Varied parameters and utility of the anal position index: a systematic review and meta-analysis.

PURPOSE: The anal position index (API) was described in 1984 by Reisner et al. Since then, it has been measured in different ethnic populations and its utility in various medical conditions have been explored. We aimed to review the literature regarding the various values reported analytically. METHOD: A Pubmed Search was carried out with the terms Anal Position Index. There were 158 articles hit by the search. The description of the API was uniform in all studies, described as ratio of anus-fourchette distance in girls and anus-scrotum distance in boys to the distance between coccyx and fourchette/scrotum. 18 relevant studies were included describing the values in different ethnic groups and describing its utility. One study was excluded from statistics due to different landmark for measurement. The studies were grouped into 4. Group A:B:C:D comprised of Newborns:Infants:All age groups:Constipated children. We carried out meta-analysis to estimate effect size (mean difference) using STATA software version 16.0. RESULTS: The different ethnic populations in which API were measured, and were Indian (2), Turkish (3), Israel (2), Taiwanese, Thai, Iranian, Spanish, Italian, and Mexican. The API was higher by 0.129 in boys. The mean API in males was 0.53:0.54:0.48:0.52 in Group A:B:C:D with an overall mean (SD) API of 0.51 (0.04). The mean API in females was 0.40:0.40:0.38:0.37 in Group A:B:C:D with an overall mean (SD) API of 0.40(0.03). Two studies were done in mice and showed the relation of API to intrauterine exposure to androgens. In children, API was mostly used to diagnose an anterior ectopic anus and see correlation with constipation. The abnormal values varied from less than 0.30-0.34 in girls and less than 0.41-0.46 in boys. The effect size by regions showed that the estimated effect size for all the regions were within 95% Confidence limits of overall estimate (0.13: 95% CI: 0.13-0.14). Therefore, it can be inferred that there was no significant ethnic variation in the study parameter. The anal position in relation to genitalia was measured in nulliparous women, menopause women, and women with levator deficiency. It was affected in vulvovaginal atrophy. Dichlorodiphenyldichloroethylene exposure during the first trimester of pregnancy was reported to alter the anal position in male infants. CONCLUSION: The anteriorly positioned anus has been associated with constipation. API been measured in various ethnic populations with insignificant variations. The API is significantly (p < 0.05) higher in males. The presence of constipation does not seem to alter API. One should adopt a single method for measurement. API should not be considered at the sole indication for any surgical intervention.

Consensus on the Management of Posterior Urethral Valves from Antenatal Period to Puberty.

The need for successful management of posterior urethral valves always captivates the minds of pediatric surgeons. Its success, however, depends on several factors ranging from prenatal preservation of upper tracts to postoperative pharmacological compliance. Regardless of measures available, some cases do not respond and progress to end stage. The management depends on several issues ranging from age and severity at presentation to long-term follow-up and prevention of secondary renal damage and managing valve bladder syndrome. This article is based on a consensus to the set of questionnaires, prepared by research section of Indian Association of Paediatric Surgeons and discussed by experienced pediatric surgeons based in different institutions in the country. Standard operating procedures for conducting a voiding cystourethrogram and cystoscopy were formulated. Age-wise contrast dosage was calculated for ready reference. Current evidence from literature was also reviewed and included to complete the topic.

Experience with the Redo Pull-Through for Hirschsprung's Disease.

AIM: This study aims to evaluate the need of Redo pull-through (Re PT) procedures for Hirschsprung's disease (HD) and suggest preventive strategies. MATERIALS AND METHODS: Patients who underwent redo procedures for HD from 1980 to 2016 by a single surgeon were retrospectively reviewed. RESULTS: Of 167 patients operated for HD, 32 underwent Re PT; 7 were previously operated by the same surgeon, while 25 were referred from outside. Indication for Re PT included residual disease including the rectal pouch following-Duhamel (12), false-negative biopsy (3), retraction of bowel (5), anorectal stricture (2), bowel twist (1), cuff inversion (2), postmyectomy continued symptoms after primary PT (1), fecal fistula (1), Re PT after surgery for adhesive intestinal obstruction (3), bleeding (1), and combination of causes, including scarred perineum (1). Age at follow-up ranged from 2.5 to 26 years. Proximal diversion was performed in 19 and 14 underwent open Scott Boley's/Soave PT and 5 ileoanal anastomosis. Of the remaining, nondiverted 13 patients, 5 underwent transanal endorectal PT and 8 underwent PT of colostomy. CONCLUSIONS: Most patients of Re PT came after an initial Duhamel's procedure. Retraction of bowel, inversion of cuff, twist, distal bowel stricture, and perianal fibrosis were found after Scott Boley procedure. Proper planning with an initial diversion, nutritional buildup, barium study evaluation, frozen section facility, experienced pathologist, and an expert surgeon are prerequisites for a successful outcome after an initial as well as Re PT.

Early vaginal replacement in cloacal malformation.

PURPOSE: We assessed the surgical outcome of cloacal malformation (CM) with emphasis on need and timing of vaginal replacement. METHODS: An ambispective study of CM was carried out including prospective cases from April 2014 to December 2017 and retrospective cases that came for routine follow-up. Early vaginal replacement was defined as that done at time of bowel pull through. Surgical procedures and associated complications were noted. The current state of urinary continence, faecal continence and renal functions was assessed. RESULTS: 18 patients with CM were studied with median age at presentation of 5 days (1 day-4 years). 18;3;2 babies underwent colostomy; vaginostomy; vesicostomy. All patients underwent posterior sagittal anorectovaginourethroplasty (PSARVUP)/ Pull through at a median age of 13 (4-46) months. Ten patients had long common channel length (> 3 cm). Six patients underwent early vaginal replacement at a median age of 14 (7-25) months with ileum; sigmoid colon; vaginal switch; hemirectum in 2;2;1;1. Three with long common channel who underwent only PSARVUP had inadequate introitus at puberty. Complications included anal mucosal prolapse, urethrovaginal fistula, perineal wound dehiscence, pyometrocolpos, bladder injury and pelvic abscess. Persistent vesicoureteric reflux remained in 8. 5;2 patients had urinary; faecal incontinence. 2 patients of uterus didelphys are having menorrhagia. One patient succumbed to sepsis at 7 months age. Renal functions remained normal in 16. One patient is undergoing dialysis. CONCLUSION: Early vaginal replacement in CM is feasible. Patients with inadequate introitus may suffer from menorrhagia. A regular follow-up is mandatory.

Comparison of the renal dynamic scan performed with 99mTc-L,L-EC and 99mTc-MAG3 in children with pelviureteric junction obstruction.

The aim was to compare the renal dynamic scan (RDS) performed with technetium-99-L,L-ethylene dicysteine (Tc-L,L-EC) and technetium-99-mercaptoacetyltriglycine (Tc-MAG3) in children with pelviureteric junction (PUJ) obstruction. A retrospective study was carried out and children with PUJ obstruction who had RDS performed with both Tc-L,L-EC and Tc-MAG3. Children with any intervention in between the two scans or a gap of more than 2 months in between renal scans were excluded. The dose of each radiotracer used was 0.1 mCi/kg (3.7 MBq/kg), with a minimum dose of 1 mCi (37 MBq). RDS was performed using the F+0 protocol. The differential renal function, Tmax, T1/2, drainage pattern, and hepatic uptake of the radiotracer were recorded and compared. A Bland-Altman plot was used to assess agreement between the two radiotracers. Sixteen children were included in the study. A total of 18 obstructed and 14 normal renal units were available to us for study. The values of differential renal function as well as Tmax and T1/2 of the two radiotracers were in agreement. In three obstructed kidneys in which T1/2 on Tc-MAG3 was greater than 20 min, Tc-L,L-EC showed T1/2 values of 13.3 min or less. Tc-L,L-EC showed nonobstructive drainage in three patients who had shown partial obstruction on Tc-MAG3 scan. The hepatic uptake of Tc-L,L-EC was also lower compared with Tc-MAG3. To conclude Tc-L,L-EC is a useful radiotracer for the evaluation of children with PUJ obstruction, with better assessment of drainage and lower hepatic uptake compared with Tc-MAG3.

Surgical modifications, additions, and alternatives to Kasai hepato-portoenterostomy to improve the outcome in biliary atresia.

Kasai hepato-portoenterostomy (HPE) is the most widely used surgical technique to restore bile flow in biliary atresia (BA). We aimed to review literature on HPE substitutes and additions to Kasai especially in advanced BA (ABA). A PubMed search was done for surgical procedures apart from or along with Kasai HPE for BA. Additional procedures to prevent cholangitis were also reviewed. Procedures and outcome were analysed. Alternative procedures done by the authors have also been described briefly. Results have been compiled in this review article. In ABA, with portal hypertension and liver cirrhosis, Kasai HPE is associated with poor outcome, increased morbidity, and even mortality. Most require liver transplant (LT). Some alternatives to HPE include exploration at porta hepatis to assess the bile flow yet avoid the major surgery (HPE) as a bridge to LT. Conduit diversion may help to combat cholangitis resistant to steroid therapy. Stoma formation is not preferred in cases listed for LT due to high risk of bleeding. Hepatocyte infusion, stem cell therapy, and synthetic liver are the future options to meet the challenges in BA. Various alternative procedures may become handy in the future especially in ABA.

Varied facets of rectal atresia and rectal stenosis.

Rectal atresia (RA) and rectal stenosis (RS) are rare anomalies with varied treatment options. A thorough literature review was done on reported cases/series of RA and RS. Based on evidence from cases managed over last 15 years, new insights into embryology were hypothesized. A comprehensive review was compiled with updated knowledge on diagnosis and management. RA is classified into five types I: II: III: IV: V as RS: RA with septal defect: RA with a fibrous cord between two atretic ends: RA with a gap: Multiple RA and/or RS. Current definitive surgical repair of these anomalies preserves the anal canal, dentate line, and sphincter complex. Most neonates with RA undergo sigmoid colostomy except few with RS who can rarely decompress adequately. Membranous RS and septal RA may seldom respond to dilatation or be amenable to transanal repair. Posterior sagittal anorectoplasty with an end-to-end/side repair is recommended for RA and most intramural RS. RS may be associated with a presacral mass and colonic/rectal motility disorders. The expected postoperative outcome is good if the normally developed anal sphincter complex is retained undamaged. Early recognition of the type of anomaly is necessary for appropriate management.

Surgical techniques for esophageal replacement in children.

PURPOSE: Surgical techniques for esophageal replacement (ER) in children include colon interposition, gastric tube, gastric transposition, and jejunal interposition. This review evaluates the merits and demerits of each. METHOD: Surgical techniques, complications, and outcome of ER are reviewed over last seven decades. RESULTS: Colon interposition is the time-tested procedure with minimal and less serious complications. Long-term complications include reflux, halitosis, colonic segment dilatation, and anastomotic stricture, sometimes requiring surgical interventions especially for dilatation and reflux. Gastric tube is technically more risky, and associated with early serious complications like prolonged leak in neck or mediastinum, graft necrosis, and ischemia leading to stricture of the tube. Long-term results are good. Gastric transposition is much simpler, can be performed in emergency and in newborns. It involves a single anastomosis in the neck. Post-operative complications include gastric stasis, bile reflux, restricted growth, and decreased pulmonary functional capacity. Jejunal interposition has not been used extensively due to short mesentery but long-term results are good in expert hands. CONCLUSION: Colon is the most preferred and safest organ for ER. Stomach is a vascular and muscular organ with lower risk of ischemia. Gastric tube is a demanding technique. Jejunum or ileum is alternative for redo cases.

Diversities of H-type anorectal malformation: a systematic review on a rare variant of the Krickenbeck classification.

Congenital H-type fistula is a rare congenital rectourogenital connection with an external anal opening in a normal or ectopic position. A systematic review was done to study the anatomical types of congenital H-type fistula, embryology, clinical presentation, relative gender distribution, associated anomalies, investigative modalities, and recent advances in treatment of these lesions. A PubMed search included H-type anorectal malformation; H-type anorectal malformations; H-type anorectal; and H-type congenital anorectal that gave 9;43;76;26 abstracts, respectively. Relevant studies and cited articles were studied omitting duplicate search. The reported incidence is 0.1-16 % of all anorectal malformation. The H-type anorectal malformation is 2.5-6 times more common in females and usually associated with a normal anus. In males, the anomaly is usually a variant with an ectopic anus or a perineal fistula. Anatomical types include anovestibular; rectovestibular; rectovaginal fistula in females and rectourethral (bulbar, prostatic, bladder neck) and rectovesical fistula in males. Variants identified include H-type fistula with perineal fistula, perineal groove, H-type sinus, H-type canal, and acquired H-type fistula. This review compiles the available literature over last six decades. Various surgical corrective procedures have been described. The high recurrence decreases with a learning curve and experience.

Impact of fetal counseling on outcome of antenatal congenital surgical anomalies.

AIM: To analyze the impact of counseling on antenatal congenital surgical anomalies (ACSA). METHODS: Cases presenting with ACSA for fetal counseling and those presenting in post-natal period following diagnosis of ACSA (PACSA) for surgical opinion were analyzed for spectrum, presentation and outcome. RESULTS: 117 cases including ACSA(68);PACSA(49) were analyzed. Gestational age at diagnosis of ACSA;PACSA was 17-37;17-39 weeks (median 24;32 weeks). Diagnoses in ACSA;PACSA included urological (26;31), neurological (10;5), congenital diaphragmatic hernia (CDH)(5;1), gastrointestinal (5;5), lung and chest anomalies (5;1), intraabdominal cysts (4;1), abdominal wall defects (4;0), tumors (3;3), limb anomaly (1;1), esophageal atresia (1;1), conjoint twins (1;0), hepatomegaly (1;0), and major cardiac anomalies (2;0). Two antenatal interventions were done for ACSA; vesicoamniotic shunt and amnioinfusion for oligohydramnios. 17;24 ACSA;PACSA required early surgical intervention in post-natal period. Nine ACSA underwent medical termination of pregnancy and 4 had intrauterine demise. Nine ACSA babies died including two CDH, one gastroschisis, one duodenal atresia, one conjoint twins, one megacystitis with motility disorder and three posterior urethral valves. All PACSA babies survived. CONCLUSION: Fetal counseling for CSA portrays true outcome of ACSA with 32.3% (22/68) mortality versus 0% for PACSA due to selection bias. However, fetal counseling ensures optimal perinatal care.

Disseminated histiocytic sarcoma in a child: a clinicopathological dichotomy with 8†years survival.

A 3-year-old girl presented with a non-healing ulcer, originating as a pustule over the right anterior chest wall of 1½ month duration associated with high fever. A subcutaneous nodule along with right apical and anterior axillary lymph nodes was palpable. Abdominal ultrasound and chest skiagram were normal. Fine needle aspiration cytology (FNAC) from the axillary lymph node was suggestive of embryonal rhabdomyosarcoma. Bone marrow aspirate was normal. The ulcer and subcutaneous nodule were excised completely with adequate margins. Histopathological examinations were compatible with malignancy of histiocytic origin with clear resected margins. The axillary nodes were free of tumour. Adjuvant chemotherapy was given for 13 months. The patient is doing well at 8 years follow-up.

Omental acinar cell carcinoma of pancreatic origin in a child: a clinicopathological rarity.

A 6-year-old boy presented with a large subhepatic mass associated with pain abdomen. Exploration revealed a tumor in lesser omentum, completely separate from the normal pancreas that was excised completely. Histopathology suggested acinar cell carcinoma of pancreatic origin in an ectopic location. The child is well at 5 months follow-up.

Male cloaca malformation: rare variant of anorectal malformation.

PURPOSE: Experience with male cloaca (MC), a single opening in perineum for passage of urine and meconeum is described. METHODS: Cases of MC were ambispectively studied, prospectively from July 2007 to April 2015 and retrospectively for last three decades. RESULTS: Seven cases of MC were identified, between the ages of newborn-4 years (median 10 days). Two missed cases underwent a colostomy, posterior sagittal anorectoplasty, and urethroplasty. Two cases underwent perineal urethrostomy and anoplasty followed by urethroplasty. In one case, part of the rectal wall was used to form urethral tube and urethrostomy. For three recent cases, posterior sagittal anorectourethroplasty was done with mobilization of rectal pouch and common channel, separation of common wall between the urethra and rectum, urethroplasty varying from 1.5 to 3 cm, perineal body reconstruction, perineal urethrostomy and anoplasty. Follow-up of 6 patients varied from 3 months to 23 years. One case is lost to follow-up. Three patients have completed repair. Complications included a discharging sinus and a urethral fistula in one case each. One patient died while awaiting urethroplasty. Two patients are awaiting formal urethroplasty. CONCLUSION: With familiarity of varying anatomy of MC, early recognition can avoid a neonatal colostomy in selected patients.

Management options of congenital pouch colon--a rare variant of anorectal malformation.

PURPOSE: Congenital pouch colon (CPC) was analysed for anatomical variations and surgical options. METHODS: Records of CPC patients managed between 1999 and 2014 were studied. CPC was classified as complete (CCPC) and incomplete (ICPC) pouch. RESULTS: Of 400 cases of high anorectal malformations, 68 cases were CPC (17%). Male:female ratio was 2:1. Fistulous communication was colovesical, colocloacal, colovaginal and absent in 42, 15, 8 and 3 cases. ICPC and CCPC was 48 (70%) and 20 (30%). In neonatal period, proximal/end colostomy (31), ileostomy (6), pouch excision with abdominoperineal pull-through (18 cases) and coloplasty with end colostomy (7) were done. 6 presented as infants including 3 referred cases of CCPC with a window colostomy. Definitive surgery was completed in 56. Severe colonic dilatation after coloplasty was noted in 5, requiring excision of coloplasty segment in 2. Histopathology of excised pouch (45) showed muscle layer disorganization, widened sub mucosa, prominent vasculature and mature and immature ganglion cells, with no hypertrophy of nerve fibres. Diarrhoea and faecal incontinence (soiling) were more frequent in patients with CCPC versus ICPC. Six neonates with CCPC died. Six are awaiting definitive surgery. CONCLUSION: CPC had 8.8% neonatal mortality. Pouch excision and definitive procedure are feasible in neonates with CPC. Coloplasty in CCPC may result in postoperative colonic dilatation.

Malignant systemic hypertension, encephalopathy and bradycardia following splenectomy for hereditary spherocytosis.

An 8-year-old girl suffering from hereditary spherocytosis underwent splenectomy for chronic severe anaemia. Surgery was uneventful and the patient had a good early postoperative recovery. On the third postoperative day, however, she developed severe headache with associated abnormal movements of upper limbs and nystagmus. She had a heart rate of 50 bpm and a blood pressure of 180/110 mm Hg. She was managed with triple antihypertensives, antiepileptics and sedatives. She recovered slowly over 2 weeks and is fine at 5 months follow-up.

Retrospective analysis of FFPE based Wilms' Tumor samples through copy number and somatic mutation related Molecular Inversion Probe Based Array.

In this report, retrospectively, we analyzed fifteen histo-pathologically characterized FFPE based Wilms' Tumor (WT) samples following an integrative approach of copy number (CN) and loss of heterozygosity (LOH) imbalances. The isolated-DNA was tested on CN and somatic-mutation related Molecular-Inversion-Probe based-Oncoscan Array™ and was analyzed through Nexus-Express OncoScan-3.0 and 7.0 software. We identified gain of 3p13.0-q29, 4p16.3-14.0, 7, 12p13.33-q24.33, and losses of 1p36.11-q44, 11p15.5-q25, 21q 22.2-22.3 and 22q11.21-13.2 in six samples (W1-6) and validated them in nine more samples (W7-9, W12-15, W17-18). Some observed that discrete deletions (1p, 1q, 10p, 10q, 13q, 20p) were specific to our samples. Maximum-LOH was observed in Ch11 as reported in previous studies. However, LOH was also observed in different regions of Ch7 including some cancer genes. The identified LOH-regions (1q21.2-q21.3, 2p24.1-23.3, 2p24.3-24.3, 3p21.3-21.1, 4p16.3, 7p11.2-p11.1, 7q31.2-31.32, 7q34-q35 and Ch 8) in W1-W6 were also validated in W7-9, W12-15 and W18. In addition, previously reported LOH of 1p and 16q region was also observed in our cases. The proven and novel onco (OG)- and tumor-suppressor genes (TSGs) involved in the CNV regions affected the major pathways like Chromatin Modification, RAS, PI3K; RAS in 14/15 cases, NOTCH/TGF-ß and Cell Cycle Apoptosis in 10/15 cases, APC in 9/15 cases and Transcriptional Regulation in 7/15 cases, PI3K and genome maintenance in 6/15 cases. This exhaustive profiling of OG and TG may help in prognosis and diagnosis of the disease after validation of all the relevant results, especially the novel ones, obtained in this research in a larger number of samples.

Neuroblastoma: outcome over a 14 year period from a tertiary care referral centre in India.

PURPOSE: To evaluate the outcome of children with neuroblastoma (NB) from a tertiary care referral centre in India. METHOD: All children with NB registered from October 1996 through July 2009 were included in the study. INSS was used for staging. All children included in the study received chemotherapy and radiation therapy appropriate for stage. Tumor resection was done when feasible. The final outcome was overall survival and it was categorized as Complete Response (CR), Partial Response (PR); No Response (NR) and Progressive Disease (PD). Analysis of three-year overall survival was done using Kaplan Meier method and Log Rank test of significance. Multivariate analysis for significance of age, site and stage was performed. RESULTS: 144 children in the age range of 1-132months (median 36) were enrolled. Only 38 (26.4%) children were below 12months. 112 (77.8%) of the tumors were abdominal and 32 (22.2%) were extra-abdominal. Stage distribution was 1+2 in 6 (4.2%); 3 in 58 (40.3%); 4 in 68 (47.2%); 4s in 12 (8.3%). 83 (57.6%) underwent gross complete resection. At the time of last follow-up, 100 (69.4%) were alive [60 CR (41.7%); 33 PR; 7 PD/NR] and 44 (30.6%) were dead [1CR; 11PR; 32 PD/NR]. The three-year OS was 60.7% [95 CI 50.4-69.5]. The OS was 69.7% for those<12months of age [95 CI 51.8-82.0] and CR was achieved in 57.9%, while for those >12months the OS was 55.3% [95 CI 42.2-66.6] and CR was achieved in 35.8% (p=0.73). All 6 (100%) patients with Stage 1 and Stage 2 disease were alive and disease free. The OS was 71.5% for Stage 3[95 CI 55.3-82.7] and CR was achieved in 56.9%, while for Stage 4 the OS was 35.7%[95 CI 19.3-52.4] and CR was achieved in 17.6% (p=0.001). The OS was 83.3% for 4s [95 CI 48.2-95.6] and CR was achieved in 75%. CONCLUSION: All the six children with Stage 1 & 2 achieved CR and were alive, while 57% of Stage 3 could achieve CR and had an OS of 71.5%. The OS (35.7%) and CR (17.6%) for Stage 4 were significantly less (p=0.001).