Long-Term Functional Outcomes and Multidisciplinary Management after Ileorectal Duhamel Pull-Through for Total Colonic Aganglionosis-20-Year Experience in a Tertiary Surgical Center.

INTRODUCTION: Management of patients with total colonic aganglionosis (TCA) is challenging for pediatric surgeons. The purpose of this study was to review our institution's 20-year experience regarding long-term outcomes and multidisciplinary team management of these patients after an ileorectal Duhamel pull-through procedure. MATERIALS AND METHODS: Retrospective review was conducted for all patients diagnosed with TCA in our tertiary institution. Data were collected on demographics, clinical presentation, complications, need for additional surgery, and long-term effects on bowel function. RESULTS: Of a total of 202 patients with Hirschsprung's disease, 13 were diagnosed with TCA (6.4%). Clinical presentation was variable. Eleven presented in neonatal period with distal bowel obstruction and two presented with constipation in early infancy. Ileorectal Duhamel pull-through was performed in all patients. Median follow-up was 13 years. Eleven are toilet trained, of whom five are fully continent. Six continue to have problems with bowel continence or constipation. One developed recurrent episode of Hirschsprung's associated enterocolitis. Two patients had stoma re-established. Patients experiencing difficulties in bowel function are jointly managed by a multidisciplinary team consisting of surgeons, gastroenterologists, pediatric psychologists, and clinical nurse specialists. CONCLUSION: TCA can be associated with significant long-term morbidity. Nearly half of the patients in this series have ongoing problems with bowel continence requiring a permanent stoma in some. Diligent follow-up coupled with inputs of a multidisciplinary team has greatly helped manage these complex patients in our institution.

Morbidity following suprapubic line insertion for videourodynamics in children.

INTRODUCTION: Previously in our unit, urodynamics were delayed after insertion of suprapubic (SP) lines. We postulated that performing urodynamics on the same day as SP line insertion would not result in increased morbidity. We retrospectively compared complications in those having urodynamics on the same day against those who had delayed urodynamics. PATIENTS AND METHODS: Notes were reviewed for patients undergoing urodynamics via SP lines from May 2009 until December 2018. In 2014 we modified our practice to allow urodynamics on the same day as SP line insertion in some patients. Patients undergoing videourodynamics would have two 5 Fr (mini Paed) SP lines inserted under general anaesthesia. Patients were divided into two groups: those that had urodynamics on the same day as SP line insertion and those that had urodynamics after an interval of more than one day. The outcome measure was the number of problems affecting those in each group. The two groups were compared using Mann-Whitney U tests and Fisher's Exact tests. RESULTS: There were a total of 211 patients with a median age of 6.5 years (range three months to 15.9 years). Urodynamics were performed on the same day in 86. Delayed Urodynamics were performed at an interval of more than one day in 125. Adverse events included pain or difficulty with voiding, increased urinary frequency, urinary incontinence, leak from catheter site, extravasation, extension of in-patient stay, visible haematuria, urethral catheterisation, and urinary tract infection. Problems affected 43 (20.4%) children. In the same day group, 11 (13.3%) patients had problems, in the delayed group 32 (25.6%) had problems; this was statistically significant (p = 0.03). The difference in combined incidence of important problems (requiring urethral catheterisation, extended admission or abandonment of urodynamics) was not statistically significant between the two groups. CONCLUSION: When using suprapubic catheters for urodynamics there is no additional morbidity when catheters are inserted on the same day as the urodynamics study compared to when urodynamic are delayed.

Post Frenotomy Massage for Ankyloglossia in Infants-Does It Improve Breastfeeding and Reduce Recurrence?

OBJECTIVES: Frenotomy is performed in breast fed infants who experience difficulty in latching after failed conservative management for ankyloglossia or tongue-tie. Though parents sometimes enquire about massage after frenotomy, neither published evidence nor clinical consensus supports this. The aim of our study was to assess if there was significant difference in breast feeding or recurrence rate between those infants who had post frenotomy massage and those who did not. METHODS: A retrospective study was conducted in a tertiary Children's hospital from January 2018 to December 2018. The tongue-tie service consisted of five pediatric surgical consultants, three of whom routinely advice post frenotomy massage. As a result, we had two groups to compare -massage and non-massage group. Total sample size (n = 599) consisted of those who were advised massage (n = 282) and those who were not advised massage (n = 317). RESULTS: Overall recurrence rate was 4/599 (0.66%) and this did not achieve statistical significance between the two groups. Breast feeding rates were also similar in both the groups. However, it is interesting to note that only 43.5% of those advised massage adhered to the massage regimen. CONCLUSIONS: Improvement in breast feeding and recurrence after frenotomy were similar between massage and non-massage groups. This confirms the lack of any additional benefit of post frenotomy massage. This study assists clinicians with decision making not to advise massage as it is unlikely to benefit infants with tongue-tie.

Perioperative morbidity of paediatric laparoscopic nephrectomy by transperitoneal and retroperitoneal approaches- any difference?

AIM: Minimal invasive approaches for paediatric nephrectomy include transperitoneal (TP) and retroperitoneal (RP); both having advantages and disadvantages. We aimed to ascertain if there was any difference in perioperative morbidities between these two approaches. METHODS: We performed a retrospective review of laparoscopic TP and RP nephrectomies performed in our institution over 10 years from May 2009 till May 2019. Outcome measures included intraoperative complications, prolonged requirement of opioid analgesics (more than 24 h), hospital stay, incidence of wound infection and urinary tract infections. Data were analysed using Fisher's exact test and Mann Whitney test. RESULTS: A total of 152 nephrectomies were performed in 139 patients; 81 were TP and 71 were RP. Age ranged from 8 months to 16 years. Median hospital stay was 2 days in both groups. There were no intraoperative complications. Outcome measures were sub-categorised as follows. Requirement of opioid analgesia for more than 24 h was documented in 2 patients in each group, leading to longer hospital stay of 3 days. A febrile urinary tract infection requiring antibiotics was detected in 4; 1 in TP and 3 in RP. Wound infection requiring antibiotics occurred in 1 patient (in RP group). No statistically significant difference was found between the two groups in any of the subcategories. CONCLUSION: TP and RP nephrectomy have similar perioperative morbidity. The decision to utilise either approach should be dependent on the surgeon's skills and experience and appropriately tailored to individual patient needs.

Posterior urethral valve and anterior rectal duplication: a new combination.

We present a rare case of association of anterior rectal duplication and posterior urethral valve (PUV). A term neonate with no antenatal concerns was admitted with urosepsis and acute renal injury at 18 days of age. History revealed a poor urinary stream and dribbling. After resuscitation and stabilisation, renal tract ultrasound and micturating cysto-urethrogram were performed. Cystourethroscopy showed PUV and a mass indenting the bladder posteriorly. MRI confirmed the presence of a cystic lesion anterior to the rectum suspicious of rectal duplication. Laparoscopic-assisted excision of the anterior rectal duplication cyst was then performed. The infant recovered uneventfully. Creatinine normalised postoperatively and has been stable at follow-up.

Long term follow up of proximal hypospadias repair-urethral stricture should be excluded in adults who present with epididymo-orchitis.

OBJECTIVE: Epididymo-orchitis (EO) is a well-known complication of urinary tract infections (UTI) in children. This is commonly seen in children who had hypospadias repair or in those who had surgery for anorectal malformation especially when it results in urethral stricture. However EO occurring as a complication of urethral stricture in adults operated for hypospadias in childhood is not well documented in the literature. MATERIAL AND METHODS: This is a retrospective review of four adults who had proximal hypospadias repair in childhood. All four men presented to us with EO. They were thoroughly investigated to rule out presence of urethral stricture. RESULTS: Three patients had urethral stricture of which two responded well to dilatation and one required cystoscopy and visual internal urethrotomy. The fourth patient did not have urethral stricture. EO in this patient is thought to be due to excessive straining during micturition causing reflux into seminal vesicles. CONCLUSION: Urethral stricture should be excluded in any adult who had a hypospadias repair and presents with EO. Urethral strictures after hypospadias surgery respond well to dilatation and to endoscopic urethrotomy.

Horseshoe kidney and associated anomalies: Single institutional review of 20 cases.

BACKGROUND: Horseshoe kidney (HSK) is a common renal fusion anomaly. We undertook this study to discuss various anomalies associated with HSKs. The objective of the study is to study various anomalies associated with HSKs and to assess the need for surgical intervention in patients with these anomalies. PATIENTS AND METHODS: This is a retrospective cohort study of twenty patients who presented to our institute with the diagnosis of HSKs. The data were analysed with regard to age at presentation, symptoms, associated anomalies, investigations and surgical intervention. RESULTS: Twenty patients were included in this study. They were referred either with a diagnosis of HSK or were diagnosed during investigations for symptoms and during workup for associated anomalies. Eleven patients were incidentally diagnosed and were asymptomatic, and there were no associated anomalies diagnosed in these patients. Nine patients were symptomatic and were diagnosed with various associated anomalies. Our results concur with recent literature review which suggests that nearly half of the patients with HSKs have associated anomalies. In the present series, all symptomatic patients with associated anomalies required surgical intervention. CONCLUSION: HSKs being a common fusion anomaly, necessitates a prompt and thorough search of the search for any associated anomalies. Many of these anomalies may require surgical intervention.

Rupture of Umbilical Hernia with Evisceration in a Newborn - A Case Report.

Most umbilical hernias in infants do not need surgery and the ring will eventually close. Occasionally few complications can arise and incarceration is most common. Spontaneous rupture of the hernia and eventual evisceration is a rarely seen complication. A 3-week-old neonate having umbilical hernia presented with rupture of the sac with evisceration of bowel within a few days of first visit. No underlying cause like umbilical sepsis was found. The baby had emergency repair of the hernia with an uneventful recovery.