Idiopathic thrombocytopenic purpura complicated with biliary atresia: a rare occurrence and literature review.

The association of biliary atresia (BA) and idiopathic thrombocytopenic purpura (ITP) is extremely rare, with only 2 cases being reported in the literature. This report describes the very rare case of a 1-year-old boy with BA complicated with ITP after cholangitis and the successful steroid treatment of ITP. We review the literature on this type of relationship between BA and ITP and discuss the clinical features of this complication. Furthermore, the possible cause of the onset of ITP complicated with BA was explored in this report.

Management and outcome in prenatally diagnosed sacrococcygeal teratomas.

BACKGROUND: The aim of the present study was to retrospectively determine the clinical factors affecting the outcome after birth in prenatally diagnosed sacrococcygeal teratomas (SCT). METHODS: Six cases of prenatal SCT were identified from January 1985 until August 2005. A retrospective review of case-notes and pathological reports was carried out. Clinical data during the perinatal period, operative findings, postoperative complications and follow up were evaluated in the patients with prenatally diagnosed SCT. RESULTS: SCT presented as type I in two neonates and type III in four between 22 and 33 weeks' gestation. Fetal intervention was not performed for any fetus. Five of six were delivered by cesarean section and the other was delivered vaginally due to small tumor size. Patients were born at between 29 and 39 weeks' gestation and weighed from 1840 to 3500 g. All patients with type III SCT presented with related diseases, including bilateral hydronephrosis, neurological deficit of the communicating peroneal nerve such as paralytic talipes equines, bladder or bowel dysfunction, high-output cardiac failure, or fetal hydrops in one of a set of fraternal twins. A baby with high-output cardiac failure and fetal hydrops underwent urgent cesarean section at 29 weeks' gestation and died 8 days after birth despite intensive care due to multi-organ failure. In five cases, surgery was successful with good outcomes maintained at follow-up of between 8 months and 14 years. CONCLUSIONS: Detailed ultrasound should be performed to rule out associated anomalies, and determine the presence or absence of hydrops in prenatally diagnosed SCT. Fetal hydrops, orthopedic impairment such as lower extremity weakness and swelling, and urinary incontinence are important clinical factors affecting the outcome after birth in prenatally diagnosed SCT. In particular, the present study indicated that the association of a fraternal twin and fetal hydrops makes it very difficult to treat SCT perinatally.

Usefulness of axial planes of helical computed tomography for diagnosis of pancreaticobiliary maljunction in early infants with negative findings on magnetic resonance cholangiopancreatography.

PURPOSE: Magnetic resonance cholangiopancreatography (MRCP) is not sufficient to detect pancreaticobiliary maljunction (PBM) in young infants because the main pancreatic duct is not visualized and respiratory artifacts occur. To our knowledge, there are no reports highlighting the diagnostic accuracy of evaluation using the axial planes of helical computed tomographic (CT) scanning with contrast medium instead of 3-dimensional (3D) reconstruction. The aim of this study was to describe our experience and the characteristics of 3 children with PBM diagnosed using the axial planes of helical CT with contrast medium, although they showed negative findings of PBM by MRCP, instead of 3D reconstruction. METHODS: Three patients aged from 1 month to 3 years were diagnosed with PBM using the axial planes of helical CT with contrast medium though MRCP could not show the common channel and/or the entrance of the common channel into the duodenum. RESULTS: In all 3 patients, PBM of the common channel was not revealed by MRCP. On the other hand, axial planes of contrast-enhanced helical CT scans showed PBM clearly. CONCLUSIONS: Our experience suggests that axial planes of the contrast-enhanced helical CT scan comprise an accurate tool for the diagnosis of fusiform-type PBM and could replace MRCP in younger children. Further studies are necessary for better assessment of the potential advantages and pitfalls of this modality.

CD56-immunostaining of the extrahepatic biliary tree as an indicator of clinical outcome in biliary atresia: a preliminary report.

The aim of this study was to evaluate the usefulness of CD56-immunostaining using extrahepatic biliary tree (EHBT) specimens as a predictive factor in biliary atresia (BA). The staining intensity of CD56-stained specimens was scored in 13 children with BA as follows: 0 = no staining, 1 = weak staining, 2 = moderate staining, and 3 = strong staining. There was strong (score 3) staining of CD56 in the EHBT specimens of 4 patients with more than 1.5 mg/dl of serum total bilirubin in accordance with a decrease of jaundice. Weak staining of CD56 was seen in the EHBT specimens of patients with no or only one episode of postoperative cholangitis. Strong staining of CD56 was noted in the EHBT specimens of 4 patients who underwent liver transplantation. Our present findings support the possibility of discriminating between good and poor prognoses of clinical outcome after Kasai portoenterostomy.

Rapidly enlarging solitary nonparasitic cyst of the liver in a child presenting as acute abdomen.

Symptomatic solitary nonparasitic cysts of the liver (SNCL) are rarely encountered in children, especially rapidly enlarging cysts presenting as acute abdomen. Therefore, it is difficult to establish the pre-operative diagnosis and to determine the treatment protocol of SNCL. While imaging techniques such as ultrasonography and computed tomography are modalities for diagnosis of SNCL, making a pre-operative diagnosis in the case of very large cysts remains difficult. We present a child with SNCL who initially presented with a rapidly enlarging cyst presenting as acute abdomen, and also provide a review of the literature. Moreover, we evaluate the surgical procedures and conclude that total excision of the cysts when possible is a suitable treatment procedure in children.

Laparoscopic liver biopsy using cup-shaped punch biopsy forceps and argon beam coagulator in children.

Needle liver biopsy is insufficient for measuring enzyme activity in liver tissue in child cases of intrahepatic cholestasis because the biopsy specimen obtained is too small. This study was undertaken to validate the feasibility of a new, relatively non-invasive laparoscopic liver biopsy technique combining the use of laparoscopic cup-shaped punch biopsy forceps (CPBF) and an argon beam coagulator (ABC) handpiece for the diagnosis and examination of liver enzyme activity in cases of intrahepatic cholestasis in children. The authors performed laparoscopic liver biopsy with the combined use of laparoscopic CPBF and an ABC handpiece in 10 children aged 4 months to 9 years old. Two 5-mm trocars were inserted in each patient after their abdomens had been filled with carbon dioxide gas at a pressure of 8 mmHg. Four to five specimens (each: 0.5 cm(3) in size) were taken at the anterior edge of the left lobe of the liver using laparoscopic CPBF. ABC was sprayed on to the cut liver surface to achieve hemostasis. The duration of the laparoscopic procedure ranged from 25 to 64 (44 +/- 12.8) min. The maximum intraoperative hemorrhage from the biopsied liver bed was 30 ml. The bleeding was easily controlled using ABC for about 1 min. There were no cases of postoperative bleeding, bile leakage from the cut surface, nor intraabdominal infection. There were also no death cases, and only one complication (hydrocele testis) was recorded. Examination by microscopy and assays of enzyme activities were performed using these biopsy specimens, which were sufficient for diagnosis in all patients. Laparoscopic liver biopsy combining the use of laparoscopic CPBF and an ABC handpiece can be performed safely, is less invasive, and provides sufficient samples for examination both by microscopy and enzyme activity assays.