Refractory pediatric nonrhabdomyosarcoma soft tissue sarcoma associated with ectopic production of beta hCG and hypercalcemia induced by PTHrP.

A 3-month-old male with a mass on the right side of his back was admitted to our hospital. The tumor was a pathologically high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). Treatment included subtotal tumor resection followed by chemotherapy. Elevation of serum beta hCG and hypercalcemia with detection of PTHrP was associated with tumor progression. The tumor was refractory to multiagent chemotherapy, and the patient died of the disease at 22 months of age. This case is novel in demonstrating a beta hCG secreting refractory NRSTS.

Pancreatobiliary reflux and the length of a common channel.

BACKGROUND/PURPOSE: Gallbladder cancer occurs frequently in patients with pancreaticobiliary maljunction due to pancreatobiliary reflux. Pancreatobiliary reflux is also detected in some patients with a relatively long common channel. This study aimed to clarify the correlation between pancreatobiliary reflux and the length of a common channel. METHODS: Two hundred and three patients, in whom both the length of a common channel and amylase level in the bile were measured, were enrolled from nine centers. RESULTS: Bile amylase level was correlated with the length of a common channel (P < 0.01). The minimum length of a common channel that could induce a markedly elevated amylase level in the bile (>1,000 mg/dl) was determined as 5 mm. We redefined high confluence of pancreatobiliary ducts (HCPBD) as cases with a common channel > or = 5 mm, in which the communication between the pancreatic and bile ducts was occluded with the sphincter contraction. Gallbladder cancer was found in 20% of 56 redefined HCPBD patients. Bile amylase level >1,000 mg/dl and biliopancreatic reflux were detected in 79 and 95% of the patients, respectively. CONCLUSIONS: Patients with a common channel > or = 5 mm (redefined HCPBD) should be monitored for the development of gallbladder cancer, as they frequently showed significant pancreatobiliary reflux.

Successful preemptive surgical division of type 2-congenital extrahepatic portosystemic shunt in children.

PURPOSE: A congenital extrahepatic portosystemic shunt (CEPS) is a rare abnormality. The shunts are classified into 2 types. Of these, a type 2-shunt is a side-to-side one, which may be treated by a simple shunt division. The aim of this retrospective study was to clarify the effects of a surgical shunt division on 4 children with type 2-CEPS. PATIENTS: Between June 2002 and June 2008, 4 children with type 2-CEPS underwent a surgical shunt division. Various clinical factors of each patient, including shunt types, shut ratios evaluated by portal scintigraphy using (123)I-iodoamphetamine, serum levels of ammonia and total bile acids before and after surgery were evaluated. FINDINGS: Two children had a conventional open surgery and the other two had a laparoscopic surgery. The serum levels of ammonia as well as total bile acids of these children decreased significantly to the normal levels within a month after the surgical shunt divisions. All the children had a better clinical course. CONCLUSIONS: A shunt division, especially by laparoscopic surgery, is an effective therapy for type 2-CEPS. To the best of our knowledge by reviewing literatures, our cases are the youngest ones treated by laparoscopic shunt division.

Laparoscopic strategy for inguinal ovarian hernias in children: when to operate for irreducible ovary.

BACKGROUND: Sliding indirect inguinal hernias containing the ovary and fallopian tube are not uncommon in younger girls. Although an irreducible ovary is not at great risk of incarceration, this condition may become a significant risk factor for torsion or strangulation. This paper describes the indications for surgery in children with irreducible ovaries. METHODS: There were 650 children (413 boys and 237 girls) with inguinal hernias treated with laparoscopic percutaneous extraperitoneal closure (LPEC) over a period of 12 years and 7 months. RESULTS: Of 237 girls with inguinal hernias, 46 had an ovary in the hernial sac, and 15 of these (age range, 4 weeks to 4 years) had irreducible ovarian hernias. Eleven were corrected laparoscopically, and their hernial orifices were repaired by LPEC. Their ovaries revealed edematous enlargement at surgery. One who was awaiting elective herniorrhaphy developed a strangulated ovarian hernia, and she needed salpingo-oophorectomy.The remaining three were corrected laparoscopically by cutting the external inguinal ring with a small skin incision.There was a high incidence of irreducible ovaries (32.6%) among younger girls with ovarian hernias,and four of them developed incarcerated ovaries. CONCLUSION: Many irreducible ovarian hernias can be corrected laparoscopically, and their hernial orifices are easily repaired by LPEC. We strongly emphasize that irreducible ovarian hernias should be treated by LPEC,as soon as possible after they are detected.

Laparoscopic percutaneous extraperitoneal closure for inguinal hernia in children: clinical outcome of 972 repairs done in 3 pediatric surgical institutions.

BACKGROUND/PURPOSE: In 1995, we developed laparoscopic percutaneous extraperitoneal closure (LPEC) to treat inguinal hernias in children. This study evaluated LPEC's safety, efficacy, and reliability in 3 hospitals. METHODS: In 2 hospitals, LPEC was the standard procedure used to repair inguinal hernias in children, and in 1 hospital, it was done optionally in girls. During LPEC, a 4.5-mm laparoscope was placed through an umbilical incision, a 2-mm grasping forceps was inserted on the left side of the umbilicus, and a 19-gauge LPEC needle with suture material was inserted at the midpoint of the right or left inguinal line. The hernial sac orifice was closed extraperitoneally by circuit suturing around the internal inguinal ring using the LPEC needle. RESULTS: Nine hundred seventy-two LPECs were performed on 711 children (age range, 18 days to 19 years): 384 boys had 500 internal inguinal rings closed and 327 girls had 472 internal inguinal rings closed. Operating time for uni- or bilateral inguinal hernias ranged from 10 to 30 minutes. No complications occurred during surgery. The recurrence rate was 0.73% during follow-up (range, 5 months to 10 years). No hydroceles or testicular atrophy occurred after surgery. CONCLUSION: Laparoscopic percutaneous extraperitoneal closure for inguinal hernia in children appears to be safe, effective, and reliable.

Is acetylcholinesterase activity in neorectum after laparoscopic endorectal pull-through method for Hirschsprung's disease a primary or a secondary condition?

Between 1996 and 2002, 22 cases of Hirschsprung's disease were treated laparoscopically by endorectal pull-through operation. The proximal margin of the resected bowel materials from these 22 patients were examined histologically by frozen sections at surgery and also after surgery using H&E staining, and complete resection of the aganglionic segment was confirmed. Eleven patients, in whom an informed consent was obtained at randomly, underwent suction biopsy from the pulled-though neorectum after surgery. Of these 11 patients, 2 revealed many AchE-positive nerve fibers in the lamina propria, the muscularis mucosa and around the ganglion cells in the submucosal plexuses. These structural changes were similar to intestinal neuronal dysplasia that was characterized by hyperganglionosis and other neuronal abnormalities. The remaining 9 revealed no AchE activity. Unexpectedly, the two patients showing AchE activity in their neorectum continued to have persistent constipation and were treated with laxatives or glycerin enema. It was suggested that their persistent constipation was caused by intestinal neuronal dysplasia showing an abnormal increase of AchE activity in spite of presence of ganglion cells of the neorectum after surgery, but it was uncertain that they were primary condition as a HaIND or secondary reactions after surgery.

No incidence of port-site recurrence after endosurgical procedure for pediatric malignancies.

Port-site recurrence (PSR) following laparoscopic procedures has been an unpredictable complication in adult cancer patients; however, no data exist about this phenomenon in the pediatric field. The aim of this study was to determine whether PSR, following endosurgical procedure for malignancies, is a typical complication or a rare event in the pediatric population. Eighty-one questionnaires were mailed to members of The Japanese Society of Pediatric Endosurgeons. They were asked to provide a list of their institutions that had experience with PSR after endosurgical procedures for pediatric malignancies. Among 29 institutions, a total of 129 endosurgical procedures for pediatric malignancies were reported; these included 85 laparoscopic and 44 thoracoscopic procedures, performed on 104 neuroblastomas, 8 hepatoblastomas, 7 nephroblastomas, and 10 other tumors. Of the 104 neuroblastomas, 83 were found by mass screening using high levels of urinary vanillylmandelic acid and homovanillic acid. Sixty-five of the 83 patients had their tumor excised, and 18 had their tumor biopsied by endosurgical procedures. Additionally, 47 of these patients did not require any postoperative chemotherapy. No incidence of PSR was reported in any of the patients that underwent endosurgical procedures. The PSR following endosurgical procedure is a rare phenomenon in the pediatric population. Both, laparoscopic and thoracoscopic procedures, are safe and recommended for treating pediatric malignancies, especially mass-screened neuroblastomas.