characterized by portal tract edema, bile duct plugging and proliferation, a small cell infiltrate, and variable amounts of giant cell formation. Bridging fibrosis is a late feature but in some cases may be present even at diagnosis. Using this protocol, about 80% of affected infants will have a positive diagnosis prior to operation. However, a liver biopsy can be traumatic and specimens require histopathologic assessment for accuracy, which can be time consuming.

While percutaneous liver biopsy is often performed, laparoscopic inspection provides the opportunity to diagnose liver cirrhosis and gallbladder atrophy. Diagnostic laparoscopy also allows cholangiography to be performed if the gallbladder is not atrophic to identify the intrahepatic bile ducts and common bile duct (CBD) to diagnose/exclude BA. Liver scintigraphy is typically abnormal in BA, with rapid uptake by hepatocytes and virtually no excretion into the bowel, even on delayed images. Laboratory investigations should include conventional liver function tests plus γ-glutamyl transpeptidase, TORCH (toxoplasmosis, other, rubella, cytomegalovirus, and herpes simplex) titers, and coagulation tests to exclude vitamin K–dependent coagulopathy.

All infant cases of BA should have parenteral vitamin K (phytomenadione, 1.0 mg/day) supplementation, usually given for several days prior to operation. The bowel should be prepared with oral kanamycin. Oral feeding is discontinued 24 to 48 hours before the operation and glycerin enemas are given. Broad-spectrum antibiotics are administered preoperatively. Our protocol is to administer antibiotics intravenously in the operating room and continue them postoperatively until the C-reactive protein (CRP) is less than 0.3 mg/dL or the leukocytosis has resolved.

Operative Technique

The baby is positioned at the foot of the operating table, and the operating surgeon stands at the infant’s feet. An assistant/camera operator is situated slightly behind and to the left of the operating surgeon, and another assistant is located on the patient’s right to assist with exposing the porta hepatis and the anastomosis. The anesthetist is positioned near the baby’s head and the scrub nurse is situated to the left of the baby ( Fig. 22-1A ). The monitor is located above the infant’s head facing the operating surgeon. Vascular access, nasogastric tube insertion, and bladder catheterization are then performed.