Single piece artificial urinary sphincter for secondary incontinence following successful repair of post traumatic urethral injury.

Post traumatic urethral injury is uncommon in children. The management of this condition is dependent on the severity of injury. Initial suprapubic cystostomy with delayed repair is the conventional treatment. Successful reconstruction of urethral injury may be followed by urethral stricture, incontinence, impotence, and retrograde ejaculation. Successful repair of post traumatic urethral injury followed by secondary incontinence in children has not been well addressed in literature. We report the management of one such child, with satisfactory outcome with implantation of a new model of single piece artificial urinary sphincter in the bulbar urethra by perineal approach.

Minimal access surgery in children:a 5 year study.

Over a period of 5 years, we analyzed our data on outcome, feasibility, and safety of Minimal Access Surgery (MAS) in 211 children. The outcome was compared objectively with age matched controls with similar diagnosis undergoing open surgery over the same period. There was no significant difference between mortality, morbidity, re-exploration rates and analgesic requirement between MAS and open surgery. There was a significant difference in the length of stay in hospital, in favour of laparoscopic cholecystectomy, appendectomy, nephrectomy, splenectomy, surgery for intra-abdominal testis compared to open surgery but not for children undergoing surgery for appendicular perforation and intestinal pathology. All parents preferred the cosmetic outcome of minimal access surgery.

Nutritional assessment of patients under hemodialysis in nepal medical college teaching hospital.

Malnutrition is very common in hemodialysis patients and is predisposed by many factors. Malnutrition is associated with increased morbidity and mortality. Total of 26 patients (16 males and 10 females) who were under hemodialysis in our center were included in the study. With the help of Malnutrition Score (MS) developed by Kalanter-Zadeh, nutritional status of the patients was assessed. Patients also underwent different anthropometric measurements such as Body Mass Index (BMI), Triceps skin fold thickness (TSF), Mid Arm Circumlference (MAC) and Mid Arm Muscle Circumference (MAMC) and laboratory investigations. Mean age of the study population was 42.58 +/- 16.32 years (range 19 to 74 years). Females were older than males. MS of the study population was 15.82 +/- 3.76 (range 9-24). Female patients were having higher MS than males (16.5 +/- 4.11 vs. 15.06 +/- 3.55). Based on MS, 22 patients (84.6%) had mild to moderate malnutrition, 2 (7.7%) patients were having severe malnutrition and remaining 2 (7.7%) had normal nutrition score. Females were having lower BMI, MAC and MAMC but higher value of TSF. Significant negative correlation was present between MS and weight, BMI, MAC and MAMC. Calculated Urea Reduction Ratio (URR) of study population was 57.27 +/- 10.89. URR was higher in females than in males (61.77 +/- 12.74 vs. 54.45 +/- 8.85). Only 23.0% of the study population had URR of >65.0%. Protein Catabolic Rate (nPCR) in our study was 0.77 +/- 0.28 g/kg/day. Malnutrition is very common in our center which is >90% when MS was considered. In our study it negatively correlated with weight, BMI, MAC and MAMC. Dialysis inadequacy was present in around 75.0% of our study population.

Factors affecting renal scarring in posterior urethral valves.

AIM: To retrospectively review the occurrence of renal scarring in patients with posterior urethral valves (PUV), and correlate it with various causative factors. METHODS: The records of 52 patients treated for PUV by the authors were reviewed. Patients with vesico-ureteric reflex (VUR) dysplasia syndrome were excluded from the study. The patients were divided into group I (no renal scarring, n=18) and group II (renal scars present, n=34) based on dimercapto-succinic acid scans. The mode of treatment, presence/absence of breakthrough urinary tract infections (UTI), presence/absence of history of nocturnal/diurnal incontinence, presence/absence of VUR, stable postoperative serum creatinine, rate of drainage of ureters on diethylene triamine penta acetic acid (DTPA) scans and management of bladder dysfunction, if any, were noted. The presence or absence of renal scarring was statistically correlated with occurrence of any of the above factors. RESULTS: Primary valve fulguration was performed in 41 patients and 11 patients had an initial vesicostomy. The median follow up was 3.5 years (range 1.5-15 years). Renal scarring was present in either kidney in 34 patients (bilateral 14, unilateral 20). The mode of initial treatment did not affect the incidence or rate of scarring. The preoperative and postoperative serum creatinine at the end of 1 year did not differ between the two groups. Presence/severity of VUR did not affect the pattern of renal scarring. A highly significant correlation between the occurrence of renal scarring and presence of diurnal incontinence (P< or =0.007, odds ratio=4.5) and breakthrough UTI (P< or =0.002, odds ratio=7.0) was observed. There was also correlation with slow drainage in the ureters on a DTPA scan (P< or =0.0005). Detrusor instability and low compliance on urodynamic assessment did not affect occurrence in the limited number of patients studied. The rate of somatic growth in both groups was retarded as compared to normal healthy counterparts. CONCLUSION: Breakthrough UTI, diurnal incontinence and poor drainage of ureters on DTPA are associated with a higher incidence of renal scarring. Mode of initial treatment, presence or absence of VUR, and bladder abnormalities do not affect renal scarring in the short term.

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.

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.

Preoperative angioembolisation for life-threatening haemorrhage from Wilms' tumour: a case report.

Wilms' tumour is one of the most common abdominal tumours of childhood. Severe perirenal bleeding resulting in consumptive coagulopathy and colonic obstruction are rare complications of Wilms' tumour. We present a case report of one patient with these two complications, their successful management with preoperative angioembolisation and emergency nephrectomy, and a review of the relevant literature.

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.

Undifferentiated embryonal sarcoma in children: beware of the solitary liver cyst.

Undifferentiated embryonal sarcoma (UES) of liver is a rare form of liver tumor in children. It may have an atypical presentation leading to delay in diagnosis. The authors report on 3 consecutive children with this condition, each of them presenting in older children who presented with this condition and an initial diagnostic dilemma. One of them presented initially with a solitary cyst, the second with severe abdominal pain, and the third with intermittent fever. Preoperative chemotherapy was successful in shrinking the tumors in 2 children to allow anatomic liver resection with tumor-free margin. Both remain free of recurrence 2 years after completing treatment.

Urinary tract infection: the urological perspective.

Urinary tract infection in babies often presents with non specific symptoms and signs. It must be considered in the differential diagnosis of a febrile sick baby or any baby with failure to thrive. In a significant proportion of babies with recurrent urinary tract infection, urological abnormality is demonstrable. The diagnosis of urinary tract infection is confirmed by the presence of pus cells and growth of microorganism in a fresh urine specimen. The voidoing history and detailed ultrasound examination in the baby including a post void evaluation of the upper and lower urinary tract can lead to the possible diagnosis in the majority. Antibiotic therapy for urinary tract infection should be followed by consideration about the need for urinary drainage at the appropriate level, particularly in cases where resolution is delayed despite antibiotics. There is a role for prophylactic antibiotics after the resolution of acute infection, at least until detailed evaluation several weeks after the acute episode, has excluded any abnormality. Micturating cytourethrogram is usually done under antibiotic cover and better done in centres with facility for fluoroscopic examination. Isotope studies have to be evaluated in the light of inherent limitations. Transient urodynamic abnormality of the urinary bladder in infancy is being increasingly recognized and should be the subject of evaluation in specific circumstances. A multidisciplinary team improves the quality of investigations, subsequent interpretation and long term care of these children. A large majority of urological abnormality in infancy and early childhood can be effectively managed by endocopic procedures. Temporary urinary diversion may be required in a small proportion. Urological reconstruction should be approached with caution, after a detailed analysis of the evolving urological tract in a baby. An aggressive approach to early diagnosis and appropriate treatment of urinary tract infection and any underlying abnormality, has been shown to significantly reduce the incidence of chronic renal failure in long term longitudinal studies in children.

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.

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.

Anorectal malformation with malrotation of gut.

Infants with anorectal anomaly have a high risk of having other congenital anomalies, but associated gastrointestinal tract anomalies are quite rare. Malrotation of gut is rarely associated with anorectal anomaly. We report two such cases of anorectal malformation with malrotation of gut. The high index of suspicion, diagnostic difficulty and surgical management with avoidance of appendicectomy in these neonates is discussed.

Testosterone therapy in microphallic hypospadias: topical or parenteral?

BACKGROUND/PURPOSE: Local or systemic application of testosterone is reported to stimulate penile growth. Intramuscular testosterone has been found to be effective in 50% of patients; however, variable results have been reported with topical testosterone. The current study is an attempt to compare the efficacy of intramuscular versus topical testosterone application. METHODS: A total of 26 consecutive patients with hypospadias and small penis (<2SD for given age) were studied prospectively. These patients were recruited alternately into group A or group B. Each group consisted of 13 patients. In group A, penile growth was accomplished by topical application of testosterone (Testoviron, oily solution containing testosterone propionate, 25 mg, and testosterone enanthate, 110 mg, equivalent to about 100 mg of testosterone, Schering, Germany) with a dose of 2 mg/kg/wk, for 3 weeks. While in group B, testosterone (same preparation as above) was administered by intramuscular injection weekly for 3 consecutive weeks. Penile length, diameter, and secondary effects were recorded before, during, and 3 weeks after the therapy by a single observer. RESULTS: Significant penile growth (P <.01) was noticed in both the groups of patients when compared with pretherapy with maximum response observed during the third week of therapy (reaching from an average pretherapy length of 2.0 cm and 1.8 cm to 3.18 cm and 3.11 cm posttherapy in group A and B patients, respectively). Seven patients in each group had growth of at least 50% compared with the initial size. The basal serum testosterone was within the normal range in both the groups. During therapy the serum testosterone was elevated above the basal level in all patients, but within the normal range except in 2 patients of group A. In these 2 children the serum testosterone level crossed the normal range. Linear growth did not alter significantly for the chronological age. Two patients of group A went on to have pubic hair, one of them had elevated testosterone level above the normal range. There was a surge in serum testosterone in all children, although significant penile enlargement was observed in 60% children in group A and 75% in group B. CONCLUSIONS: Although the desired therapeutic effect of testosterone was achieved in both the groups, this study failed to show any significant difference between the 2 routes of administration. However, in group A, (topical) serum testosterone crossed the normal range in 15% of patients and was associated with significant reversible side effects.

Choledochal cyst associated with portal hypertension.

Portal hypertension is a known complication of delayed presentation of choledochal cyst. In the literature, choledochal cyst presenting with portal hypertension has not been addressed adequately. The aetiology of portal hypertension in these cases has not been well studied, but it may be related to compression of the cyst over the portal vein, secondary biliary cirrhosis, or even extrahepatic portal venous thrombosis. We present our experience with four cases of choledochal cysts with associated portal hypertension over a 10-year period (1991-2001). Gastrointestinal bleeding, splenomegaly or incidental discovery of oesophageal varices led to the diagnosis of associated portal hypertension. Excision of choledochal cyst and bilioenteric bypass may not be feasible in those patients with massive collaterals in the region of porta with secondary portal hypertension. An initial approach of internal drainage (endoscopic/operative) of the cyst may allow regression of collaterals and subsequent safe excision of the cyst in this difficult group of patients. The liver function and histopathology dictated the final outcome.

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.

Crohn's disease: disastrous consequences of late diagnosis.

Chronic and recurrent perianal abscess is an uncommon condition in children. Tuberculosis is thought to be the common etiology for such a presentation in India. We report a case of a child with colonic and perianal disease due to Crohn's disease and emphasize the disastrous complication due to delayed diagnosis.