Primary repair vs Delayed staged repair in infants with primary obstructive megaureters and their long term outcomes - A single centre experience.

BACKGROUND: There is paucity of literature in the management of infants with primary obstructive megaureters undergoing upfront primary repair with an extravesical technique of ureteral re implantation (with or without ureteral tailoring). OBJECTIVE: To compare 2 different approaches in the management of infants with unilateral primary obstructive megaureters. STUDY DESIGN: This was a retrospective analysis of a prospectively maintained data base between 2005 and 2021. Infants <1 year with unilateral primary obstructive megaureter were included. They were divided into 2 groups: those who underwent an upfront extravesical ureteric reimplantation with or without ureteral tailoring during infancy -Primary Repair (PR), and those who initially underwent a low end cutaneous ureterostomy during infancy followed by take down of ureterostomy and intravesical ureteric reimplantation after 1 year of age -Delayed staged repair (DSR). Children presenting with sepsis, in whom a diversion was imperative, were excluded. All children were followed up annually after their definitive repair with a renal ultrasound, diuretic renogram, estimated glomerular filtration rate and assessment of voiding dysfunction if present. The 1st year and 3rd year follow up details were collated and analysed. Failure was defined as persistent obstructive pattern on renogram with worsening differential renal function or presence of high grade reflux with recurrent breakthrough urinary tract infection; both of which necessitated a redo reimplantation following the definitive surgery. RESULTS: There were 18 infants in Primary repair and 16 infants in Delayed Staged Repair. Urinary tract infections was the commonest presenting symptom amongst both groups i.e. > 50%.The post operative complication rate was 11% in Primary repair and 31% in Delayed Staged Repair. One child in each of the groups (2 girls) required redo reimplantation (5.8%). At the end of the 3rd year follow up (from the definitive repair) there was significant reduction in the hydronephrosis, improvement in the differential renal function with no evidence of obstruction and improvement in the estimated glomerular filtration rate amongst all in both groups which was statistically significant i.e. p < 0.05. The success rate was 94.4% in Primary Repair and 93.75% in Delayed Staged Repair. The mean follow up was 9.7 years amongst those undergone Primary Repair and 9 years amongst those undergone Delayed Staged Repair. DISCUSSION AND CONCLUSION: Primary extravesical ureteral reimplantation may be considered as the preferred line of management of unilateral obstructed megaureters during infancy.

Thoracic Tumors in Children and their Long-Term Outcomes: A Single-Center Experience.

Aims: This study aims to review our experience in children with thoracic tumors managed by different surgical approaches, and to evaluate their long-term outcomes in relation to their functional status and quality of life. Subjects and Methods: This is a retrospective study (2011-2021). Children <18 years with tumors of the thorax (lung, mediastinum, and thoracic cage) were included. All included were diagnosed, managed, and followed up based on a departmental protocol. Children alive were followed up annually to monitor the development of chest wall/spinal deformities and assessed regarding their quality of life (Lansky play-performance scale) and pulmonary functions. Information regarding their demography, clinical presentations, diagnosis, treatment administered, outcome, and follow-up details were collated and analyzed. Results: Twenty-two children with thoracic tumors were included (2011-2021). Of which, 6/22 are benign and 16/22 are malignant lesions. About 14/22 children are alive on a regular follow-up until 2021, with a mean follow-up of 6 years (benign) and 6.25 years (malignant). About 3/22 children with malignant tumors requiring thoracotomy with rib resections developed scoliosis with a severely restrictive pattern on pulmonary functions, having a mild-to-moderate restriction of play on quality of life grading. Conclusions: Early follow-up of children who have undergone various surgical approaches for thoracic tumors based on the quality of life assessment and pulmonary function tests helps in planning early intervention if needed, especially in those who have undergone thoracotomy with rib resections, thereby improving their long-term functional status.

Pelviureteric Junction Obstruction Due to Vascular Anomalies in Children - Simple Surgical Options.

Aims: Pelviureteric junction obstruction (PUJO) due to aberrant lower polar artery is conventionally managed with pyeloplasty. We present our experience of managing PUJO due to "vascular" anomalies-aberrant lower polar artery and vascular adhesions with simpler surgical options. Subjects and Methods: This is a protocol based, retrospective study of PUJO. Preoperative investigations included ultrasonography (USG) and diuretic renogram. An intraoperative methylene blue test (MBT) assessed transit across the Pelviureteric junction (PUJ) after release of vascular compression. Surgical management included adhesiolysis for vascular adhesions and pyelopyelostomy anterior to the aberrant polar artery. Postoperative studies were repeated after 3 and 6 months. Results: Fourteen of 144 PUJO (9.7%) were "vascular" obstructions. Those with vascular adhesions (six) were largely infants with antenatal hydronephrosis. Children with aberrant lower polar artery (eight) were older, had fleeting symptoms, minimally increased pelvic diameter and subtle impairment on diuretic renogram. Majority were term males with urinary tract infection. The MBT showed normal transit across the PUJ in all. Postoperatively, there was progressive improvement on USG and diuretic renogram after 3 and 6 months. None had any complication or redosurgeries. At a mean follow-up of 41.2 months, all are asymptomatic. Conclusions: PUJO due to extrinsic vascular anomalies is rare. Intraoperative evaluation with the MBT ruled out associated intrinsic pathology. We describe two simple surgical alternatives preserving the normal PUJ - adhesiolysis for vascular adhesions and pyelopyelostomy for aberrant lower polar artery. The preliminary outcomes are comparable to conventional pyeloplasty.

Metachronous Venous Malformation Masquerading as an Obscure Gastrointestinal Bleed.

Vascular malformation of the gastrointestinal (GI) tract along with metachronous lesions elsewhere in the body is extremely rare. We report an unusual case of a female child, with a history of venous malformation excision over the right leg presenting with severe iron-deficiency anemia. Although venous malformations of the small bowel are a rare cause of occult GI bleed, in the presence of cutaneous vascular lesions, there should be a high index of suspicion of a vascular malformation, along with the other causes of GI bleed in children.

Varicocelectomy in adolescents - Does it safeguard future fertility? A single centre experience.

BACKGROUND: There is paucity of literature comparing varicocelectomy to observant management amongst adolescent boys with hormonal and semen abnormalities resulting from high grade unilateral varicoceles and consequent testicular volume loss. Furthermore, it is not known whether surgical correction in such adolescents improves paternity rates in future compared to their non-operated cohort. OBJECTIVE: The primary objective was to compare adolescent boys with unilateral high grade varicocele with associated ipsilateral testicular volume loss who were operated versus those who were not, in relation to their fertility markers (hormonal, semen parameters, and testicular volume) over a 5 year follow up period. The secondary objective was to compare the paternity rates in the respective groups over long term. STUDY DESIGN: This was a single center, retrospective study of a prospectively maintained database conducted from 2010 to 2020, based on a standardized protocol. All adolescent boys >15 years of age (middle and late adolescence), with grade II or III unilateral varicoceles with abnormal fertility markers, who were operated (Group A) and not operated (Group B) were included. The changes in hormonal assay, sonographic assessment, semen analysis at presentation, 1st year and the 5th year follow up amongst both the groups were collated and analysed. Primary paternity rates amongst both the groups was documented by telephonic or email conversations. RESULTS: Of the 182 boys referred for varicocele management, 110 boys (Group A -70 boys and Group B - 40 boys) satisfied our inclusion criteria and were analysed. Mean age at presentation amongst Group A boys was 16.5 years (15-18 years) and Group B boys was 16 years (15-18 years). Grade III varicoceles were more predominant amongst both the groups. There was a significant improvement in all Group A boys (operated) in the fertility markers from the time at presentation to the 5th year follow up (p < 0.001). In Group B, (boys not operated) there was no significant improvement in the above parameters. The testicular catch up growth was 92% at the 5th year follow-up in Group A and 42% in Group B. At long term follow-up, the paternity rate was 80% and 36% in Group A and B respectively. DISCUSSION AND CONCLUSION: In adolescent boys in whom hormonal assay, testicular volumes and semen characteristics are negatively affected by high grade unilateral varicoceles, surgical correction could normalize these values, thereby safeguarding their fertility in the long term.

A single centre experience of percutaneous nephrolithotomy in infants and its long-term outcomes.

BACKGROUND: Renal stones in infants requiring Percutaneous nephrolithotomy (PCNL) is rare. There is insufficient literature on the long-term implications of PCNL in growing kidneys of these children. OBJECTIVES: To review our experience of PCNL amongst infants i.e., < 1year of age and to analyse the safety and efficacy of this procedure and assess its long-term renal outcomes. STUDY DESIGN: This was a retrospective analysis of a prospectively maintained data base between 2005 and 2020. All infants with unilateral renal stones >12 mm underwent PCNL. Changes in the serum creatinine, estimated glomerular filtration rate and renal size prior to the PCNL and at the last follow up were monitored. The demographics, clinical profile, operative details, post-operative complications and follow up data were collated and analyzed. RESULTS: 86 children were diagnosed with renal stones of whom, 24 infants met our inclusion criteria and were included in the review. The average age was 9.75 months with fever being the commonest presenting symptom. Five infants were diagnosed with metabolic abnormalities, hypercalciuria being the commonest. Majority of the infants (22) had single stones and the lower calyx was the commonest site (50%). The mean stone burden was 19.5 mm. The stone free rate was 91% during the primary PCNL, which increased to 100% after re-do PCNL. The overall complication rate was 16% which was graded by the modified Clavien Dindo scale for surgical complications. The median follow up period was 144 months and average age at the last follow up was 10.5 years. At the last follow up, a mean serum creatinine of 0.4 mg/dl, mean estimated glomerular filtration rate of 98 ml/min/1.72 m2 and a mean renal size of 8.3 cm was recorded, which was comparable to that of a normal child, thus signifying no deterioration of renal functions and renal growth. Three children showed the presence of cortical scars on an isotope scan at follow up. However, all the 24 operated renal units demonstrated preserved renal functions. DISCUSSION & CONCLUSION: PCNL performed during infancy does not hinder the growth potential of the kidney. Each of the 24 children achieved the target renal size and estimated glomerular filtration rate corresponding to the duration and body size at the end of the follow-up. Thus, PCNL in infants <1 year of age is safe and effective with no adverse effects at long term follow-up.

Paediatric Buccal Mucosal Graft Urethroplasty for Non-Hypospadias Urethral Strictures: A Single Centre Experience with Long Term Outcomes.

OBJECTIVE: To review our experience in the management of paediatric urethral strictures with buccal mucosal graft urethroplasty and its long term outcomes. METHODS: This was a retrospective analysis of a prospectively maintained data base between 2009-2019. Circumcised children with long segment urethral strictures (> 1.5 cm) were included. They were characterized as either peno-bulbar (PBS) or isolated bulbar strictures (IBS) based on a standardized protocol. All children underwent single stage dorsal onlay buccal mucosal graft urethroplasty by either the Kulkarni technique (PBS group) or the Barbagli technique (IBS group). All children were followed up at 3 months, then annually thereafter with flow rates at each visit. Success was defined as a flow rate > 10ml/sec with a bell-shaped curve and absence of need for any secondary procedures. RESULTS: 28 children underwent buccal mucosal graft uretheroplasty.16 children were diagnosed with PBS & 12 children with IBS. The median age was 7.5 years (2-17 years) in PBS and 5.5 years (3-10 years) in IBS. Iatrogenic injury was the commonest aetiology ie 71 % (20/28). Mean length of stricture was 4 cm (3-5.5 cm) in PBS and 2.5 cm (2-3.5 cm) in IBS. Median follow up was 96 months (24-120 months) in PBS and 90 months (36-120 months) in IBS. The success rate was 87.5% (14/16) in PBS and 83.3% (10/12) in IBS. CONCLUSION: Single stage dorsal on lay buccal mucosal graft urethroplasty, ie Kulkarni and Barbagli techniques are safe and feasible in children with long segment urethral strictures with good long-term outcomes.

Application of the "Yang-Monti principle" in children with iatrogenic ureteral injuries.

BACKGROUND: Iatrogenic ureteral injuries in children are rare, due to its retroperitoneal position. The Yang -Monti ileal substitution serves as a good surgical option in such injuries associated with long segment defects of the ureter. OBJECTIVES: To review our experience using the Yang Monti principle of ileal ureter substitution in children with long segment ureteric defects and analyse its intermediate term outcomes. STUDY DESIGN: This was a prospective study, conducted from 2014 to 2019. All children with iatrogenic ureteral injuries with delayed presentations, undergoing the Yang Monti principle of ileal ureter substitution, were included (as illustrated in summary image). These injuries were divided into upper, mid, and distal ureteral injuries and were diagnosed based on a standardized protocol. At follow up, findings of the CT urogram, functional isotope renogram, and renal functions were the key points monitored. The demographic, clinical profile, operative, post-operative complications and follow up data were collated and analyzed. RESULTS: 22 children were diagnosed with iatrogenic ureteral injuries. Of these, 14 children with delayed presentations underwent the Yang Monti principle of ileal ureter substitution. The primary pathology was gangrenous appendicitis (9 cases), calculus cholecystitis (3 cases) and torsion ovary (2 cases). Laparoscopic procedures were the commonest approach for the injury (10). The average age was 11.5 years, with a male predominance. The right ureter was affected in 12 cases. The average time to detection was 7 days. All children underwent an ultrasound guided nephrostomy tube insertion at presentation and the mid ureter (9) was most affected. The mean length of ureteric injury was 3.75 cm. Intra operatively, one ileal segment (2), two ileal segments (11) and three ileal segments (1) were used according to the length of the ureteric defect. Complications were urinary tract infections (2), adhesions (3) and leak (2) which were conservatively managed with no redo surgeries. The median follow up period was 4 years. At follow up CT urogram and isotope diuretic renogram were normal, with preserved renal functions and no metabolic complications in all. DISCUSSION & CONCLUSION: In children associated with iatrogenic ureteral injuries of long lengths, the "Yang Monti ileal substitution" serves as a good and simple option with lesser complications permitting the preservation of the renal function at follow up.