Seok Joo Han

Biliary Atresia: Color Doppler US Findings in Neonates and Infants

PURPOSE To describe color Doppler ultrasonographic (US) findings in livers of neonates with biliary atresia (BA) and to compare them with US findings in livers of neonates with non-BA and control subjects. MATERIALS AND METHODS Institutional review board approval was obtained; acquisition of informed consent was exempted. US and color Doppler US findings were retrospectively reviewed in 64 patients with neonatal cholestasis and 19 control subjects. BA and non-BA were confirmed in 29 and 35 patients, respectively. Three pediatric radiologists assessed US and color Doppler US images, independently documented their findings, and resolved discrepancies by consensus. Triangular cord (TC) sign, gallbladder length, and hepatic artery and portal vein diameters were evaluated on US images. The presence of hepatic subcapsular flow was evaluated on color Doppler US images. Diagnostic value of TC sign and hepatic subcapsular flow in the diagnosis of BA were evaluated. Significance of hepatic artery and portal vein diameters in each group was assessed. RESULTS In the diagnosis of BA, sensitivity and specificity of the TC sign on US images were 62% and 100%, respectively. On color Doppler US images, hepatic subcapsular flow was detected in all patients with BA and in five patients with non-BA. At the first review, there was a discrepancy between radiologists in interpretation of hepatic subcapsular flow in patients with non-BA. However, consensus was reached at the second review. There was no hepatic subcapsular flow in control subjects. Sensitivity and specificity of hepatic subcapsular flow on color Doppler US images were 100% and 80%-86%, respectively, on the basis of individual interpretations of reviewers. Sensitivity and specificity of hepatic subcapsular flow on color Doppler US images were 100% and 86%, respectively, on the basis of consensus reading. Mean diameter of the hepatic artery in patients with BA (2.1 mm +/- 0.7 [standard deviation]) was significantly larger than that in patients with non-BA (1.5 mm +/- 0.4, P < .001) and control subjects (1.5 mm +/- 0.4, P = .001). CONCLUSION The presence of hepatic subcapsular flow is useful for differentiating between BA and other causes of neonatal jaundice.

Assessment of liver fibrosis and cirrhosis by aspartate aminotransferase-to-platelet ratio index in children with biliary atresia

Background: In patients with biliary atresia (BA), liver fibrosis and cirrhosis commonly occur even after Kasai hepatoportoenterostomy. Although liver biopsy is the gold standard to evaluate liver fibrosis, it is invasive and may result in life-threatening complications. The aspartate aminotransferase-to-platelet ratio index (APRI) is a safe and simple method to assess liver fibrosis in patients with chronic liver diseases. To use APRI as a postoperative follow-up tool, we validated the diagnostic power of APRI for the degree of liver fibrosis in postoperative patients with BA. Patients and Methods: Patients with newly diagnosed BA who underwent the Kasai procedure between March 2006 and May 2009 were analyzed. Several laboratory tests including APRI were performed. Liver wedge biopsy specimens were obtained during the surgical procedure, and histopathologic analyses were performed using the Metavir classification. Results: Thirty-five patients (12 boys, median age of 1.9 months) were enrolled. Metavir scores were F1 in 0, F2 in 11, F3 in 11, and F4 in 13 patients. The areas under the receiver operating characteristics curves for F ≥ 3 and F = 4 were 0.92 and 0.91, respectively. Distinct optimal cutoff values of APRI for F ≥ 3 and F = 4 were obtained (1.01 and 1.41, respectively). Clinical outcomes of patients were significantly different between 2 groups (noncirrhosis vs cirrhosis) based on APRI before and 3 months after the Kasai procedure. Conclusion: APRI may be used as a simple and readily available tool for assessing liver fibrosis without additional risks in patients with BA during postoperative follow-up care.

Hepatic Fibrosis Scan for Liver Stiffness Score Measurement: A Useful Preendoscopic Screening Test for the Detection of Varices in Postoperative Patients With Biliary Atresia

Objective:Even after successful Kasai portoenterostomy, progressive hepatic fibrosis in postoperative patients with biliary atresia (BA) can be associated with portal hypertension and esophageal or gastric varices. Therefore, early diagnosis and close follow-up of varices are important. We investigated the correlation between the liver stiffness scores measured by FibroScan and the presence of esophageal or gastric varices to examine the usefulness of FibroScan as a preendoscopic screening test for varices. Patients and Methods:A total of 49 of 81 children with BA following successful Kasai operations were enrolled in this study. FibroScan and endoscopic examination were performed prospectively. Results:There were 22 males (44.9%) and the mean age of the patients was 3.8 ± 2.7 years. Esophageal or gastric varices were present in 30 patients (Vx group) and absent in 19 (nVx group). The mean liver stiffness score was significantly higher in the Vx group (21.35 ± 10.31 kPa in the Vx group versus 9.75 ± 8.61 kPa in the nVx group, P < 0.001). The optimal cutoff value of the liver stiffness score for the prediction of a varix was 9.7 kPa with a sensitivity of 0.97 and a specificity of 0.80. Conclusions:Liver stiffness scores measured by FibroScan correlate well with the presence of esophageal or gastric varices. FibroScan is a novel, noninvasive, and useful screening method for the preendoscopic detection of varices in postoperative patients with BA.

Spinal dysraphism with anorectal malformation: lumbosacral magnetic resonance imaging evaluation of 120 patients

PURPOSE We evaluated the prevalence of spinal dysraphism (SD) in patients with anorectal malformation (ARM) by magnetic resonance imaging (MRI). METHODS From January 2002 to March 2009, 120 patients with ARM who underwent anorectal reconstruction were evaluated for SD with sacral plain film, spinal ultrasonography (US), and lumbosacral MRI. We adopted Krickenbeck international classification of ARM. RESULTS Spinal dysraphism was present in 41 (34.2%) of 120 patients with ARM, 3 (13.0%) of 23 patients with perineal fistula, 7 (29.2%) of 24 patients with vestibular fistula, 4 (36.4%) of 11 patients with rectovesical fistula, 18 (40.9%) of 44 patients with rectourethral fistula, and 9 (60.0%) of 15 patients with cloacal anomaly (P = .04). Among 41 patients having SD detected by MR, 26 patients (26/41; 63.4%) underwent detethering surgery for tethered spinal cord. The mean sacral ratio (SR) in patients who underwent detethering surgery (0.54 +/- 0.19) was significantly lower than in patients who did not undergo detethering surgery (0.69 +/- 0.13; P < .001). The optimal cutoff for the SR value predicting SD requiring detethering surgery was 0.605, with sensitivity of 65.4% and specificity of 77.7%. CONCLUSIONS Spinal dysraphism is common in patients with ARM, and its prevalence is higher in patients with complex ARM. Spinal anomalies can occur even with benign types of ARM and, therefore, that all patients should be screened. Magnetic resonance imaging is useful in detecting occult SD that may be missed by conventional radiologic evaluation, physical examination, and spinal US. We further recommend a lumbosacral MRI examination in those whose SR is lower than 0.6.

Actual half-life of alpha-fetoprotein as a prognostic tool in pediatric malignant tumors

In a retrospective study, the prognostic value of monitoring the decay of alpha-fetoprotein (AFP) was assessed. Serum AFP was determined serially in 18 children with malignant germ-cell or hepatic tumors: 7 endodermal sinus tumor, 3 embryonal carcinoma, 5 malignant teratoma, 2 hepatoblastomas, and 1 hepatocellular carcinoma. The actual half-life (AHL) of AFP was computed after surgical resection of the tumor. In group 1, which had complete resection and no recurrence during follow-up (n = 13), the AHL of AFP was 4.0 ± 0.9 days. In group 2, which had incomplete resection or recurrence during follow-up (n = 5), the AHL of AFP was 24.8 ± 20 days, significantly longer than that of group 1 (P = 0.0026). The increased AHL of AFP indicated residual active tumor after surgical resection. The AHL of AFP may be more sensitive than serial monitoring of AFP in detecting preclinical recurrence after surgical resection of AFP-secreting tumors. Treatment strategies can be based on AFP clearance, and prospective clinical trials are warranted.

An unusual case of monozygotic epigastric heteropagus twinning

Epigastric heteropagus refers to unequal and asymmetric conjoined twins in which the dependent component (parasite) is smaller and usually attached to the epigastrium of the dominant component (autosite). Epigastric heteropagus is a very rare type of conjoined twins. It is generally assumed that conjoined twins represent a form of monozygotic twinning, but there has been no confirmation of this monozygotic hypothesis. Epigastric heteropagus twins differ in several ways from symmetrical conjoined twins. These include male preponderance, and no major connection of vessels, bowels, or bones. These significant characteristics suggest to the authors that the fusion of two fertilized ova and the atrophy of one fetus resulted in this interesting malformation. However, a DNA study indicated monozygotic origin in their case. The possible pathogenesis was ischemic atrophy of the body structure of the monozygotic conjoined twins at an early gestational age.

Clinical experience with conservative surgery for vaginal endodermal sinus tumor

Two cases of vaginal endodermal sinus tumor (EST), a rare pediatric malignancy, were managed with conservative surgery followed by adjuvant chemotherapy. The first case involved a 9-month-old girl with EST of the vagina, who was treated with a partial vaginectomy and VAC regimen (vincristine, actinomycin D, cyclophosphamide) during a 2-year period. The serum alpha-fetoprotein (AFP) level returned to normal after excision of the tumor, and it remained normal throughout the treatment period. There was no evidence of disease 30 months after diagnosis. The second case involved an 8-month-old girl with EST of the vagina, who was treated with local excision of the mass through a vaginotomy. The VAC regimen was administered, but the serum AFP level remained elevated. A follow-up abdominopelvic computed tomography scan, taken 4 months after the operation, showed local recurrence of the tumor. The VAC regimen was then changed to a BEP regimen (bleomycin, etoposide, cisplatin). The serum AFP level returned to normal after 2 courses of the new regimen, and no tumor was visible on the follow-up magnetic resonance imaging study. For vaginal EST, primary conservative surgery and adjuvant chemotherapy are attractive measures to preserve both reproductive and sexual function. The extent of conservative surgery requires at least a partial vaginectomy. Simple tumor excision may not be adequate to achieve cure or to prevent local recurrence, even with adjuvant chemotherapy. The serum AFP level is useful for diagnosing and monitoring vaginal EST in the infant.

Transient elastography and sonography for prediction of liver fibrosis in infants with biliary atresia.

The purpose of this study was to assess the diagnostic performance of transient elastography and sonography for noninvasive evaluation of liver fibrosis in infants with biliary atresia.

Agranular platelets as a cardinal feature of ARC syndrome.

We aimed to describe abnormal platelet morphology and its clinical significance in infants who were diagnosed with arthrogryposis renal dysfunction and cholestasis (ARC) syndrome. We collected all of the cases of ARC syndrome referred to a single pediatric referral center. In all patients, platelet counts and analysis of platelet morphology were performed with peripheral blood smear specimens. Electron microscopy images were obtained to examine the ultrastructure of the platelets. Over the 12-year period, 12 cases of ARC syndrome were identified. The sex ratio (male:female) was 1:1. The median birth weight was 3.15 kg (range, 2.3 to 3.8 kg). Failure to thrive was observed in all the patients. The major cause of death was recurrent febrile illness and pneumonia. The median age at death was 8.9 months (range, 2.6 to 28.8 kg). Their median body weight at death was 3.1 kg (range, 2.6 to 6.0 kg). Close examination of their peripheral blood smear (n=11) specimens showed large, pale, agranular platelets similar to those seen in gray platelet syndrome. Electron microscopic images of the platelets (n=7) revealed a lack of α; granules. Agranular platelets are a common finding in ARC syndrome. Agranular platelets should be considered as a cardinal feature of ARC syndrome and can be useful as a noninvasive diagnostic marker for ARC syndrome.

Vulvar lipomas in children: an analysis of 7 cases

PURPOSE Vulvar lipomas are a type of rare benign soft tissue tumor. This study describes 7 cases of vulvar lipomas in children, and it evaluates the clinical characteristics of this disease. METHOD We performed a retrospective review of pediatric patients at our institution who were diagnosed with vulvar lipomas between January 1998 and December 2007. RESULTS Seven girls who were between the ages of 7 and 11 years were included in the study. In 6 cases, the lipomas were located on the right side of the vulva. In 4 of those 6 cases, the lipomas were found on the right anterolateral side. Five cases had gross swelling of the vulva with a poorly demarcated mass, but 2 cases had a well-demarcated or pedunculated mass. The initial tentative diagnoses made before imaging studies were right inguinal hernia in 4 cases and Bartholins cyst in 2 cases. Preoperative imaging studies of ultrasound, computed tomography, and magnetic resonance imaging showed lipomatous characteristics of these tumors. All cases underwent surgical excision. Subsequent histopathologic examination demonstrated lipomas in each case. CONCLUSIONS Vulvar lipomas in children tend to occur on the right side, especially the right anterolateral side. It is necessary to differentiate this tumor from an inguinal hernia. Radiologic studies such as ultrasonography, computed tomography, and magnetic resonance imaging are useful for making the proper diagnosis. Surgical excision is the treatment of choice.