Endoscopic Management of Vesicoureteral Reflux and Long-term Follow-up.

OBJECTIVE: To report our experience with endoscopic management of vesicoureteral reflux (VUR) by injection of a tissue bulking substance - Dextranomer/ hyaluronic acid co-polymer at vesicoureteric junction. DESIGN: Retrospective analyses of case records. SETTING: Pediatric Surgery department in a tertiary care government Institute. PARTICIPANTS: 500 children (767 renal units) consecutively referred to the out-patient department with vesicoureteral reflux noted on micturating cysto-urethrogram (MCU) over a period of 13 years (2004-2016). INTERVENTION: Preoperative VUR grading and renal scars on radionuclide scans were documented. Dextranomer hyaluronic acid copolymer was injected through a cystoscope at the vesicoureteral junction as a day care procedure under short anesthesia. Patients were followed (average duration 27.3 mo) with clinical assessment, periodic urine cultures and renal scans. MAIN OUTCOME MEASURE: Cessation of VUR and symptomatic relief / clinical success postoperatively at 3 months. RESULTS: Complete symptomatic relief was obtained in 482 (96.4%) patients. In 681 units where MCU was available, 614 (90%) units showed resolution of VUR. CONCLUSIONS: Endoscopic injection of tissue bulking substances at vesicoureteric junction to stop VUR seems to be an effective intervention.

Acetylcholinesterase histochemistry (AChE)--A helpful technique in the diagnosis and in aiding the operative procedures of Hirschsprung disease.

BACKGROUND: Hirschsprung's disease (HD) is an anomaly characterized by the absence of myenteric and submucosal ganglion cells (GC) in the distal alimentary tract. Diagnosis of HD is made by the absence of GC and missing out on even a single ganglion cell can be very devastating. Acetylcholinesterase (AChE) histochemistry, done on frozen sections is said to be a very useful ancillary technique in the diagnosis and in aiding the operative procedures of HD. METHODS: To assess this, 73 samples from 42 suspected/known cases of HD were subjected to frozen section analysis with rapid haematoxylin and eosin, toluidin blue stain along with AChE histochemistry. The remnant sample was paraffin embedded for routine haematoxylin and eosin staining. RESULTS: On frozen section analysis, 33 samples showed absence of ganglion cells, AChE histochemistry showed a positive staining pattern in 17 samples and paraffin embedded routine, H&E stained sections showed absence of ganglion cells in 19 samples. Sensitivity and specificity of both tests ie frozen section rapid H&E/AChE histochemistry in the diagnosis of HD, were calculated taking paraffin embedded H&E stained sections as the gold standard. Sensitivity of frozen section rapid H&E in the diagnosis of HD is 57.57 % and specificity is 79.10 %. The p-value is <0.0001, which is significant. The sensitivity of AChE histochemistry in the diagnosis of HD is 90.47 % and specificity is 96.36 %. The p-value is <0.0001, which is significant. CONCLUSIONS: Acetylcholineesterase (AChE) histochemistry is a very useful ancillary technique in the diagnosis and in aiding the operative procedures of HD. It acts as a double check in the diagnosis of HD.

Posterior urethral valves: Morphological normalization of posterior urethra after fulguration is a significant factor in prognosis.

AIM: To assess the changes in urethral morphology 3 months post fulguration of posterior urethral valves (PUVs) on micturating cystourethrogram (MCUG) and correlate these changes with the overall clinical status of the patient. MATERIALS AND METHODS: A total of 217 children, managed for PUVs during a period of 6 years in a single surgical unit were prospectively studied. The ratio of the diameters of the prostatic and bulbar urethras (PU/BU) was calculated on the pre- and post-fulguration MCUG films. They were categorized into three groups based on the degree of normalization of posterior urethra (post-fulguration PU/BU ratio). GROUP A: Of the 133 patients, 131 had normal urinary stream and 4 (3%) had nocturnal enuresis. Vesicoureteral reflux (VUR), initially seen in 83 units (31% units), regressed completely at a mean duration of 6 months in 41 units (49%). Of the 152 non-VUR, hydroureteronephrosis (HUN) units, 11 were poorly functioning kidneys. Persistent slow but unobstructed drainage was seen in 23 units (16%) over a period of 1.5-5 years (mean 2.5 years). Group B: All the 11 patients had a normal stream. Four (36.4%) had daytime frequency for a mean duration of 1 year and one (9%) had nocturnal enuresis for 1 year. Grade IV-V VUR was seen in five patients (three bilateral), which regressed completely by 3 months in five units (62.5%). In the non-VUR, HUN patients, slow (but unobstructed) drainage was persistent in two units (14%) at 3 years. Group C: Of the 16 patients, only 5 (31.3%) were asymptomatic. Six patients (nine units) had persistent VUR for 6 months to 3 years. Of the 20 units with HUN, 17 (85%) were persistent at 1-4 years (mean 2 years). Eight patients (50%) required a second fulguration while 3 (18.7%) required urethral dilatation for stricture following which all parameters improved. CONCLUSIONS: Adequacy of fulguration should be assessed by a properly performed MCUG. A postop PU/BU ratio >3 SD (1.92) should alert to an incomplete fulguration or stricture. Patients within normal range ratio have faster recovery of slow draining units, reflux and less voiding dysfunction. There is a strong correlation between incomplete fulguration and persistent slow draining units, uremia, voiding dysfunction and urinary tract infections.

Surgical management and outcome analysis of stage III pediatric empyema thoracis.

AIM: Report of 125 pediatric patients of empyema thoracis treated by open decortication, highlighting the presentation, delay in referral, operative findings, the response to surgical intervention and follow-up. MATERIALS AND METHODS: All the children who underwent open decortication for stage III empyema thoracis during the study period were included. Preoperative workup included hemogram, serum protein, chest radiographs and contrast-enhanced computed tomographic (CECT) scan of the chest. RESULTS: One hundred and twenty-five patients (81 males, 44 females) (age 3 months-12 years, mean 4.9 years) were operated during a 4.5-year period. Among them, two children underwent bilateral thoracotomies. Also, 81.6% patients were referred 3 weeks after the onset of disease (mean duration 9 weeks). Intercostal chest drainage (ICD) had been inserted in (119) 95% cases. Thickened pleura, multiloculated pus and lung involvement were invariably seen on CECT scan. Bronchopleural fistula was present in 10 patients and empyema necessitatis in 2. Decortication, removal of necrotic tissue and closure of air leaks was performed in all the patients. Necrotizing pneumonia was seen in (35) 27.5% cases. Mean duration of postoperative ICD was 7 days. Follow-up ranged from 3 months to 4 years (mean 12 months). There was no mortality. Six patients had proven tuberculosis. CONCLUSIONS: The duration of the disease had a direct relationship with the thickness of the pleura and injury to the underlying lung. Delayed referral causes irreversible changes in the lung prolonging recovery. Only 18% presented within the early period of the disease. Meticulous open surgical debridement gives gratifying results. The status of the lung at the end of surgery is a major prognostic factor.

Gastric lipoma.

We report a 12-year-old-boy with gastric lipoma. Upper gastrointestinal (GI) endoscopy with biopsy and abdominal computed tomogram (CT) scan revealed the diagnosis. Open surgical excision of the mass with stomach preservation was done. The clinical presentation and management are discussed and the literature reviewed here. This is the sixth pediatric case reported in the English literature.

Extrahepatic portal hypertension: quality of life and somatic growth after surgery.

AIM: This study was carried out in order to determine whether children with extrahepatic portal hypertension show any improvement in growth parameters and quality of life after elective surgery. PATIENTS AND METHODS: All patients with extrahepatic portal hypertension who underwent surgery between April 1999 and March 2002 were studied prospectively. Height and weight before and after surgery were converted into Z scores for comparison. The quality of life was assessed by changes in scholastic ability, physical activity, social interaction, and economic effects on the family. RESULTS: At the end of a minimum follow-up period of 1 year, of the 30 children, 50 % and 76 % showed improvement in weight and height Z scores, respectively, compared to their scores while on medical management. Z scores below - 2 SD for weight and height were seen in 57 % and 37 % of patients prior to surgery, and only in 40 % and 20 %, respectively at the end of follow-up, which ranged from 1 - 4 years. The improvement in height and weight was more in those who had undergone splenectomy with either devascularization or central splenorenal shunt compared to those who had undergone side-to-side lienorenal shunt without splenectomy. The children, who had dropped out of school while on medical management, either rejoined school or attended vocational courses after surgery. There was an improvement in school performance and physical activity in 85 % of the children. Improvement in personality was seen in 73 % of the affected patients. The cost incurred for surgery was one-third of the amount spent while on medical management. CONCLUSIONS: Growth parameters improved significantly in children after surgical intervention for portal hypertension. Overall improvement in scholastic abilities, physical activity, and social interaction was noted in a majority of the patients. As a one-time procedure in a developing country, surgery is also more cost effective.

Case report: bilateral multilocular cystic nephromas: a rare occurrence.

We present a rare case of bilateral renal cystic tumours in an 18-month-old female child, which proved to be cystic nephromas on imaging and histopathology. Although unilateral cystic nephromas are known in children; the occurrence of bilateral tumours is rare.

Primary vesicoureteral reflux: progress of disease, somatic growth and renal parameters.

Thirty children (45 units) in the age group 1 day-12 yrs with primary vesicoureteral reflux were studied prospectively and periodically assessed for renal function, scarring, grade of reflux and somatic growth parameters. Four children (6 units) with grade IV reflux underwent ureteric reimplantation. Complete resolution with medical management was seen in all 12 units of grade I-III reflux and in 5 of the remaining 27 units of grade IV-V reflux over 6 months-6 yrs. This group showed highly significant improvement in height . The weight gain of the above 2 groups was statistically significant compared to those with persistent reflux. Focal defects were seen initially in 62 percent refluxing units. None of the patients showed deterioration in renal function or formation of new scars over the next 1-7 years. Three children on conservative management showed persistent growth retardation with associated breakthrough infection, hypertension, multiple renal scars and poor renal functional volume.

Extrahepatic portal hypertension in children: observations on three surgical procedures.

This paper presents a comparative prospective study of three modalities of surgical treatment for extrahepatic portal hypertension in children: central splenorenal shunt after splenectomy (CSS), side-to-side lienorenal shunt (SSLR) without splenectomy, and splenectomy and gastroesophageal devascularization (SGD). In an 18-month period, 27 procedures were performed: 10 CSS, 10 SSLR, and seven SGD. The outcomes were evaluated by fall in portal pressures, hematological parameters, shunt patency, splenic regression, and disappearance of esophageal varices. All three procedures were comparable in the fall of portal pressure after surgery. The average blood loss and operating time were statistically significant in favor of SSLR compared with CSS. At 3-month follow-up, shunt patency was confirmed by duplex Doppler study in all the patients in the SSLR group and in nine out of 10 patients in the CSS group. In the CSS and SGD groups, hypersplenism resolved in all the patients. In the SSLR group, blood counts improved in only five out of eight affected children. No patient re-bled during a follow-up of 3-5 years. There were no cases of hepatic encephalopathy or overwhelming postsplenectomy sepsis. In conclusion, CSS is useful when there is a large spleen, severe hypersplenism, and a shuntable splenic vein. SSLR is suitable when there is only mild splenomegaly, mild hypersplenism, and a shuntable splenic vein. Splenectomy and devascularization is the choice when there is no shuntable splenic vein.

Risk factors in the primary management of anorectal malformations in Northern India.

The purpose of this study was to determine the major factors influencing the survival of babies with anorectal malformation (ARM) during the primary management in the neonatal period in a tertiary referral center. The outcome of 125 neonates with ARM, treated in a single hospital during a 2-year period, was analyzed retrospectively with particular reference to birth weight, time of arrival, and the type of ARM, including pouch colon and major associated anomalies, to see whether any of them had a significant effect on initial survival. High ARM (HARM) was present in 75, low ARM (LARM) in 36, and pouch colon in 14 babies. There were a total of 28 deaths (22%). In babies with isolated HARM weighing >2.5 kg and brought to the hospital within the first 48 h of life, the survival was 100%, which dropped to 80% when brought later than 48 h. Of the babies with birth weight <2.5 kg and isolated HARM but who were brought to the hospital within the first 48 h of life, 80% survived, whereas of those reaching the hospital after 48 h, only 55% survived. Two babies with pouch colon and two of the four babies with LARM and associated anomalies died. In the HARM group, of the 31 babies with major associated anomalies, only 37% with birth weight <2.5 kg and 58% with birth weight >2.5 kg survived. Low birth weight, major associated anomalies, and delay in referral were the three important factors that influenced the outcomes of babies with ARM during the neonatal period.

Oesophageal atresia and biliary atresia: a rare association.

Oesophageal atresia is known to be associated with cardiovascular, gastrointestinal, genitourinary and skeletal anomalies. Its association with biliary atresia is very rare and only a few cases have been reported in the literature. Although biliary atresia could be part of a multiple anomaly syndrome, its isolated occurrence is even rare. We present a case of oesophageal atresia associated with biliary atresia.

An audit of neonatal colostomy for high anorectal malformation: the developing world perspective.

A high divided sigmoid colostomy has been recommended for staged management of high anorectal malformation. We audited our cases of neonatal colostomy for high anorectal malformation to assess its effectiveness. A retrospective study was carried out of all surgical newborns admitted with high imperforate anus as the single diagnosis at our centre between December 1998 and December 2000. Morbidity and mortality were analysed after retrospective stratification into two groups (group A: birth weight >2.5 kg; group B: birth weight <2.5 kg). The chi square test was used to test the statistical significance in terms of outcome in the two groups. Overall mortality was 16%. Group A consisted of 34 babies: 30 with divided sigmoid colostomy and four with transverse loop colostomy. One baby with a divided sigmoid colostomy died from wound complications and septicaemia (mortality 2.9%). All four babies with transverse loop colostomy done under local anaesthesia survived, despite being sick on arrival. Group B consisted of 16 babies: 15 with sigmoid colostomy and one with transverse loop colostomy, with seven deaths (44%). None of the five babies with transverse loop colostomy done under local anaesthesia died, despite being sick on arrival, whereas all eight babies who died had undergone sigmoid colostomy under general anaesthesia. The difference in the outcomes of babies in groups A and B is highly significant ( p <.01). Sick, small (<2.5 kg) and septic babies arriving late to the unit do not appear to tolerate general anaesthesia and divided sigmoid colostomy well, despite that procedure's long-term advantages. Divided sigmoid colostomy has produced excellent results in babies >2.5 kg, but in the context of the developing world and limited critical care availability, transverse loop colostomy under local anaesthesia may save lives.

Fundal tube esophagoplasty for esophageal reconstruction in atresia.

PURPOSE: This is a report of 4 patients with long gap/pure esophageal atresia, who, after an initial gastrostomy and cervical esophagostomy at birth, were treated with a new technique called fundal tube esophagoplasty. METHODS: The technique consisted of mobilization of the distal esophageal stump, division of the left gastric artery, and mobilization of upper half of stomach. Both walls of the stomach were incised in the region of the fundus starting on the lesser curvature in such a way that a tube was created out of the fundus, and the native esophageal stump appears to be an extension of this neoesophagus. This neoesophagus was brought to the neck by the retrosternal route and in a second stage, esophago-esophageal anastomosis was made. Thal's fundoplication was added in 2 cases. RESULTS: All the stages have been completed successfully in 4 babies. They have been followed up for periods ranging from 8 to 24 months after restoration of esophageal continuity. All the children are thriving. CONCLUSIONS: Fundal tube esophagoplasty (1) serves to provide a neoesophagus of natural caliber, (2) utilizes native esophagus in reconstruction, (3) does not produce a space-occupying problem in the mediastinum, and (4) preserves the gastric reservoir function. In this early experience, it appears to fulfill all the requirements of a good esophageal substitute.

Pelviureteric junction obstruction: how much is the extent of the upper ureter with defective innervation needing resection?

BACKGROUND/PURPOSE: It is well realized that Anderson-Hyne's pyeloplasty fails to improve the drainage pattern and renal functional parameters in a few cases of hydronephrosis because of pelviureteric junction (PUJ) obstruction. The purpose of this study is to delineate the lower extent of the abnormally innervated pelviureteric junction by S-100 immunohistochemistry, which requires surgical removal. METHODS: Thirty pelviureteric junction (PUJ) specimens were analyzed by histology and by S-100 immunohistochemistry after serial sectioning of the resected ureter and compared with the variables age, calyceal separation, parenchymal thickness, differential renal function, glomerular filtration rate (GFR), and the measured constricted ureteral segment on gross examination. RESULTS: No significant correlation was noted (P >.05) when the histology was analyzed to find any association with any of the variables studied. The length of the visible constricted segment ranged from 2 mm to 15 mm (mean, 5.37 mm). The abnormally innervated segment was much longer than the length of the visible constricted segment in 24 and of the same length in 5 specimens. However, in 1 case, the abnormal innervation segment was shorter than the macroscopic constriction by 3 mm. The maximum difference in length between the visible constriction and the lower limit of defective innervation was 8 mm. CONCLUSIONS: The abnormally innervated ureteral segment below the PUJ in hydronephrosis is longer than the visible constricted segment at the time of surgery, and the ureter should be excised at least 8 mm or more beyond the visible lower limit of the constricted segment in cases of Anderson-Hyne's pyeloplasty.

Congenital diaphragmatic hernia associated with aplasia of left lobe of the liver.

A case of left-sided diaphragmatic hernia associated with congenital aplasia/agenesis of the left lobe (left lateral segment) of the liver is reported. This association of defects, to the best of the authors' knowledge, has not been reported previously.

Adrenocortical tumors in childhood.

Adrenocortical tumors (ACT) are rare in childhood. Determination of malignant potential is difficult. To assess the presentation, clinical behaviour, and histology of these tumors in our center, the records of nine patients with ACTs between 1989 and 2000 were analyzed. The age, sex, clinical presentation, investigations, treatment, follow-up data, and current status were determined. The pathological features were reanalyzed by our pathologist. The endocrine profile of the tumors was also retrieved from the records. There were two males and seven females; the median age at referral was 2.5 years. Two patients (one male, one female) presented with clinical features of Cushing's syndrome with hypertension. Four girls had features of Cushing's syndrome with associated virilization. One 11-year-old girl presented with hypertension, hypokalemia, and pseudoparalysis due to an aldosterone-secreting tumor. One girl presented with precocious pubic hair and clitoromegaly. One male had evidence of true precocious puberty. The endocrine profile was available in eight cases: cortisol levels were raised in four, testosterone in four, and aldosterone in one. One boy had pubertal levels of follicle-stimulating hormone and testosterone. Surgery was the only modality of therapy used. Histopathology showed capsular invasion in four patients and vascular invasion in two. The median tumor weight was 80 g (15-550 g) and the size ranged from 1.5 x 1.5 to 12 x 12 cm. Five patients are alive without tumor recurrence at a median follow-up time of 2 years. Tumor recurrence was seen in two patients, one with capsular rupture during surgery and another with a tumor weight of 16 g with associated capsular and vascular invasion. Atypical modes of presentation like pseudoparalysis must be kept in mind. Histologic criteria for malignancy in ACT are unreliable. Tumor weight may not always be accurate in predicting tumor behaviour. Complete surgical excision remains the only effective and potentially curative treatment.

Recurrent tracheoesophageal fistula after foreign body impaction.

We describe a case of recurrent tracheoesophageal fistula in a child previously operated for esophageal atresia and tracheoesophageal fistula following impaction of a foreign body. After extracting the foreign body, the diagnosis of recurrence was confirmed by esophagogram and bronchoscopy. The fistula was approached through transpleural thoracotomy and repair effected without significant difficulty. The possibility of unrecognized foreign body impactions as the etiological factor for late recurrence of tracheoesophageal fistula is discussed.

Infected esophageal duplication cyst simulating empyema.

Duplications of the esophagus are the second most common duplication of the gastrointestinal tract. The children with esophageal duplication cyst usually present with respiratory distress or as asymptomatic thoracic mass found on incidental chest x-ray. We report a case of infected esophageal duplication cyst initially confused with empyema in a two years old boy.

Prospective analysis of renal function and somatic growth in neonatal posterior urethral valves.

AIM: The present prospective study focuses on the effect of transurethral fulguration (TUF) and vesicostomy on the renal functions and somatic growth in posterior urethral valves (PUV). PATIENTS AND METHODS: 37 consecutive neonates were diagnosed and treated for PUV with vesicostomy (18 patients) and TUF (19 patients). Postoperative stable creatinine values, renal function and somatic growth were recorded. Standard anthropometric techniques and standard statistical methods were used to compute distance statistics for body weight and crown-heel length at age intervals of 3 months for the first year. OBSERVATIONS: The distance values of body weight and crown-heel length of these groups of children were less than the 5th percentile of the National Centre For Health Statistics (NCHS) and healthy Punjabi infants showing growth retardation in the first year of life. Pre- and postoperative mean serum creatinine of the fulguration group was 1.5 +/- 1.5 mg/dl and 0.8 +/- 0.3 mg/dl and for vesicostomy it was 2.4 +/- 2.2 mg/dl, and 0.9 +/- 0.7 mg/dl respectively. The babies of the fulguration group showed better growth attainments than the babies of the vesicostomy group at the end of one year. CONCLUSION: At the end of one year, babies of the fulguration group had a similar growth velocity compared to the vesicostomy group, though in general they showed retarded growth compared to healthy counterparts. The modality of treatment chosen did not seem to affect the renal functions or somatic growth in the short-term follow-up.