Extravesical vs. intravesical ureteric reimplantation for primary vesicoureteral reflux: A systematic review and meta-analysis.

Purpose: This study aims to compare the outcomes of extravesical (EVUR) and intravesical (IVUR) ureteric reimplantation for primary vesicoureteral reflux (VUR) via systematic review and meta-analysis. Methods: Literature review from Medline, Embase, and Cochrane since inception to March 2022 was performed. Meta-analysis was conducted on eligible randomized controlled trials (RCT) and observational cohort studies (OCS) comparing outcomes between EVUR and IVUR. Results: Twelve studies were included, comprising 577 patients (778 ureters) operated by EVUR and 395 patients (635 ureters) by IVUR. Pre-operative VUR grade, postoperative VUR persistence and hydronephrosis was not statistically significant. EVUR had shorter operative time [mean differences (MD) -22.91 min; 95% confidence interval (CI), -44.53 to -1.30, P = 0.04] and hospital stay (MD -2.09 days; 95% CI, -2.82 to -1.36, P < 0.00001) compared to IVUR. Bilateral EVUR had higher risk of postoperative acute urinary retention (ARU) (8.1%) compared to bilateral IVUR (1.7%) (OR = 4.40; 95% CI, 1.33-14.58, P = 0.02). No patient undergoing unilateral EVUR or IVUR experienced ARU. Conclusion: Both EVUR and IVUR are equally effective in correcting primary VUR. Operative time and hospital stay are shorter after EVUR compared to IVUR. However, bilateral EVUR is associated with higher risk of postoperative ARU.

Pediatric Solid Tumor Care and Multidisciplinary Tumor Boards in Low- and Middle-Income Countries in Southeast Asia.

PURPOSE: Pediatric solid tumors require coordinated multidisciplinary specialist care. However, expertise and resources to conduct multidisciplinary tumor boards (MDTBs) are lacking in low- and middle-income countries (LMICs). We aimed to profile the landscape of pediatric solid tumor care and practices and perceptions on MDTBs among pediatric solid tumor units (PSTUs) in Southeast Asian LMICs. METHODS: Using online surveys, availability of specialty manpower and MDTBs among PSTUs was first determined. From the subset of PSTUs with MDTBs, one pediatric surgeon and one pediatric oncologist from each center were queried using 5-point Likert scale questions adapted from published questionnaires. RESULTS: In 37 (80.4%) of 46 identified PSTUs, availability of pediatric-trained specialists was as follows: oncologists, 94.6%; surgeons, 91.9%; radiologists, 54.1%; pathologists, 40.5%; radiation oncologists, 29.7%; nuclear medicine physicians, 13.5%; and nurses, 81.1%. Availability of pediatric-trained surgeons, radiologists, and pathologists was significantly associated with the existence of MDTBs (P = .037, .005, and .022, respectively). Among 43 (89.6%) of 48 respondents from 24 PSTUs with MDTBs, 90.5% of oncologists reported > 50% oncology-dedicated workload versus 22.7% of surgeons. Views on benefits and barriers did not significantly differ between oncologists and surgeons. The majority agreed that MDTBs helped to improve accuracy of treatment recommendations and team competence. Complex cases, insufficient radiology and pathology preparation, and need for supplementary investigations were the top barriers. CONCLUSION: This first known profile of pediatric solid tumor care in Southeast Asia found that availability of pediatric-trained subspecialists was a significant prerequisite for pediatric MDTBs in this region. Most PSTUs lacked pediatric-trained pathologists and radiologists. Correspondingly, gaps in radiographic and pathologic diagnoses were the most common limitations for MDTBs. Greater emphasis on holistic multidisciplinary subspecialty development is needed to advance pediatric solid tumor care in Southeast Asia.

Time course response of inflammatory markers in pediatric appendicitis.

PURPOSE: We aim to evaluate the diagnostic value and time course response of the triple inflammatory markers: white blood cell count (WBC), neutrophil percentage (Neu), and C-reactive protein (CRP) in pediatric acute appendicitis. METHODS: A retrospective review of clinical data pertaining to 1391 patients admitted with suspicion for pediatric appendicitis from 2012 to 2017 was conducted. Triple inflammatory markers were acquired upon admission. Appendicitis was confirmed histologically post-appendectomy. The diagnostic value and time course response of these markers was trended in relation to the duration of abdominal pain on admission. RESULTS: 718 patients had histologically confirmed appendicitis. WBC and Neu demonstrate high sensitivity for early appendicitis at 94.6% and 80.0% at Day 1, while CRP demonstrates highest sensitivity of 97.9% at Day 4. The triple markers had poor overall diagnostic value when interpreted individually, however, had a high combined sensitivity of 99.7% and negative predictive value of 98.7% regardless of duration of disease. Overall negative appendectomy rate was 6.7% (n = 52). Among 19 patients with triple markers negative who underwent appendectomy, 17 (89.5%) were histologically normal. CONCLUSIONS: The triple inflammatory markers have limited diagnostic value when interpreted individually, but are strong discriminators of pediatric appendicitis when combined. Their high sensitivity and negative predictive value could potentially help patients avoid unnecessary admissions or costly imaging studies, and reduce negative appendectomy rates. In addition, their objective nature confers an advantage over existing clinical scoring systems which comprise subjective elements.

Anorectal malformation & Hirschsprung's disease: A cross-sectional comparison of quality of life and bowel function to healthy controls.

BACKGROUND: Patients with anorectal malformation (ARM) and Hirschsprung's disease (HD) face long-term disturbance in bowel function even after definitive surgery. This study evaluates the quality of life (QOL) of patients with ARM and HD, and compares them to healthy controls using self-report questionnaires. METHODOLOGY: A prospective study was performed recruiting patients with ARM or HD from September 2013 to December 2014 who had primary surgery done in our institution at least 2 years prior to participation. Age-matched and gender-matched controls were enrolled from our patients with minor outpatient complaints. All participants completed the following PedsQL™ scales (maximum score 100): 4.0 Generic Core Scales, 3.0 General Well-Being (GWB) Scale and 2.0 Family Impact (FI) Module. All were also scored on bowel function (BFS), with a maximum score 20. Appropriate statistical analysis was performed, with significance level <0.05. RESULTS: There were 193 participants: 87 controls, 62 ARM, 44 HD. When comparing Core, GWB and FI scores, there were no significant differences between groups although controls had best scores indicating best QOL and general wellbeing, with least impact of the child's health on the family. BFS was significantly different with controls having best and ARM worst scores. There were no significant differences in scores between parent and child indicating intradyad consistency. There was significant positive correlation between BFS and Core (p<0.0001), and between BFS and GWB scores (p<0.005); and significant negative correlation between BFS and FI scores (p<0.0001). CONCLUSIONS: Bowel function impacts quality of life. Those with ARM and HD can achieve good quality of life comparable to controls, based on patient and caregiver self-reported outcomes. TYPE OF STUDY: Prospective comparative study LEVEL OF EVIDENCE: Level II.

A new 2-step risk-stratification clinical score for suspected appendicitis in children.

AIM: The aim of this study was to develop a new Children's Appendicitis Score (CAS) by combining 3 inflammatory markers and a set of predictors for suspected appendicitis in children. METHODS: 374 children aged 4-16years with suspicion of appendicitis were prospectively enrolled for the derivation cohort. Demographic characteristics, clinical features, laboratory, and histology data were collected. The outcome measure was the histological presence or absence of appendicitis. Backward logistic regression was employed to select predictors for construction of a score. Diagnostic performance of CAS was compared with the Pediatric Appendicitis Score (PAS) on a separate validation cohort. RESULTS: The combination of normal white blood cell count (WBC), neutrophil percentage, and C-reactive protein (CRP) had a 100% negative predictive value for appendicitis. We assigned 'coefficient A' as 'zero' when all triple markers were negative and 'one' when any one markers was positive. A second component of 6 predictors was identified for construction of the 'raw score': Localized right-lower-quadrant pain, generalized guarding, constant characteristic of pain, pain on percussion or coughing, WBC≥14000/L and CRP≥24g/L. CAS was generated by multiplying 'coefficient A' by 'raw score'. CONCLUSION: CAS is superior to PAS in ruling out appendicitis. Risk stratification of equivocal patients could guide the need for further diagnostic imaging examination.

Undescended testis: Level of knowledge among potential referring health-care providers.

AIM: Studies report that most boys with undescended testis(UDT) are referred and operated beyond the recommended age of 1 year, possibly due to lack of awareness of treatment guidelines. We investigate the level of knowledge of UDT among potential referring health-care providers. METHOD: We devised a survey on the clinical features and appropriate management of UDT. Using convenience sampling, we approached health-care professionals with regular contact with paediatric patients and final year medical students. Respondents were allowed to remain anonymous. They were categorised according to specialty and level of experience/training. RESULTS: Of 1179 approached, 203 responded. Thirty-six (24%) of 149 qualified doctors had never seen a case of UDT. Median score was 6 (range 1-9). There was no significant difference in scores when comparing specialty. Mean scores decreased significantly in trend according to level of experience. When questioned regarding timings of referral and orchidopexy, 24% of qualified doctors would not refer until 9 months of age, and 66% thought orchidopexy should be done after 1 year old. Half would stop examining for UDT after 2 years old. CONCLUSIONS: Inexperience with UDT and outdated knowledge may contribute to delays in referral for UDT. Many would stop examining for UDT at 2 years old, placing undue reliance on accurate physical examination in early childhood and indicating lack of awareness of the ascending testis. Community health initiatives must emphasise recent changes in guidelines for management of UDT.

Undescended testis: 513 patients' characteristics, age at orchidopexy and patterns of referral.

OBJECTIVE: Undescended testis (UDT) affects 1-6% of males. Current recommendations are to correct maldescent by 1 year of age. We identify the population characteristics of children referred and managed for UDT, age at referral and orchidopexy, and patterns of referral. DESIGN, SETTING AND PATIENTS: Retrospective 5-year review of all patients operated for UDT from 2007 to 2011 in our institution. Patient demographics, neonatal diagnosis of UDT, age at referral, referral source and age at first orchidopexy were recorded. Data are reported as median (range). RESULTS: There were 513 boys with 576 undescended gonads; 450 (88%) had unilateral UDT. Congenital (present at birth) UDT was diagnosed in 287 (56%) children. Seventy-nine (15%) were premature births, 41 (8%) had associated major genitourinary abnormalities. Median age at referral was 1.1 (0-16.2) years; median age at first orchidopexy was 1.6 (0-17.2) years. When corrected for age, those with a history of prematurity and associated major genitourinary malformations were referred and operated on earlier. There was no difference in age at referral and orchidopexy when comparing unilateral versus bilateral maldescent, and palpability of UDT. Of those with congenital UDT, 70% were operated at beyond 1 year of age. Those referred from public tertiary hospitals were younger than those referred from community clinics (p<0.0001) and private healthcare institutions (p=0.003). CONCLUSIONS: Despite early diagnosis in many patients with UDT, most are referred and operated after 1 year of age, even in congenital UDT. Premature babies, those with major genitourinary anomalies, and those seen in public tertiary hospitals are referred earlier. Community health initiatives must emphasise prompt referral to allay the impact of delayed surgery.

Anomalies associated with anorectal malformations according to the Krickenbeck anatomic classification.

PURPOSE: We evaluated the incidence of congenital anomalies associated with anorectal malformations (ARMs) in relation to the anatomic type of ARM as defined by the Krickenbeck classification. METHODS: We reviewed 99 children with ARM in our institution from 2002 to 2011. Data were collected on patient demographics, type of ARM, and associated congenital anomalies, which were categorized according to organ systems. Statistical analysis was performed for comparison between groups using 'perineal fistula' as the base group. RESULTS: There were 62 (63%) male patients. The majority had perineal fistulas (35, 35%). Seventy-seven (78%) had at least one associated malformation. The most frequent malformations seen were genitourinary (28, 28%) and spinal anomalies (26, 26%). Those with rectovesical fistula had the highest proportion of genitourinary malformations (Odds Ratio [OR], 41.3; 95% confidence interval [CI], 4.7-363.4). Those with cloaca (OR, 49.5; 95% CI, 3.4-718.9) and those with rectovestibular fistula (OR, 12.4; 95% CI, 2.3-65.6) were most likely to have major spinal abnormalities, with tethered cord seen in all groups. The rectovestibular group was also most likely to have other associated malformations (OR, 8.6; 95% CI, 2.2-32.8). CONCLUSION: More than 75% of children with anorectal malformation have other associated malformations. Genitourinary anomalies are the most common. Major spinal anomalies are seen in all groups, affecting nearly half of those with rectovestibular fistula and those without fistula. The incidence of associated malformations in the rectovestibular group is higher than described in the literature. Thorough systematic evaluation of all infants with ARM should be done regardless of type of ARM.

Gene expression of TRK neurotrophin receptors in advanced neuroblastomas in Singapore--a pilot study.

The clinical hallmark of neuroblastoma is heterogeneity. Biologically, ploidy and N-Myc amplification are currently the only 2 features used to define risk group and to determine therapy. Tyrosine kinase neurotrophin receptors (Trks, including TrkA, TrkB, and TrkC) are important in the clinical and biological behavior of neuroblastomas. The authors aim to study Trks gene expression in their local population of advanced neuroblastoma patients. Multiplex reverse transcriptase-polymerase chain reaction (RT-PCR) assay on the expression of TrkA, TrkB, TrkB-truncated, and TrkC was performed on a total of 19 advanced neuroblastoma archival tumors, diagnosed in KK Women's and Children's Hospital between 2003 and 2007. Of the 19 tumors investigated, Trks expression was present in 14 (73.6%) cases. Of these cases, 8 (42.1%), 10 (52.6%), 7 (36.8%), and 6 (31.6%) expressed TrkA, TrkB, TrkB-truncated, and TrkC receptor mRNAs, respectively. Subsequently, the authors compared Trks expression with N-Myc amplification status of the 19 patients. N-Myc was amplified in 5 (26.3%) of the cases. Within the non-N-Myc-amplified group, Trks expression was present in 9 (64%) of the 14 cases. The significant expression of Trk isoforms among advanced neuroblastoma cases as evident from this study support their role as possible risk assessment tools alongside N-Myc amplification status.

Appendicitis in immunosuppressed children: Still a diagnostic and therapeutic dilemma?

Acute appendicitis in the immunosuppressed patients are often difficult to diagnose and are associated with increased morbidity and mortality. Recent debates on primary appendicitis and typhlitis-associated appendicitis have contributed to the diagnostic and therapeutic dilemma. We retrospectively reviewed all our immunosuppressed patients with appendicitis. The most frequent presenting symptom was fever followed by non-specific intermittent abdominal pain. The median delay in diagnosis was 4 days. CT scan was useful in making the diagnosis, but the presence of adjacent bowel thickening has contributed to the dilemma in diagnosis and treatment.

Neonatal intestinal volvulus due to a persistent right vitelline artery.

We report a case of neonatal intestinal volvulus around a persistent right vitelline artery, presenting as an aberrant parieto-mesenteric band on exploratory laparotomy. To our knowledge, this is the first case report in the English literature of a persistent right vitelline artery causing axial intestinal volvulus in a neonate. A review of the literature and the embryopathogenesis is discussed, as well as the importance of emergent diagnoses of such lesions.

Role of surgery in the era of highly successful air enema reduction of intussusception.

BACKGROUND: Despite routine use of air enema reduction in childhood intussusceptions, some still require operative management. This study evaluated the role of surgery and identified factors associated with failed air enema reduction and bowel resection. METHODS: We reviewed 24 patients who underwent laparotomies for intussusception between 1 July 1999 and 31 July 2002. Demographic data, clinical presentations, investigations, surgical interventions and their outcomes were reviewed. RESULTS: Twenty-four (14.5%) of 166 patients treated for intussusceptions between 1 July 1999 and 31 July 2002 underwent laparotomies. A significant proportion (45.8%) was younger than 3 months and older than 36 months of age. Intussusception was diagnosed on ultrasonography in 21 patients. Eighteen underwent attempted air enema reduction. Ileocolic intussusceptions occurred in 54.2% of patients. Five patients had small bowel intussusceptions, all of whom required bowel resection. Seven patients (29.2%) had pathological lead points. Presence of pathological lead points and intussusceptions occurring outside the ileocolic region strongly predicted the need for bowel resection. CONCLUSION: Air enema reductions are less likely to succeed in patients less than 3 months old and those more than 3 years old. Bowel resection is most likely required when pathological lead points are present and when intussusceptions occur outside the ileocolic region. Early surgical intervention may obviate the need for bowel resection in selected patients, thereby reducing surgical morbidity.

Kikuchi disease in Asian children.

OBJECTIVES: Kikuchi disease is a benign, self-limiting condition of unknown aetiology, often manifesting as cervical lymphadenopathy and fever. The disease usually manifests itself in adulthood and paediatric reports are uncommon. The aims of this study are to report the features of Kikuchi disease in the local paediatric population, and to raise the awareness of this entity among clinicians involved in treating these children. METHODS: This is a retrospective series of nine paediatric cases of Kikuchi disease treated in KK Women's and Children's Hospital, Singapore. The clinical presentation, subsequent clinical course and laboratory findings of these patients were reviewed. RESULTS: The patients ranged in age from 7 to 16 years, and included six males and three females. All nine patients presented with fever and cervical lymphadenopathy. Hepatosplenomegaly was present in one child. The erythrocyte sedimentation rate was raised to variable extents, but there was no other significant haematological, serological or microbiological abnormality. In all cases the diagnosis was made on the basis of histological findings. CONCLUSIONS: Kikuchi disease in the paediatric population is not as uncommon as previously thought, and should be considered in the differential diagnosis of any child with fever of unknown origin or cervical lymphadenopathy. Early recognition will minimize unnecessary investigations and prolonged empirical treatments.

Is early laparoscopic appendectomy feasible in children with acute appendicitis presenting with an appendiceal mass? A prospective study.

BACKGROUND: The management of an appendiceal mass remains controversial with 2 schools of thought; early surgical intervention vs nonoperative management with or without interval appendectomy. The aim is to determine the role and safety of early laparoscopic appendectomy (LA) in children with acute appendicitis presenting with an appendiceal mass. METHODS: This is a prospective study of 88 consecutive pediatric patients who underwent attempted LA for suspected acute appendicitis at KK Women's and Children's Hospital, Singapore, between May and October 2003. RESULTS: A total of 88 patients with a mean age of 10 +/- 3 years (range, 3-16 years) underwent LA for an appendiceal mass (n = 22), simple appendicitis (n = 36), other complicated (gangrenous or perforated) appendicitis (n = 23), and a normal appendix (n = 7). There were 7 conversions to open appendicectomy, 3 of which occurred in patients with an appendiceal mass. There were no perioperative or postoperative mortalities. Morbidity occurred in only one patient who underwent LA for perforated appendicitis. He had prolonged sepsis that resolved after 2 weeks of intravenous antibiotics. None of the patients with an appendiceal mass developed complications. Patients who underwent early LA for an appendiceal mass had a statistically significant (P < .05) longer operating time (median, 103 minutes; interquartile range, 90-151 minutes, vs median, 87 minutes; interquartile range, 71-112 minutes), prolonged time to ambulation (median, 2.0 days; interquartile range, 2-2.5 days, vs median, 1.0 days; interquartile, 1-2 days), increased time to resumption of diet (median, 4 days; interquartile, 3-5 days, vs median, 2 days; interquartile, 2-3 days), and longer postoperative stay (median, 6.0 days; interquartile, 5.5-6.5 days, vs median, 4.0 days; interquartile, 3-5.5 days) compared with patients presenting with appendicitis without mass formation. However, there was no statistical difference in these parameters when LA for an appendiceal mass was compared with LA for other complicated appendicitis (perforated and gangrenous). CONCLUSION: Although early LA for an appendiceal mass is a technically demanding procedure, it can be performed safely in children with minimal morbidity and mortality. In an era where patients' demand for "key-hole" surgery is rising, early LA is a safe and viable option in the management of children with an appendiceal mass. It also offers the advantage of avoiding misdiagnoses and the need for a second hospitalization.