Premalignant/malignant histology in excised choledochal cyst specimens from children. Experience and literature review.

BACKGROUND: Dysplasia, carcinoma in situ, and other malignant transformation or premalignant/malignant histopathology (PMMH) seem uncommon in pediatric choledochal cyst (CC). A literature review and the authors' experience are presented. METHODS: All reports about PMMH in CC patients 15 years old or younger published in English and all cases of PMMH in specimens excised from CC patients 15 years old or younger by the authors were reviewed. RESULTS: Of 20 published reports, PMMH was adenocarcinoma (n = 4), sarcoma (n = 4), and dysplasia (n = 12). Treatment for malignancies was primary pancreaticoduodenectomy (PD; n = 2) or cyst excision/hepaticojejunostomy (Ex/HJ; n = 6). Outcomes at the time of writing for malignancies: 2 deaths, 4 survivors after follow-up of 2 years, and 2 lost to follow-up. No dysplasia case has undergone malignant transformation. The authors have experienced 7 cases of PMMH; adenocarcinoma in situ (AIS; n = 1) and dysplasia (n = 6). CONCLUSIONS: The present study identified the youngest cases of AIS and dysplasia from specimens excised when they were 3 years old and 4 months old, respectively. Both are published for the first time as evidence that PMMH can complicate CC in young patients. Long-term protocolized postoperative follow-up is mandatory when PMMH is diagnosed in pediatric CC.

Prednisolone administration monitored by postoperative stool color achieves high jaundice clearance after laparoscopic portoenterostomy for biliary atresia.

PURPOSE: Stool color (SC) for monitoring prednisolone use in biliary atresia (BA) patients after laparoscopic portoenterostomy (LPE) was reviewed. METHODS: Subjects were 47 post-LPE BA patients given a reducing dose course of intravenous prednisolone. The course started at 4 mg/kg/day and gradually reduced, ultimately reaching a final total dose (TD) of 31.5 mg/kg. Normal SC indicated a course could progress until finished and was repeated until jaundice clearance (JC) was achieved. Abnormal SC persisting for two consecutive courses was the absolute indication for redo or liver transplantation (LTx). RESULTS: JC was achieved in 38/47 (80.9%) LPE cases and 4/6 redos to give an overall JC rate (JCR) of 42/47 (89.4%). Outcomes after one course (n = 5; JCR: 80.0%; median TD: 30.0 mg/kg, interquartile range [IQR: 26.0-31.5]), two courses (n = 10; JCR: 90.0%; median TD: 62.5 mg/kg [IQR: 60.8-66.0]), three courses (n = 13; JCR: 92.3%; median TD: 90.0 mg/kg [IQR: 86.0-90.0]), four courses (n = 10; JCR: 80.0%; median TD: 120.0 mg/kg [IQR: 116.7-123.3]), five courses (n = 7; JCR: 100%; median TD: 156.0 mg/kg [IQR: 154.3-157.5]), six courses (n = 1; JCR: 100%; TD: 189.0 mg/kg), ten courses (n = 1; JCR: 100%; TD: 308 mg/kg). CONCLUSION: Indications for repeat prednisolone and timing of redo/LTx based on SC monitoring appeared effective based on high JCR and successful redo/LTx. LEVELS OF EVIDENCE: III.

Comparison of robotic assistance and laparoscopy for pediatric choledochal cyst: advantages of robotic assistance.

AIM: Surgery for pediatric choledochal cyst (CC), complete excision (CE), and Roux-en-Y hepaticojejunostomy anastomosis (HJA) can be performed using laparoscopy (Lap), robotic-assistance (Rob; da Vinci Xi/Si), or both (Lap/Rob). METHODS: Lap was used exclusively between 2009 and 2021 (n = 31) and Rob was introduced in 2017 (n = 23). All subjects were matched for age, weight, BMI, and episodes of preoperative pancreatitis. For Rob, the first 15/23 were Lap-CE/Rob-HJA and the last 8/23 were Rob-CE/Rob-HJA. RESULTS: Total anastomotic time (TAT), TAT per suture during HJA, and time taken for dissection during CE were significantly shorter with less variance for Rob, although overall operative times were similar. Serum amylase on postoperative days 3, 5, and 7 were significantly higher for Lap. Times taken to ambulate, for return of bowel sounds, and discharge home were all significantly shorter for Rob. All postoperative complications occurred after Lap; HJA leak (n = 1; 3.2%), HJA stricture (n = 1; 3.2%), both treated by open re-HJA; and pancreatic fistula (n = 6; 19%), all treated conservatively. CONCLUSION: Dissection and recovery were faster with Rob while overcoming Lap-associated shortcomings to prevent complications associated with suturing. Both CE and HJA were safer and more reliable with Rob, a reflection of Rob's superiority.

Prospective evaluation of common hepatic duct histopathology at the time of choledochal cyst excision ranging from children to adults.

PURPOSE: To evaluate common hepatic duct just distal to the HE anastomosis (d-CHD) prospectively for mucosal damage, inflammation, fibrosis, dysplasia, carcinoma in situ, malignant transformation, effects of serum amylase, and symptoms at presentation in CC cases ranging from children to adults. METHODS: Cross-sections of d-CHD obtained at cyst excision 2018-2023 from 65 CC patients; 40 children (< 15 years old), 25 adults (≥ 15) were examined with hematoxylin and eosin, Ki-67, S100P, IMP3, p53, and Masson's trichrome to determine an inflammation score (IS), fibrosis score (FS), and damaged mucosa rate (DMR; damaged mucosa expressed as a percentage of the internal circumference). RESULTS: Mean age at cyst excision ("age") was 18.2 years (range: 3 months-74 years). Significant inverse correlations were found for age and DMR (p = 0.002), age and IS (p = 0.011), and age and Ki-67 (p = 0.01). FS did not correlate with age (p = 0.32) despite significantly increased IS in children. Dysplasia was identified in a 4-month-old girl with cystic CC. Serum amylase was elevated in high DMR subjects. CONCLUSIONS: High DMR, high IS, and evidence of dysplasia in pediatric CC suggest children are at risk for serious sequelae best managed by precise histopathology, protocolized follow-up, and awareness that premalignant histopathology can arise in infancy.

Long-term outcomes of male imperforate anus with recto-urethral fistula: laparoscopy-assisted anorectoplasty versus posterior sagittal anorectoplasty.

PURPOSE: A five-parameter fecal continence evaluation questionnaire (FCEQ) and incidence of complications were used for long-term assessment of laparoscopy-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) for treating male imperforate anus (MIA) with rectobulbar (RB) or rectoprostatic (RP) fistulas. METHODS: Subjects were 64 consecutive Japanese MIA patients with RB or RP fistulas treated at a single institution between 1995 and 2021. FCEQ data collected retrospectively were used to calculate a fecal continence evaluation (FCE) score (best = 10) and coefficient of variation for FCE (FCECV). The statistical significance threshold was defined at p < 0.05. RESULTS: Fistulas were RB (n = 40; LAARP = 25/40, PSARP = 15/40) or RP (n = 24; LAARP = 17/24, PSARP = 7/24). Mean ages at surgery and status of the sacrum were similar (p = 0.06, 0.05 and 0.51). FCE scores in RP-LAARP were consistently higher with less FCECV but differences were only statistically significant from 7 years postoperatively (p < 0.05). While FCE scores for RB-LAARP and RB-PSARP were similar (p = 0.99), FCECV were lower for RB-LAARP compared with RB-PSARP. LAARP was associated with less-wound infections, but greater incidence of anal mucosal prolapse unrelated to preoperative status of the sacrum. CONCLUSION: Long-term postoperative FCEQ assessment favored LAARP for treating MIA with either RB or RP fistulas.

Mid-term outcome of postoperative biliary atresia patients according to level of transection of the biliary remnant and depth of suturing.

PURPOSE: Open portoenterostomy (PE) for biliary atresia (BA) is currently more extended (EP) than the original (OP). Typical OP techniques, shallow transection of the biliary remnant and shallow suturing, both lost in EP, were revived as a modified procedure (MP). Postoperative outcomes of EP and MP were compared. METHODS: Subjects were 55 consecutive BA patients treated by EP (n = 18) or MP (n = 37) at a single center between 2004 and 2021. RESULTS: Mean follow-up duration was: MP: 15.5 years (range 0.1-12.3 years) and EP: 15.5 years (range 0.38-17.1 years). The ratio of jaundice free (JF; total bilirubin ≤ 1.2 mg/dL) subjects was significantly higher in MP (78.4%) versus EP (50%); p > 0.05, the incidence of bile lakes at the porta hepatis was significantly higher in MP (7/37: 18.9%) versus EP (0/18: 0%); p > 0.05, and Kaplan-Meier analysis showed JF survival with the native liver (JF + SNL) was significantly better in MP (26/37: 70.2%) versus EP (4/18: 22.2%); p > 0.05. All other criteria were similar. Of note, time taken to become JF and the incidence of cholangitis were not significantly different. CONCLUSIONS: Shallow transection and shallow suturing would appear to influence postoperative outcome. The etiology of bile lake formation in MP requires urgent confirmation.

Successful engraftment of bladder organoids in de-epithelialized mouse colon.

PURPOSE: To engraft bladder organoids (BO) on de-epithelialized mouse colon using an epithelial replacement technique. METHODS: BO cultured using bladder specimens from enhanced green fluorescent protein (EGFP) transgenic mice were engrafted to replace proximal colon epithelium stripped from an approximately 1 cm long target site in syngeneic wild-type recipient mice (n = 9) by exposure to ethylenediaminetetraacetic acid by infusion and flushing with phosphate buffered saline. Target sites were harvested on postoperative days 2, 7, and 28 for hematoxylin-eosin staining and immunofluorescence. RESULTS: Histology on postoperative days 7 and 28 showed BO derived EGFP + cells forming multiple layers on the luminal surface of the colon. Immunohistochemistry showed that EGFP + areas were positive for CK5 and CK14, markers for basal and immature subtype urothelium, respectively, but negative for CA2, a marker for colonic epithelium. Ki67 was detected predominantly in the basal parts of EGFP + areas on postoperative day 7 and day 28. CONCLUSIONS: This is the first report of successful engraftment of BO in de-epithelialized colon with urothelial tissue reconstituted by actively proliferating cells. This technique could be developed for augmentation cystoplasty to prevent bladder calculi formation and malignant transformation.

Japan's comprehensive undescended testis screening program: incidence of ascending testis after screening.

PURPOSE: Screening for undescended testis (UDT) in Japan is performed as a neonate, then at 1, 3, 10, and 18 months old, and 3 years old. Incidence of ascending testis (AT) after screening was reviewed. METHODS: All orchiopexy/orchiectomy at a single institute between July 2005 and June 2022 were reviewed retrospectively. RESULTS: 376 boys had 422 procedures; 54/422 (12.8%) were in 48 boys ≥ 4 years old (mean age: 6.7 years; range: 4-13); testes were normal (n = 22; 40.7%), small (n = 25; 46.2%), or atrophied (n = 7; 1.3%). There were 47 orchiopexies and 7 orchiectomies for atrophy. Incidence of AT in boys ≥ 4 years old was 24/422 (5.7%). Of these, 16/422 (3.8%) developed after normal descent and 8/422 (1.9%) were associated with retractile testis (AT + RET). Other indications included delayed treatment for UDT (n = 13), late referral by pediatricians (n = 10), and iatrogenic UDT (n = 6). Surgical intervention in boys ≥ 4 years old (12.8%) was less than that reported in the West (range: 30-50%) as was AT: (5.7% versus 15.4%) and AT + RET (1.9% versus 13.8%). CONCLUSIONS: Comprehensive UDT screening probably contributed to the lower incidence of surgery and AT (especially AT + RET) in boys ≥ 4 years old.

Significant neonatal intraoperative cerebral and renal oxygen desaturation identified with near-infrared spectroscopy.

INTRODUCTION: Near-infrared spectroscopy (NIRS) was used to monitor intraoperative regional oxygen saturation (rSO2) during open (Op) and minimally invasive (MI) surgery performed in neonates (N) and children. MATERIALS AND METHODS: NIRS sensors were applied to the forehead and flanks for cerebral rSO2 (C-rSO2) and renal rSO2 (R-rSO2), respectively. MI included laparoscopy (La), retroperitoneoscopy (Re) and thoracoscopy (Th). In children, Op and MI were major operations taking at least 3 h (MOp; MMI). Pathological desaturation (PD) was defined as > 20% deterioration in rSO2. RESULTS: Mean ages at surgery were N: 5.2 ± 8.2 days, MOp: 2.4 ± 2.9 years, and MMI: 3.8 ± 4.3 years. Despite significantly shorter operative times in N (169 ± 94 min; p < 0.0001), PD was significantly worse; PD(C-rSO2): N = 14/35 (40.0%) versus MOp = 3/36 (8.3%) and MMI = 7/58 (12.1%); p = 0.0006, and PD(R-rSO2): N = 27/35 (77.1%) versus MOp = 6/36 (16.7%) and MMI = 7/58 (12.1%); p < 0.0001, respectively. PD(R-rSO2) occurred immediately with visceral reduction in NOp (Fig. 1) and PD was frequent during NMI(Th) (Fig. 2). rSO2 was stable throughout MOp and MMI (Fig. 3). Fig. 1 Pathological desaturation in renal rSO2 after visceral reduction for gastroschisis. Renal rSO2 deteriorated immediately after viscera were returned to the abdominal cavity rSO2 regional oxygen saturation Fig. 2 Fragility of tissue perfusion during thoracoscopic lung lobectomy in a neonate. Pathological desaturation occurred frequently during neonatal thoracoscopic surgery rSO2 regional oxygen saturation Fig. 3 Changes in cerebral and renal rSO2 according to operative time. Cerebral and renal rSO2 did not appear to change according to operative time during major open and major minimally invasive surgery in children. rSO2 regional oxygen saturation CONCLUSIONS: NIRS is a non-invasive technique for monitoring rSO2 as an indicator of intraoperative stress and vascular perfusion. PD was so significant in neonates that intraoperative NIRS is highly recommended during thoracoscopy and procedures requiring visceral manipulation.

Levator ani asymmetry and deviation in high-type anorectal malformation evaluated by magnetic resonance imaging.

INTRODUCTION: The levator ani (LA) complex in high-type imperforate anus (H-IA), low-type imperforate anus (L-IA), and Hirschsprung's disease (HD) patients as controls were documented using magnetic resonance imaging (MRI) and compared for symmetry. MATERIALS AND METHODS: Mean left:right LA thickness ratio (LA ratio), and deviation of the LA from the pubococcygeal line (PCL; LA angle) were calculated from thin-slice MRI images (axial 2 mm, coronal 2 mm, and sagittal 3 mm) of the puborectalis and pubococcygeus taken parallel to the PCL under sedation in H-IA (n=14), L-IA (n=16), and HD (n=9). RESULTS: MRI scans were performed between January 2018 and June 2021. LA were significantly thinner in H-IA (1.78±0.46 mm) compared with L-IA (2.97±0.55 mm) and controls (2.87±0.32 mm), p<0.0001. LA ratio was significantly lower in H-IA (0.71±0.15) compared with L-IA (0.93±0.04), and controls (0.91±0.06), p<0.0001. Mean LA-angle was significantly different in H-IA, 10.8° (range 6°-19°), versus L-IA and controls, both zero degrees (range 0°-5°), p<0.0001, respectively. CONCLUSIONS: LA was confirmed to be significantly asymmetric in H-IA. Because outcome of surgical repair involving a midline incision, such as posterior sagittal anorectoplasty could be impaired, pediatric surgeons are advised to plan surgical intervention for H-IA carefully and appropriately.

High intestinal vascular permeability in a murine model for Hirschsprung's disease: implications for postoperative Hirschsprung-associated enterocolitis.

PURPOSE: Intestinal vascular permeability (VP) in a murine model for Hirschsprung's disease (HD) and postoperative Hirschsprung-associated enterocolitis (HAEC) were investigated. METHODS: Intestinal VP was determined using a Miles assay using 1% Evans blue injected into a superficial temporal vein of newborn endothelin receptor-B KO HD model (KO) and syngeneic wild-type (WT) mice (n = 5, respectively). Extravasated Evans blue in normoganglionic ileum (Ng-I), normoganglionic proximal colon (Ng-PC) and aganglionic distal colon (Ag-DC) was quantified by absorbance at 620 nm. Quantitative polymerase chain reaction (qPCR) for Vascular Endothelial Growth Factor A (VEGF-A), VEGF-B, CDH5, SELE and CD31, and immunofluorescence for CD31 were performed. RESULTS: VP was significantly higher in Ng-I, Ng-PC, and Ag-DC from KO than WT (p < 0.01, p < 0.05, and p < 0.05, respectively). qPCR demonstrated upregulated VEGF-A in Ng-I and Ag-DC, VEGF-B in Ng-I, and SELE in Ng-I and Ng-PC (p < 0.05, p < 0.05, p < 0.05, p < 0.01 and p < 0.05, respectively), and downregulated CDH5 in Ng-I and Ng-PC from KO (p < 0.05, respectively). Expression of CD31 mRNA in Ng-I and Ag-DC from KO was significantly higher on qPCR (p < 0.05) but differences on immunofluorescence were not significant. CONCLUSIONS: VP may be etiologic for postoperative HAEC throughout the intestinal tract even after excision of aganglionic bowel.

Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining "successful" portoenterostomy.

PURPOSE: Native liver survivors (NLS) after portoenterostomy (PE) for biliary atresia (BA) with normal biomarkers defined as total bilirubin (T-Bil), aspartate aminotransferase (AST)/alanine aminotransferase (ALT) for liver function (LF), cholinesterase (ChE), platelet count (PC), and absence of portal hypertension (PHT) were reviewed to redefine "successful" PE. METHODS: 92 post-PE BA patients were classified as NLS-1: normal biomarkers, PHT (-); NLS-2: at least one abnormal biomarker, PHT (-); NLS-3: normal biomarkers, PHT ( +); NLS-4: abnormal biomarkers, PHT ( +) and reviewed for a maximum 32 years. RESULTS: As of June 2022, 55/92 (59.8%) had received liver transplants and 37/92 (40.2%) were NLS. NLS patients were classified as excellent outcome (EO): NLS-1 (n = 10; 27.0%) or non-EO: NLS-2: (n = 8; 21.6%), NLS-3: (n = 6; 16.2%), and NLS-4: (n = 13; 35.1%). Compared with non-EO, EO had PE earlier (50.5 versus 65 days; not significant; p = 0.08), significantly earlier onset of symptoms (13 days versus 32 days; p = 0.01) and significantly shorter jaundice-clearance (JC; 34.5 days versus 56.0 days; p < 0.001). Durations of follow-up were similar: 13 years in EO, 18.5 years in NLS-2, 20 years in NLS-3, and 15 years in NLS-4. CONCLUSION: Incidence of "successful" PE or EO is low and correlated with early onset of symptoms and quicker JC.

Oncologic safety of Carrel patch hepaticojejunostomy for treating cystic-type choledochal cyst in children based on 20-plus years follow-up.

AIM: During choledochal cyst (CC) excision, the hepaticojejunostomy anastomosis (HJA) can be performed conventionally (CHJA) or with a Carrel patch (CPA). CPA can increase CHD diameter to 10-13 mm, preventing anastomotic stenosis and intrahepatic bile duct (IHBD) stones but may be at risk for malignant transformation. METHODS: The medical records of 83 cystic-type CC with CHD ≤ 9 mm followed up for at least 20 years were reviewed retrospectively. Available excised CC specimens (70/83) were re-examined blindly for pre-malignant changes. A questionnaire about suturing narrow lumens was conducted. RESULTS: All 83 had pancreaticobiliary maljunction. Group data were similar. Anastomoses were CPA (n = 43) and CHJA (n = 40). Mean diameter for CPA was 11.4 mm (range: 10-13 mm); for CHJA was 7.4 mm (range: 5-9 mm). Mean follow-up was 27.7 years (range: 20-42). Postoperative anastomotic stenoses were less after CPA: 1/43 (2.3%) versus 5/40 (12.5%) (p = 0.10), but CHJA had significantly more postoperative IHBD stones: 0% versus 4/40 (10.0%) (p < 0.05). All IHBD stone patients had anastomotic stenosis. Excised specimens showed no pre-malignant cytology. Lumen diameter ≤ 9 mm was considered challenging by 10/10 surgical trainees and ≤ 7 mm by 16/22 pediatric surgeons. CONCLUSIONS: CPA appears to be oncologically safe because of the absence of malignant transformation for at least 20 years.

Quality of life after type-A esophageal atresia surgery: changes over time and effect of pre-anastomotic elongation.

AIM: To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients. METHODS: Modified gastrointestinal quality-of-life index surveys were administered to postoperative EA patients who were at least 7 years old at evaluation to assess three topics about general lifestyle (GL), five topics about EA, and four topics about mental health (MH). For MH, caregivers were also interviewed, but separately. Subjects were divided according to age: children (7-12 years old), teenagers (13-19), and adults (20 and over) and compared according to Foker or Kimura elongation (FK) or bougienage stretching (BS). RESULTS: There were 22 patients evaluated. Responses for GL, EA, and MH did not differ significantly between age groups, but MH responses by caregivers for subjects who were children or teenagers scored significantly lower than responses they made themselves. For primary esophageal elongation technique (PET), age at esophagoesophagostomy was significantly higher in FK. Despite FK scoring 15.1 versus 12.4 for BS during EA evaluation, this difference was not statistically significant. CONCLUSION: Changes in QOL responses according to age were unremarkable. However, discrepancies in MH indicate that subjects felt better than their caregivers thought. PET did not appear to influence QOL.

Changes in postoperative quality of life of pediatric total colonic aganglionosis patients: effect of pull-through technique.

AIM: To assess mid-/long-term quality of life (QOL) of total colonic aganglionosis (TCA) patients. METHODS: Modified pre-existing QOL assessment tools for general lifestyle (GL), bowel function (BF), and mental health (MH) were administered to postoperative TCA patients from five institutions, who were at least 7 years old to compare Duhamel (with pouch) and Swenson/Soave (without pouch) techniques between children (Ch 7-12 years old), teenagers (Tn 13-19), and adults (Ad 20 and over). For MH, caregivers were also interviewed, but separately. Maximum scores were 12 for GL/MH and 18 for BF. RESULTS: There were 32 subjects. GL and BF scores increased significantly from Ch (GL 4.8 ± 2.5, BF: 11.3 ± 4.6) to Tn (GL 7.8 ± 2.6, BF 16.2 ± 3.0); scores for MH did not change significantly. Mean caregiver MH scores were significantly lower than mean subject MH scores for all age groups (subject scores: 10.1, 10.7, 10.7 versus caregiver scores: 6.8, 7.8, 8.1 for Ch, Tn, Ad, respectively). PT technique/presence of a pouch did not influence the incidence of enterocolitis or QOL scores. CONCLUSION: MH responses showed subjects felt better than caregivers believed. This discrepancy could cause conflict despite steadily improving GL/BF. QOL was unaffected by PT technique/presence of a pouch.

Recovery of bowel function after transperitoneal or retroperitoneal laparoscopic pyeloplasty. A multi-center study.

AIM: To document the recovery of bowel function (BF) in children after transperitoneal (TP) or retroperitoneal (RP) laparoscopic pyeloplasty. METHODS: Data were obtained retrospectively from four centers between 2008 and 2019 for TP (n = 51) and RP (n = 58). Each surgeon chose which technique to perform. RESULTS: Subject demographics were not significantly different. Differences in operative times were not significant (RP: 241 min versus TP: 225 min). Mean duration/requirement for postoperative epidural/intravenous analgesia were not significantly different (TP: 1.4 days versus RP: 1.3 days) and (TP: 66.7% versus RP: 67.2%), respectively. Postoperative nasogastric (NG) intubation was more common in RP (TP: 19.6% versus RP: 44.8%; p < .05). NG aspiration (TP: 0.15 mL/kg/hr versus RP: 0.16 mL/kg/hr), nausea (TP: 31.4% versus RP: 17.2%), and vomiting (TP: 19.6% versus RP: 15.5%) were not significantly different. There were no perioperative complications (including ileus). Abdominal distention was problematic in one case per group (TP: 2.0% versus RP: 1.7%). Times for oral liquid (TP: 0.69 day versus RP: 0.83 day), solid food (TP: 0.88 day versus RP 1.07 days), and the first bowel movement (TP: 2.86 days versus RP: 2.79 days), were not significantly different. CONCLUSIONS: BF recovery would appear to be consistent, independent of technique.

Decreased incidence of intussusception during the COVID-19 pandemic. Trends in pediatric surgical emergencies.

PURPOSE: Recent reports suggest that the COVID-19 pandemic may be influencing disease morbidity. The purpose of this study was to investigate pandemic-related changes in the incidence of pediatric surgical emergencies. METHODS: Data from patients with one of 8 typical conditions considered to be pediatric emergencies who presented at 3 hospitals close to central Tokyo were collated retrospectively from accident and emergency (AE) department records for 2020 and compared with data for 3 years prior to 2020. RESULTS: All subjects had similar demographic profiles. The total number of pediatric AE attendances from 2017 to 2020 was 2880 (2017: n = 600, 2018: n = 736, 2019: n = 817, and 2020: n = 727). Annual attendances were similar. Of the 8 conditions, there were significantly less cases of intussusception in 2020 than previously (23/727; 3.1% versus 132/2153; 6.1%) p < 0.01 and the number of emergency surgical interventions for intussusception was also significantly less in 2020 (0/23; 0% versus 13/132; 9.8%) p < 0.01. CONCLUSION: The implementation of preventative measures to combat the COVID-19 pandemic in 2020 would appear to have influenced the etiopathogenesis of intussusception enough to significantly decrease its overall incidence and the requirement for emergency surgical intervention.

Atypical rectoscrotal and rectoperineal fistula in male anorectal malformations: a case series of non-detaching strategies for preventing urethral injury.

Anorectal malformations (ARM) represent a broad spectrum of patients with different level of the rectum and type of a fistula. Standardized approaches are usually successful, but patients occasionally present with an unusual course of fistula which requires a modified surgical strategy. We present here three male ARM patients with an atypical fistula which did not have connection with the urinary tract, but ran near the fistula. Case 1 has a low-type ARM with a rectoscrotal fistula running deep and partly involved in the corpus spongiosum. Anorectoplasty was performed through an anterior sagittal incision and the anterior wall of the fistula was laid open leaving the posterior wall undetached. Case 2 was diagnosed with an intermediate-type ARM with a long rectoscrotal fistula running near and parallel the urethra. Posterior sagittal anorectoplasty (PSARP) was performed leaving the fistula untouched. Case 3 presented with an intermediate-type ARM with a rectoperineal fistula adherent to the urethra. The patient was treated by PSARP leaving the fistula and part of the muscle coat of the rectum in situ. All the cases were smoothly discharged and no urological complication nor problem associated with the residual fistula was observed at the latest follow-up (17 months-2 years). Preoperative distal colostography with the aid of diverting colostomy was importantly useful for deciding surgical procedure.

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.

How best to expose the entire surgical anal canal in the operative field during transanal pull-through for Hirschsprung's disease: a crucial step that determines success.

PURPOSE: During transanal pull-through (TAPT) for Hirschsprung's disease (HD), exposing the entire surgical anal canal (SAC) including the squamo-columnar junction, or anorectal line (ARL) is a crucial step for minimizing problematic postoperative bowel function. We present a hint for exposing the entire SAC. METHOD: Histologically, the ARL represents the junction of proximal unilayer columnar colorectal mucosa with distal stratified squamous epithelium and is the proximal limit of the SAC. It is an obvious landmark; proximal mucosa is vivid pink and distal mucosa is more whitish. We use the Lone Star (LS) self-retaining retractor system to expose the ARL. Before we attach the LS hooks to the anal sinuses on the dentate line full-circle, we place 3/0 sutures at 0, 3, 6, and 9 o'clock around the anus to expose the anal sinuses. If the patient's buttocks cannot be positioned as described or the patient is too high on the table, the LS ring does not sit well, resulting only in dilatation and lengthening of the SAC without prolapse. By hanging the patient's buttocks 5 cm over the end of the table, the LS ring sits snugly and the ARL and entire SAC prolapse to the anal verge. RESULTS: Good positioning, as described, greatly facilitated dissection in 61/68 TAPT cases, while poor exposure hindered treatment in 7/68. CONCLUSION: Thorough exposure of the entire SAC, which is crucial for adequate TAPT, is greatly facilitated by patient positioning.