Outcome Analysis of Reduction and Nonreduction Dismembered Pyeloplasty in Ureteropelvic Junction Obstruction: A Randomized, Prospective, Comparative Study.

Objectives: The objective of this study is to compare the changes in renal function and drainage following open dismembered pyeloplasty with and without renal pelvis reduction. Materials and Methods: Randomized prospective study of children with ureteropelvic junction obstruction undergoing pyeloplasty with (Group 1) and without (Group 2) pelvis reduction over an 18-month period. Postoperative function and drainage were assessed by ethylene dicysteine (EC) scan and intravenous urography (IVU) and renal pelvis size by ultrasonography. Results: Forty-two patients (2 months-11 years) participated. The mean preoperative EC scan function was Group 1: 45.88% ± 14.42% (5%-80%) and Group 2: 39.22% ± 9.75% (21%-53%). (P = 0.117). The mean postoperative EC scan function of Group 1 was 42.64% ± 9.62% (17%-54%) and 43.75% ± 9.88% (17%-58%) and of Group 2 was 44.77% ± 12.82% (20%-68%) and 42.25% ± 8.56% (23%-58%) at 3 months (P = 0.584) and ≥ 1year (P = 0.385), respectively, with no significant difference. None required re-do pyeloplasty. The number of patients with slow drainage, especially at 3 months and also at ≥1-year postoperative period on EC scan was slightly higher in Group 2 compared to Group 1 but did not attain statistical significance. There was postoperative improvement in function and drainage on IVU with no significant difference between the two groups, (P = 0.214; P = 0.99, respectively). At a mean follow-up of 45.5 months, Group 2 also showed significant reduction in pelvis size on ultrasound (P = 0.011). Conclusion: Postoperative function remained stable in both groups. More number of patients achieved unobstructed drainage by 3 mo postoperative after reduction pyeloplasty but drainage patterns were mostly similar between reduction and nonreduction of pelvis group in late follow-up.

Benign Renal Tumors in Pediatric Age Group: Retrospective Analysis.

BACKGROUND: Benign renal tumors are extremely rare and were studied here. This series also includes a renal teratoma in a horseshoe kidney, probably only the second in the pediatric literature. MATERIALS AND METHODS: Retrospective review of children with benign renal tumors operated between 2006 and 2018 at one center. RESULTS: Twelve patients (M:F ratio 10:2), age range 3 weeks (31-week gestation) to 13 years presented with large palpable renal swelling (n = 12) and hematuria (n = 3). Computed tomography (CT) scan showed features typical of the tumor. Final histopathology (age group [mean]) showed: multilocular cystic nephroma (MLCN) - n = 5 (41.7%), (11-16 months [13.6]); congenital mesoblastic nephroma (CMN) - n = 4 (33.3%) (classic 1, cellular 3) (0.75-5 months [2.125]); mature cystic teratoma - n = 1 (8.3%): (48 months, in a horseshoe kidney), and angiomyolipoma (AML) - n = 2 (16.7%) (144 months [sporadic] and 156 months [tuberous sclerosis]) One patient with cystic teratoma with no calcification on CT scan received pre-operative chemotherapy as fine-needle aspiration cytology (FNAC) reported malignant small blue cell tumor. Nephroureterectomy with Gerota's fascia could be done easily in all without intraoperative complications. Delay in presentation in MLCN and CMN led to increased symptoms and CT scan changes. All patients did well in 1.5-12 years (median 3 years) follow-up including cellular mesoblastic nephroma. CONCLUSIONS: Benign renal tumors often occur in specific age groups but may overlap that of Wilms tumor. Proper interpretation of clinical presentation, CT scan, and FNAC findings help in avoiding preoperative chemotherapy. Upfront nephroureterectomy is curative. Histopathological findings decide further treatment. Children with AML and tuberous sclerosis need lifelong follow-up.

Co-Existing Pediatric Ureteropelvic Junction Obstruction and Vesicoureteric Reflux: Prevalence and Implications.

PURPOSE: The purpose of this study is to ascertain the coexistence of ipsilateral vesicoureteric reflux (VUR) with ureteropelvic junction obstruction (UPJO) and to compare postpyeloplasty outcome in patients with and without associated VUR. MATERIALS AND METHODS: Prospective study from 2014 to 2016 of consecutive children (n = 135) undergoing pyeloplasty. Data of patients without (Group 1) and with (Group 2) associated ipsilateral VUR were compared. RESULTS: Thirty-five patients (25.9%) had ipsilateral VUR along with UPJO (Group 2). This group showed the following unique features: (1) Higher percentage of infants (31/35) compared to Group 1 (62/100) (P = 0.003) (2) VUR in the contralateral (normal) kidney in 21/35 (60%) cases and nil in Group 1 (3) Significantly less preoperative differential renal function in children above 1 year (P = 0.007) (4) Presence of renal scars (18 units) and pyelonephritic changes (6 units) in Group 2 at the 1-year follow-up dimercaptosuccinic acid renal scan. Both groups showed improvement in function 3 months after pyeloplasty with no statistically significant difference. Improvement in drainage on the renal scan was better in Group 1 at 3 months postoperative (P = 0.015) as well as between 3 months and 1-year follow-up (P = 0.052). CONCLUSION: The prevalence of VUR was 25.9% in this study and 33.3% in ≤1 year age group. There was a loss of function in delayed presenters with associated ipsilateral VUR. There was delayed drainage postpyeloplasty in patients with VUR. A preoperative voiding cystourethrogram should be done in children <1 year age before pyeloplasty so that associated VUR if detected can be concurrently managed along with pyeloplasty and preserve nephrons affected by the dual pathology.

Anorectal Malformations in Males: Pros and Cons of Neonatal versus Staged Reconstruction for High and Intermediate Varieties.

BACKGROUND: High and intermediate types of anorectal malformations (ARMs) in male neonates may be managed either by primary neonatal reconstruction without colostomy cover or by traditional policy of staged reconstruction after neonatal colostomy. Posterior sagittal anorectoplasty (PSARP) is the current widely practiced reconstructive technique with varied results. AIM: To assess our functional results of PSARP without colostomy in male neonates with high and intermediate ARMs compared to 3-stage (neonatal colostomy - PSARP - colostomy closure) methodology in a high volume tertiary care institution of a developing country. PATIENTS AND METHODS: The number of colostomies performed for male high/intermediate anomalies and the number of babies who completed 3-stage reconstruction during a 10-year period is analyzed. The outcome of primary neonatal PSARPs during the same period was analyzed. Eighty primary PSARPs were compared to 81 staged reconstructions for outcome analysis, using Kelly score. RESULTS: A total of 453 colostomies were performed, but only 253 of them completed all stages of reconstruction (52%). Good continence was achieved in 45% of cases of primary PSARP versus 26% in 3-staged surgery. CONCLUSIONS: Primary PSARP in neonatal period without colostomy is a good option for high and intermediate ARMs in males if the treating surgeon is reasonably skilled in neonatal surgery and PSARP procedures.

Dysphagia because of unilateral internal jugular vein phlebectasia in an infant.

Phlebectasia affecting the internal jugular vein is a rare cause of a benign neck swelling in children. They are mostly asymptomatic and therefore managed conservatively. Ligation of the vein and excision is usually avoided owing to the worry of raised intracranial pressure. We report a case of a large right internal jugular vein phlebectasia, causing dysphagia in a 7month old male child. Contrast enhanced computed tomography with 3-D reconstruction helped in pre-operative anatomical delineation, especially of the lower extent. It was excised through a cervico-thoracic approach with postoperative amelioration of symptoms. Contrary to expectation, the ectasia was not friable and was covered with a pseudocapsule of fibrofatty tissue, making excision easy.

Outcome analysis of pediatric pyeloplasty in units with less than 20% differential renal function.

OBJECTIVE: This study was conducted to assess ureteropelvic junction obstruction (UPJO) units with differential renal function (DRF) ≤20%, 3 months post-pyeloplasty as well as complications and stability of function in long term follow up. MATERIAL AND METHODS: In this prospective study, children below 12 years age with unilateral UPJO and DRF ≤20% undergoing open Anderson-Hynes dismembered pyeloplasty from 2002-2014 excluding associated vesicoureteric reflux were included. Drainage and function were assessed with isotope [ethylene di-cysteine (EC)] scan at 3 months and later during yearly follow-up as well as by intravenous urography (IVU). RESULTS: Of a total 744 patients with UPJO in the study period, 112 had DRF ≤20%. Thirty four (30%) underwent percutaneous nephrostomy. Ten with no function underwent nephrectomy. 102 (mean age 4.7 years) with DRF 0-9% (n = 40) and 10-20% (n = 62) formed the study group. Hypertension (3), small kidney (13) and crossing vessels (9) were associated. Follow up ranged from 1-8 years (4.6 ± 1.34 years). Six patients were lost to follow up. There was significant improvement in drainage in the remaining 96 patients. Mean DRF and IVU function showed highly significant improvement (p < .001) in those with clinical signs and symptoms (n = 85), compared to asymptomatic patients in preoperative 0-9% and 10-20% group. The mean rise in DRF was significantly more in the preoperative 0-9% group, palpable mass presentation and by age at 5-12 years in the 0-9% group and 1-5 years in the 10-20% group. In the symptomatic group, except for intermittent UTIs (n = 8) and pain (n = 3) which subsided 1-2 years after surgery, all patients had resolution of initial complaints. Hypertension resolved in two patients with crossing vessels. No patient required re-do pyeloplasty or developed hypertension during followup. There was no significant difference (p = 1.000) in mean DRF between 3 month and 1-8 years post-operative scans (n = 53). CONCLUSIONS: This study which appears to be the largest in the literature of pediatric UPJO with DRF ≤20%, conclusively shows that there is statistically significant improvement in function after pyeloplasty which remains stable during the period of growth with no long term complications. Nephrectomy should be considered only in patients with nil or <5% uptake on isotope imaging with associated (a) no parenchyma on imaging or on exploration, (b) pyonephrosis, (c) hypertension or (d) minimal urine output on percutaneous nephrostomy.

Biliary Fasciola gigantica infestation in a nonendemic area--An intraoperative surprise.

A 7year old girl infected with the zoonotic trematode, Fasciola gigantica is reported because of the extreme rarity of this condition in our region. Because of the overlap in symptomatology and radiological features, the more common amebic/pyogenic liver abscess in the initial hepatic migratory phase and later choledochal cyst/biliary ascariasis when the parasite was finally located in the extrahepatic bile ducts, were thought of delaying effective treatment. The diagnosis was confirmed only by surgical exploration. The characteristic contrast enhanced computed tomography scan features retrospectively identified were multiple clustered hypodense lesions in the liver with peripheral enhancement in the acute hepatic migratory phase, and periportal tracking in the previously affected areas of the liver with biliary dilatation and a linear hypointense lesion within the common bile duct in the chronic phase. Although a known association, she did not have eosinophilia. This child, who became symptomatic at the age of 5.5years, also appears to be one of the youngest patients reported with Fasciola gigantica.

Comparative analysis of spherical and fusiform choledochal cyst based on three-dimensional magnetic resonance cholangiopancreatography, biliary amylase, and histopathological examination.

AIMS: The aim was to compare biliary amylase, common channel, and gall bladder/liver histopathology between spherical and fusiform choledochal cysts. MATERIALS AND METHODS: Children undergoing cyst excision with Roux-en-Y hepatico-jejunostomy over a 17 months period were prospectively studied. The common channel was assessed by three-dimensional (3D) magnetic resonance cholangiopancreatography (MRCP). RESULTS: Among 22 patients (spherical = 10, fusiform = 12), there was a higher incidence of spherical cysts in infants (5/7-71.4%) and fusiform cysts in older children (10/15-66.7%) (P = 0.09). Common channel identified in 14 (64%) cases was long (>10 mm) in 5 (38.2%) (one spherical, four fusiform [P = 0.5]) with associated high biliary amylase levels (>500 IU/L) in four (one spherical, three fusiform) (P = 0.05). Exact point of junction of common bile duct with pancreatic duct was visible with coronal half-Fourier-acquisition single-shot turbo spin-echo and 3D sampling perfection with application with optimize contrast using different flip angle evolution sequence using a reformatted plane of axis, particularly coronal, and coronal oblique orientation. Biliary amylase was raised (>100 IU/L) in 4 (40%) in the spherical group, compared to 8 (67%) in the fusiform group. Mean biliary amylase was similar in gallbladder and cyst in spherical but higher in gallbladder in fusiform cysts. Mean biliary amylase was <500 IU/L in 85.7% infants. Five out of 7 infants had liver fibrosis or cirrhosis on histopathology (P = 0.05). There were no dysplastic changes in the gallbladder epithelium. CONCLUSION: Three-dimensional MRCP delineated the common channel in two-third cases especially in coronal and coronal oblique orientation. The long common channel may have an etiological role in fusiform cysts. Spherical cysts, especially in infants, have a higher incidence of obstructive cholangiopathy.

Isolated Ileal Pancreatic Heterotopia Causing Intussusception with Gangrene.

Isolated ileal heterotopic pancreatic tissue as a lead point of intussusception in children is extremely rare. We report a 6-month-old female with ileoileal intussusception and a 4-year-old female with ileocolic intussusception both associated with gangrene. A review of literature suggests a higher prevalence of gangrene probably secondary to the pancreatic exocrine tissue.

Laparoscopic nephrectomy in children for benign conditions: indications and outcome.

AIM: To analyze the indications and outcome of laparoscopic nephrectomy for benign non-functioning kidneys in children. MATERIALS AND METHODS: The data of all patients operated over a 10 year period was retrospectively analyzed. RESULTS: There were 56 children, aged 4 months to 12 years with a male: female ratio of 2.3:1. The most common presentation in boys and girls was urinary tract infection (UTI) (61.5% and 47.05% respectively). Incontinence due to ectopic ureter was a close second in girls (41.17%). The most common underlying conditions were vesico-ureteric reflux (42.85%) and multicystic dysplastic kidney (23.2%). There were 6 nephrectomies, 4 heminephroureterectomies and the remaining nephroureterectomies. All children tolerated the surgery well. One patient underwent a concomitant cholecystectomy. The post-operative problems encountered were UTI (1), urine retention (1), pyonephrosis in the opposite kidney and development of contra-lateral reflux (1). All others had resolution of pre-operative symptoms with good cosmesis. CONCLUSIONS: As per available literature, this appears to be the largest Indian series of pediatric laparoscopic nephrectomies for benign non-functioning kidneys. Laparoscopic approach gives excellent results provided pre-operative investigations rule out other causes for the symptoms with which the patient presents. Often it is not the kidney but the dilated dysplastic ureter which is the seat of stasis and infection or pain and therefore should be completely removed.

Long segment jejuno-ileal duplication cyst with ectopic gastric mucosa detected on 99mTc-pertechnetate scintigraphy.

Enteric duplication cysts (EDC) are uncommon congenital anomalies that may occur anywhere along the digestive tract. Ectopic gastric mucosa (EGM), another rare condition, is usually present as short segments in the small intestine and may be associated with EDC. Abdominal scintigraphy with 99mTc pertechnetate may be useful in the diagnosis, since the radiotracer is concentrated by functioning gastric mucosa. In this case report, the authors describe a child with a 150 cm long jejuno-ileal duplication cyst containing EGM identified by intense 99mTc pertechnetate uptake on scintigraphy without any pharmacological intervention.

Caecal duplication cyst mimicking intussusception.

Cystic caecal duplication is a rare congenital anomaly. We report a case of an infant, who presented with bilious vomiting and abdominal distension. On ultrasonography, intussusception with a cyst as a lead point was present in the right iliac fossa. On exploration, there was no evidence of intussusception, only caecal duplication cyst was found. To date, only three cases of caecal duplication which mimicked intussusception have been reported in the English literature.

Duplicated gall bladder with duodenal duplication cyst.

A four year old boy with a duplicated gall bladder communicating with a duplication cyst of the duodenum is reported for the first time in the literature. It is also reported for the absence of any connection with the biliary system.

Potential diagnostic role of renal scintigraphy in the management of patients with high anorectal malformation.

Urological problems are the leading associated anomalies in patients with anorectal malformation (ARM). In this study, we evaluated the role of scintigraphy in managing patients with high ARM. The records of infants with urologic anomalies on abdominal ultrasound and referred for scintigraphic evaluation were retrospectively analyzed. Diuretic renography in these patients was performed using (99m)Tc-diethyl triamine penta-acetic acid ((99m)Tc-DTPA) or (99m)Tc-ethylene cysteine ((99m)Tc-EC) while cortical scintigraphy was performed with (99m)Tc-dimercaptosuccinate (DMSA). Whenever available, EC was preferred in children of age less than 1 year. Forty patients (38 males, 2 females) were analyzed. The mean age of the population was 25 months (range 15 days-21 years). Eighteen of the forty patients were less than 3 months of age. Twenty seven of the patients were less than one year of age. The most common renal anomaly was unilaterally non-visualized kidney affecting 15 out of the 40 patients. The visualised kidney was abnormal in 8/15 patients. Hydronephrotic changes were noticed in 12 of 40 patients of whom 2 had pelvi ureteric junction obstruction that was confirmed and treated surgically. Horse shoe kidney was present in 1 patient while 2 had an ectopic kidney. Scarring/ pyelonephritic changes were found in 7/40 patients. Five patients had already progressed to chronic renal failure at the time of scintigraphy. Only 8 patients of the 40 had bilaterally normal kidneys. In conclusion, this study suggests that urologic abnormalities often found in infants with high ARM may remain clinically silent and eventually lead to chronic renal failure. Proper evaluation by diuretic renography and cortical scintigraphy can lead to early identification of potentially treatable conditions hence reducing the likelihood of developing severe renal damage.

Addition of midazolam to continuous postoperative epidural bupivacaine infusion reduces requirement for rescue analgesia in children undergoing upper abdominal and flank surgery.

STUDY OBJECTIVE: To investigate the effect of adding midazolam to continuous epidural infusion of bupivacaine for postoperative analgesia in children. DESIGN: Prospective, randomized, double-blind, controlled study. SETTING: Tertiary-care center. PATIENTS: 44 ASA physical status I and II children in age groups of two to 10 years, undergoing elective upper abdominal and flank surgery. INTERVENTIONS: At the end of surgery, patients were randomly allocated to receive epidural infusion of 0.125% bupivacaine alone (Group B) or with 20 microg/kg/hr midazolam (Group BM) for 12 hours at the rate of 0.2 mL/kg/hr. MEASUREMENTS: Pain, motor block, and sedation were assessed at predetermined times over 24 hours. Intravenous fentanyl was used as rescue analgesic for the first 12 hours, and tramadol for next 12 hours. Patients were followed at one week, one month, and one year for any neurological deficits. MAIN RESULTS: The number of patients requiring rescue analgesia during infusion was significantly lower in Group BM (7 vs. 17 in Group B; P < 0.001). Time to first rescue analgesia was significantly prolonged in Group BM compared with Group B (P < 0.001). Frequency of fentanyl (P < 0.001) and tramadol (P = 0.001) administration as rescue analgesia was significantly less in Group BM. Significantly lower median pain scores were obtained in Group BM than Group B at all time intervals (P < 0.05). Greater sedation scores were noted in Group BM at all time intervals postoperatively except at 4 hours (P < 0.05). No motor block was observed in any child during the study. No neurological deficit was reported in any child in the one year of follow-up. CONCLUSION: Addition of 20 microg/kg/hr of midazolam to postoperative continuous epidural infusion of 0.125% bupivacaine reduces the requirement for rescue analgesia in children following upper abdominal and flank surgery.

Mature gastric teratoma: recurrence in adulthood.

A case of mature gastric teratoma developing recurrence after 2 decades after complete excision during infancy is being reported. The patient was successfully managed by surgical resection. Pathologic examination confirmed it to be a mature teratoma.

Giant isolated cavernous hemangioma of the stomach.

Giant isolate cavernous hemangiomas of the stomach are extremely rare. An eight year old boy with recurrent episodes of massive hematemesis and involvement of both the anterior and posterior walls of the stomach is presented. Awareness of the lesion, a combination of endoscopy and contrast enhanced CT scan are helpful in reaching a diagnosis. Surgical excision is curative.