Yasuyuki Higashimoto

Role of pediatric endoscopic retrograde cholangiopancreatography in an era stressing less-invasive imaging modalities.

Background:The significance of pediatric endoscopic retrograde cholangiopancreatography (ERCP) according to age or disease variation is inconclusive. This study aimed to evaluate the usefulness of pediatric ERCP in diagnosing or treating small children with pancreaticobiliary disorders, including choledochal cyst (CC) and biliary atresia (BA). Methods:From 1980 to 2011, 235 ERCPs were performed in 220 pediatric patients (median age, 2 years) at our institution. Underlying pathology was CC in 92 patients (3 years), BA in 62 patients (55 days), and others in 66 patients. Success and complication rates, and ERCP findings were retrospectively analyzed. Results:The overall success rate was 96%. Rates for individual pathologies were 99% for CC, 92% for BA, and 96% for others. The success rate was 100% among children >3 years, but 92% when limited to infants. Post-ERCP hyperamylasemia and duodenal perforation occurred in 9% and 0.4% of cases, respectively. Regarding preoperative evaluation of the pancreaticobiliary system in CC, ERCP clearly delineated pancreaticobiliary maljunction (79%) and the pancreatic duct (94%), whereas it visualized the common bile duct and intrahepatic bile duct at relatively low rates (77% and 33%, respectively). ERCP successfully identified 16 cases (18%) with non-BA of 90 patients with suspected BA. Moreover, ERCP demonstrated only pancreatic duct in 70% of all BA. Conclusions:ERCP among children including infants with CC and BA can be performed with fairly satisfactory results. Although pediatric ERCP can also help describe the pancreaticobiliary system in detail, its indication should be deliberately considered when anatomical information from less-invasive imaging modalities is insufficient.

Successful endoscopic balloon dilatation for hypertrophic pyloric stenosis

The authors successfully applied endoscopic balloon dilatation for the treatment of hypertrophic pyloric stenosis (HPS). The patient was an infant girl who had undergone repair of a giant omphalocele. Endoscopic balloon dilatation was performed using a 9-mm endoscope and an 8-mm polyethylene terephthalate (PET) balloon dilator. Dilatation was performed three times for 10 minutes. Vomiting continued after the dilatation. At the second session, dilatation was performed using a 12-mm PET balloon dilator. The 9-mm endoscope then passed through the pylorus. The patient has had no episodes of vomiting since the second treatment. This procedure is an important therapeutic option for selected patients with HPS.

Contralateral incidence after repair of unilateral inguinal hernia in infants and children

The parents of 206 patients with unilateral inguinal hernia who underwent hernia repair at Kobe Childrens Hospital in 1985 were interviewed by telephone to determine the incidence of contralateral occurence. There were 129 males and 77 females. The side of the initial hernia was the right in 120 patients and the left in 86. The age at presentation of the initial hernia ranged from 11 days to 13 years. There were 12 contralateral occurences (5.8%) These were seen in 8 males (6.6%) and 4 females (4.6%), in 6 after repair on the right side (5.0%) and 6 after repair on the left (6.9%). There was no preponderance of contralateral incidence in relation to age, sex, or original side in our series. Contralateral exploration is recommended only under circumstances in which abdominal pressure is increased by such conditions as ascites or a ventriculoperitoneal shunt.

Congenital true diverticula of the esophagus: A case report

Pseudodiverticulosis secondary to gastroesophageal reflux is a common disease in adults, but true esophageal diverticula are rare in infants and children. A 5-year-old boy was well until the age of 1 1/2 years when he started vomiting. An upper gastrointestinal series showed two diverticula bulging from the posterior right side of the middle esophagus associated with slight hiatal hernia and short esophagus. Diverticulectomy, the Collis-Nissen antireflux procedure, and pyloroplasty were performed simultaneously through a left thoracoabdominal incision. Histological examination of the diverticula showed that the wall of each diverticulum consisted of a full-thickness of esophageal wall. Because there was no tracheal remnant in the diverticula, this lesion is more likely to be a true diverticulum than a duplication.

A case of intra-abdominal desmoplastic small-round-cell tumor with elevated serum CA125.

Abstract An 11-year-old girl with a malignant intra-abdominal desmoplastic small-round-cell tumor (DSRCT) and a high serum level of the tumor marker CA 125 is reported. A high serum CA 125 level may be a specific marker for DSRCT, and thus may permit early diagnosis and treatment of this fast-growing tumor.

Diagnosis of biliary atresia can not be excluded by declining trend of serum direct bilirubin

The aim of this paper is to investigate the clinical courses of patients with biliary atresia (BA) during neonatal period. We examined 19 patients with BA, who underwent blood tests including direct bilirubin (D-Bil) within 20 days of age, in 3 tertiary hospitals in Japan. The first blood sample was collected at 8.4±6.5 days of age. The acholic stool was observed within 2 weeks of age in 16 cases (84.2%). Decrease of T-Bil was observed in all the subjects, with a range of reduction of 6.5±3.3 mg/dL, from 10.4±7.5 to 29.8±9.1 days of age. Decrease of D-Bil was also observed in 17 out of 19 cases (89%), with a range of reduction of 1.1±1.0 mg/dL, from 15.5±8.0 to 24.9±9.6 days of age. A significant decrease of D-Bil was observed in 2 cases of biliary atresia splenic malformation syndrome. We therefore conclude that clinicians treating icteric infants should not exclude a diagnosis of BA even if the level of D-Bil has a declining tendency.

Systemic and Local Cytokine Profile in Biliary Atresia

Purpose Systemic and local immune environments in human biliary atresia (BA) were analyzed. Methods Plasma concentrations of 19 inflammatory components in 16 preoperative BA patients (median age, 51 days), 13 normal controls (NCs) (44 days), and 15 cholestatic controls (CC) (23 days) were measured using flow cytometry, and compared according to post‐Kasai outcomes in BA patients. Hepatic mRNA levels of representative helper T (Th) cell cytokines and forkhead box protein 3 (FoxP3) quantified by real‐time reverse transcription polymerase chain reaction were compared between BA and non‐BA. Results No significant differences were observed between BA and control in serum Th1, Th2, or macrophage markers, while soluble cellular adhesion molecule (CAM) levels were significantly higher in BA (p < 0.0001 for NC; p = 0.0003 for CC). No statistically significant difference was discovered between BA with favorable and unfavorable prognosis. Hepatic mRNA levels of interferon‐gamma or interleukin‐4 showed no significant differences between BA and non‐BA, while FoxP3 was significantly higher in BA (p = 0.01). Conclusions A skewed bias toward specific Th‐oriented immunity was not demonstrated in either the systemic or local environment in the early stage of human BA, with patient prognoses not necessarily revealed by preoperative plasma inflammatory component levels. CAM and regulatory T cell roles and functions warrant further investigation.

Histological features of primary tumors after induction or high-dose chemotherapy in high-risk neuroblastoma

PurposeIn the recent years in Japan, an increasing number of patients with neuroblastoma (NB) are being treated by the “delayed local treatment (DL)” policy, undergoing surgery after the completion of high-dose chemotherapy with hematopoietic stem cell rescue (HDC). We reviewed the histopathological findings of second-look operations, including those of patients treated with DL.PatientsFrom 1998 to 2013, 26 patients with high-risk NB underwent radical operation following chemotherapy. Surgery was performed after induction chemotherapy in 17 cases (standard; STD), whereas 9 cases completed induction chemotherapy and HDC before undergoing tumor resection (DL). The amount of necrosis and the degree of differentiation within the post-treatment tumor were assessed.ResultsEighty-eight percent of the tumors showed necrosis in more than 1/3 of the specimen. Two DL cases showed complete disappearance of viable tumor cells. Amount of necrosis did not affect the prognosis of the patient. Tumors with immature, poorly differentiated phenotypes showed an extremely aggressive thereafter. Though not statistically proven, 123I-MIBG (metaiodobenzylguanidine) uptake may be correlated with the amount of viable cells remaining within the tumor, but not with the degree of differentiation.ConclusionsOur results support the previous reports advocating that tumors that sustain unfavorable histology after chemotherapy behave aggressively thereafter.

Successful reversal of persistent fetal circulation by inhaled nitric oxide in a newborn with overwhelming sepsis

The authors report a case of successful reversal of persistent fetal circulation (PFC) by inhaled nitric oxide (NO) therapy in a child with overwhelming sepsis. Criteria for extracorporeal membrane oxygenation (ECMO) were reached but due to an intracranial hemorrhage ECMO was contraindicated. Inhaled NO in combination with surfactant administration caused a dramatic reversal of the PFC. Inhaled NO therapy may be a useful and easily accessible measure for the treatment of PFC. It is more simple, and therefore may be more widely applicable, than ECMO.