Can Intraoperative Video Recordings Contribute to Improving Laparoscopic Percutaneous Extraperitoneal Closure in Children with Inguinal Hernia and Prevent Recurrence? A Pilot Study.

Aim: We reviewed intraoperative video recordings (IVRs) of laparoscopic percutaneous extraperitoneal closure (LPEC) for inguinal hernia in children blindly to assess performance. Methods: IVRs of 183 LPEC performed between April 2013 and March 2016, graded by the operating surgeon as difficult (D; n = 8), straightforward (S; n = 96), or easy (E; n = 79), were scored by a panel of reviewers with advanced (group A; >400 LPEC cases; n = 5), intermediate (group I; 50-150 cases; n = 5), and basic (group B; <10 cases; n = 5) experience, according to suturing, dissection plane, vas/vessel dissection, bleeding, and peritoneal injury. They also allocated a recurrence risk rank (RRR; highest = 6; lowest = 1) for each IVR. Mean score variance for each IVR was also compared between reviewers. Results: There was one recurrence (R; 4-year-old male; level E). RRR were: 1, 2, and 2 for reviewers A, I, and B, respectively. Reviewer A scores for "suturing" and "bleeding," and reviewer I scores for "dissection plane" and "peritoneal injury" correlated significantly with RRR. No reviewer B scores correlated with RRR. Score variance between A and I and A and B for cases D1 and D2 were statistically significant. Conclusion: Advanced reviewers showed greatest variance, questioning the validity of whether experience alone improves surgical technique.

Combination therapy with traditional medicines for perianal abscess in children.

BACKGROUND: Combination therapy with two different traditional medicine formulations called hainosankyuto and juzentaihoto (TJ-122 and TJ-48; Tsumura & Co, Tokyo, Japan) may be effective for perianal abscess (PA), but their effectiveness has not been established. The present study investigated the effectiveness of combination therapy with TJ-122 and TJ-48 as the most effective conservative treatment for PA. METHODS: We identified 69 patients with PA under 2 years of age and divided them into four groups according to the formulations used: group 1, TJ-122 (n = 17); group 2, TJ-48 (n = 14); group 3, TJ-122 and TJ-48 (n = 19); and group 4, no traditional medicines (n = 19). Treatment was continued for 3-6 months after resolution of the PA in groups 1 and 2, and for 1 year in group 3. Age at presentation, duration of purulent discharge (PD), frequency of surgical intervention (incision and drainage [ID]; fistulotomy/fistulectomy), and recurrence rates were statistically analyzed. RESULTS: Mean age (months) was 8.6 ± 9.2, 6.9 ± 7.3, 5.2 ± 4.7, and 3.8 ± 3.1 in groups 1, 2, 3, and 4, respectively (P = n.s.). Mean PD (weeks) was 2.5 ± 2.2, 7.1 ± 10.8, 2.0 ± 0.0, and 2.7 ± 1.0, respectively. Duration of PD was significantly longer in group 2 than in groups 1 and 3 (P < 0.05). Mean number of ID procedures was 1.0 ± 0.2, 2.3 ± 0.5, 0, and 1.6 ± 0.2, respectively. Group 1 had significantly less ID than group 2 (P < 0.01). Recurrence rates were 6%, 36%, 0%, and 32%, respectively. Groups 1 and 3 had significantly less recurrence than group 2 (P < 0.05), and group 3 had significantly less recurrence than group 4 (P < 0.01). CONCLUSIONS: Combination therapy with TJ-122 and TJ-48 decreased recurrence and surgical intervention to zero in this study, demonstrating high effectiveness for treating PA in children.

Management of inguinal hernia in children can be enhanced by closer follow-up by consultant pediatric surgeons.

AIM: To determine if follow-up by consultant pediatric surgeons (CPS) affects morbidity due to incarceration (INC) in children with indirect inguinal hernia (IH). METHODS: We educate parents so they can identify possible INC and advise them to attend our emergency department anytime for immediate review by on-call CPS. RESULTS: We reviewed 3,493 cases of IH by grouping them according to age at diagnosis: neonatal (G1; n = 96), 2-3 months (G2; n = 331), 4-6 months (G3; n = 118), 7-12 months (G4; n = 193), and over 12 months (G5; n = 2,755). Data per group were: mean gestational age (weeks): 32.1, 38.0, 36.4, 37.4, 38.7; mean birth weight (g): 1,645, 2,736, 2,471, 2,769, 2,930; mean age at elective hernia repair (HR) (months): 11.3, 4.9, 10.1, 12.9, 56.5; mean weight at elective HR (kg): 6.8, 6.4, 7.3, 9.1, 17.4; mean duration from diagnosis to elective HR (months): 10.9, 3.1, 6.3, 3.6, 3.0; mean follow-up: 6.7 years. Overall, INC occurred in 203/3,493 during follow-up. Incidence per group was: G1: 4/96, G2: 62/331, G3: 6/118, G4: 47/193, G5: 84/2,755. All INC were reduced manually without complications. HR complications occurred in 7/3,493 (0.2%). CONCLUSIONS: With CPS follow-up, INC can be managed without morbidity, allowing elective HR to be performed later with fewer complications.

Primary anastomosis for meconium peritonitis: first choice of treatment.

PURPOSE: Newborn surgery for meconium peritonitis (MP) is sometimes very difficult owing to severe adhesions and bleeding. The aim of this study was to reveal the benefit of primary anastomosis (PA) for MP by comparing PA with multistep operations (MO). PATIENTS AND METHODS: We retrospectively reviewed 38 patients with MP who underwent surgery in our institution from 1983 to 2009. From 1983 to 2000, we essentially used MO. After 2001, we used PA with the exception of 1 patient. We performed MO on 20 patients (group A) and PA on 18 patients (group B). RESULTS: Mortality was 4 in 20 in group A and 1 in 18 in group B. Three patients in group A and 2 in group B required reoperation because of complications. After 2001, 14 of 16 patients underwent PA. Of the 2 patients for whom PA could not be performed, one was postresuscitation from cardiopulmonary arrest and the other was an extremely low-birth-weight infant. The only mortality among the patients who underwent PA occurred in a very low-birth-weight infant who died from intraoperative hepatic hemorrhage. CONCLUSION: PA can be performed for almost all patients with MP except for extremely low-birth-weight infants.

Immature enteric neurons in Ncx/Hox11L.1 deficient intestinal neuronal dysplasia model mice.

AIM: The Ncx/Hox11L.1 gene is required for adequate development of enteric neurons in mice and Ncx/Hox11L.1 deficient (Ncx-/-) mice are used as a model for human intestinal neuronal dysplasia (IND) because of similar histopathology (hyperganglionosis), however, some 50% of Ncx-/- mice develop megacolon with a caliber change in the proximal colon, and die when 21-35 days old. We used polysialylated neural cell adhesion molecule (PSA-NCAM) to examine the maturity of enteric neurons in Ncx-/- mice to further understand the etiology of IND. METHODS: PSA-NCAM immunoreactivity was measured in specimens taken 1 cm proximal to the ileocecal valve (ileum), 1 cm distal to the ileocecal valve (proximal colon), and 1 cm proximal to the anus (distal colon) from 63 mice (Ncx-/-: n = 14, Ncx+/-: n = 30, and Ncx+/+: n = 19) on days 14 (D14), 21 (D21), and 27 or later (>D27). RESULTS: PSA-NCAM was positive (indicating immaturity) in proximal colon (submucosal and myenteric plexuses) from 8/14 (57%) Ncx-/- mice (2/4 on D14, 4/6 on D21, and 2/4 on >D27) and from 5/30 (17%) Ncx+/- mice (0/2 in D14, 2/13 in D21, and 3/15 in >D27). PSA-NCAM was negative (indicating maturity) in all other specimens. The incidence of PSA-NCAM positive neurons in Ncx-/- appeared to be correlated with the mortality rate seen in IND mice. CONCLUSIONS: Our data suggest that colonic dysmotility and pathology seen in Ncx-/- mice may be due to persistence of immature neurons in the proximal colon, which could also be the case in human IND and warrants further investigation.

Endoscopy-assisted laparoscopic excision of rectourethral fistula in a male with imperforate anus.

We report a surgical technique that we developed to facilitate complete excision of rectourethral fistula (RUF)in male patients with imperforate anus (IA). A 6-month-old boy with rectobulbar urethral fistula (bulbar-RUF)had laparoscopic repair of IA. During laparoscopic dissection of the RUF, a fine flexible endoscope was inserted into the rectum through an opening made in the anterior rectal wall. Endoscopy of the rectum allowed the level of laparoscopic dissection to be observed intraluminally, allowing the bulbar-RUF to be excised exactly at its distal end. He is well after follow-up of 9 months with no evidence of residual RUF on radiologic investigations.We have since used this technique to treat another IA patient with prostatic-RUF successfully.

The mechanism of intestinal motility in homozygous mutant Ncx/Hox11L.1-deficient mice--a model for intestinal neuronal dysplasia.

PURPOSE: Homozygous mutant Ncx/Hox11L.1-deficient (Ncx-/-) mice develop mega-ileo-ceco-colon with a caliber change in the proximal colon. This study investigated the mechanism of intestinal motility in these mice. METHOD: Five-week-old male and female Ncx-/- mice with mega-ileo-ceco-colon (n = 8) were compared with age-matched male BDF1 mice used as controls (n = 8). All mice were sacrificed, and uniform-sized strips of jejunum, ileum, proximal colon, and distal colon were exposed to electrical field stimulation and pretreatment with atropine sulfate, guanethidine, or tetrodotoxin. Contractile responses were recorded and compared. RESULTS: Longitudinal muscle from strips of jejunum and ileum from all mice (BDF1 and Ncx-/-) did not respond to electrical field stimulation, whereas ileal circular muscle contracted in BDF1 mice and contracted and relaxed in Ncx-/- mice. Pretreatment with atropine sulfate and guanethidine inhibited the responses of circular muscle of distal colon and ileum in BDF1 mice significantly (P < .05), but no effect was observed in Ncx-/- mice. CONCLUSION: In ileum, BDF1 mice have cholinergic and adrenergic dominant contraction patterns, whereas Ncx-/- mice have relaxation-dominant patterns because of nonadrenergic, noncholinergic nerves. Based on this, there would appear to be some kind of variation in the gastrointestinal nerve supply in Ncx-/- mice.

Does abnormal expression of acetylcholine receptors in Hirschsprung's disease induce acetylcholine esterase positive nerve fibres?

OBJECTIVE: Alpha bungarotoxin (alpha-BTX) is a neurotoxin isolated from the venom of Bungarus multicinctus that binds specifically to the beta-subunits of nicotinic acetylcholine receptors (nAChR) on myotube membranes. The purpose of the present study was to investigate the distribution of alpha-BTX-sensitive nAChR in Hirschsprung's disease (HD) to understand the histopathological features of HD, especially the increase in acetylcholine esterase (AChE) positive nerve fibres. METHODS: Confocal microscopy was used to study the expression of FITC (fluorescein isothiocyanate)-alpha-BTX, anti-synaptophysin (A-SY) antibody, and anti-neurofilament (A-NF) antibody to determine the distribution of nAChR and ganglion cell and nerve fibres in colon specimens from five cases of HD and three normal controls. RESULTS: Quantitative assessment of the immunoreactivity of colonic muscle and colonic mucosal epithelium from an aganglionic segment of HD bowel demonstrated markedly increased nAChR compared with colonic muscle and colonic mucosal epithelium from a ganglionic segment of HD bowel and normal bowel (p < 0.0001, respectively), both of which have only a few positive nAChR. In colonic muscle from aganglionic and transitional segments of HD, there were many nAChR around hypertrophic nerve trunks identified by A-NF and A-SY staining. CONCLUSION: We suggest that abnormal expression of nAChR in HD might be implicated in causing gastrointestinal dysmotility because of their localization around hypertrophic nerve trunks.

Maternal microchimerism in biliary atresia.

BACKGROUND: The aim of this study was to determine the existence and extent of maternal microchimerism in the livers of biliary atresia (BA) patients. METHODS: Two series of investigations were performed based on the sex of our subjects. Subjects for series I were men, of which 6 had BA. Livers were analyzed using X and Y chromosome probes and fluorescent in situ hybridization. Subjects for series II were woman. Nine BA cases and their mothers were HLA typed (class I). Daughter livers were also tested for antibodies to maternal and other HLA. Two cases of neonatal hepatitis, 2 cases of Alagille syndrome, and 1 case of Byler syndrome acted as controls. RESULTS: All male BA livers were found to contain a mixture of cells with 1 and 2 X chromosomes (ie, XY or XX). All livers from male controls had only 1 X chromosome (ie, XY). All female BA subjects had varying intensities of antimaternal HLA class I (HLA-A) antibodies in their bile duct epithelium and hepatocytes (strong, 5; mild, 3; weak, 1). The liver from the female control did not display any antimaternal HLA class I antibodies (HLA-Ab). CONCLUSION: Our preliminary data appear to show that maternal microchimerism is present within the livers of patients with progressive postnatal type BA. We suggest that BA could in fact be a graft-vs-host disease masquerading as an autoimmune reaction triggered by maternal microchimerism, and we intend to pursue this hypothesis further to clarify the etiology of BA.

A new rapid acetylcholinesterase staining kit for diagnosing Hirschsprung's disease.

Conventional acetylcholinesterase (AChE) histochemistry is both time consuming and complicated and requires the mixing of reagents that are toxic to the human body. We developed a rapid technique for performing AChE histochemistry, which has already been published, and now present a kit for performing AChE histochemistry that is a further improvement. Rectal suction biopsy specimens taken from 20 constipated patients and three full thickness biopsy specimens taken from 4 Hirschsprung's disease (HD) patients during pull-through surgery from aganglionic, transitional, and ganglionic bowel segments were tested using our rapid technique and the new kit. Each specimen was incubated for only 6 min. All ganglion cells stained clearly for AchE in just 6 min using both techniques. However, the kit was able to stain AchE positive nerve fibers more clearly and did not detect endogenous peroxidase-containing histiocytes, as did the earlier rapid technique. The kit could also detect AchE positive nerve fibers in the circular and longitudinal muscle layers, unlike the earlier rapid technique. The kit allows AChE histochemistry to be performed rapidly with complete accuracy, without any risk for toxicity. Moreover, the kit provides more focused information on AchE distribution in the bowel itself without any extraneous staining and can be used for diagnosing HD and allied disorders as well as establishing the exact level of innervation for pull-through resection.

The role of monocyte chemoattractant protein-1 in biliary atresia.

BACKGROUND: The aim of this study was to explain the role of monocyte chemoattractant protein-1 (MCP-1) in biliary atresia (BA). METHODS: Concentrations of serum MCP-1 and collagen type IV were measured in 38 patients with BA by using commercially available kits. MCP-1 was also assessed in liver biopsy specimens by using immunohistochemistry. Subjects were classified into groups. Group 1 comprised BA patients with normal liver function (n = 13), group II comprised BA patients with moderate liver dysfunction (n = 18), group III comprised BA patients older than 20 years awaiting liver transplantation (n = 7), and the control group comprised age-matched patients without evidence of liver disease (n = 23). RESULTS: Serum MCP-1 levels were significantly increased in group II compared with group I (P < .0001) and the control group (P < .0001). Serum MCP-1 levels in group III were lower than in the control group (P < .0001). There was a significant linear correlation between serum MCP-1 levels and type IV collagen levels in group II. Group II subjects with portal hypertension (PH) had higher MCP-1 levels than those without PH (P = .0009). Biopsy specimens showed MCP-1 was expressed mainly on biliary epithelial cells, vascular endothelial cells, and hepatocytes in group II. CONCLUSIONS: These findings suggest that MCP-1 probably plays a significant role in the development of progressive liver fibrosis in BA.

Obstruction due to rectal cuff after laparoscopy-assisted transanal endorectal pull-through for Hirschsprung's disease.

We report a case of persistent obstruction after laparoscopy-assisted transanal endorectal pullthrough for Hirschsprung's disease in a 4-week-old boy with biopsy-proven HD. Before pull-through, the posterior rectal cuff was split along its entire length cranially, starting from the dentate line. Initial recovery was uneventful; however, signs of obstruction developed 3 weeks postoperatively. Reoperation through a posterior sagittal approach confirmed a residual rectal cuff surrounding the neo-rectum circumferentially. The dorsal side of the residual rectal cuff was removed completely. At follow-up 5 years later, he defecates 2 to 4 times a day with occasional staining. We hypothesize that the persistent postoperative obstruction was caused by a long residual rectal cuff that spontaneously reapproximated and/or became folded during pull-through. Therefore, a shorter cuff with near-total posterior excision should be strongly considered during laparoscopy-assisted transanal endorectal pull-through for Hirschsprung's disease.

Jejuno-jejunal intussusception secondary to submucosal lymphangioma in a child.

Small bowel submucosal lymphangioma in children is extremely rare. We present the case of a 5-year-old boy with intussusception secondary to a submucosal lymphangioma in the jejunum. To the best of our knowledge, this is the first report of intussusception secondary to a small intestinal submucosal lymphangioma in a child.

Gastroschisis reduction using "Applied Alexis", a wound protector and retractor.

We used an Applied Alexis (Applied Medical Resources Corp, USA) wound protector and retractor (WPAR) instead of a "spring loaded silo" (SLS) for gastroschisis reduction because SLS is currently unavailable in Japan. Our patient was a 1,707 g female diagnosed prenatally with gastroschisis born at 35 weeks gestation by spontaneous vaginal delivery. Attempted primary closure failed and a WPAR was used as a silo. A piece of the bottom ring of the WPAR was cut out and a smaller ring was made from it, so it would fit into the patient's abdomen. Eviscerated organs were inserted into the retractor through the bottom ring, and the bottom ring was placed into the abdominal cavity through the gastroschisis defect without suturing to complete the silo. Operating time was 20 minutes. Three days later, all eviscerated organs had been reduced into the peritoneal cavity, the silo was removed, and the gastroschisis defect closed without difficulty. Postoperative recovery was uneventful and the WPAR was an excellent alternative for making a silo.

Neuroblastoma detected by mass screening: the Tumor Board's role in its treatment.

Japan has a nationwide mass-screening program for neuroblastoma in 6-month-old infants. Neuroblastoma can regress spontaneously, and some institutions observe selected cases. We evaluated the management of screened neuroblastoma at our hospital since 1997 when an observation program was introduced. Criteria for the observation program were stage-I, stage-II, or stage-IVs tumors, urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels <40 microg/mg creatinine, tumor <5 cm in diameter, no invasion to the intraspinal canal or great vessels, and parental consent to participate. Patients who did not meet observation criteria underwent surgery or mild chemotherapy according to the location of the tumor. If patients met observation criteria after chemotherapy, surgical intervention was no longer performed. Thirty-six patients attended our hospital for screened neuroblastoma from 1997 to 2002. Thirty-three patients who were managed at our hospital participated in this study. Ten subjects met observation criteria. Tumors regressed in 7 patients (mean follow-up period 36.3 months) with corresponding decreases in VMA and HVA levels (group A). Three underwent surgery (group B) because of increasing VMA and HVA levels, increase in tumor size, or guardian's request. Twenty-three subjects did not meet observation criteria. Four patients underwent primary surgery (group C), and 19 patients had chemotherapy initially. Fourteen patients met observation criteria after chemotherapy and two are still having chemotherapy (group D). Three patients required surgery due to insufficient regression of their tumors (group E). Fourteen subjects in group D had marked decreases in VMA and HVA levels and tumor size (mean follow-up period 29.1 months), and tumors were not detected using imaging techniques in 8 patients. Histological examination of all resected specimens during the study period showed favorable histology and no N-myc amplification. There was no evidence of unfavorable prognosis in any of the 33 subjects, although 1 patient who underwent primary surgery had a vanishing kidney 1 year later and 1 patient had multiple bony metastases after complete resection of tumor, which was treated by chemotherapy. Until the real significance of mass screening for neuroblastoma as a public health measure is confirmed, observation with careful follow-up should be adopted more extensively because it has a favorable outcome in many cases, and is associated with minimal therapeutic complications.