The patient was a 6-year-old boy with a history of neonatal jaundice that was treated with phototherapy. In terms of family history, few relatives from the maternal family developed cholelithiasis in the past. The patient developed hemolytic crisis induced by parvovirus infection and received a diagnosis of HS from his previous physician. During the course of observation, the fatigability and anemia persisted. In addition, gallbladder stones were observed using ultrasound (US) in the previous hospital. Therefore, the patient was referred to our hospital for splenectomy. While waiting for an operation, calculous cholecystitis occurred twice and antibiotics were administered for a month. Blood tests revealed the following results: white blood cell count, 6300/?l; Hb level, 8.3 g/dl; T-bil level, 2.8 mg/dl; and D-bil level, 0.7 mg/dl, suggesting anemia and jaundice associated with a higher unconjugated bilirubin level. No other abnormalities were detected in the biochemical examination or coagulation test. Abdominal US revealed a contracted gallbladder that contained multiple small stones of 2–3 mm (Fig. 1).

Fig. 1

Abdominal ultrasound reveals a slight contracted gallbladder that contains multiple small stones measuring 2–3 mm.

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Abdominal CT revealed an enlarged spleen (91 × 51 mm) and accumulated small stones inside the gallbladder. Dilatation of the common bile duct or pancreatic duct was not observed (Fig. 2).

Fig. 2

Abdominal CT shows an enlarged spleen (arrowhead) and accumulated small stones inside the gallbladder (arrow). Dilation of the common bile duct or pancreatic duct was not observed.

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MRCP revealed small stones in the gallbladder but not in the common bile duct or intrahepatic bile duct. No other anatomical abnormality was observed in the biliary system. Hence, a diagnosis of HS complicated by gallstones was made, and laparoscopic splenectomy and gallbladder-preserving cholecystolithotomy were simultaneously performed after obtaining consent from the patient’s family.

During the operation, a wound retractor was first set through a vertical incision in the umbilical region. A 12-mm port was inserted on the right hypochondrium and was guided toward outside the gallbladder fundus through the incision site of the port (Fig. 3A). While holding the bottom of the gallbladder with two forceps, a small hole was made in the gallbladder and bile was aspirated. Then, we made a small window of approximately 1 cm and fixed the gallbladder wall and skin at four points (upper/lower and left/right) using a 3-0 absorbable suture to prevent any leakage of bile into the peritoneal cavity. From the same window, six black gallstones (2–3 mm) were removed using a curette (Fig. 3B). A choledochofiberscope (OLYMPUS CHF-P20Q Choledochoscope) was inserted through the window, confirming that there were no residual stones inside the gallbladder (Fig. 3C).

Fig. 3

A, The gallbladder fundus is lifted from a port of the right hypochondrium. B, Incision was made to open the gallbladder and the gallbladder and the abdominal wall were fixed to remove gallstones with curette. C, A choledochofiberscope was inserted in the gallbladder, confirming that there were no residual stones inside. D, The schema of the operative method.

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Subsequently, a tube (6 Fr.) was inserted in the gallbladder, and direct cholangiography was performed to confirm the absence of stone shadow in the common bile duct. The incision of the gallbladder was closed with 3-0 absorbable sutures, and the organ was returned to the peritoneal cavity. The 12-mm port was reinserted into the same site, and laparoscopic splenectomy was performed as a routine procedure. The operating time was 2 h and 58 min, and the amount of blood loss was 45 ml. The patient did not develop any postoperative complication. Anemia improved, and the patient was discharged 5 days after the operation. To date, i.e., a year after the operation, no recurrent cholelithiasis or gallstone reformation has been observed. Using the ellipsoid method, abdominal US revealed 64% shrinkage in the gallbladder size [2], suggesting that a successful contractile function of the gallbladder has been maintained (Fig. 4).

Fig. 4

The postoperative ultrasound shows no recurrence of gallstones, and the contraction of the gallbladder remains normal after a meal.

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