Role of urodynamics in male patients of high-anorectal malformations: a prospective study.

PURPOSE: Association of spinal or vertebral anomalies and the iatrogenic denervation during surgical correction of anorectal malformation patients especially in boys can lead to neurogenic bladder inthese subset of patients. The paucity of literature with regard to urodynamic studies focusing exclusively in male children with high-anorectal malformations (HARM) lead us to analyze the urodynamic changes. The objective was to study urodynamic profile in male patients who have undergone surgery for anorectal malformation. METHODS: Male high-anorectal malformation patients who had completed all the stages of repair were prospective studied. Following the basic work up, all patients based on the urodynamics were categorized into 2 groups as safe or unsafe bladders. Unsafe bladder was defined as detrusor pressure > 40 cm (high detrusor pressure) or pressure variability of 15 cm of water (detrusor overactivity) or significant post-void residue. MRI was limited to patients with only abnormal urodynamics to rule out spinal causes of neurogenic bladder and due to financial constraints, it could not be offered to all patients. RESULTS: 41 HARM meet the exclusion criteria. All patients were asymptomatic with none having history of urinary tract infections. Ultrasound showed bladder wall thickening in 31.7% patients. UDS revealed reduction in bladder capacity and compliance was noted in 31.7% and 30% patients, respectively. Elevated detrusor pressures (> 40 cm of water) were noted in 10% (4/41), detrusor overactivity with concomitant elevated detrusor pressures in 19.5% (8/41) and normal UDS in 70% (29/41). 13 patients (31.7%) had abnormal cystometric parameters with 12(30%) having unsafe bladders. MRI confirmed sacral agenesis in 1 patient with unsafe bladder. CONCLUSION: Urodynamics can demonstrate occult neurovesical dysfunction in patients with HARM. This would help in early renal protective therapy and prevent the burden of long-term sequelae of neurovesical dysfunction in HARM patients.

Outcome Analysis of Patients of Congenital Hydrocephalus with Ventriculoperitoneal Shunt at a Tertiary Care Hospital in North India.

INTRODUCTION: Ventriculoperitoneal (VP) shunt is a commonly performed neurosurgical procedure in pediatric surgical practice. This study is aimed at determining the outcome of congenital hydrocephalus patients who underwent shunt surgery at our center. MATERIALS AND METHODS: A total of 278 operated cases of congenital hydrocephalus with a minimum follow-up of 6 months were analyzed in terms of complications. Hydrocephalus associated with intracranial hemorrhage, neural tube defects, and ventriculitis were excluded from the study. RESULTS: Complications were observed in 32.0% (89/278) of cases. Shunt block and chamber migration were the most common complications (29.21%). Abdominal wound complications were seen in 4.49% of cases. Shunt exposure due to erosion of overlying skin was noted in 6 patients. Shunt infection was diagnosed in only 9 patients (4.4%). New-onset seizures were seen in 4 cases. Overall, 21% of patients required redo shunt and there were 2 mortalities. CONCLUSION: VP shunt continues to have high complication rates. Shunt migration and new-onset seizure disorders are important late complications. In spite of inherent problems, shunt surgery is the procedure of choice until a safer effective alternative is available.

Double Encephalocele: An Unusual Presentation.

The occurrence of multiple neural tube defects in a single patient is rare; the cases reported in the past are mostly of thoracic and lumbosacral region. Double occipital encephaloceles are rare; only four cases have been reported till date. Here we report a case of double encephalocele-one in the vertex and the other in the occiput, and a literature study on similar reports.

Abdominoperineal Pull-through with Simultaneous Extravesical Detrussoraphy: An Alternative Surgical Technique for Congenital Pouch Colon with High-grade Vesicoureteric Reflux.

Congenital pouch colon (CPC) is frequently associated with vesicoureteric reflux (VUR). These patients require long-term antibiotic prophylaxis and/or an additional surgical intervention for the management of the refluxing system. We propose a single-stage alternative approach in these patients. Two patients diagnosed to have CPC underwent pouch excision and an end colostomy at birth. Further evaluation revealed high-grade reflux in both the patients. At 6 months of age, definitive abdominoperineal pull-through (APPT) surgery along with extravesical detrusorrhaphy was performed. In the follow-up at 1 year, they are thriving well with no urinary complaints. Micturating cystourethrogram revealed complete resolution of VUR. This approach takes the advantage of the anesthesia for APPT and offers a relatively simple and quick solution for the refluxing system, thus, enabling the stoppage of antibiotic prophylaxis and obviating the need for a future endoscopy/surgery.

Health-related Quality of Life in Children with Congenital Hydrocephalus and the Parental Concern: An Analysis in a Developing Nation.

PURPOSE: To analyze quality of life of children operated for congenital hydrocephalus and the concern of parents in taking care of these children. METHODS: Thirty patients who underwent ventriculo-peritoneal shunt were randomly selected with minimum gap of 1 year between surgery and study. Canadian validated questionnaire was used. Overall health score (OHS) and parental concern score (PCS) were correlated with gender, family type and number of surgeries. RESULTS: Mean OHS was 159.43 which was summation of physical health (mean 45.76), social-emotional (mean 80.03) and cognitive health scores (mean 33.66). Mean OHS was 151.57 for males and 177.77 for females (p-value 0.233). Nuclear and joint families had mean OHS of 160.36 and 158.89 respectively (p-value 0.944). Those who underwent one surgery had mean OHS of 167.48 and PCS of 23.10 whereas mean OHS was 140.66 and PCS was 27.78 for those with multiple procedures. Mean PCS for males was 26.71 and for females was 19.33 (p-value 0.036 statistically significant). This was not statistically significant between nuclear (24.73) and joint families (24.26). CONCLUSIONS: Quality of life of survivors of hydrocephalus is reasonably good even in developing countries due to keen parental involvement irrespective of gender, family type and number of surgeries.

Is urodynamic evaluation a must in all operated cases of open neural tube defects.

BACKGROUND: Spina bifida is a common cause of pediatric neurogenic bladder. It causes renal failure in almost 100% of patients if the associated detrusor sphincter dyssynergia (DSD) is inadequately managed. Detrusor instability and high detrusor pressures (Figure) have been implicated as the major factors predictive of renal damage in these patients. Urodynamic studies provide early identification of "at risk" kidneys so that appropriate intervention can be made. However, the role in post-operative patients of spina bifida who have no clinical manifestations remains unclear. OBJECTIVE: To study the bladder dynamics in asymptomatic post-operative patients of spina bifida and to determine whether routine urodynamic study is justifiable. MATERIAL AND METHODS: Urodynamics was performed on 15 operated patients of spina bifida who did not have any neurological deficit and were asymptomatic. RESULTS: The mean age of the patients was 4.97 years. None of the patients had any urological complaints with their ultrasonography being normal. None had scars on nuclear scan. Of the 15 patients, 12 (80%) had abnormal findings on urodynamic assessment. Three patients (20%) had detrusor pressures greater than 40 cm of H2O. One patient had significant residual urine and detrusor instability. DISCUSSION: The use of urodynamic studies in asymptomatic patients of spina bifida remains controversial, with one school of thought advocating early invasive urodynamic testing. In contrast, some favor noninvasive sonological monitoring, reserving invasive tests only for patients with renal tract dilatation. In our subset of patients none had renal tract dilatation but three patients (20%) had "at risk" bladders. These patients would benefit from early intervention aimed at renal preservation. The study is limited by a small sample size because of the relative rarity of the patient profile included. A further multicenter study with a case-control design could conclusively indicate the role of urodynamic testing in these patients. CONCLUSION: Patients of spina bifida, even when asymptomatic, have a high incidence of unsafe bladders. Early identification and appropriate measures can protect kidneys from long-term damage, hence urodynamic profiling is mandatory for identification of potentially high-risk bladders.

Role of gabapentin and anticholinergics in management of neurogenic bladder after repair of spina bifida - a randomized controlled study.

BACKGROUND: Anticholinergics are well established in the management of neurogenic bladders. However, some patients do have sub-optimal response or severe side effects. This study is designed to assess and compare efficacy of gabapentin with oxybutynin in neurogenic bladders after surgery for spina bifida. METHODS: Patients were randomized into three groups after urodynamic studies and started on oxybutynin, gabapentin, and combination of both, respectively. Thorough clinical and urodynamic reassessment was done at 6months and one year after starting treatment. RESULTS: Forty-four patients (3-19years) were studied. Improvement was noted in symptoms as well as urodynamic parameters in all groups. Maximal improvement of symptom score was with combination of drugs at 1year. In urodynamic studies, compliance, pressures, and capacity showed improvement, which was significant between the groups at both six months and 1year for bladder pressures and volume. Improvement in compliance though marked was not statistically significant. Best response was seen in group receiving both drugs. Gabapentin was better tolerated than oxybutynin. CONCLUSION: Gabapentin is a good alternative to oxybutynin for management of neurogenic bladder, both as monotherapy and as an add-on therapy. It has potential application in patients with inadequate response to anticholinergics. LEVEL OF EVIDENCE: prospective competitive treatment study - level II.

Emergent repair of bronchial transection: both right and left main bronchial lung ventilation at surgery makes it easy for all.

Maintaining adequate gas exchange is crucial during surgical repair of tracheobronchial injuries in children and reflects the skills of the anaesthetist and the surgeon. We present a 7-year-old girl who underwent urgent repair of the bronchial transection and novel use of ventilation of the transected lung helped accomplish the repair successfully.

Long Upper Pouch in Esophageal Atresia: A Rare Variant.

The earliest clinical sign of esophageal atresia (EA) is excessive salivation and the diagnosis is made by failure to pass an infant feeding tube (IFT) into the stomach. The diagnostic errors may occur due to presence of an unusually long upper pouch, when the IFT seems to pass into the stomach. We describe one such case and review the relevant literature.