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Introduction: A modified pull-through (PT) distinguished by complete full-thickness removal of the posterior rectal cuff, initially developed as an open procedure in 1980, has been performed with l...

The incidence of perforation during antibiotic therapy (AT) of children triaged as non-complicated acute appendicitis (NC-Ap) was investigated. Abdominal ultrasonography (US) and/or computed tomography (CT) scans from cases of perforation identified at appendectomy for failed AT were reassessed blindly by a panel of board-certified specialists for any evidence of pre-AT morbidity suggestive of perforation. Of 521 cases triaged as NC-Ap, symptoms resolved with AT in 452 cases (86.8%). All 69/521 (13.2%) cases with persistent symptoms had urgent appendectomy, and 12/521 (2.3%) were found to have perforated. Blind reassessment of US and/or CT scans from these cases identified seven with evidence of perforation when they were triaged as NC-Ap. Thus, the actual incidence of perforation during AT for NC-Ap was actually 12–7 = 5/521 (0.95%). Perforation is generally believed to be a complication of AT, but inappropriate triaging of cases for AT can bias results by artificially inflating the number of perforations, in this study, by more than double. We are the first to assess the unbiased incidence of perforation during AT for NC-Ap, by reassessing pre-AT US and/or CT scans. The incidence of perforation during AT is actually negligible.

Purpose To report the sequelae of and preventive strategies for selected lower urinary tract (LUT) complications, i.e., posterior urethral diverticulum (PUD), intraoperative LUT injuries, postoperative dysuria, and fistula recurrence in male imperforate anus (IA) with rectourethral/rectovesical (RU/RV) fistula after laparoscopy-assisted anorectoplasty (LAARP) or posterior sagittal anorectoplasty (PSARP). Methods 153 boys with IA and RU/RV fistula treated 1986–2019 by LAARP (n = 56) or PSARP (n = 97) at two unrelated institutes were studied retrospectively. Results After mean follow-up of 17.0 years (range: 36.5 days-32.0 years), the overall incidences of LUT complications were: LAARP (6/56; 10.7%); PSARP (7/97; 7.2%); p = 0.55, comprising PUD: LAARP (n = 5), PSARP (n = 0); p = 0.006; injuries: LAARP (n = 0), PSARP (n = 5); p = 0.16; dysuria: LAARP (n = 1), PSARP (n = 1); p>0.999; and recurrence: LAARP (n = 0), PSARP (n = 1); p>0.999. Mean onset of PUD was 5.1 years (range: 1.0–15.1 years). Treatment: PUD: surgery (n = 2/5), conservative (n = 3/5); injuries: intraoperative repair (n = 5/5); dysuria: conservative (n = 2/2), and recurrence: redo PSARP (n = 1/1). Conclusions Strategies devised to improve dissection accuracy resolved the specific technical issues causing LUT complications (remnant RU fistula dissection in LAARP and blind posterior access in PSARP). Currently, the incidence of new cases of PUD and LUT injuries is zero. Level of Evidence: Level III

Dysgerminoma is a malignant ovarian germ cell tumor, and unlike sex-cord stromal tumors, endocrine manifestation is considered rare. Here, we report the first case of dysgerminoma presenting precocious puberty. The patient is a 7-year-old girl who presented with a breast development in Tanner stage 3. Serum estradiol (E2) was markedly elevated while luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were suppressed below the detection limit. Microscopically, the right ovarian mass displayed nests of large polygonal cells and fibrous septa which were focally concentrated by theca-like plump spindle cells. Immunohistochemistry revealed that the spindle cells expressed various steroidogenic enzymes involved in estrogen biosynthesis including P450 aromatase. The tumor was diagnosed with pure dysgerminoma with estrogen-producing functioning stroma. After the operation, serum E2 declined below the detection limit; LH and FSH returned within the normal range. This case demonstrates that even a conventional dysgerminoma can present endocrine manifestation through functioning stroma.

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 1...

Objective Three criteria (age at first pale stool, age at portoenterostomy, and duration from the first pale stool to portoenterostomy) were assessed for prognostic value in biliary atresia. Methods The medical records of 116 consecutive biliary atresia patients treated by portoenterostomy after liver transplantation became available in Japan in 1989 were identified and data from 96 were analyzed retrospectively for this study. The impact of each criterion on clearance of jaundice to normal levels (total serum bilirubin ≤1.2 mg/dL) and survival with the native liver as indicators of outcome were compared according to time (≤30 days, 31−60 days, and ≥61 days). Results Age at first pale stool was ≤30 days in 53, 31−60 days in 26, ≥61 days in 17; age at portoenterostomy was ≤30 days in 7, 31−60 days in 35, ≥61 days in 54, and duration pre-portoenterostomy was ≤30 days in 50, 31−60 days in 36, ≥61 days in 10. Survival with the native liver was not significantly influenced by age at first pale stool or age at portoenterostomy, but prolonged duration (≥61 days) reduced survival with the native liver significantly (p = 0.003). Clearance of jaundice to normal levels was not affected by any criterion at any time.

Detailed implications of age at laparoscopic transanal pull-through (LTAPT) on postoperative bowel function (POBF) in Hirschsprung’s disease (HD) are somewhat obscure because of a spectrum of factors. Age at surgery was used to categorize 106 consecutive postoperative HD cases treated by our modified LTAPT (JLTPAT) between 1997 and 2015; group A

Aim Early diagnosis is essential for the successful management of biliary atresia (BA). We assessed the efficacy of our diagnostic strategies for BA in the laparoscopic era. Methods A retrospective review of the medical records of 132 infants presenting with suspected BA between 1998 and 2018 was performed to assess the efficacy of “basic” tests (blood biochemistry, abdominal ultrasound, and Tc-99m N-pyrydoxyl-5-methyltriptophane liver scintigraphy) and the value of laparoscopic assessment of the porta hepatis and/or cholangiography for choosing between Kasai portoenterostomy (PE) or cholangiodrainage (CD) for treatment. Results BA was excluded on “basic” tests in 7 cases: 109/125 had BA diagnosed laparoscopically and proceeded immediately to PE. Nonatrophic gallbladder cases (n = 10) had CD and other morbidity diagnosed on liver biopsy (Bx; n = 6). Sensitivity, specificity, and accuracy of diagnosis using “basic” tests were 100%, 30.4%, and 87.9%, respectively. Accuracy of laparoscopy without Bx was 100%, allowing immediate progression to PE without waiting for Bx results or trauma from Bx to heal. “Basic” tests and laparoscopy can be completed within one week. Conclusions A combination of “basic” tests and laparoscopy can diagnose BA promptly and accurately without Bx. Bx is essentially unnecessary for diagnosing BA if laparoscopy is performed. Type of study Study of diagnostic test. Level of evidence Level III.

Aim The purpose of this study was to determine the incidence of ureteric obstruction (UB) and requirement for Cohen antireflux surgery (CAS) after Deflux Ⓡ treatment (DT) for vesicoureteric reflux (VUR). Methods Between 2011 and 2017, 494 ureters (VUR severity ≤ grade III: N=291 or >grade IV: N=203) were treated by DT at a mean age of 4.5 (range: 0.2–24) years. Epidural Catheter Assistance (ECA) was used to exclude UB by injecting diluted indigo carmine solution (1–3mL) into an epidural catheter inserted into a ureter after DT and confirming dye flow within 15min. ECA+: N=181 ureters; ECA−: N=313 ureters. Results In ECA+, UB was detected in 5/181 (2.7%) ureters (grade II: N=1, III: N=3, and IV: N=1) treated by leaving the ECA catheter in situ overnight (N=4) or double J stent (DJS) insertion for 1month (N=2). After mean follow-up of 1.9years, one grade III DJS case has residual grade II VUR. In ECA−, 3/313 (0.9%) cases developed UB. One resolved, and one required DJS. CAS was required for 17/494 (3.4%) ureters and hindered by DT in 5/17 (29.4%) ureters. All are sequelae-free after mean follow-up of 1.8years. Conclusions UB may be more frequent than reported (3.3% versus 0.6%–1.8%). ECA identifies potential UB. Type of study Treatment study. Level of evidence Level III.

Total bilirubin (T-bil) is used universally for monitoring post-portoenterostomy (PE) biliary atresia (BA) patients although other biochemical markers [BM; AST/ALT and platelet count (PC)] are also prognostic. We compared open PE (OPE) with laparoscopic PE (LPE) using T-bil, AST/ALT, and PC (3BM) as more comprehensive indicators of postoperative clinical status.Subjects were 31 PE cases (LPE: n = 17; OPE: n = 14). BA classification was type III (n = 16), type II (n = 1) in LPE and type III (n = 12), type I (n = 1), type II (n = 1) in OPE.Mean ages and weights at PE were similar: 65.5 days, 4.4 kg (LPE) versus 69.3 days, 4.1 kg (OPE); and mean follow-up was 2.5 years for both LPE and OPE. Jaundice clearance (T-bil ≤1.2 mg/dL) was achieved in 16/17 (94.1 %) after LPE versus 10/14 (71.4 %) after OPE (p = NS), but 3BM were closer to normal after OPE. At the time of review, 13/17 LPE cases (76.5 %) were alive with native livers and 4/17 had received LTx (23.5 %) and 10/14 OPE cases (71.4 %) were alive with native livers and 4/14 had received LTx (28.6 %).Although JC was better after LPE, 3BM were better after OPE. Further follow-up will prove the comprehensive prognostic value of 3BM.

Purpose We reviewed our post-Kasai portoenterostomy biliary atresia (BA) patients who required liver transplantation (LTx) for deterioration in native liver (NL) function to investigate mortality in relation to age at LTx. Methods BA patients indicated for LTx when less than 18years old (U18; n=17) and when 18 or older (18+; n=13) were compared. All achieved jaundice clearance postoperatively (TBil ≤1.2mg/dL (≈20μmol/L)). Results In U18, living-donor (LD) LTxs were performed at a median of 6.1years (range: 0.5–16.7; n=14) and cadaveric (CD) LTxs at a median of 1.3years (1.1–1.5; n=3). In 18+, LDLTxs were performed at a median of 28years (18–37; n=8), and 1 case died from graft versus host disease. CDLTxs were indicated in 5, but 4 died at a median of 30years (26–32), a mean of 1.4years (0.7–1.8) after NL deterioration commenced. One case is awaiting CDLTx. At the time of review, all U18 and 7 LDLTx cases in 18+ were clinically stable. Mortality rates were 0% in U18 and 38% in 18+ (P=.006). Conclusion Our results highlight the extremely grave prognosis for long-term BA patients requiring LTx when 18 or older because of poor donor availability in Japan. Level of evidence Level III.

Oral-facial-digital syndrome type 1 (OFD1; MIM 311200) is characterized by multiple anomalies of the oral cavity, face and digits. We report a family with OFD1, where two female siblings and their mother shared the same mutation of the responsible gene (OFD1) c.1193_1196delAATC. Phenotypic variability was observed among them; the mother showed minimal features of OFD1, whereas her two daughters showed partial features and the full spectrum of OFD1, respectively. Thus, OFD1 was suspected only after a health check-up during pregnancy of the second patient showing fetal brain anomaly and maternal polycystic kidney. For these reasons, there was a delay in the recognition of OFD1 in this family. Patients with OFD1 show phenotypic variability, which poses challenges for genetic counseling.

Introduction In an imperforate anus, colostography often fails to identify recto-urethral fistula (RUF). Thus, surgeons must always assume an RUF is present, despite colostography findings, and dissect the distal rectal pouch (RP) with caution. We report the usefulness of intraoperative colonoscopy (IOC) for excluding RUF and, thus, facilitating safe dissection of the RP. Methods We used IOC in six cases of imperforate anus. All had right transverse colostomy initially after birth. Distal colostography excluded RUF in five cases and was inconclusive in one. Laparoscopy was used to free the RP carefully from the bladder neck in all cases. Near the prostate, a 4-mm fine, flexible colonoscope was inserted into the RP through the anterior rectal wall to observe the laparoscopic dissection of the RP, which was attached closely to the prostate/bulbar urethra intraluminally to prevent injury to the urethra. The mucosa of the distal end of the RP was mucosectomized or diathermied, and the colon was pulled-through. Mean age at surgery was 11 months. Results IOC excluded RUF under direct vision in all cases, which enabled the dissection of the RP to be monitored and to proceed smoothly. At follow-up (mean: 31 months), all cases were well. Conclusions IOC can be used to exclude RUF and facilitate safe dissection of the RP in imperforate anus.

Introduction: Laparoscopic appendectomy (LA) can be performed safely and effectively using endoloops (EL) or endostaples (ES). We compared EL and ES for stump closure during LA for complicated appendicitis in children. Methods: All LA for complicated appendicitis performed between July 2005 and August 2009 were assessed prospectively. EL were used in 37 procedures and ES in 31. Apart from choice of technique which was the personal preference of the attending surgeon, all patients were managed according to the same intraoperative and postoperative protocols. Results: There was no significant difference between mean age at operation; gender ratio; mean preoperative and postoperative white blood cell; mean preoperative and postoperative white blood cell C-reactive protein; histopathology; mean operating time (EL: 71 minutes; ES: 64 minutes); mean hospitalization (EL: 5.3 days; ES: 5.1 days); febrile period (EL: 2.1 days; ES: 1.9 days); white blood cell normalization (EL: 2.6 days; ES: 2.4 days); and intravenous antibiotic usage (EL: 3.8 days; ES: 3.7 days). There were no intraoperative complications or ICU admissions in either group, but two EL cases required conversion to open surgery (P=NS). Incidences of intra-abdominal abscess (EL: n=1 or 2.7%; ES: n=1 or 3.2%), transient ileus (EL: n=2 or 5.4%; ES: n=2 or 6.4%), small bowel obstruction (EL: n=0; ES: n=0), and wound infection (EL: n=1 or 2.7%; ES: n=1 or 3.2 %) were not significantly different. Rate of rehospitalization for EL was 2.7% (n=1; colitis), and for ES, it was 3.2% (n=1; intra-abdominal abscess) (P=NS). Mean cost for EL was US$890, and for ES, it was US$1300. Conclusion: This is the first prospective study comparing EL and ES during LA for complicated appendicitis in children. ES is more expensive, but there was no significant difference in morbidity for this technique, particularly with regard to incidence of postoperative intra-abdominal abscess.

We report a case of massive melena caused by a carcinoid of the small intestine. A 28-year-old woman was admitted to our department after presenting with massive melena. The source of the bleeding could not be localized by upper or lower gastrointestinal endoscopy, computed tomography (CT), or labeled red blood cell scintigraphy. Enteroscopy allowed visualization only up to the jejunum, and we could not localize the lesion. The melena subsided with conservative treatment, but the patient was readmitted 4 months later when she suffered another episode of massive melena. A contrast CT scan performed immediately showed extravascular leakage and the retention of contrast medium in the ileum. Thus, she underwent an emergency surgery, during which endoscopy confirmed a small ileal tumor accompanied by pulsating bleeding from the exposed blood vessels at its center. The small intestine was partially resected, including the swollen lymph nodes, the size of small beans. Pathological examination confirmed a carcinoid tumor 1 cm in diameter, with an arterial rupture at its center and lymph node metastasis.

症例は35歳の女性. 平成17年8月13日より腹痛を自覚するようになり, さらに8月17日より嘔吐も出現するため, 当院を受診した. 腸閉塞と診断され緊急入院となった. イレウスと診断し, 入院後イレウス管を挿入した. イレウス管造影検査で回腸末端の狭窄像と腹部CT検査で回盲部の腫瘤像を認めたため, 回腸子宮内膜症による腸閉塞と診断し開腹手術を施行した. 開腹所見では黄色透明の腹水を少量認め, ダグラス窩左側は子宮, チョコレート嚢胞およびS状結腸が癒着するfrozen pelvisの状態であった. 回腸末端が盲腸に強固に癒着しており, 剥離不可能であったため回盲部切除術を施行した. 回腸子宮内膜症は術前に診断を確定することは非常に困難とされているが, 今回, イレウス管造影検査と腹部CT検査で術前の診断が可能であったので報告した.

Background In 2007, we began using the anorectal line (ARL) as the landmark for commencing rectal mucosal dissection (RMD) instead of the dentate line (DL) during laparoscopy-assisted transanal pull-through (L-TAPT) for Hirschsprung's disease (HD). We conducted a medium-term prospective comparison of postoperative fecal continence (POFC) between DL and ARL cases to follow our short-term study. Methods POFC is assessed by scoring frequency of motions, severity of staining, severity of perianal erosions, anal shape, requirement for medications, sensation of rectal fullness, and ability to distinguish flatus from stool on a scale of 0 to 2 (maximum: 14). Results Patient demographics were similar for ARL (2007–2014: n = 33) and DL (1997–2006: n = 41). There were no intraoperative complications and 2 cases of postoperative colitis in both ARL (6.1%) and DL (4.9%). Mean annual medium-term POFC scores for the 4–7 term of this study were consistently better in ARL: 9.7 ± 1.4*, 10.1 ± 1.6*, 10.6 ± 1.6, and 11.3 ± 1.4* in ARL and 8.6 ± 1.5, 9.1 ± 1.6, 9.8 ± 1.9, 10.0 ± 1.6 in DL (*: p Conclusions Medium-term POFC is better when the ARL is used as the landmark for RMD during L-TAPT for HD.

We modified conventional hepatic portoenterostomy by pulling the remnant fibrous mass caudally and placing 5 or 6 numbered and individually clamped absorbable 5-0 double-needle sutures horizontally in the liver surface of the posterior side of the remnant fibrous mass before excision and apposing the posterior and anterior margins of the hepatic portoenterostomy to resemble a flattened isosceles triangle. Our technique was used to treat 14 cases of biliary atresia. Good postoperative biliary excretion (serum total bilirubin

Prednisolone is used routinely after portoenterostomy (PE) in patients with biliary atresia (BA). The authors reviewed their patients with BA post-PE to assess prednisolone protocols.Severity of fibrosis at PE (moderate or severe), age at PE (30-70 days), size of bile ductules in the fibrotic biliary remnant at the porta hepatis (100 microm), and type of BA (uncorrectable type) were used as criteria for selecting 63 subjects from our patients with BA post-PE. Subjects were divided into 5 groups according to prednisolone dosage: group 1, no prednisolone; groups 2 to 4, single courses of intravenous prednisolone commencing on day 7 post-PE administered in decreasing dose for 3 days each as follows: group 2, 6, 4, and 2 mg; group 3, 10, 5, and 2.5 mg; group 4, 20, 15, 10, 5, and 2.5 mg; group 5, same as group 4, but stool color was used to monitor bile excretion and a course was restarted from 20 mg whenever stools began to turn pale. If necessary, single courses were repeated until serum total bilirubin was less than 2.0 mg/dL. Protocol efficacy was assessed by comparing the number of patients who became jaundice free, the period taken to become jaundice free, and the incidence of side effects related to prednisolone.The number of patients who became jaundice free in the no prednisolone group (group 1, 7/12 or 58.3%) was not significantly different from the number in the single-course groups (group 2, 8/12 or 66.6%; group 3, 10/13 or 76.9%; and group 4, 11/15 or 73.3%). The number in the stool-monitored group (group 5, 10/11 or 90.9%) was significantly greater (P.05). The mean period taken to become jaundice free in group 1 (82.6 +/- 29.1 days) was not significantly different from the single-course groups (group 2, 74.5 +/- 29.3 days; group 3, 49.6 +/- 19.8 days; and group 4, 48.3 +/- 26.0 days). The mean period taken in the stool-monitored group (group 5, 33.3 +/- 6.4 days) was significantly shorter (P.05). The number of subjects who developed cholangitis after becoming jaundice free was not significantly different (group 1, 2/7; group 2, 2/8; group 3, 2/10; group 4, 2/11; group 5, 2/10). There were no prednisolone-related complications identified in any subject.These results provide strong evidence that large-dose prednisolone therapy with stool color monitoring of bile flow has a positive impact on the time taken for patients with BA post-PE to become jaundice free and the number of patients who remain jaundice free.

We assessed the effect of high partial pressure of arterial carbon dioxide (PaCO2) due to pneumoperitoneum (PP) on growth (height/weight) and development (gross/fine motor function, receptive/expressive communication, and social interaction), by comparing outcome after portoenterostomy (PE) for biliary atresia (BA) using laparoscopic PE (LPE: n = 13) and open PE (OPE: n = 13) cases performed between 2005 and 2014. Our PE is based on Kasai’s original PE. All data were collated prospectively. Differences in duration of follow-up (LPE: 38.8 months; OPE: 38.1 months), jaundice clearance (LPE: 12/13 = 92.3 %; OPE: 9/13 = 69.2 %), survival with the native liver (LPE: 10/13 = 76.9 %; OPE: 9/13 = 69.2 %), incidence of cholangitis, hypersplenism, and incidence of esophageal varices were not significant. Mean intraoperative PaCO2 was significantly higher in LPE (LPE: 50.1 mmHg; OPE: 40.7 mmHg, p

Generally, open portoenterostomy (PE) involves a wide extended anastomosis and all sutures are deep [extended PE (EP)]. In contrast, the anastomosis in Kasai’s PE (KP), our modified open Kasai PE (MK), and our laparoscopic modified Kasai PE (lapMK) involve shallow suturing, especially at the 2 and 10 o’clock positions where the right and left bile ducts would be normally. We compared outcomes of 36 consecutive biliary atresia (BA) patients treated by three types of PE at a single institution during the period 2005–2014; EP (n = 13), MK (n = 11), and lapMK (n = 12). We compared age at PE, time taken to become jaundice-free (total bilirubin ≤1.2 mg/dL; JF time), proportion of JF subjects [JF ratio (JFR)], steroid dosage, incidence of cholangitis, postoperative liver function and CRP, presence of hypersplenism, requirement for liver transplantation (LTx), and JF survival with the native liver (JF+NL) as indicators of outcome. Patient demographics, steroid dosage, JF time, incidence of cholangitis, presence of hypersplenism, operating time, blood loss and postoperative biochemistry were similar for all groups. However, JFR was significantly higher for lapMK (100 %) versus EP (46.2 %) (p

Purpose: We have previously demonstrated that spontaneous bacterial translocation (BT) occurs, in newborn rabbits and correlates strongly with small bowel colonization (BC). Birth stress, specifically hypoxia, is believed to increase this pathologic process and thus lead to sepsis. This study investigated the relationship between BT and acute hypoxia in newborn rabbits. Methods: Four hundred seventeen rabbit pups (aged 0, 2 to 4, 6, and 28 days) were divided into four groups according to the type of hypoxic stress: 9% O 2 for 1 hour, 9% O 2 + 12% CO 2 for 1 or 4 hours, and 21% O 2 (control animals). The animals were killed 1.5 or 20 hours after the stress. Sterile specimens of mesenteric lymph nodes (MLN), spleen, liver, small bowel, and large bowel were incubated aerobically at 37°C for 24 hours in thioglycolate broth, and subsequently plated on both MacConkey and Colistin Naladixic Acid media. After 24 hours, the growth on both plates was recorded. χ 2 analysis was used, and P values of less than .05 were considered significant. Results: BC of the small bowel and BT to the MLN were low in the first 4 days of life in the hypoxic groups (range, 0% to 21% BC, 0% to 6% BT) and the control group (range, 4% to 30% BC, 3% to 12% BT). After an increase in BC at 6 days of age, the rate of BT increased to 25% to 29% in control animals. The rate of BT in the hypoxic groups (25%) did not differ significantly from that of the controls ( P > .05). Additionally, killing at 20 hours ( v 1.5 hours) was not associated with an increase in the incidence of BT. None of the stress groups had a significant increase in BT compared with the controls. Importantly, although 4 hours of 9% O 2 + 12% CO 2 resulted in a 30% mortality rate, the incidence of BT was no higher than that of the control animals (13% v 29%; P > .05). Conclusion: Severe hypoxic stress in newborn rabbits does not increase the incidence of BT. Because the incidence of BT correlates with that of BC, and because BC is the same in the control and hypoxic animals, the sepsis observed in hypoxic newborns probably is not related to an increased incidence of BT.

Purpose: The aim of this study was to review the outcome of a protocol the authors proposed for preventing Roux-en-Y hepatico-jejunostomy (RYHJ) related complications. Methods: In 1986 the authors adopted the following protocol for improving RYHJ to minimize risks for RY-related complications: (1) end-to-end anastomosis if possible; (2) if end-to-side anastomosis is unavoidable, the common hepatic duct should be anastomosed as close as possible to the closed end of the blind pouch (BP); (3) careful selection of the vascular supply to the RY jejunal limb; (4) length of the RY jejunal limb should not be predetermined, but individualized; (5) approximate the native jejunum to the RY limb. The authors reviewed 92 children who had surgery for CC (mean age at surgery, 4.1 years) at our institution between 1986 and 2002 to assess the effectiveness of their recommendations. Results: After the adoption of the authors' recommendations for RYHJ, they have experienced no RYHJ-related complications after a mean follow-up period of 7.6 years (range, 4 months to 16 years). Conclusions: RYHJ in children is different from that in adults, because the RY limb or BP can grow and elongate as the child grows. The authors' recommendations appear to have successfully prevented RY-related complications up to the time of this review, and they highly recommend their use.

To assess whether ursodeoxycholic acid (UDCA) treatment has any beneficial effect on essential fatty acid (EFA) deficiency in patients who have had a Kasai operation for extrahepatic atresia (EBA), responses of serum fatty acids to UDCA administration (15 mg/kg/d) were investigated in eight jaundice-free patients and in eight patients with jaundice (serum total bilirubin ≥ 1.0 mg/dL). All patients were also given taurine supplementation (100 mg/kg/d). Serum fatty acid composition was determined before and 6 months after UDCA treatment. Serum total bile acid concentration and serum total bilirubin value, as a part of conventional liver function tests, were measured before and during UDCA therapy. Before UDCA treatment, the concentrations of linoleic acid and arachidonic acid were significantly lower ( P > .05 for the former; P > .01 for the latter) in both the jaundice and jaundice-free groups than in the controls. After 6 months of treatment, the linoleic acid concentration significantly increased ( P > .05), to the normal range, in the jaundice-free group, but not in the jaundice group. The arachidonic acid concentration did not increase significantly in either group. The serum total bile acid concentration was lower in six of the eight jaundice-free patients and in four of the eight jaundice patients. The serum total bilirubin value decreased in six of the eight jaundice-free patients and in four of the eight jaundice patients; however, the degree of improvement was not statistically significant in either group. No side effects developed, and there were no changes in blood chemistry values unrelated to liver disease. Results of the study indicate that UDCA therapy may be a significant benefit in the treatment of EFA deficiency associated with postoperative EBA, particularly for patients without jaundice.

The aim of this study was to examine the innervation pattern of the muscle layers of normoganglionic pulled-through bowel in patients with Hirschsprung's disease (HD) by observing the distribution of nerve terminals comprising neuromuscular junctions and synapses. As a marker for nerve terminals, monoclonal antibody (MAb) 171B5 was used. Pulled-through normoganglionic bowel from 12 patients with HD that was either biopsied or resected at operation and normal bowel specimens from 7 age-matched controls were labelled using an immuno-histochemical technique with MAb 171B5. In all specimens of the control group and 9 patients of the HD group, numerous neuromuscular junctions were demonstrated in the muscle layers of the normoganglionic bowel and numerous synapses in the myenteric plexuses. However, in the specimens of 3 patients of the HD group abnormal distribution of nerve terminals was seen in the normoganglionic bowel. Two of these 3 patients had abnormal postoperative defecation. Based on our study, there appears to be a small number of HD patients who have an innervation disorder of the muscle layers in the proximal, normoganglionic bowel pulled through at surgery, which hitherto have been thought to be normal.

The aim of this study was to compare endoloops and endostaples for closing the stump of the appendix during laparoscopic appendectomy (LA) for uncomplicated appendicitis in children.All LA performed for appendicitis from 2005 to 2009 were analyzed prospectively. Cases of complicated appendicitis such as perforated appendicitis or intra-abdominal abscess were excluded, leaving 75 cases closed with loops and 81 cases closed with staples. Choice of technique was determined by the attending surgeon's preference. All patients were managed according to the same pre-, intra-, and postoperative protocols.There were no significant differences between mean age at surgery, gender ratio, preoperative mean white blood cell count and mean C-reactive protein, histopathologic findings, mean duration of surgery, and mean hospitalization. There were no intra-operative complications in either group, but one loop case required conversion to open surgery (P = NS). There were no significant differences in the incidences of intra-abdominal abscess, transient ileus, small bowel obstruction, and wound infection. The postoperative readmission rate for management of complications was 4.0% for loop cases and 2.5% for staple cases (P = NS). Overall, using staples for a standard LA (anesthesia and stump closure) was U.S. $405 more expensive than when loops were used.This is the first prospective study comparing endoloops with endostaples for closing the stump of the appendix during LA for uncomplicated appendicitis in children. Although stapling proved to be more expensive, the choice of technique should reflect the surgeon's experience and confidence to ensure that the patient has the safest possible LA.

We assessed ductal plate malformation (DPM) as a prognostic factor for mid-/long-term outcome in post-hepatoportoenterostomy (HPE) biliary atresia patients.Of 78 cases of HPE performed between 1989 and 2009, biopsy specimens were available for 43. Cytokeratin 19 was used to identify DPM and preoperative status, postoperative serum total bilirubin, time taken to become jaundice-free, duration of jaundice-free period, steroid usage, incidences of cholangitis, bile lakes, and varices, and requirement for reoperation and liver transplantation were compared.DPM was detected in 21/43 (48.8%) subjects [DPM-positive (DPM+)]. Differences were identified for maximum postoperative serum total bilirubin: DPM+ (12.28 ± 1.00 mg/dL) versus DPM-negative (DPM-) (9.67 ± 0.71 mg/dL) (P 0.05) and total steroid usage: DPM+ (98.3 ± 11.8 mg/kg) versus DPM- (83.4 ± 15.9 mg/kg) (P = NS). However, total steroid dose in jaundice-free cases was significantly higher in DPM+ [75.3 ± 10.0 mg/kg (n = 14) vs. 48.1 ± 6.4 mg/kg (n = 19); P 0.05)]. There were no differences for complications. Mean duration of follow-up was significantly shorter for DPM+ (60.9 ± 38.4 vs. 113.8 ± 56.9 months; P 0.05).Postoperative total bilirubin was higher in DPM+ cases, thus higher doses of steroids were required. However, DPM+ did not appear to influence the incidence of complications mid-/long-term.

A 9-year-old girl presented with a chief complaint of abdominal pain. Esophagogastroduodenal endoscopy (EGD) identified a long and large gastric trichobezoar extending into the duodenum. We attempted endoscopic retrieval after informed consent was obtained from the patient's mother. Initially, a gasper with 5-prolongs, commonly used for retrieval of endoscopically excised polyps, failed to remove the whole trichobezoar. When a net was used instead, it proved impossible to remove the trichobezoar completely. Therefore, we withdrew the scope from the mouth, leaving the net grasping the tricobezoar firmly in the stomach. Subsequently, we were able to retrieve about 70% of the trichobezoar manually by grasping the snare part of the net directly. A second pass found no deep laceration or perforation endoscopically. The remaining trichobezoar was completely retrieved with the net. The procedure was completed within 15 min. The retrieved specimens were 34 cm in length and 100 g in weight. The patient was discharged uneventfully 5 d thereafter. She was advised to visit a psychiatrist to avoid suffering from a relapse. Follow-up EGD showed no trichobezoar, and the patient's frontal hair grew back.

Symptomatic hepatic duct diverticulum located outside the liver is extremely rare, with only one reported case. We encountered this anomaly and present our experience, focusing on surgical management. A 17-year-old-girl was referred to our institute because of recurrent right upper quadrant pain. At presentation, serum amylase, lipase, bilirubin, and transaminases were normal. White blood cell count and C-reactive protein were slightly raised. Ultrasonography and magnetic resonance cholangiopancreatography (MRCP) revealed a diverticulum-like lesion on the right side of the bile duct system appearing to overlap the gallbladder. The provisional diagnosis was type II choledochal cyst. Pancreaticobiliary malunion (PBMU) could not be confirmed on MRCP. The origin of the lesion could not be identified initially at laparotomy, but after repeated intraoperative cholangiography (IC) studies, the lesion was found to originate from a normal-sized right hepatic duct via a narrow duct. It was relatively easy to ligate the narrow duct and treat the lesion smoothly. IC also showed that the common bile duct was not dilated and that PBMU was absent. Histopathology showed the lesion to be a diverticulum of the bile duct epithelium. This is only the second report of a symptomatic hepatic duct diverticulum located outside the liver. Based on our experience, IC is particularly useful for the complete understanding of anomalous hepatobiliary duct anatomy and for planning surgical treatment.

We report two cases of rare cystic dilatations of the ejaculatory system. In case 1, a 6-month-old boy was referred to us for the management of recurrent epididymo-orchitis (E-O) complicating open drainage and a colostomy was performed elsewhere for a purulent rectal discharge thought to be rectal duplication. Diagnostic imaging showed a retrovesical cyst. Urethrocystoscopy showed a swelling of the verumontanum. No fistula was seen between the cyst and rectum on colonoscopy. At laparotomy, both ejaculatory ducts and seminal vesicles were found to be fused into a mass with cystic dilatation of the ejaculatory duct. Intraoperative histopathology of the cyst identified a metaplastic epithelial lesion. The cyst was excised with bilateral vasoligation. Since surgery, 8 years ago, urination and defecation have been normal. In case 2, a 4-month-old boy presented with fever and a swollen right scrotum. Ultrasonography showed a retrovesical cyst. Right grade IV vesicoureteral reflux diagnosed on voiding cystourethrography was treated by ureter reimplantation (Cohen) but complicated by recurrent E-O. Urethrocystoscopy with retrograde contrast via the utriculus showed that the cyst opened on the verumontanum, that both ejaculatory ducts opened into the cyst, and there was reflux into the right vas deferens. Right vasoligation alone was performed through a scrotal approach. Although the cyst was not excised, there has been no recurrence of E-O nor enlargement of the cyst for 6 years. Cysts of the ejaculatory system should be considered in the etiology of recurrent E-O in prepubertal children and a high index of awareness is recommended.

Purpose The aim of this study was to describe a new technique for the surgical management of prenatally diagnosed small bowel atresia. Methods Under general anesthesia, a 5-mm trocar was inserted using an open technique through an intraumbilical incision. The proximal atretic bowel end was identified using laparoscopy and mobilized toward the umbilicus using an additional 3-mm trocar inserted in the left lower quadrant. The umbilical trocar then was removed, and a ring retractor was inserted into the trocar site and used to expand the wound to deliver both atretic bowel ends. The bowel was repaired and returned to the abdomen through the umbilical wound. The umbilical fascia and skin were closed conventionally. Results Three patients were reviewed. Two had minimal abdominal distension, and the atretic bowel ends could be identified easily; laparoscopy-assisted surgery was successful. The third case had significant dilatation, and laparotomy was required. Postoperatively, there was minimal abdominal scarring, and the umbilicus was normal in appearance. Conclusions Although this experience is limited to 3 patients, this technique is simple, safe, and virtually scar free and can be applied for the treatment of neonates with prenatally diagnosed small bowel atresia, especially if there is minimal abdominal distension at birth.

Using monoclonal antibody 171B5 against synaptic vesicle proteins, neuromuscular junctions and synapses in the muscle layers of the bowel affected by hypoganglionosis were labeled and their distribution analysed. In the hypoganglionic bowel, there were less synapses in the myenteric plexus and few neuromuscular junctions compared to normal bowels. The bowel dysmotility of the patient with hypoganglionosis appears to be due to inadequate innervation between ganglion cells and smooth muscle cells.

Background/Purpose: Anorectal achalasia (AA) may persist after pull-through (PT) for Hirschsprung's disease (HD), which may cause postoperative enterocolitis (POE) and constipation. The authors modified the Soave PT (modified Soave PT, MSPT) to eliminate AA, and present their results. Methods: This was a 16-year retrospective review of 43 patients with histologically proven HD of the rectosigmoid or sigmoid colon treated by MSPT. The MSPT ivolves excision of the posterior rectal cuff and an intraoperative internal sphincterotomy, allowing the PT colon to fit nicely. Results: Mean age at MSPT was 16.7 months (16 were ≤3 months old [37%]; 7 were neonates [16%]). Mean follow-up was 9.2 years. Six of 43 cases (14%) had preoperative enterocolitis; only 2 of 43 (5%) had single episodes of POE. At review, 37 of 43 were older than 4 years; 29 (78%) had normal bowel function (14 had experienced soiling after MSPT, which resolved after a mean of 6.4 years); and 8 (21%) had problematic bowel function: 3 had occasional soiling, 1 had soiling only before defecation, 3 (8%) had constipation requiring laxatives or enemas, and 1 had significant soiling. Conclusion: MSPT is safe and may contribute to a reduction in the incidence of POE and constipation.

Purpose: Epidermal growth factor (EGF), which is present in breast milk, has both trophic and maturational effects on intestinal mucosa. The aim of this study is to deermine the effect of EGF on spontaneous intestinal bacterial translocation (BT) in formula-fed newborn rabbits, who have a high incidence of BT compared with breast-fed newborn rabbits. Methods: Sixty-one rabbit pups were divided into three groups: EGF(−), n=24, EGF(+), n=22, and breast-fed animals, n=15. Both the EGF(−) and EGF(+) groups were gavage fed a standard artificial formula three times daily. EGF was administered subcutaneously three times daily (1.5 μg/g body weight per day) in the EGF(+) group. The breast-fed group was fed by their mothers ad libitum. At 7 days of age, all rabbits were killed, and the mesenteric lymph nodes (MLN), liver, and spleen were cultured qualitatively for bacterial growth, while the cecum and ileum were quantitatively cultured. To determine the effect of EGF on mucus-producing cells, goblet cell numbers in the small intestine were quantified histologically. Results: There was no BT to MLN, spleen, or liver in the breast-fed group. The incidence of BT to MLN and spleen was significantly lower in the EGF(+) compared with EGF(−) group; (EGF[+]: MLN, 45%; spleen, 32%; Liver, 27%; EGF[−]: MLN, 79%; Spleen 67%; Liver 29%; in EGF[+] MLN and Spleen P v EGF[−]). There was no significant difference in cecal and ileal bacterial colonization between the EGF(+) and EGF(−) groups. The number of goblet cells in the small intestine was significantly lower in the EGF(−) group compared with the EGF(+) group as follows: EGF(+), 14 ± 3; EGF(−), 9 ± 3; breast-fed, 11 ± 5 goblet cells per 100 epithelial cell nuclei; P = .013. Conclusions: (1) EGF caused a significant decrease in spontaneous bacterial translocation in formula-fed newborn rabbits and was associated with an increase in the goblet cell number of the small intestine. (2) These changes occurred in spite of the fact that no changes in small bowel bacterial colonization were observed. (3) These results suggest, but do not prove, that EGF may provide protection for neonates from gut origin infection by improving the mucosal barrier function through increased goblet cell production, thus decreasing the incidence of spontaneous bacterial translocation in the newborn.

Purpose: Delayed gastric emptying (DGE) in children with gastroesophageal reflux (GER) is often treated with a gastric emptying procedure. Although pyloroplasty is the most common gastric emptying procedure performed, pyloromyotomy is easier to perform and is associated with less morbidity. The aim of this study was to compare the efficacy of pyloromyotomy and pyloroplasty in children with DGE and GER undergoing a fundoplication. Materials and Methods: We reviewed the charts of 54 patients with DGE who underwent pyloromyotomy (n = 29), or pyloroplasty (n = 25) along with a fundoplication. A technetium 99-labeled sulfur colloid liquid-phase gastric emptying study (GES) was performed in the pre-and early postoperative period (within 6 months after operation). Normal stomach emptying was defined as greater than 40% at 1 hour. Comparisons were made with regard to postoperative complication rate, incidence of redo fundoplication, length of postoperative hospital stay, and pre- and postoperative GES. Results: The pyloroplasty and pyloromyotomy group were comparable in terms of age, sex, operative indications, and neurological status. There was no significant difference in the GES between the two groups preoperatively. There was a trend toward a decreased incidence of early postoperative complications including gas bloat, wound infection, pneumonia, dysphagia, bowel obstruction and dumping syndrome in the pyloromyotomy (8, 28%) when compared with the pyloroplasty group (12, 48%, P = .10). The mean postoperative hospital stay was 10.6 ± 1.4 days for the pyloroplasty group and 7.6 ± 1.0 days for the pyloromyotomy group ( P + .08). The incidence of a redo fundoplication was 8% in the pyloroplasty and 7% in the pyloromyotomy group. Postoperative gastric emptying increased significantly in both groups (pyloroplasty group, from 18.1 ± 3.1 to 49.5 ± 7.9%, P = .0005; pyloromyotomy group, from 19.3 ± 2.1 to 41.2 ± 3.7%, P = .0001). There was no significant difference in the postoperative GES between the two groups ( P = .289). Conclusion: Both pyloroplasty and pyloromyotomy performed in conjunction with a fundoplication resulted in a significant increase in early postoperative gastric emptying. There was no advantage of pyloroplasty over pyloromyotomy during this follow-up period. These data suggest that pyloromyotomy is an effective gastric emptying procedure in children with GER and DGE.

Thyroglossal duct cyst (TGDC) is one of the more common causes of a pediatric neck mass. Lingual TGDC, which is located at the base of the tongue, is an unusual variant. Because of the oral pharyngeal location, lingual TGDC may cause dysphagia and respiratory distress. Previous investigators have advocated the use of a formal Sistrunk procedure for lingual TGDC. Herein the authors describe three children with a lingual TGDC in whom marsupialization of the cyst was performed, without excision. The follow-up period ranges from 2 to 5 years, and there has been no recurrence. Because of the low morbidity and high success rate associated with this approach, the authors recommend it for the treatment of lingual TGDC.

The authors have previously demonstrated spontaneous bacterial translocation (BT) in newborn rabbits and its resolution with aging. It is hypothesized that this spontaneous BT was associated with an immature gut-associated lymphoid tissue (GALT). The aim of the present study was to characterize the cellular populations of the GALT in rabbits at various ages and to correlate this with the frequency of BT. Small bowel (SB) sections and mesenteric lymph nodes (MLN) were harvested and cultured (aerobically) from New Zealand White rabbits at 0, 6, 14, 28, and more than 90 days of age for determination of bacterial colonization (BC) and BT. Portions of ileum (n = 6 for each age) were simultaneously harvested for immunoperoxidase staining. Total T cells (CDS+), expressed as the number of positive cells/1000 nuclei and activated T cells (CD25+), expressed as the number of positive cells/1000 nuclei and as the ratio of CD25+/CD5+ cells, were analyzed for each tissue. Positive cells were counted in 30 villi by light microscopy. The incidence of BT rose as BC increased in the small bowel and peaked at 6 days of age; BT then decreased with age. CD5+ cells in the small bowel villi at 0 days of age were few (2.5 positive cells/1000 nuclei) and the number significantly increased with age (6 days, 6.5; 14 days, 19.0; 28 days, 31.6; adult, 136.6 positive cells/1,000 nuclei). The distribution of T cells started in the crypts, and with advancing age, cells were found all the way to the top of the villi. The number of CD25+ cells in the villi increased with age. The CD25+/CD5+ ratio in the small bowel villi peaked at 6 days of age. These results demonstrate an inverse relationship between the number of CD5+ cells in the intestinal villi and the incidence of bacterial translocation. The elevation of activated T cells (CD25+) at 6 days of age may be the result of an immunologic activation during the time of peak bacterial translocation. These data suggest that maturity of the GALT leads to a loss of spontaneous bacterial translocation in the newborn period. Modalities that supplement the GALT may help reduce bacterial translocation.

To investigate the beneficial effects of food restriction on systemic lupus erythematosus in NZB x NZW F1 mice, we separated the mice into three groups. One was fed a diet in which total food intake was reduced to 60% of normal from age 2 mo onward, while the animals were still healthy (group 2R). A second group was selected at age 7 mo based on a positive lupus nephritis (proteinuria) and fed the 40% restricted diet thereafter (group 7R) ; a third group was allowed to consume food ad libitum (control). All control mice died of renal disease by age 14 mo, whereas all mice in group 2R and 80% of those in group 7R were living at that age. Measurements of anti-double stranded DNA antibody concentrations in sera and in supernatants of in vitro spleen cell cultures revealed that the production of the immunoglobulin G, but not immunoglobulin M, class of antibodies was markedly and significantly reduced in food-restricted mice. Age-associated changes in lymphocyte subsets seen in control mice, i.e., increases in B :T and CD4 :CD8 T cell ratios, decreases in NTA260 + T cell subsets, and increases in aberrant activated NTA204 + CD4 + T cells and cycling cells, were all significantly lessened in underfed mice. Food restriction did not suppress the secondary acquired antibody responses to a foreign antigen. Thus, the beneficial effects of food restriction in these mice may be related to the lessening of the age-related onset of T cell subset abnormalities, including activation of autoreactive T cells. This results in the arrest of the differentiation and affinity maturation of autoantibody-producing B cells in the process of immunoglobulin M to immunoglobulin G isotype switching, an event which is critical in the pathogenesis of systemic lupus erythematosus. J. Nutr. 125 : 2316-2324, 1995.

This study investigated the relationship between bacterial translocation (BT) and small and large bowel bacterial colonization in the neonatal rabbit. The authors have previously shown that spontaneous BT occurs in rabbits during the first week of life, but is less frequent before the third day of life. The authors have also shown that bacterial colonization of the small bowel, is delayed until 3 to 5 days of age, and is preceded by colonization of the large bowel. Therefore, the authors hypothesizes that BT is dependent on small bowel, not large bowel, colonization. New Zealand White rabbit pups (n = 255) were divided into four groups, formula-fed (FF), formula-fed plus antibiotic (FFAB), breast-fed (BF) and unfed controls (UC). Animals from each group were killed on day 1 to 4 of life. Small bowel (SB) and large bowel (LB) specimens, mesenteric lymph nodes (MLN), spleen (SPL), and liver (LIV) were obtained from each rabbit, incubated for 24 hours in thioglycolate broth, and plated on both MacConkey and Colistin Naladixic Acid media in an aerobic environment. After 24 hours, growth on either plate was recorded. Bacterial colonization was significantly greater in FF versus UC, BF, and FFAB rabbit groups. In addition, large bowel colonization was significantly increased compared with small bowel colonization in all feeding groups (P.05). Bacterial translocation to MLN, SPL, and LIV ranged from 10% to 27% in all groups. Gram-negative bacteria predominated over gram-positive bacteria in translocation to the various tissues sampled.(ABSTRACT TRUNCATED AT 250 WORDS)

A new reliable immunohistochemical method for diagnosing Hirschsprung's disease (HD) using our unique monoclonal antibody (MAb) 171B5 against synaptic vesicles is described. Fresh frozen sections of rectal tissues were used from 13 patients with HD aged 2 weeks to 13 months; 9 had rectosigmoid HD and 4 had total colonic aganglionosis (TCA). Comparable normal colonic and rectal specimens were also obtained from 13 age-matched controls. All specimens were labeled with MAb 171B5, to demonstrate neuronal innervation patterns of both mucosa and submucosa. In all control specimens, many synapses arranged in variciform plexuses were seen in the lamina propria, a moderate number in the muscularis mucosae, and dense clusters in the submucosal plexus. In all aganglionic specimens, only scanty numbers of synapses which were not organized in variciform plexuses were seen in the lamina propria, none in the muscularis mucosae, and a few in the submucosa. These findings suggest that MAb 171B5 immunohistochemistry on the lamina propria alone can differentiate between normal and aganglionic bowel and appears to be a reliable and useful method for detecting HD on suction rectal biopsy.