Yew Wei Tan - Academia.edu (original) (raw)

Papers by Yew Wei Tan

Research paper thumbnail of Late Diagnosis of Hirschsprung's Disease: Definition and Implication on Core Outcomes

European Journal of Pediatric Surgery, Mar 9, 2022

INTRODUCTION Late diagnosis of Hirschsprung's disease (LDHD) may carry a poor prognosis. Its... more INTRODUCTION Late diagnosis of Hirschsprung's disease (LDHD) may carry a poor prognosis. Its definition remains unclear and its implication on HD-related core outcomes has not been fully reported. METHODS A single-center 20-year series was reviewed to include HD with follow-up of 1 year or more post pull-through (PT) and aged 5 years or older. We investigated six core outcomes derived from NETS1HD study by comparing the groups dichotomized by four time points using age at diagnosis (44-week gestation, 6 months, 1 year, and 3 years). Following establishment of definition of LDHD, the outcomes and complications were compared with timely diagnosis of HD (TDHD). RESULTS Forty-nine out of eighty-six HD were included. The definition of LDHD was found to be HD diagnosed at 1 year of age or later because 3/6 core outcomes were significantly worse than TDHD. Nine patients (18%) had LDHD-median age at diagnosis 42 months (12-89) and PT performed at 57 months (12-103), mostly Soave (73%); a covering stoma was performed in 7/9, significantly higher than TDHD in 10/40 (p = 0.001). LDHD was associated with increased unplanned surgery (78% vs. 30%, p = 0.019), fecal incontinence (100% vs. 62%, p = 0.01), and permanent stoma (33% vs. 5%, p = 0.037). Major complications (56% vs. 20%, p = 0.043) and redo PT (33% vs. 5%, p = 0.037) were also higher in LDHD. CONCLUSION LDHD could be defined as HD diagnosis at or over 1 year of age. LDHD was associated with more preoperative stoma, major postoperative complications, unplanned reoperation, and worse HD-related core outcomes.

Research paper thumbnail of A Critical Analysis of Rectal Biopsy to Exclude Hirschsprung's Disease

European Journal of Pediatric Surgery, 2021

Introduction Most Hirschsprung's disease (HD) are diagnosed in young children with increased ... more Introduction Most Hirschsprung's disease (HD) are diagnosed in young children with increased risk (”red flag”). Older children (>6 months) require open rectal biopsy (ORB) with its own impact on risk and resources. We investigated if “red flag”, age, and sex used in combination could exclude HD. Materials and Methods “Red flags” are risk factors associated with HD, including neonatal bowel obstruction, genetic association, failure of passage of meconium in <48 hours, infantile constipation, distension with vomiting, or family history. All rectal biopsies (2015–2018) were reviewed for indications, methods, and histopathological findings. Logistic regression analysis was adopted to assess predictive value of “red flag,” age, and sex (p 6 months old (62 vs. 30%*), and female gender (54 vs. 16%*), compared with HD. In the absence of “red flag,” 7/82 (9%) had HD (negative predictive value = 91%). Logistic regression analysis found absent “red flag” predicted non-HD biopsy with ...

Research paper thumbnail of Coagulation Gene Expression Profiling in Infants with Necrotizing Enterocolitis

Journal of pediatric gastroenterology and nutrition, Dec 31, 2016

Coagulopathy and mesenteric thrombosis are common in premature neonates with necrotizing enteroco... more Coagulopathy and mesenteric thrombosis are common in premature neonates with necrotizing enterocolitis (NEC). This pilot study aimed to investigate the hypothesis that there are changes in the gene expression related to the coagulation and anticoagulation systems in NEC. Consecutive neonates (n = 11) with NEC (Bell's stages 2-3) were recruited. Two comparison groups, matched for birth weight and corrected gestational age, were selected based on: the absence of inflammation and coagulopathy (healthy control, n = 10); or the presence of a confirmed blood infection (sepsis control, n = 12). A pathway specific quantitative PCR array was used to determine the expression of 94 genes involved in human blood coagulation and anticoagulation cascade. Twelve genes of the coagulation and anticoagulation systems were significantly altered in the NEC patients compared to healthy controls. In particular, ELANE, CD63, PROS1, HGF, F12 were significantly up-regulated (mean fold changes +2.74, p &...

Research paper thumbnail of Coagulopathy in Necrotising Enterocolitis: Modulation of Gene Expression Microarrays Analysis to Study Gene Expression

ABSTRACT Stefano Giuliani, MD PhD1, Yew Wei Tan, MD1, Dongling Zheng, PhD2, Nigel Kennea, MD1 and... more ABSTRACT Stefano Giuliani, MD PhD1, Yew Wei Tan, MD1, Dongling Zheng, PhD2, Nigel Kennea, MD1 and Yalda Jamshidi, PhD2, (1)St.George&#39;s Healthcare NHS Trust and University, London, United Kingdom, (2)St.George&#39;s University of London, London, United Kingdom Purpose: Severe coagulopathy and thrombosis of the mesentery are common findings in advanced necrotising enterocolitis (NEC) and they are associated with high morbidity and mortality. There is no knowledge about how the expression of genes involved in the coagulation and anticoagulation cascades are altered during the advanced phases of the disease. The aim of the study was to investigate differences in the coagulation and anticoagulation gene expression between advanced NEC patients and a control group. Methods: Consecutive patients with necrotizing enterocolitis stage 2 and 3 (Bell&#39;s classification) were recruited over 6 month&#39;s period. Patients included had a birth weight &gt;800 gram and corrected gestational age (cGA) less than 37 weeks. A control group, matched for weight and cGA, was selected from infants without sepsis or coagulopathy. Each infant had a 0.5ml blood sample taken for gene expression analysis. RNA was extracted from pelleted cells for gene expression analysis using the Human Blood Coagulation 96 StellARRay qPCR array (Lonza). We employed a sensitive quantitative real-time PCR method to compare gene expression by means of a &#39;ÔHuman Blood Coagulation&#39;&#39; qPCR array. Clinical end points analysed were differences in: haemoglobin, platelet, clotting profile, laparotomy and survival between the NEC and control. Continuous data used Student t-test and Mann-Whitney U test as appropriate, nominal data used Fisher&#39;s exact test, and statistical significance was P&lt;0.05. Results: 8 controls (median gestational age 29+6 weeks, birth weight 1165g, 23 days old) and 7 NEC (median gestation 28+4 weeks, birth weight 840g, 18 days old) were included in the study. Significantly lower platelet counts were found in the NEC compared to the control group (67 v 340 x10^9/L, p=0.002). Mortality in both groups were comparable (NEC 3/7 v control 1/8, P=0.185), with emergency laparotomy indicated in one of 7 NEC patients. 10 genes associated with the coagulation and anticoagulation cascades were identified to be significantly up or down-regulated in the NEC group compared to the controls. These were genes involved in proteolysis of extracellular matrix (up-regulation of CD63, ELANE, PLAU, ASGR2), sensors of proteolysis (down-regulation of F2RL1), activation of coagulation (F12, PROC) and platelet aggregation (down-regulation of P2RY12). Conclusion: This is the first study defining significant alterations in the gene expression of the coagulation and anticoagulation systems in patients with advanced NEC. These findings support the hypothesis that coagulopathy plays an important role in advanced NEC and points to a need for further research to understand the relationship of coagulation and NEC disease progression.

Research paper thumbnail of Laparoscopic Toupet Fundoplication

Video Atlas of Pediatric Endosurgery (VAPE), 2021

Research paper thumbnail of Neonatal Surgical Conditions: Congenital Diaphragmatic Hernia and Short Bowel Syndrome

The dramatic improvements in the outcomes of neonatal surgery are due largely to advances in neon... more The dramatic improvements in the outcomes of neonatal surgery are due largely to advances in neonatal care and in that sense, all directly of interest to the neonatologist. However there are areas where our understanding of the factors affecting outcomes is still evolving and are therefore areas of intense research. The conditions we have chosen to discuss are those where optimum medical management is the outcome determinant and surgery itself is secondary. These are congenital diaphragmatic hernia and short bowel syndrome.

Research paper thumbnail of Optimal management of gastrojejunal tube in the ENFit era — Interventions that changed practice

Journal of Pediatric Surgery

Research paper thumbnail of Treatment of megarectum in anorectal malformation with emphasis on preventive aspects: 17 years experience

Pediatric Surgery International

Research paper thumbnail of Prognostic Value of the Oxygenation Index to Predict Survival and Timing of Surgery in Infants with Congenital Diaphragmatic Hernia

Journal of Pediatric Surgery

Research paper thumbnail of Morgagni hernia repair in children over two decades: Institutional experience, systematic review, and meta-analysis of 296 patients

Journal of pediatric surgery, Jan 13, 2018

Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use i... more Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use in MH repair to curb recurrence. A systematic review and meta-analysis were performed to study the recurrence and complications associated with minimally invasive surgery and the use of patch. We retrospectively reviewed all cases of MH who underwent first-time repair in 2012-2017 in our institution to determine recurrence and complication rate. A MEDLINE search related to minimally invasive surgery (MIS) and patch repair of MH was conducted for systematic review. Eligible articles published from 1997-2017 with follow-up data available were included. Primary outcomes measured were recurrence and complication. Meta-analysis to compare open versus MIS and primary versus patch repair in the MIS group were performed in comparative cohorts. Continuous data were presented as median (range), and statistical significance was P<0.05. In our institution, 12 consecutive patients aged 17-month-old...

Research paper thumbnail of Duhamel operation for children with distal colonic dysmotility

Pediatric Surgery International

To report outcomes of children with constipation refractory to medical management and manometrica... more To report outcomes of children with constipation refractory to medical management and manometrically proven distal colonic dysmotility, managed with rectosigmoidectomy followed by Duhamel operation (Duhamel). Children who underwent a Duhamel from 2009 onwards for intractable constipation and left colonic dysmotility were retrospectively reviewed. The primary end point was resolution of constipation, and secondary end point was postoperative complications. Continuous data were median (range). 11 patients (4 males) had Duhamel at 11 years (5-16) with constipation started from 2 years (1-8). Hirschsprung&amp;amp;#39;s disease was excluded. All Duhamels were performed with a covering ileostomy: 9 following a Hartmann procedure, one following a previously failed reversal of Hartmann, and one Duhamel performed with a pre-existing ileostomy. All ileostomies were subsequently closed. Median resection length was 22 cm (11-31). Length of stay was 8 days (5-23). Follow-up was 5 years (0.5-7). Age at final review was 15 years (10-18). Resolution of constipation occurred in nine patients (4 required antegrade continence enemas (ACE), 5 with laxative); two had persistent constipation and faecal incontinence despite ACE, ultimately requiring an ileostomy. Two postoperative small bowel obstructions required laparotomy. Duhamel performed in children with manometrically proven distal colonic dysmotility yielded 82% resolution of refractory constipation; half of them subsequently needed ACE.

Research paper thumbnail of The management of boys under 3 months of age with an inguinal hernia and ipsilateral palpable undescended testis

Journal of Pediatric Surgery

The optimal management for boys under 3 months of age with an indirect inguinal hernia (IIH) and ... more The optimal management for boys under 3 months of age with an indirect inguinal hernia (IIH) and ipsilateral palpable undescended testis (IPUDT) is unknown. We aimed to: 1) determine the current practice for managing these boys across the UK, and 2) compare outcomes of different treatment strategies. We undertook two studies. Firstly, we completed a National Survey of all surgeons on the British Association of Paediatric Surgeons email list in 2014. Subsequently, we undertook a multi-centre, retrospective, 10-year (2005-2015) review across 4 pediatric surgery centers of boys under 3months of age with concomitant IIH and IPUDT. Primary outcome was testicular atrophy. Secondary outcomes included need for subsequent orchidopexy, testicular ascent and hernia recurrence. Data are presented as median (range). Chi-squared test and multivariate binomial logistic regression analysis were used for analysis; p&amp;amp;amp;amp;lt;0.05 was considered significant. Survey: Consultant practice varies widely across the UK, with a tendency towards performing concurrent orchidopexy at the time of herniotomy under 3 months of age. Concurrent orchidopexy is favored less in cases where the hernia is symptomatic. Case Series Review: Forty-one boys with 43 concomitant IIH and IPUDT were identified, and all included. 32 (74%) hernias were reducible, 11 (26%) were symptomatic requiring urgent or emergency repair. Post-conceptual age at surgery was 45weeks (36-65). Primary operations included: 29 (67%) open hernia repair and standard orchidopexy, 8 (19%) open hernia repair with future orchidopexy if required, 4 (9%) laparoscopic hernia repair with future orchidopexy if required, 2 (5%) open hernia repair and suturing of the testis to the inverted scrotum without scrotal incision. Variation in atrophy rate between different surgical approaches did not reach statistical significance (p=0.42). Overall atrophy rate was 18%. If hernia repair alone was undertaken (8 open and 4 laparoscopic), the testis did not descend in 8 patients, requiring subsequent orchidopexy (67%); if orchidopexy was undertaken at the time of hernia repair, 1 in 29 required a repeat orchidopexy (3%) (p=0.0001). No hernia recurred. This study suggests that orchidopexy at the time of inguinal herniotomy does not increase the risk of testicular atrophy in boys under 3months of age.

Research paper thumbnail of Corrigendum: A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis

Scientific reports, Jul 17, 2017

Research paper thumbnail of A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis

Scientific reports, Jan 27, 2017

The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) s... more The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) surgery for revalidation of neonatal surgeons. The aim of this study was therefore to calculate the number of infants in the UK/Ireland with surgical NEC and describe outcomes that could be used for national benchmarking and counselling of parents. A prospective nationwide cohort study of every infant requiring surgical intervention for NEC in the UK was conducted between 01/03/13 and 28/02/14. Primary outcome was mortality at 28-days. Secondary outcomes included discharge, post-operative complication, and TPN requirement. 236 infants were included, 43(18%) of whom died, and eight(3%) of whom were discharged prior to 28-days post decision to intervene surgically. Sixty infants who underwent laparotomy (27%) experienced a complication, and 67(35%) of those who were alive at 28 days were parenteral nutrition free. Following multi-variable modelling, presence of a non-cardiac congenital anom...

Research paper thumbnail of Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis

The Journal of pediatrics, Jan 8, 2016

To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities ... more To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities (IRAs) in asymptomatic patients with heterotaxy. PubMed, Embase, and Cinahl were searched electronically to determine the overall incidence of IRAs in heterotaxy; the detection rate of IRAs associated with screening; the incidence of midgut volvulus in patients without screening; and the incidence of morbidity and mortality after prophylactic and emergency Ladd procedures. Relevant data were computed with a meta-analysis of proportions. Between-study heterogeneity was assessed with the I(2) statistic. From 276 papers identified, 24 studies with a total of 1433 patients with heterotaxy were included for systematic review. No randomized study was identified. True incidence of IRA in heterotaxy could not be ascertained through meta-analysis. In patients who underwent screening, the incidence of IRA was 58%. Acute midgut volvulus occurred in 5.8% of those who did not undergo screening. Posto...

Research paper thumbnail of Extensive Necrotising Enterocolitis: Objective Evaluation of the Role of Second-Look Laparotomy in Bowel Salvage and Survival

World Journal of Surgery, 2015

We investigated the role and outcome of a planned second-look laparotomy (SLL) in preserving bowe... more We investigated the role and outcome of a planned second-look laparotomy (SLL) in preserving bowel in extensive necrotizing enterocolitis (NEC). Extensive NECs managed surgically in a tertiary centre in 2006-2009 were retrospectively studied to include patients planned for an SLL. End points were bowel salvage rate and survival outcomes. Results were median (ranges), and statistical significance was P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05. In 4 years, 34 NECs required a laparotomy, and 9 extensive NECs who required an SLL were included. The gestation at birth was 27 (24-38) weeks, birth weight was 1120 (580-2835) g, and first laparotomy performed on day 34 (2-77) of life, with SLL performed 2 (1-3) days after initial laparotomy. Commonest indications for SLL were doubtful bowel viability and physiological instability. 3 died before SLL. Patients who survived to have an SLL (n = 6) had remaining small bowel length of 41 (25-70) cm, overall small bowel salvage rate 51 % (0-100 %), and 30-day survival 5/6 (83 %). Four patients survived for 1 year, their length of NICU stay was 114 (76-120) postoperative days, time on PN was 84 postoperative days (71 days-17 months), including one patient with short bowel syndrome who achieved enteral autonomy at 17 months; one late mortality had short bowel syndrome after further bowel resection for bowel obstruction, developed intestinal failure associated liver disease, and died before 1 year of life following liver transplant. SLL is a viable approach for extensive NEC. It offered bowel salvage rate of 51 % and long-term PN-free survival of 44 %, in the patient group who would have had significant risk of mortality and major morbidity.

Research paper thumbnail of Carpal scaphoid fracture in the skeletally immature: a single centre one-year prospective study

Acta orthopaedica Belgica, 2009

We report up-to-date one-year prospective data on the incidence of scaphoid fracture in skeletall... more We report up-to-date one-year prospective data on the incidence of scaphoid fracture in skeletally immature patients managed in a Fracture Clinic of a Level I Trauma Centre in a University Hospital in the UK. All scaphoid fractures were immobilised until union was achieved. One hundred twenty one skeletally immature patients were referred for a suspected fracture of the scaphoid. Fourteen patients (11.5%) did have a scaphoid fracture, an incidence of 15 per 100,000 (0.55% of all paediatric injuries referred). Mean delay in being seen was 2.6 days, and mean follow-up time was 52.3 days. Only plain radiography was used to diagnose and follow up scaphoid fractures. Fractures of the scaphoid in skeletally immature individuals are uncommon, are usually undisplaced, occur more commonly in the distal portion of the bone, and carry a good prognosis.

Research paper thumbnail of A comparison of open and laparoscopic restorative proctocolectomy in children

Pediatric Surgery International, 2009

Aim To compare laparoscopic restorative proctocolectomy (RP) in children with the same operation ... more Aim To compare laparoscopic restorative proctocolectomy (RP) in children with the same operation performed using open surgery. Methods Case-control comparison of two consecutive cohorts undergoing procto-colectomy by one surgeon. Outcome variables measured were: operating time, blood loss, hospital stay and complication rates. Results Twenty-two children, mean age 13 years, underwent RP. Eleven laparoscopic cases were compared with the preceding open case. There was no difference in operating time (443 vs. 403 min, P = 0.479), blood loss (159 vs. 417 ml, P = 0.082), complication rate (4 vs. 1) or hospital stay (14 vs. 19 days, P = 0.192). Conclusions Laparoscopic RP is feasible and safe in children. The small number of children recruited may mask benefits in blood loss and hospital stay for laparoscopic surgery.

Research paper thumbnail of Correlation of gastric heterotopia and Meckel’s diverticular bleeding in children: a unique association

Pediatric Surgery International, 2014

Meckel&#39;s diverticular bleeding is the commonest cause of major gastrointestinal (GI) haem... more Meckel&#39;s diverticular bleeding is the commonest cause of major gastrointestinal (GI) haemorrhage in children. We hypothesise that gastric heterotopia (GH) is associated with increased risk of bleeding and, therefore, may affect initial management. Paediatric patients with histological diagnosis of Meckel&#39;s diverticulum (MD) following diverticulectomy from 1995 to 2011 were reviewed retrospectively, focusing on histological correlation to bleeding. GH identified on histology was regarded as GH+, and where GH was not found was regarded as GH-. Two-tailed Chi-square test and Fisher&#39;s exact test were used, p &lt; 0.05 was statistically significant. Thirty-nine patients underwent Meckel&#39;s diverticulectomy. The number of patients with GH+, GH-, and bowel necrosis were 21, 15, and 3. Eighteen children with MD had GI bleeding. GI bleeding was more commonly associated with GH+ (14/21) than GH- (4/15) (67 vs 27 %, p = 0.02). Further analysis revealed all four GH- bleeding originated from other sources than the MD. GH+ had no significant association with other presentations. GH+ was present in 8/8 (100 %) positive Meckel&#39;s scans and 2/5 (40 %) negative scans. Meckel&#39;s scan had a sensitivity of 8/10 (80 %), and specificity of 3/3 (100 %). This study confirmed that GH positive MD is a unique entity presenting typically with GI bleeding.

Research paper thumbnail of Bracka’s staged repair of proximal hypospadias - revisiting a versatile technique

Journal of Pediatric Urology, 2012

Proximal hypospadias is associated with poorly defined urethral plate and often with chordee. A t... more Proximal hypospadias is associated with poorly defined urethral plate and often with chordee. A two-staged Bracka's repair is reproducible and has been used routinely in our practice. We present the key steps of this technique on a 13-month-old boy. Patient and method: This boy presented with proximal penile hypospadias, hooded foreskin and mild chordee. He underwent stage one Bracka's repair. The steps included: 1) Artificial erection test to define extent of chordee; 2) inner preputial graft harvest and preparation; 3) glans and urethral plate incision down to corpora cavernosa; 4) partial release of chordee by division of aberrant corpus spongiosum, without degloving of penile skin; 5) laying of preputial graft; 6) dressing. Result: The patient had catheter removed on second, and dressing removed on seventh, postoperative days, without complication. The patient is planned for second stage repair in 6 months. Our standard approach includes either removal of catheter on the second or seventh post-operative day, according to surgeon preference. The three senior surgeons have used this method in 54 patients without significant complication. The graft has taken in 100% of cases. Conclusion: The Bracka's staged repair of proximal hypospadias is a versatile technique that gives reproducible and sound results.

Research paper thumbnail of Late Diagnosis of Hirschsprung's Disease: Definition and Implication on Core Outcomes

European Journal of Pediatric Surgery, Mar 9, 2022

INTRODUCTION Late diagnosis of Hirschsprung's disease (LDHD) may carry a poor prognosis. Its... more INTRODUCTION Late diagnosis of Hirschsprung's disease (LDHD) may carry a poor prognosis. Its definition remains unclear and its implication on HD-related core outcomes has not been fully reported. METHODS A single-center 20-year series was reviewed to include HD with follow-up of 1 year or more post pull-through (PT) and aged 5 years or older. We investigated six core outcomes derived from NETS1HD study by comparing the groups dichotomized by four time points using age at diagnosis (44-week gestation, 6 months, 1 year, and 3 years). Following establishment of definition of LDHD, the outcomes and complications were compared with timely diagnosis of HD (TDHD). RESULTS Forty-nine out of eighty-six HD were included. The definition of LDHD was found to be HD diagnosed at 1 year of age or later because 3/6 core outcomes were significantly worse than TDHD. Nine patients (18%) had LDHD-median age at diagnosis 42 months (12-89) and PT performed at 57 months (12-103), mostly Soave (73%); a covering stoma was performed in 7/9, significantly higher than TDHD in 10/40 (p = 0.001). LDHD was associated with increased unplanned surgery (78% vs. 30%, p = 0.019), fecal incontinence (100% vs. 62%, p = 0.01), and permanent stoma (33% vs. 5%, p = 0.037). Major complications (56% vs. 20%, p = 0.043) and redo PT (33% vs. 5%, p = 0.037) were also higher in LDHD. CONCLUSION LDHD could be defined as HD diagnosis at or over 1 year of age. LDHD was associated with more preoperative stoma, major postoperative complications, unplanned reoperation, and worse HD-related core outcomes.

Research paper thumbnail of A Critical Analysis of Rectal Biopsy to Exclude Hirschsprung's Disease

European Journal of Pediatric Surgery, 2021

Introduction Most Hirschsprung's disease (HD) are diagnosed in young children with increased ... more Introduction Most Hirschsprung's disease (HD) are diagnosed in young children with increased risk (”red flag”). Older children (>6 months) require open rectal biopsy (ORB) with its own impact on risk and resources. We investigated if “red flag”, age, and sex used in combination could exclude HD. Materials and Methods “Red flags” are risk factors associated with HD, including neonatal bowel obstruction, genetic association, failure of passage of meconium in <48 hours, infantile constipation, distension with vomiting, or family history. All rectal biopsies (2015–2018) were reviewed for indications, methods, and histopathological findings. Logistic regression analysis was adopted to assess predictive value of “red flag,” age, and sex (p 6 months old (62 vs. 30%*), and female gender (54 vs. 16%*), compared with HD. In the absence of “red flag,” 7/82 (9%) had HD (negative predictive value = 91%). Logistic regression analysis found absent “red flag” predicted non-HD biopsy with ...

Research paper thumbnail of Coagulation Gene Expression Profiling in Infants with Necrotizing Enterocolitis

Journal of pediatric gastroenterology and nutrition, Dec 31, 2016

Coagulopathy and mesenteric thrombosis are common in premature neonates with necrotizing enteroco... more Coagulopathy and mesenteric thrombosis are common in premature neonates with necrotizing enterocolitis (NEC). This pilot study aimed to investigate the hypothesis that there are changes in the gene expression related to the coagulation and anticoagulation systems in NEC. Consecutive neonates (n = 11) with NEC (Bell's stages 2-3) were recruited. Two comparison groups, matched for birth weight and corrected gestational age, were selected based on: the absence of inflammation and coagulopathy (healthy control, n = 10); or the presence of a confirmed blood infection (sepsis control, n = 12). A pathway specific quantitative PCR array was used to determine the expression of 94 genes involved in human blood coagulation and anticoagulation cascade. Twelve genes of the coagulation and anticoagulation systems were significantly altered in the NEC patients compared to healthy controls. In particular, ELANE, CD63, PROS1, HGF, F12 were significantly up-regulated (mean fold changes +2.74, p &...

Research paper thumbnail of Coagulopathy in Necrotising Enterocolitis: Modulation of Gene Expression Microarrays Analysis to Study Gene Expression

ABSTRACT Stefano Giuliani, MD PhD1, Yew Wei Tan, MD1, Dongling Zheng, PhD2, Nigel Kennea, MD1 and... more ABSTRACT Stefano Giuliani, MD PhD1, Yew Wei Tan, MD1, Dongling Zheng, PhD2, Nigel Kennea, MD1 and Yalda Jamshidi, PhD2, (1)St.George&#39;s Healthcare NHS Trust and University, London, United Kingdom, (2)St.George&#39;s University of London, London, United Kingdom Purpose: Severe coagulopathy and thrombosis of the mesentery are common findings in advanced necrotising enterocolitis (NEC) and they are associated with high morbidity and mortality. There is no knowledge about how the expression of genes involved in the coagulation and anticoagulation cascades are altered during the advanced phases of the disease. The aim of the study was to investigate differences in the coagulation and anticoagulation gene expression between advanced NEC patients and a control group. Methods: Consecutive patients with necrotizing enterocolitis stage 2 and 3 (Bell&#39;s classification) were recruited over 6 month&#39;s period. Patients included had a birth weight &gt;800 gram and corrected gestational age (cGA) less than 37 weeks. A control group, matched for weight and cGA, was selected from infants without sepsis or coagulopathy. Each infant had a 0.5ml blood sample taken for gene expression analysis. RNA was extracted from pelleted cells for gene expression analysis using the Human Blood Coagulation 96 StellARRay qPCR array (Lonza). We employed a sensitive quantitative real-time PCR method to compare gene expression by means of a &#39;ÔHuman Blood Coagulation&#39;&#39; qPCR array. Clinical end points analysed were differences in: haemoglobin, platelet, clotting profile, laparotomy and survival between the NEC and control. Continuous data used Student t-test and Mann-Whitney U test as appropriate, nominal data used Fisher&#39;s exact test, and statistical significance was P&lt;0.05. Results: 8 controls (median gestational age 29+6 weeks, birth weight 1165g, 23 days old) and 7 NEC (median gestation 28+4 weeks, birth weight 840g, 18 days old) were included in the study. Significantly lower platelet counts were found in the NEC compared to the control group (67 v 340 x10^9/L, p=0.002). Mortality in both groups were comparable (NEC 3/7 v control 1/8, P=0.185), with emergency laparotomy indicated in one of 7 NEC patients. 10 genes associated with the coagulation and anticoagulation cascades were identified to be significantly up or down-regulated in the NEC group compared to the controls. These were genes involved in proteolysis of extracellular matrix (up-regulation of CD63, ELANE, PLAU, ASGR2), sensors of proteolysis (down-regulation of F2RL1), activation of coagulation (F12, PROC) and platelet aggregation (down-regulation of P2RY12). Conclusion: This is the first study defining significant alterations in the gene expression of the coagulation and anticoagulation systems in patients with advanced NEC. These findings support the hypothesis that coagulopathy plays an important role in advanced NEC and points to a need for further research to understand the relationship of coagulation and NEC disease progression.

Research paper thumbnail of Laparoscopic Toupet Fundoplication

Video Atlas of Pediatric Endosurgery (VAPE), 2021

Research paper thumbnail of Neonatal Surgical Conditions: Congenital Diaphragmatic Hernia and Short Bowel Syndrome

The dramatic improvements in the outcomes of neonatal surgery are due largely to advances in neon... more The dramatic improvements in the outcomes of neonatal surgery are due largely to advances in neonatal care and in that sense, all directly of interest to the neonatologist. However there are areas where our understanding of the factors affecting outcomes is still evolving and are therefore areas of intense research. The conditions we have chosen to discuss are those where optimum medical management is the outcome determinant and surgery itself is secondary. These are congenital diaphragmatic hernia and short bowel syndrome.

Research paper thumbnail of Optimal management of gastrojejunal tube in the ENFit era — Interventions that changed practice

Journal of Pediatric Surgery

Research paper thumbnail of Treatment of megarectum in anorectal malformation with emphasis on preventive aspects: 17 years experience

Pediatric Surgery International

Research paper thumbnail of Prognostic Value of the Oxygenation Index to Predict Survival and Timing of Surgery in Infants with Congenital Diaphragmatic Hernia

Journal of Pediatric Surgery

Research paper thumbnail of Morgagni hernia repair in children over two decades: Institutional experience, systematic review, and meta-analysis of 296 patients

Journal of pediatric surgery, Jan 13, 2018

Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use i... more Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use in MH repair to curb recurrence. A systematic review and meta-analysis were performed to study the recurrence and complications associated with minimally invasive surgery and the use of patch. We retrospectively reviewed all cases of MH who underwent first-time repair in 2012-2017 in our institution to determine recurrence and complication rate. A MEDLINE search related to minimally invasive surgery (MIS) and patch repair of MH was conducted for systematic review. Eligible articles published from 1997-2017 with follow-up data available were included. Primary outcomes measured were recurrence and complication. Meta-analysis to compare open versus MIS and primary versus patch repair in the MIS group were performed in comparative cohorts. Continuous data were presented as median (range), and statistical significance was P<0.05. In our institution, 12 consecutive patients aged 17-month-old...

Research paper thumbnail of Duhamel operation for children with distal colonic dysmotility

Pediatric Surgery International

To report outcomes of children with constipation refractory to medical management and manometrica... more To report outcomes of children with constipation refractory to medical management and manometrically proven distal colonic dysmotility, managed with rectosigmoidectomy followed by Duhamel operation (Duhamel). Children who underwent a Duhamel from 2009 onwards for intractable constipation and left colonic dysmotility were retrospectively reviewed. The primary end point was resolution of constipation, and secondary end point was postoperative complications. Continuous data were median (range). 11 patients (4 males) had Duhamel at 11 years (5-16) with constipation started from 2 years (1-8). Hirschsprung&amp;amp;#39;s disease was excluded. All Duhamels were performed with a covering ileostomy: 9 following a Hartmann procedure, one following a previously failed reversal of Hartmann, and one Duhamel performed with a pre-existing ileostomy. All ileostomies were subsequently closed. Median resection length was 22 cm (11-31). Length of stay was 8 days (5-23). Follow-up was 5 years (0.5-7). Age at final review was 15 years (10-18). Resolution of constipation occurred in nine patients (4 required antegrade continence enemas (ACE), 5 with laxative); two had persistent constipation and faecal incontinence despite ACE, ultimately requiring an ileostomy. Two postoperative small bowel obstructions required laparotomy. Duhamel performed in children with manometrically proven distal colonic dysmotility yielded 82% resolution of refractory constipation; half of them subsequently needed ACE.

Research paper thumbnail of The management of boys under 3 months of age with an inguinal hernia and ipsilateral palpable undescended testis

Journal of Pediatric Surgery

The optimal management for boys under 3 months of age with an indirect inguinal hernia (IIH) and ... more The optimal management for boys under 3 months of age with an indirect inguinal hernia (IIH) and ipsilateral palpable undescended testis (IPUDT) is unknown. We aimed to: 1) determine the current practice for managing these boys across the UK, and 2) compare outcomes of different treatment strategies. We undertook two studies. Firstly, we completed a National Survey of all surgeons on the British Association of Paediatric Surgeons email list in 2014. Subsequently, we undertook a multi-centre, retrospective, 10-year (2005-2015) review across 4 pediatric surgery centers of boys under 3months of age with concomitant IIH and IPUDT. Primary outcome was testicular atrophy. Secondary outcomes included need for subsequent orchidopexy, testicular ascent and hernia recurrence. Data are presented as median (range). Chi-squared test and multivariate binomial logistic regression analysis were used for analysis; p&amp;amp;amp;amp;lt;0.05 was considered significant. Survey: Consultant practice varies widely across the UK, with a tendency towards performing concurrent orchidopexy at the time of herniotomy under 3 months of age. Concurrent orchidopexy is favored less in cases where the hernia is symptomatic. Case Series Review: Forty-one boys with 43 concomitant IIH and IPUDT were identified, and all included. 32 (74%) hernias were reducible, 11 (26%) were symptomatic requiring urgent or emergency repair. Post-conceptual age at surgery was 45weeks (36-65). Primary operations included: 29 (67%) open hernia repair and standard orchidopexy, 8 (19%) open hernia repair with future orchidopexy if required, 4 (9%) laparoscopic hernia repair with future orchidopexy if required, 2 (5%) open hernia repair and suturing of the testis to the inverted scrotum without scrotal incision. Variation in atrophy rate between different surgical approaches did not reach statistical significance (p=0.42). Overall atrophy rate was 18%. If hernia repair alone was undertaken (8 open and 4 laparoscopic), the testis did not descend in 8 patients, requiring subsequent orchidopexy (67%); if orchidopexy was undertaken at the time of hernia repair, 1 in 29 required a repeat orchidopexy (3%) (p=0.0001). No hernia recurred. This study suggests that orchidopexy at the time of inguinal herniotomy does not increase the risk of testicular atrophy in boys under 3months of age.

Research paper thumbnail of Corrigendum: A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis

Scientific reports, Jul 17, 2017

Research paper thumbnail of A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis

Scientific reports, Jan 27, 2017

The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) s... more The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) surgery for revalidation of neonatal surgeons. The aim of this study was therefore to calculate the number of infants in the UK/Ireland with surgical NEC and describe outcomes that could be used for national benchmarking and counselling of parents. A prospective nationwide cohort study of every infant requiring surgical intervention for NEC in the UK was conducted between 01/03/13 and 28/02/14. Primary outcome was mortality at 28-days. Secondary outcomes included discharge, post-operative complication, and TPN requirement. 236 infants were included, 43(18%) of whom died, and eight(3%) of whom were discharged prior to 28-days post decision to intervene surgically. Sixty infants who underwent laparotomy (27%) experienced a complication, and 67(35%) of those who were alive at 28 days were parenteral nutrition free. Following multi-variable modelling, presence of a non-cardiac congenital anom...

Research paper thumbnail of Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis

The Journal of pediatrics, Jan 8, 2016

To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities ... more To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities (IRAs) in asymptomatic patients with heterotaxy. PubMed, Embase, and Cinahl were searched electronically to determine the overall incidence of IRAs in heterotaxy; the detection rate of IRAs associated with screening; the incidence of midgut volvulus in patients without screening; and the incidence of morbidity and mortality after prophylactic and emergency Ladd procedures. Relevant data were computed with a meta-analysis of proportions. Between-study heterogeneity was assessed with the I(2) statistic. From 276 papers identified, 24 studies with a total of 1433 patients with heterotaxy were included for systematic review. No randomized study was identified. True incidence of IRA in heterotaxy could not be ascertained through meta-analysis. In patients who underwent screening, the incidence of IRA was 58%. Acute midgut volvulus occurred in 5.8% of those who did not undergo screening. Posto...

Research paper thumbnail of Extensive Necrotising Enterocolitis: Objective Evaluation of the Role of Second-Look Laparotomy in Bowel Salvage and Survival

World Journal of Surgery, 2015

We investigated the role and outcome of a planned second-look laparotomy (SLL) in preserving bowe... more We investigated the role and outcome of a planned second-look laparotomy (SLL) in preserving bowel in extensive necrotizing enterocolitis (NEC). Extensive NECs managed surgically in a tertiary centre in 2006-2009 were retrospectively studied to include patients planned for an SLL. End points were bowel salvage rate and survival outcomes. Results were median (ranges), and statistical significance was P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05. In 4 years, 34 NECs required a laparotomy, and 9 extensive NECs who required an SLL were included. The gestation at birth was 27 (24-38) weeks, birth weight was 1120 (580-2835) g, and first laparotomy performed on day 34 (2-77) of life, with SLL performed 2 (1-3) days after initial laparotomy. Commonest indications for SLL were doubtful bowel viability and physiological instability. 3 died before SLL. Patients who survived to have an SLL (n = 6) had remaining small bowel length of 41 (25-70) cm, overall small bowel salvage rate 51 % (0-100 %), and 30-day survival 5/6 (83 %). Four patients survived for 1 year, their length of NICU stay was 114 (76-120) postoperative days, time on PN was 84 postoperative days (71 days-17 months), including one patient with short bowel syndrome who achieved enteral autonomy at 17 months; one late mortality had short bowel syndrome after further bowel resection for bowel obstruction, developed intestinal failure associated liver disease, and died before 1 year of life following liver transplant. SLL is a viable approach for extensive NEC. It offered bowel salvage rate of 51 % and long-term PN-free survival of 44 %, in the patient group who would have had significant risk of mortality and major morbidity.

Research paper thumbnail of Carpal scaphoid fracture in the skeletally immature: a single centre one-year prospective study

Acta orthopaedica Belgica, 2009

We report up-to-date one-year prospective data on the incidence of scaphoid fracture in skeletall... more We report up-to-date one-year prospective data on the incidence of scaphoid fracture in skeletally immature patients managed in a Fracture Clinic of a Level I Trauma Centre in a University Hospital in the UK. All scaphoid fractures were immobilised until union was achieved. One hundred twenty one skeletally immature patients were referred for a suspected fracture of the scaphoid. Fourteen patients (11.5%) did have a scaphoid fracture, an incidence of 15 per 100,000 (0.55% of all paediatric injuries referred). Mean delay in being seen was 2.6 days, and mean follow-up time was 52.3 days. Only plain radiography was used to diagnose and follow up scaphoid fractures. Fractures of the scaphoid in skeletally immature individuals are uncommon, are usually undisplaced, occur more commonly in the distal portion of the bone, and carry a good prognosis.

Research paper thumbnail of A comparison of open and laparoscopic restorative proctocolectomy in children

Pediatric Surgery International, 2009

Aim To compare laparoscopic restorative proctocolectomy (RP) in children with the same operation ... more Aim To compare laparoscopic restorative proctocolectomy (RP) in children with the same operation performed using open surgery. Methods Case-control comparison of two consecutive cohorts undergoing procto-colectomy by one surgeon. Outcome variables measured were: operating time, blood loss, hospital stay and complication rates. Results Twenty-two children, mean age 13 years, underwent RP. Eleven laparoscopic cases were compared with the preceding open case. There was no difference in operating time (443 vs. 403 min, P = 0.479), blood loss (159 vs. 417 ml, P = 0.082), complication rate (4 vs. 1) or hospital stay (14 vs. 19 days, P = 0.192). Conclusions Laparoscopic RP is feasible and safe in children. The small number of children recruited may mask benefits in blood loss and hospital stay for laparoscopic surgery.

Research paper thumbnail of Correlation of gastric heterotopia and Meckel’s diverticular bleeding in children: a unique association

Pediatric Surgery International, 2014

Meckel&#39;s diverticular bleeding is the commonest cause of major gastrointestinal (GI) haem... more Meckel&#39;s diverticular bleeding is the commonest cause of major gastrointestinal (GI) haemorrhage in children. We hypothesise that gastric heterotopia (GH) is associated with increased risk of bleeding and, therefore, may affect initial management. Paediatric patients with histological diagnosis of Meckel&#39;s diverticulum (MD) following diverticulectomy from 1995 to 2011 were reviewed retrospectively, focusing on histological correlation to bleeding. GH identified on histology was regarded as GH+, and where GH was not found was regarded as GH-. Two-tailed Chi-square test and Fisher&#39;s exact test were used, p &lt; 0.05 was statistically significant. Thirty-nine patients underwent Meckel&#39;s diverticulectomy. The number of patients with GH+, GH-, and bowel necrosis were 21, 15, and 3. Eighteen children with MD had GI bleeding. GI bleeding was more commonly associated with GH+ (14/21) than GH- (4/15) (67 vs 27 %, p = 0.02). Further analysis revealed all four GH- bleeding originated from other sources than the MD. GH+ had no significant association with other presentations. GH+ was present in 8/8 (100 %) positive Meckel&#39;s scans and 2/5 (40 %) negative scans. Meckel&#39;s scan had a sensitivity of 8/10 (80 %), and specificity of 3/3 (100 %). This study confirmed that GH positive MD is a unique entity presenting typically with GI bleeding.

Research paper thumbnail of Bracka’s staged repair of proximal hypospadias - revisiting a versatile technique

Journal of Pediatric Urology, 2012

Proximal hypospadias is associated with poorly defined urethral plate and often with chordee. A t... more Proximal hypospadias is associated with poorly defined urethral plate and often with chordee. A two-staged Bracka's repair is reproducible and has been used routinely in our practice. We present the key steps of this technique on a 13-month-old boy. Patient and method: This boy presented with proximal penile hypospadias, hooded foreskin and mild chordee. He underwent stage one Bracka's repair. The steps included: 1) Artificial erection test to define extent of chordee; 2) inner preputial graft harvest and preparation; 3) glans and urethral plate incision down to corpora cavernosa; 4) partial release of chordee by division of aberrant corpus spongiosum, without degloving of penile skin; 5) laying of preputial graft; 6) dressing. Result: The patient had catheter removed on second, and dressing removed on seventh, postoperative days, without complication. The patient is planned for second stage repair in 6 months. Our standard approach includes either removal of catheter on the second or seventh post-operative day, according to surgeon preference. The three senior surgeons have used this method in 54 patients without significant complication. The graft has taken in 100% of cases. Conclusion: The Bracka's staged repair of proximal hypospadias is a versatile technique that gives reproducible and sound results.