Lumboscopic-Assisted Pyeloplasty: A Single-Port, Retroperitoneoscopic Approach for Children with Pelvi-Ureteric Junction Obstruction.

INTRODUCTION: Pelvi-ureteric junction obstruction (PUJO) is one of the most common conditions presenting to a pediatric urologist. As laparoscopic or robotic-assisted pyeloplasty, either transperitoneal or retroperitoneal, involves intracorporeal suturing skills and has a long learning curve, they have not gained popularity among beginners in laparoscopy. OBJECTIVE: We conducted a study to assess the results of a single-port, retroperitoneoscopic approach to renal access, i.e. lumboscopic-assisted pyeloplasty (LAP), by single surgeon at our institute. MATERIALS AND METHODS: A retrospective review of all children who underwent LAP from July 2013 to March 2018 was conducted. Patients who presented with PUJO and required surgical treatment were included. A single-port lumboscopy using coaxial telescope was performed in prone position in all patients. The renal pelvis was dissected and retrieved through the port site followed by extracorporeal hand-sewn pyeloplasty over a double-J stent or a nephrostent. The operative time, postoperative pain, surgical complications, duration of hospital stay, follow-up, and cosmesis at 6 months postsurgery were evaluated. RESULTS: A total of 96 children were included (72 males and 24 females), with the age at operation ranging from 3 months to 10 years (mean = 4.9 years). All patients had an uneventful postoperative recovery. Two patients had a superficial wound infection, and one patient was converted to open approach due to excessive bleeding. The average operating time was 80 ± 22.5 min, the median duration of hospital stay was 3 days, and the average scar length at 3 months was 15.6 ± 0.4 mm. Follow-up renogram (diethylenetriamine pentaacetic acid) showed satisfactory postpyeloplasty drainage pattern in 93 children while three showed obstructive drainage curves. CONCLUSION: LAP can be performed safely with minimal retroperitoneal dissection, excellent cosmetic results, and minimal postoperative pain in children with PUJO. It has a shorter learning curve as compared to laparoscopic pyeloplasty as it involves time tested extracorporeal hand-sewn anastomosis.

A Clinical Approach to Diagnosis of Ambiguous Genitalia.

Disorders of sex development (DSD) are a sensitive and stressful condition for the family as well as the treating physician to deal with. The main issue in managing such cases is sex assignment. The decision is influenced by the cultural background, the sex of rearing, clinical features, the biochemical parameters including hormonal studies, the imaging reports, parental preference, fertility potential, and the assessment of mental make-up of the child when possible. In third world countries, there is diagnostic dilemma as most children with DSD present late and a detailed-lengthy work-up often delay their definitive treatment. In this article, the authors try to identify the important clinical features in children presenting with various types of DSD, which may aid in making a quick provisional clinical diagnosis and expediting the diagnostic work-up. The data have been gathered from 38 years of experience of the senior author while managing about 1200 cases of DSD in the pediatric intersex clinic at the tertiary care level institute.

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.

Surgical Surprise during Posterior Sagittal Anorectoplasy: A Rectal Duplication.

An 8-month-old boy with anorectal malformation (ARM) was incidentally found to have double rectal pouches during posterior sagittal anorectoplasty. The distal blind-ending pouch was excised, and the larger proximal pouch was tapered and anorectoplasty performed. The excised pouch was confirmed as rectal duplication cyst. One must be aware of such uncommon associations with ARM.

Hydrometrocolpos etiology and management: past beckons the present.

Hydrometrocolpos is a rare condition in which the uterus and the vagina are grossly distended with a retained fluid other than pus or blood. It may present during the neonatal period or later at puberty. Most cases reported earlier were stillbirths and were diagnosed only on autopsy. Antenatal diagnosis is now possible with the advent of ultrasound. An early diagnosis and speedy management is the key to survival. Many previous case reports have focused on the varied clinical presentations, multiple causes, associated syndromes and/or the radiological diagnosis of this condition. However, management options for different types of hydrometrocolpos have not yet been concisely discussed. We have reviewed the literature and tried to summarize the management options applicable to most case scenarios of hydrometrocolpos.

A review of genetic factors contributing to the etiopathogenesis of anorectal malformations.

BACKGROUND: Anorectal malformation (ARM) is a common congenital anomaly with a wide clinical spectrum. Recently, many genetic and molecular studies have been conducted worldwide highlighting the contribution of genetic factors in its etiology. We summarize the current literature on such genetic factors. MATERIALS AND METHODS: Literature search was done using different combinations of terms related to genetics in anorectal malformations. From 2012 to June 2017, articles published in the English literature and studies conducted on human population were included. OBSERVATIONS AND RESULTS: A paradigm shift was observed from the earlier studies concentrating on genetic aberrations in specific pathways to genome wide arrays exploring single nucleotide polymorphisms (SNPs) and copy number variations (CNVs) in ARM patients. Rare CNVs (including 79 genes) and SNPs have been found to genetically contribute to ARM. Out of disrupted 79 genes one such putative gene is DKK4. Down regulation of CDX-1 gene has also been implicated in isolated ARM patients. In syndromic ARM de novo microdeletion at 17q12 and a few others have been identified. CONCLUSION: Major genetic aberrations proposed in the pathogenesis of ARM affect members of the Wnt, Hox (homebox) genes, Sonic hedgehog (Shh) and Gli2, Bmp4, Fgf and CDX1 signalling pathways; probable targets of future molecular gene therapy.

Ratio of Preoperative and Postoperative Serum Bilirubin Levels Predicts Early Outcome Following Biliary Atresia Surgery.

AIM: This study investigates the fall in total serum bilirubin levels within 1 week after surgery, as a marker to predict early outcome in biliary atresia (BA) patients post-Kasai portoenterostomy (KP). METHODS: The ratio of total serum bilirubin levels at the 7th postoperative day to the preoperative level (TB7/TB0) in patients undergoing KP was calculated (January 2011-July 2015). Patients were stratified after 3-months follow-up into outcome groups depending on the clinical clearance of jaundice and TB7/TB0 ratio was correlated to outcome and liver histopathological changes in these groups. RESULTS: Sixty-one patients (M:F = 44:17), median age 75 days were included. At the end of 3 months, 27 (44.39%) were anicteric while 26 (42.6%) were still clinically jaundiced. Patients with a higher median value of TB7/TB0, that is, 0.856 were more likely to have jaundice at the end of 3 months as compared to patients with a lower median value of 0.615 (P < 0.0001). A cutoff TB7/TB0 ratio >0.723 predicted the KP outcome with 84.6% sensitivity and 81.5% specificity. The difference in TB7/TB0 ratio between patients with varying severity of liver histopathological changes was also significant, namely, cholestasis (P = 0.01), hepatocellular damage (P = 0.03), portal inflammation (P = 0.04), and portal fibrosis (P = 0.02). CONCLUSIONS: The rapidity of fall in the total serum bilirubin levels within 1 week post-KP was able to predict the likely outcome in BA patients.

Chronic pelvic pain and dysmenorrhea disguising a rare submucosal leiomyoma of the urinary bladder.

Representing 0.43% of all urinary bladder neoplasms, leiomyomas are rare mesenchymal tumours with a benign pathophysiology. There have only been approximately 250 cases published on this subject, necessitating further inquiry into this disease and effective management protocols. Treatment options may include a broad spectrum of surgical interventions, from minimally invasive resection to radical cystectomy, depending on the location, size and symptoms associated with the tumour. To date, few cases of leiomyoma have resulted in recurrence after removal, and zero have reported malignant transformation. Described here in detail is a woman in her early 40s who presented with a history of chronic pelvic pain and irregular vaginal bleeding. The urology team completed further evaluation after imaging discovered a concerning bladder lesion. Eventually, she underwent transurethral resection, with the subsequent pathology revealing a rare diagnosis of leiomyoma in the urinary bladder.

Penile Osteosarcoma: A Rare Extraosseous Site.

Primary penile extraosseous osteosarcoma (EOS) ranks the most uncommon amongst the differential penile masses, with only nine cases reported so far. In this report, we share the management of a 67-year-old Hispanic male who presented with a painful mass over his distal penile shaft and glans for the last two months. After initial imaging and complete blood investigations, he underwent partial penectomy. Histology revealed high-grade sarcoma, with osteoid production, favoring high-grade extra-skeletal osteosarcoma, with tumor necrosis involving approximately 5% of the tumor volume. The patient had bilateral palpable inguinal lymphadenopathy, which was seen even on a pre-op CT scan. The patient thus underwent bilateral robotic superficial and deep inguinal standard template lymph node dissection three weeks after his partial penectomy. His pathology was negative for malignancy in all examined lymph nodes. At his last follow-up, five months post his primary surgery, he had been doing well without concerns for recurrence.

Does preoperative screening VCUG affect the outcomes and complications of pyeloplasty in patients with ureteropelvic junction obstruction?

INTRODUCTION: The role of voiding cystourethrogram (VCUG) in evaluating vesicoureteral reflux (VUR) in patients with known ureteropelvic junction obstruction (UPJO) remains unclear. While VCUG is frequently performed, the incidence of concomitant VUR and UPJO is low, and VUR is often low-grade with high rates of spontaneous regression. OBJECTIVE: To analyze the clinical relevance of VCUG in patients with UPJO by determining its incidence and studying the difference in clinical outcomes between patients with known, unknown, and negative VUR. STUDY DESIGN: Retrospective review of patients with UPJO who underwent pyeloplasty from 2012 to 2020 with <18 years-old, unilateral UPJO, postoperative follow-up of ≥2 months and had at least 1 renal ultrasound (US) after pyeloplasty. Results were compared among 3 groups: patients who underwent VCUG before pyeloplasty and were found to have VUR (group 1), patients who underwent VCUG before pyeloplasty without VUR (group 2), and patients who did not have a VCUG before pyeloplasty (group 3). RESULTS: A total of 275 patients met the inclusion criteria, of which 21 patients were classified in group 1, 166 patients in group 2, and 88 patients in group 3 (Table). The age at preoperative VCUG was 14.7 ± 32.9 months in group 1 and 15.17 ± 35.8 months in group 2 (p = 0.960). Overall, the incidence of concomitant UPJO and VUR was 11.2%. In group 1 the initial VUR grade was 5 in 2 patients, 4 in 3, 3 in 5, 2 in 7, and 1 in 4 patients. Of these, only 1 patient required ureteral reimplantation after pyeloplasty. Post-pyeloplasty, no significant differences were observed in complications (p = 0.7436), length of follow up (p = 0.3212), SFU grade 4 hydronephrosis (p = 0.2247), postoperative UTIs (p = 0.1047) and pyeloplasty success rate (p = 0.4206) among the 3 groups. Despite the use of antibiotic prophylaxis being significantly different amongst the three groups (p < 0.001), it was not associated with a lower incidence of postoperative UTIs (group 1 p = 0.068, group 2 p = 0.486, group 3 p = 1). In patients with reflux, an increase in age was associated with a decrease in the rates of complications (p = 0.019). CONCLUSION: We found no significant difference in the outcomes in patients who had a preoperative VCUG as compared to those who did not. The preoperative diagnosis of VUR by VCUG changed the management in less than 1% of the study population and thus its role in patients with UPJO should be reevaluated.

Need for urodynamic evaluation as a regular follow-up tool in assessment of long-term urological outcomes in patients with sacrococcygeal teratoma.

AIM: To assess the long-term urologic outcomes in follow-up of patients of sacrococcygeal teratoma (SCT) using urodynamic study (UDS) in addition to clinical and radiologic evaluation. METHODS: A prospective study of clinical, radiological and urodynamic evaluation in patients with SCT who underwent resection between January 2002-June 2015 and were followed up till January 2016 was conducted. RESULTS: Total 57 patients, 42 (73.7%) females and 15 (26.3%) males with 35 (62.4%) following treatment for benign and 22 (38.5%) for malignant disease were included. Twenty-eight of 57 (49.12%) had urological problems. Clinical complaints in 21 (36.8%) patients included stress urinary incontinence-14 (66.7%), enuresis-9 (42.9%), and poor stream or dribbling of urine-6 (28.6%). Eight of 51 patients (15.7%) had abnormal ultrasound findings, which included contracted, trabeculated thick walled bladder (3), bilateral hydronephrosis (3) and significant post void residue (PVR) (6). Seven of 57 underwent micturating cystourethrogram (MCU), 5 had an abnormal report[significant PVR (4), small trabeculated bladder (3), reflux (2) and large capacity bladder (1)]. Urodynamic study was done in 27 patients, 18/27 (66.7%) had abnormalities. Six patients without any clinical or ultrasonographic abnormalities had abnormal UDS. Total 28 (49.12%) had urological comorbidities. Three patients had overactive bladder, five dysfunctional voiding, one underactive bladder and one had giggle incontinence. Children were managed by behaviour therapy and pharmacotherapy. CONCLUSION: Urodynamic evaluation could detect abnormalities in patients who had no urinary complaints or abnormality on ultrasound. The abnormalities have a potential for progressive upper tract damage. Urodynamics should be an integral part of urological surveillance in patients operated for SCT. TYPE OF STUDY: Prognostic study. LEVEL OF EVIDENCE: Level II (Prospective cohort study).

Total midgut duplication: a ticking time bomb.

A day-old neonate presented with bowel obstruction and an abdominal mass. Exploratory laparotomy revealed complete tubular midgut duplication from duodeno-jejunal junction up to terminal ileum which was communicating with the ileum distally. At the proximal end, another 5×5 cm duplication cyst was identified and excised. Postoperatively, complaints were relieved. During follow-up, 99m-Tc-pertechnetate-SPECT scan showed ectopic gastric mucosa in lower abdomen and in the right hemithorax. CECT-chest showed a 3×3 cm foregut duplication cyst, but there were no respiratory symptoms. While being planned for an elective surgery, he presented at 6 months of age in emergency with massive bleed per-rectum and shock. He underwent Wrenn procedure without any injury to the normal bowel. The thoracic foregut duplication cyst was excised later. Total midgut duplication, though benign, may present with life-threatening haemorrhage if left untreated. Presence of one such lesion warrants a search for others. Mucosal stripping is a simple and safe alternate to resection.

Antenatally Diagnosed Surgical Conditions: Fetus As Our Patient.

In today's era of improved antenatal care and screening modalities (3D high-resolution fetal ultrasound, fetal magnetic resonance imaging, fetal echocardiography, maternal serum markers and fetal blood sampling), an early diagnosis of surgical fetal abnormalities is routinely possible. A thorough knowledge about the incidence of such defects, the means of detection and evaluation, the scope of fetal intervention, postnatal management and long-term outcomes of common surgical conditions diagnosed antenatally is essential. This knowledge would not only help in proper patient care and management but also to ensure appropriate counselling of the expectant parents. This article highlights the fetal anomalies which are amenable to some form of fetal intervention from a pediatric surgical perspective as also those which can be treated after birth.

Peutz-Jeghers syndrome: need for early screening.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant cancer-predisposing condition characterised by intestinal hamartomatous polyps and distinct melanin depositions in skin and mucosa. Small intestinal cancer in patients with PJS usually presents by the third decade. A 7-year-old-PJS boy presented with recurrent episodes of colicky abdominal pain and melena requiring repeated blood transfusions. Abdominal CT scan revealed multiple jejunal polyps with jejunoileal intussusception. On exploration, the intussuscepted bowel was resected along with its mesentery and anastomosed. Simultaneously, multiple enterotomies with resection of palpable polyps were performed. The resected bowel showed well-differentiated stage 2A adenocarcinoma with clear resected margins. Postoperatively, the complaints were relieved. On follow-up, he was asymptomatic and is now on yearly cancer surveillance. This is probably the youngest reported case of small bowel cancer in PJS.

Congenital colonic stenosis with absent caecum and appendix: a rare association.

Congenital colonic stenosis is a rare condition with less than 20 cases reported in the literature since 1966. We report an interesting case of a 7-month-old baby girl who presented with features suggestive of acute intestinal obstruction. On exploration, it was a case of ascending colon stenosis with absence of caecum and appendix. A double barrel ileocolostomy was performed. The histopathology confirmed the diagnosis of colonic stenosis and ruled out the presence of Hirschsprung's disease in the distal colon. The child underwent second stage surgery (stoma closure) after 9 months. In complex cases of congenital colonic stenosis, an early decompressive surgery followed by a delayed second stage closure is recommended in patients with poor general condition.

'Mutiny on the crown': two cases of rare cephalic malformations.

The presentation and management of two bizarre congenital cephalic curiosities at the two extremes of the clinical spectrum are discussed herewith. Case 1: presented to us as a neonate with a scalp swelling mirror-imaging her head and face. The journey from clinics to wards and to the operation theatre and to her home is introspected. Case 2: presented to us as dicephalous dibrachius dipus parapagus conjoined twins. The detailed work-up of individual organ systems, the multidisciplinary approach to management and the final outcome are discussed. This is an unsolved mystery for the anatomists, paediatric surgeons, radiologists and the medical fraternity at large.