Management of non-functioning kidney due to pelvi-ureteric junction obstruction in pediatric age group: an observational study.

INTRODUCTION: A non-functional kidney (NFK) has been defined as one having paper-thin parenchyma, and split renal function (SRF) of < 10% on a nuclear scan. There are differences of opinion about nephrectomy or pyeloplasty in these patients. The present study was conducted to assess our management strategy of renal salvage for NFK. MATERIALS AND METHODS: It was a retrospective cohort study from January 2015 to July 2022, patients having SRF < 10% were included. These patients underwent ultrasound-guided percutaneous nephrostomy (PCN). A repeat nuclear scan was performed after 3 months. If SRF increased to > 10%, pyeloplasty was performed. RESULTS: Fifteen patients were managed. The mean age was 24.67 ± 23.61 months. Male to female ratio was 4:1. The initial mean SRF was 6.67 ± 2.85, which improved to 16.80 ± 4.69 after 3 months of placing the PCN (p < 0.001). The corresponding changes in the mean effective renal plasma flow (ERPF) were 60.13 ± 24.08 to 106.53 ± 24.61 (p < 0.001). There was no complaint after the placement of PCN. All patients underwent dismembered pyeloplasty. CONCLUSION: In NFK due to PUJO, expectant treatment in form of PCN followed by pyeloplasty appears to be the primary treatment modality, and nephrectomy may not be needed in any of them.

Differential expression profiling of onco and tumor-suppressor genes from major-signaling pathways in Wilms' tumor.

PURPOSE: Wilms' tumor is the most-frequent malignant-kidney tumor in children under 3-4 years of age and is caused by genetic alterations of oncogenes (OG) and tumor-suppressor genes (TG). Wilms' tumor has been linked to many OG-&-TG. However, only WT1 has a proven role in the development of this embryonic-tumor. METHODS: The study investigates the level of mRNA expression of 16 OGs and 20 TGs involved in key-signaling pathways, including chromatin modification; RAS; APC; Cell Cycle/Apoptosis; Transcriptional Regulation; PI3K; NOTCH-&-HH; PI3K & RAS of 24-fresh Wilms'-tumor cases by capture-and-reporter probe Code-Sets chemistry, as CNVs in these pathway genes have been reported. RESULTS: Upon extensively investigating, MEN1, MLL2, MLL3, PBRM1, PRDM1, SMARCB1, SETD2, WT1, PTPN11, KRAS, HRAS, NF1, APC, RB1, FUBP1, BCOR, U2AF1, PIK3CA, PTEN, EBXW7, SMO, ALK, CBL, EP300-and-GATA1 were found to be significantly up-regulated in 58.34, 62.5, 79.17, 91.67, 58, 66.66,54, 58.34, 66.67, 75, 62.5, 62.5, 58, 79.17, 79.17, 75, 70.84, 50, 50, 75, 66.66, 62.50, 61.66, 58.34-and-62.50% of cases respectively, whereas BRAF, NF2, CDH1, BCL2, FGFR3, ERBB2, MET, RET, EGFR-and-GATA2 were significantly down regulated in 58, 87.50, 79.16, 54.16, 79.17, 91.66, 66.66, 58.33, 91.66-and-62.50% of cases, respectively. Interestingly, the WT1 gene was five-fold down regulated in 41.66% of cases only. CONCLUSION: Hence, extensive profiling of OGs and TGs association of major-signaling pathways in Wilms' tumor cases may aid in disease diagnosis. PBRM1 (up-regulated in 91.67% of cases), ERBB2 and EGFR (down-regulated in 91.66 and 91.66% of cases, respectively) could be marker genes. However, validation of all relevant results in a larger number of samples is required.

Evaluation and management of "low" anorectal malformation in male children: an observational study.

PURPOSE: ARM with perineal fistula has been traditionally defined as low ARM (LARM). This study was conducted to evaluate LARM in male patients with an emphasis on the role of various factors on the outcome and follow-up of them. MATERIALS AND METHODS: It was a retrospective cohort study. The clinical presentation, associated anomalies, and complications were assessed. The operative procedures included cutback anoplasty and others. The patients were followed in the outpatient department. The complications were assessed and managed accordingly. RESULTS: During the study period of 8 years, 301 patients were admitted. The complaints included absent or abnormal anal opening, abdominal distension, constipation, and peritonitis. Most of the children (n = 214) presented in the neonatal period. The most common clinical presentation was the perineal fistula. The most common associated anomaly was urologic. Fourteen patients were referred from other centers after complications. The most common problem in follow-up was constipation. CONCLUSION: LARM in male patients may have a diverse presentation. The associated anomalies need proper assessment. Awareness may avoid delayed presentation and unwanted complications. When managed by an expert, the condition can be effectively managed. Regular follow-up is important.

The wandering ventriculoperitoneal shunt and the scope of its salvage.

PURPOSE: Distal shunt tube migration following ventriculoperitoneal (VP) shunt placement in children is mostly managed by an initial shunt diversion/removal and subsequent replacement. Lately, shunt salvage is being used as an alternative in certain conditions. We have focused on the situations where one can consider or disregard shunt salvage in such cases. METHOD: A retrospective study of children treated for distal shunt migration following VP shunt placement between January 2013 and December 2019. RESULT: Seventeen children were managed for over 7 years. These included cutaneous extrusions (n = 4), hollow viscus perforation (n = 6), inguinal hernias (n = 5), and umbilical extrusion (n = 2). The surgical treatment varied from a cutaneous wound closure (with a tube in situ), temporary external shunt diversion, and laparotomy with shunt reposition into the peritoneal cavity. Shunt salvage was possible in three cases, whereas in 2 cases even though shunt salvage was possible, it was not feasible due to a short residual shunt length. CONCLUSION: VP shunt salvage is possible in certain cases of distal shunt migration with a functional uninfected shunt. Small cutaneous extrusions can be covered by a local skin flap. Also, one should consider the residual intraperitoneal shunt length before its salvage in small children.

Evaluation and management of rectovaginal fistula in anorectal malformation: an observational study.

PURPOSE: The rectovaginal fistula (RVF) is a type of female ARM in which the rectum terminates in the vagina. Due to its rarity, there are limited reports on its presentation, management, and follow-up. This paper deals with the clinical presentation, management, and outcome of RVF. METHODS: It was a retrospective cohort study of 10 years. The patients were evaluated for age, clinical presentation, associated anomalies, any prior surgical interventions performed elsewhere, and complications. After workup, the patients underwent three stages of surgery. RESULTS: Fifty-six patients of RVF were managed. The median age was 13.48 months. The associated anomalies were present in 37 (66%) patients. Posterosagittal and anterosagittal anorectoplasty (PSARP and ASARP) were performed in 29 and 6 patients, respectively. Abdominoperineal pull-through (APPT) was performed in 16 patients of congenital pouch colon. The complications of the first stage included stomal stenosis (4) and stomal prolapse (3). Constipation was present in 39 patients 2 years after the third surgery. CONCLUSIONS: RVF is a distinct entity, which needs careful clinical examination. With proper planning for diagnosis and treatment, it can be managed at specialized centers. Care may be needed for the associated anomalies. The follow-up is an integral part of its management.

Bracka Urethroplasty with Buccal Mucosa Graft: Ergonomic Management of Penile Skin Dartos in the First Stage to Facilitate Second-stage Neourethral Coverage.

AIMS: The aim of the study was to report a new technique of ergonomic penile skin-dartos management during buccal mucosa graft (BMG) to provide adequate penile skin-dartos for neourethral coverage at the time of second-stage tubularization. MATERIALS AND METHODS: Ten proximal hypospadias with severe chordee underwent first-stage surgery with a new technique. An incision along the urethral plate margin and preputial edge was used to split inner prepuce off preputial dartos and penile degloving leaving inner prepuce attached to corona. Urethral plate was divided into the subfascial plane. Penile dartos was bisected in the dorsal midline. Distal half of penile skin-dartos bifurcated and joined to inner preputial edges. Mobilized and lateralized penile skin-dartos was sutured flanking edges of BMG. The second-stage tubularization after 6 months provided neourethral double dartos coverage with eccentric suture lines. RESULTS: Adequate dartos for neourethral coverage during second-stage tubularization was available in all. Subcoronal urethrocutaneous fistula occurred in one that was repaired. CONCLUSIONS: Ergonomic management of inner-preputial skin and ventral transfer of penile skin-dartos helps in providing neourethral coverage during subsequent second-stage tubularization to minimize the occurrence of complications.

Giant Occipital Encephalocele - Challenges in Management.

INTRODUCTION: Giant occipital encephalocele (GOE) is a term used when the size of the OE is greater than or equal to the size of the head. It has been limited to case reports, with only sporadic exclusive series. This is a series of GOE managed at our center over time with emphasis on practical problems faced in management. MATERIALS AND METHODS: This was a retrospective observational study. The patients were evaluated for the age of presentation, sex, and head size. Any associated neural tube defect was also looked for. Imaging was used for associated brain anomalies and to plan the surgical procedure. The requirement of ventriculoperitoneal (VP) shunt was also assessed. RESULTS: During the study period of 7 years, 11 patients of GOE were admitted. Apart from one, all other patients were <1 year of age. Nine patients underwent surgical intervention, which included excision and repair of swelling with or without VP shunt placement. The content of the sac was only cerebrospinal fluid (CSF) in six patients and CSF and gliotic brain tissue in remaining patients. The attendants of two patients did not give consent for surgery and left against medical advice. CONCLUSION: GOE is an uncommon entity with limited information about management. Careful evaluation, proper imaging of patient, and care during intraoperative and postoperative periods with emphasis of factors determining the prognosis may provide satisfactory results.

Evaluation of Effect of Propranolol on Serum Vascular Endothelial Growth Factor and Tissue Inhibitor of Metalloproteinase-2 Levels in Infantile Hemangioma.

BACKGROUND: Infantile hemangioma is the most common tumor of infancy. Currently, propranolol is a preferred drug for treating hemangioma. The exact mechanism of action of propranolol is not known. In this study, we attempted to assess whether propranolol has any effect on vascular endothelial growth factor (VEGF) and tissue inhibitor of metalloproteinase-2 (TIMP-2) over a period of time, and if it is there, how long it affects it. MATERIALS AND METHODS: Propranolol was administered in the dosage of 2-3 mg/kg. The first serum sample was collected before starting the propranolol treatment. Thereafter, samples were collected at monthly intervals up to a total of six samples. The samples were assessed for TIMP-2 and VEGF using enzyme-linked immunosorbent assay kit. RESULTS: The duration of this study was from June 2016 to November 2017. The total number of patients in this study was 15. Thirteen patients responded to treatment. The mean age of patients was 7.1 months. The mean value of baseline VEGF was 0.234 ± 0.059 and that of TIMP-2 was 1.338 ± 0.679. As compared to baseline value, the P value was statistically not significant in any of sequential values. In category-wise analysis, apart from statistically significant value in the 6th month in excellent category and good response category in the 1st month, all other values did not reveal any significant change in VEGF analysis. The analysis of TIMP-2 revealed a significant change in the levels from Sample 2 to Sample 6 in the excellent response group; however, the levels did not show a specific trend either increasing or decreasing. CONCLUSION: Despite its beneficial action in regression of hemangioma, the exact mechanism is yet to be identified. The exact duration of treatment needs further evaluation.

Management Strategy of Meconium Ileus-Outcome Analysis.

BACKGROUND: Meconium ileus (MI) is defined as an intestinal obstruction caused by the impaction of inspissated meconium in the terminal ileum. In this study, we have evaluated the nonoperative management of patients of simple MI without fluoroscopic support -an important requisite of the Noblett's criteria. Besides this, surgical management in cases of failed conservative management and complicated MI was also assessed. MATERIALS AND METHODS: This was a retrospective observational study. Various clinical and radiological parameters were evaluated. Conservative management included the use of water-soluble contrast diatrizoate meglumine and diatrizoate sodium. In case of nonpassage of meconium in 24 h from first intervention, exploratory laparotomy with ileostomy was performed. All complicated MI underwent exploratory laparotomy with creation of stoma as and when needed. RESULTS: The duration of this study was 6½ years. Twenty-five neonates of MI were admitted. Of these, 22 had simple MI and remaining three had complicated MI. Eighteen neonates responded to the conservative management. In four neonates, who did not respond, exploratory laparotomy was performed. All three neonates having complicated MI underwent exploratory laparotomy. One patient expired in follow-up. CONCLUSION: MI is an important neonatal emergency, which needed immediate attention of a pediatric surgeon. Proper evaluation of the patient, careful application of principals of conservative management, and timely surgical intervention may fetch satisfactory results.

Single-nucleotide and copy-number variance related to severity of hypospadias.

BACKGROUND: The genetic association of hypospadias-risk studies has been conducted in Caucasians, Chinese-Han populations and few in Indian populations. However, no comprehensive approach has been followed to assess genetic involvement in the severity of the disorder. METHODS: The study evaluated to establish the correlation between genotyped single nucleotide and copy number variants (SNPs/CNVs) and severity of hypospadias by an association in a total 30 SNPs in genes related to sex hormone-biosynthesis and metabolism; embryonic-development and phospholipase-D-signalling pathways on 138 surgery-confirmed hypospadias-cases from North India (84 penile and 28 cases of penoscrotal-hypospadias as compared with 31 cases of glanular + coronal), and analyzed and identified CNVs in four familial cases (18 members) and three paired-sporadic cases (6 members) using array-based comparative-genomic-hybridization and validated in 32 hypospadias samples by TaqMan assay. RESULTS: Based on odds ratio at 95% CI, Z Statistic and Significance Levels, STS gene-rs17268974 was associated with Penile-Hypospadias and 9-SNPs [seven-SNPs (rs5934740; rs5934842; rs5934913; rs6639811; rs3923341; rs17268974; rs5934937)] of STS gene; rs7562326-SRD5A2 and rs1877031-STARD3 were associated with penoscrotal-hypospadias. On aggregate analysis with p < 0.001, we identified homozygous-loss of Ch7:q34 (PRSS3P2, PRSS2). On validation in previously CNV-characterized and new (32 hypospadias cases), we identified PRSS3P2-loss in most of the grade 3 and 4 hypospadias. Hence, Grade 1 and 2 (coronal and granular) show no-PRSS3P2-loss and no-association with SNPs in STS; SRD5A2; STARD3-gene but Grade 3 and 4 (Penile and Penoscrotal) show PRSS3P2-loss accompanied with the association of SNPs in STS; SRD5A2; STARD3. CONCLUSIONS: Hence, homozygous-loss of PRSS3P2 accompanied with the association of STS; SRD5A2; STARD3 may link to the severity of the disease.

Anorectal Malformations in Adolescent Females: A Retrospective Study.

CONTEXT: While most of the females with anorectal malformations (ARMs) present in either neonatal period or early infancy, a small percentage presents in their adolescence. AIM: The aim is to study the causes of delay in presentation, management, and postoperative outcome in these cases. SETTINGS AND DESIGN: This is a retrospective observational study conducted in the Department of Paediatric Surgery, King George's Medical University, Lucknow. SUBJECTS AND METHODS: Records of females with ARM managed in one unit of the department from 2010 to 2015 were scrutinized. Of these, record of females who primarily presented in adolescence was reviewed. Data regarding the demographics, clinicoradiological presentation, management, postoperative stay, and follow-up were analyzed. Causes for delay in presentation were looked for. STATISTICAL ANALYSIS USED: Statistical analysis used was not required. RESULTS: Out of 627 cases of ARM managed over 5 years, ten girls (5.3%) presented between 12 and 18 years (average 14.4 years). Main reasons for the delay in presentation were misinformation, illiteracy, and poverty. These comprised of anovestibular fistula (n = 5), anterior ectopic anus (n = 3), perineal canal (n = 1), and rectovestibular fistula (n = 1). Three (30%) girls had no problem apart from an abnormal anal orifice. Five (50%) girls had constipation as a major additional symptom. Four cases had a normal to mildly enlarged rectum on contrast study, whereas the rectum was moderately to hugely dilated in the remaining six cases. Six cases were managed with posterior sagittal anorectoplasty while an anterior sagittal anorectoplasty was done in four. Minor wound dehiscence developed in two cases. There was no mortality. In a mean follow-up of 16 months (8-26), constipation and soiling were seen in 5 (50%) girls. The overall outcome in these girls was satisfactory. CONCLUSION: Presentation of females with ARM in adolescence is not uncommon in the third world. A primary pull through is possible in these girls with mild-to-moderate rectal dilatations. A diverting colostomy before pull through is always a safe option in cases with severely dilated rectum and also otherwise. Moreover, a greater awareness regarding these malformations in the general public is required.

IschioPubic Osteotomy, A Simple and Effective Technique for Pelvic Ring Closure in Repair of Classic Exstrophy Bladder.

AIM: The aim of this study is to report the technique and outcome of ischiopubic (IP) osteotomy for pelvic ring closure in classic exstrophy bladder. METHODS: A total of 85 male classic exstrophies were selected based on trapezoid-shaped space between IP ramus on three-dimensional computed tomography pelvis. Using midline scroto-perineal approach, after bladder plate mobilization and radical corporal detachment; the pelvic surface of superior pubic ramus (SPR) was exposed. Above the obturator canal, H-shaped incision was made on periosteum of the SPR. The horizontal line of H was placed above the obturator canal. Rectangular periosteal flaps above and below the horizontal line was raised. In the subperiosteal plane, curved hemostats encircled the SPR that were divided using a bone drill. On the medial aspect of ischial tuberosity, a notch was created as hinge using a bone drill. Forks of bone holding forceps hooked the pubic bone and tightened for its midline approximation, lengthening of the SPR and inward rotation of IP ramus. Linea alba and pubic bones were approximated with interrupted Polygalactin sutures. RESULTS: Midline approximation of pubic bone and linea alba was possible in all. There was no injury to obturator nerve, vessels, or other structures . CONCLUSIONS: IP osteotomy is the safe and effective technique of pelvic ring closure in patients with specific pelvic configuration.

Migration of Ventriculoperitoneal Shunt into a Hernia Sac: An Unusual Complication of Ventriculoperitoneal Shunt Surgery in Children.

We report 2 cases of ventriculoperitoneal (VP) shunt migration into an inguinal hernia sac. In both cases hernia manifested itself on the right side in late infancy. We attempted to analyse the anatomical and mechanical factors leading to shunt migration as seen in the X-rays of our cases.

Analysis of the Efficacy of Elastography in Comparison with Dynamic Renal Nuclear Scintigraphy in the Evaluation of Unilateral Pelvi-Ureteric Junction Obstruction.

INTRODUCTION: In hydronephrosis due to pelviureteric junction obstruction (PUJO), an obstruction to urine flow may lead to increased pelvic pressure, which may cause interstitial fibrosis and renal impairment. Recently, there have been reports on renal pelvic assessment using ultrasound elastography (USE). This study was conducted to see if USE can evaluate PUJO and if it can be correlated to the findings of the dynamic renal nuclear scan. MATERIAL AND METHODS: In this observational study, only patients with unilateral PUJO underwent acoustic radiation force impulse (ARFI) elastography. A rectangular region of interest (ROI) measuring 5 × 10 mm was positioned on the cortex region of the upper, mid, and lower poles of the affected kidney. Three valid measurements were obtained, from which a mean value was calculated. A dynamic renal nuclear scan using Technetium-99m ethylene dicysteine (EC or TC99 m EC) was obtained and split renal function (SRF) was used for comparison. RESULTS: In the group of 20 patients, the mean age was 3.37 years. The mean SRF of the affected kidney was 26.65 %, and the corresponding USE value was 0.45 kpa. The Spearman's rho correlation coefficient for SRF and USE was 1 and 0.672, respectively (p = 0.001). Elastography was not feasible if SRF was less than 20 %. CONCLUSION: USE may be able to comment on the renal functional status of hydronephrosis. If USE is reported as non-feasible, it may suggest that renal function is grossly compromised. It may serve as an alternative diagnostic modality for renal functional evaluation. LEVEL OF EVIDENCE: Level II, Prospective Cohort Study.

Rethinking Fistula Preservation in Anorectal Malformation Surgery: A Histopathological Perspective.

OBJECTIVE: This study aims to assess the histopathological characteristics of the fistulous tissue in males with anorectal malformation (ARM) to determine its suitability for use in constructing a neoanus. METHODS: This prospective observational study included male ARM patients with recto-urethral fistula. All other types were excluded. The tissue specimens comprised 0.5 to 2.0 cm of the most distal part of the rectal pouch and the fistulous tissue. Hematoxylin and eosin-stained sections were prepared. Histological features, viz. internal sphincter, anal grand and crypts, ganglion cells, presence of subepithelial fibrosis, thickened nerve trunks, and metaplasia, were evaluated. RESULTS: Of 65 patients of ARM admitted, 24 met the inclusion criteria. Gross and microscopic internal sphincter was not found in any sample. The anal mucosa was visualized in all samples; however, crypts were irregular and distorted architecture was seen in 4 (16.67%) samples. Urothelial metaplasia was identified in 10 (41.67%) samples. Adequate ganglion cells were seen only in 6 (25%) samples. Significant submucosal fibrosis was seen in all samples. Thickened nerve trunks were identified in 4 (16.67%) samples. CONCLUSIONS: All normal anal histological features could not be found together in the fistula tissue. With the absence of normal features, such as internal anal sphincter muscles, and the presence of abnormal histopathological features, such as subepithelial fibrosis, thickened nerve trunks, and metaplasia, there are high chances of abnormal bowel function like constipation if the fistulous tissue is used for neoanus construction. It may have an impact on the quality of life of patients.

Y-type urethral duplication in children: Management strategy at our center.

AIMS: Report of seven children with Y-type urethral duplication (YUD). MATERIALS AND METHODS: (A) Four staged operations were performed in patients having extensive perineal dissection (for rectourinary fistula separation and anterior mobilization of ventral urethra (VU)), tension rectocutaneous anastomosis, and children who were not toilet trained). These stages are (1) diversion sigmoid colostomy with anterior mobilization of VU as perineal urethrostomy via anterior sagittal approach; (2) Orthotopic urethral (OU) reconstruction; (3) anastomosis of OU and perineal urethra; (4) colostomy closure with management of complications. (B) The patients having VU onto the perineum underwent single stage urethral reconstruction. RESULTS: The VU was urethrorectal/urethroanal in five and urethroperineal in two. Low anorectal malformation and upper urinary tract anomalies were present in 57.1% (4/7) and 14.7% (1/7) patients, respectively. Buccal mucosa free graft, transverse inner preputial flap, and perineal skin were tubularized for OU reconstruction. Mean age at 1(st) , 2(nd) , 3(rd) , and 4(th) surgery was 5 ± 0.78, 28 ± 0.78, 36 ± 0.78, 49 ± 0.78 months respectively. Three patients needed surgery for complications (urethrocutaneous fistula in two and urethral diverticulum in one) in a mean 3.12 ± 0.34 years of follow-up. Final uroflowmetry and fecal continence were good in all patients. CONCLUSIONS: The YUD is a difficult entity to manage. Although, staged procedure appears to be time consuming, but good and promising results can be achieved by staging the procedure.

Bladder exstrophy: Comparison of anatomical bladder neck repair with innervation preserving sphincteroplasty versus Young-Dees-Leadbetter bladder neck reconstruction.

AIM: To evaluate the outcome of innervation preserving sphincteroplasty along with anatomical bladder neck reconstruction (IPS-ABNR) compared to classic Young-Dees-Leadbetter (YDL) bladder neck reconstruction in exstrophy with insufficient bladder capacity requiring detubularized-ileocystoplasty. MATERIALS AND METHODS: Sixteen male patients of exstrophy bladder who required ileocystoplasty from 2004 to 2010 were randomized into group A (n = 7) and group B (n = 9). After detubularized-ileocystoplasty with Mitrofanoff stoma and ureteric reimplantation in all, group A received YDL bladder neck repair while group B received IPS-ABNR repair through a midline scrotoperineal approach. Outcome measurement included operative and postoperative problems, continence, and upper tract status. RESULTS: In group A, two had incompetent bladder neck with gross incontinence, while four had a dry interval of more than 3 h without the ability of voiding per urethra. In group B, seven patients had dry interval of more than 3 h with an ability of urethral voiding and midstream holding in five. CONCLUSIONS: Exstrophy patients requiring augmentation cystoplasty and repaired with IPS-ABNR can achieve dynamic bladder outlet resistance with adequate leak point pressure and ability to void voluntarily with midstream holding capability. The children had the satisfaction of voiding per urethra with ability to stop in midstream similar to that in normal children.

Retrieval of a retained umbilical vein catheter at 6 months.

Umbilical venous catheterisation is carried out for the care of neonates who have very low birth weight and are critically ill. It is uncommon for catheter fragments to become fractured and retained. Here, we describe a case of a retained fractured umbilical vein catheter in an infant. An endovascular procedure to retrieve a catheter is the usual management option when the catheter has been accidentally cut and has moved proximally into a major vessel or inside the heart. It is rare to need open exploration and retrieval.

Congenital anterior urethral valves and diverticula.

AIMS OF STUDY: Anterior urethral valves (AUVs) are rare congenital anomalies causing lower urinary tract obstruction in children. The present study highlights the clinical approach in identifying the condition and its treatment options. METHOD: Single centre, retrospective study of children. RESULT: Over a 16 year duration, 14 children were diagnosed with AUVs and diverticula with a mean age of 15 months. Clinical features included straining at micturation and a palpable penile swelling. Diagnosis was confirmed with voiding cystourethrogram and cystoscopy. An open surgical procedure was performed in 12 cases and endoscopic intervention done in one. On follow up 13 patients showed restoration of a normal voiding stream and normal renal function. One child died of chronic renal failure due to a delayed presentation, severe back-pressure changes, urinary ascites, and urosepsis. One patient developed a small urethrocutaneous fistula that healed spontaneously. CONCLUSION: AUV, though rare, should be recognizable due to the combination of a poor urinary stream and visible swelling in the ventral aspect of penis. Open surgical excision is the method of choice, and the outcome is excellent. LEVEL OF EVIDENCE: Level IV.

Intralesional steroid in the era of propranolol for infantile hemangioma-Do we need it?

INTRODUCTION: Despite its effectivity, there are reports of poor response to propranolol in the treatment of infantile hemangioma (IH). The literature is limited to the type of IH that fails to respond to propranolol. This study was conducted to analyze which types of hemangiomas respond poorly to propranolol and the effects of intralesional triamcinolone (IL TMC) in them. MATERIAL AND METHODS: In this prospective cohort study, IH was classified as superficial, deep, and mixed. The clinical details were recorded. Propranolol was started in the patients at a dose of 1 mg/kg/day and increased to 2-3 mg/kg/day. The response to the treatment was evaluated as excellent, good, poor, and no response. IL TMC was given in the non-responding group at a dose of 1-2 mg/kg at one-month interval for a total of six doses after stopping propranolol. RESULTS: Ninety-six patients (median age, 7 months; M/F = 2:1) were treated. Superficial hemangioma was present in 40 (41.7%), deep in 10 (10.4%), and mixed in 46 (47.9%) patients. The response was statistically better if initiated within four months of age. It was not influenced by the sex, number, site, or size. The response was statistically better in superficial hemangioma. IL TMC was administered in the 16 patients. The response was good or excellent in 10 patients. CONCLUSION: Propranolol will be used as a first-line drug for IH. All superficial IHs are likely to respond. There will be a possibility of non-responding mixed or deep IH. Use of IL TMC seems reasonable for IH not responding to propranolol.