Natsumi Tanaka

Diagnosis and management of biliary cystic malformations in neonates

PURPOSE Recent advances in ultrasonography have made it possible to identify biliary atresia (BA) and choledochal cyst (CC) with biliary cystic malformations (BCM) both prenatally and neonatally. The early differential diagnosis between BA and CC is extremely important because operations must be performed as soon as possible before the livers of BA patients advance to an irreversible cirrhotic stage. The aim of this study was to differentiate patients with BCM and to determine the best course of management in the neonatal period. METHODS The medical records of patients that were diagnosed with BCM by a prenatal or neonatal ultrasound between 1997 and 2008 were reviewed. We retrospectively divided the BCM patients into the BA and CC groups and then compared the results of ultrasound, computed tomography, and laboratory tests between the 2 groups. RESULTS Ten patients were enrolled in the study. The median age at the time of corrective surgery was 74 days (range, 24-206 days). All of the BA cases received an operation by the time they were 60 days old. In the BA group (5 patients), the mean cyst size was 15 mm, the mean direct bilirubin (D-Bil) was 3.3 mg/dL, and the mean total bile acid (TBA) was 138.1 µmol/L at 30 days of age, whereas in the CC group (5 patients), cyst size, D-Bil, and TBA were 40 mm, 0.9 mg/dL, and 46.9 µmol/L, respectively. These differences between the 2 groups were statistically significant. All of the patients with CC successfully cleared their jaundice, whereas 4 patients with BA subsequently required liver transplantation for liver failure. In our study, all patients with BCM less than 21 mm, D-Bil greater than 2.5 mg/dL, and TBA greater than 111 µmol/L in the neonatal period were diagnosed with BA. CONCLUSIONS Our data suggested that patients with BCM smaller than 21 mm, D-Bil higher than 2.5 mg/dL, and TBA higher than 111 µmol/L in the neonatal period were more likely to have BA than CC. This potential diagnosis should be surgically examined and corrected as soon as possible.

Graft fibrosis in patients with biliary atresia after pediatric living‐related liver transplantation

Ueno T, Tanaka N, Ihara Y, Takama Y, Yamada H, Mushiake S, Fukuzawa M. Graft fibrosis in patients with biliary atresia after pediatric living‐related liver transplantation. 
Pediatr Transplantation 2011: 15: 470–475.

Mature teratoma arising from an intraabdominal testis in a 2-month-old boy: case report and review of intraabdominal testicular tumors in children

Tumors arising in an undescended testis are rare in infants. We report a mature teratoma in an intraabdominal testis of a 2-month-old boy. He presented with a large left-sided abdominal mass and nonpalpable left testis. Abdominal ultrasound and computed tomography showed a well-defined, sizable cystic tumor with focal calcification in the lower abdomen. Tumor markers were all within normal limits. Surgical resection revealed a large tumor arising from an intraabdominal left testis, and histologic analysis identified a mature teratoma. The tumor was completely removed, and there was no recurrence at follow-up 3 years later. The histologic condition of prepubertal intraabdominal testicular (IAT) tumors is quite different from that of testicular tumors in childhood descended testes and postpubertal IAT tumors. A total of 30 cases of IAT tumors in children, including our case, were reviewed to elucidate this issue.

Magnetic compression anastomosis for postoperative biliary atresia

We report a case of successful magnetic compression anastomosis (MCA) for obstructed cyst‐jejunostomy in a young woman who had undergone surgery for type 1 biliary atresia (BA) on day 78 of life. A 16‐year‐old girl was admitted with obstructive jaundice. Jaundice resolved with percutaneous trans‐hepatic cholangiodrainage (PTCD) but contrast medium injected from the PTCD tube did not flow through the anastomosis. Magnets were placed on each side of the anastomosis, in the cyst and the jejunum, to compress the partition. On postoperative day (POD) 6, the anastomosis was recanalized and the PTCD tube placed trans‐anastomotically until POD 245. The patient remained free from jaundice after removal of the PTCD tube. MCA can be a useful and less invasive procedure for treating biliary tract anastomotic obstruction in patients with BA.

Fibroadenoma in adolescent females after living donor liver transplantation

Breast FA is the most common breast tumor diagnosed in young women. Female renal transplant recipients on CsA have an increased risk of developing FA. However, reports of FA after LDLT have not been described. Our objectives were to determine the incidence of FA, analyze risk factors for FA, and evaluate treatment strategies in adolescent females after LDLT. A total of 18 female patients aged 10‐19 years who underwent LDLT and survived at least one year after transplantation were enrolled in our study. The incidence of FA was 11.1%. To determine pre‐ or post‐transplant conditions that are associated with FA after transplantation, the patients were divided into two groups according to the presence or absence of FA: FA group (n=2) and non‐FA group (n=16). There were no differences in mean age at LDLT, mean age at breast evaluation, and mean duration between transplantation and breast evaluation between the two groups. However, there was a difference in the immunosuppressive regimen between the two groups. The FA group was maintained on CsA, whereas the non‐FA group was maintained on tacrolimus. CsA might be implicated in FA development in adolescent females after LDLT.