Major surgical conditions of childhood and their lifelong implications: comprehensive review.

BACKGROUND: In recent decades, the survival of children with congenital anomalies and paediatric cancer has improved dramatically such that there has been a steady shift towards understanding their lifelong health outcomes. Paediatric surgeons will actively manage such conditions in childhood and adolescence, however, adult surgeons must later care for these 'grown-ups' in adulthood. This article aims to highlight some of those rare disorders encountered by paediatric surgeons requiring long-term follow-up, their management in childhood and their survivorship impact, in order that the adult specialist may be better equipped with skills and knowledge to manage these patients into adulthood. METHODS: A comprehensive literature review was performed to identify relevant publications. Research studies, review articles and guidelines were sought, focusing on the paediatric management and long-term outcomes of surgical conditions of childhood. The article has been written for adult surgeon readership. RESULTS: This article describes the aforementioned conditions, their management in childhood and their lifelong implications, including: oesophageal atresia, tracheo-oesophageal fistula, malrotation, short bowel syndrome, duodenal atresia, gastroschisis, exomphalos, choledochal malformations, biliary atresia, Hirschsprung disease, anorectal malformations, congenital diaphragmatic hernia, congenital lung lesions and paediatric cancer. CONCLUSION: The increasing survivorship of children affected by surgical conditions will translate into a growing population of adults with lifelong conditions and specialist healthcare needs. The importance of transition from childhood to adulthood is becoming realized. It is hoped that this timely review will enthuse the readership to offer care for such vulnerable patients, and to collaborate with paediatric surgeons in providing successful and seamless transitional care.

Is Roux-en-Y Feeding Jejunostomy a Safe and Effective Operation in Children? A Systematic Review Exploring Outcomes.

OBJECTIVES: Roux-en-Y jejunostomy (REYJ) may establish feeding in children with foregut dysmotility or severe gastro-esophageal reflux disease (GERD). Nevertheless, concerns have been raised about safety and efficacy. We, therefore, evaluated outcomes of REYJ by systematic review to determine if this was a satisfactory option for achieving enteral autonomy in children with complex nutritional needs. METHODS: A PRISMA-adherent systematic review was conducted of studies reporting children undergoing feeding REYJ. Two authors performed processes independently; the senior author resolved disagreements. Embase, CINAHL and Medline were searched (inception-01/21). Additional databases, references, and 'grey' literature were searched. Methodological Index for Non-randomized Studies (MINORS) and a bespoke system assessed methodological quality. RESULTS: Of 362 articles, 10 met eligibility criteria (9 retrospective series; 1 conference proceeding). Unpublished data were also attained. Interobserver agreement for MINORS (kappa = 0.47) and bespoke scoring (kappa = 0.58) were moderate. After consensus, median MINORS score was 37.5% (IQR 6.3%) and bespoke 50% (IQR 20.8%), indicating poor methodological quality. One hundred sixty-four patients were reported (age range: 2 months to 19 years). Time to full feeds and length of stay were inadequately reported but most achieved enteral autonomy. No studies reported patient/caregiver-questionnaires. Seventy-six complications were documented (Clavien-Dindo grading was infeasible). Morbidity included peristomal leakage (N = 26), internal hernia/volvulus (N = 8), and SSI (N = 7). Thirty-eight patients died (2 procedure-attributable) during follow-up (range: 1 month to 15 years). CONCLUSIONS: Up to 50% patients experience complications after REYJ (often minor) with 23% patients dying during follow-up, often comorbidity-attributable. REYJ can achieve enteral autonomy although parents/caregivers of children should be counselled accordingly.

Retrospective analysis of testicular outcomes following laparoscopic two-stage Fowler Stephens orchidopexy.

AIM OF THE STUDY: Few large series report outcomes for laparoscopic two-stage Fowler Stephens orchidopexy (LFSO). The aims were to evaluate testicular outcomes and to identify factors predictive of successful outcome. METHOD: A retrospective case series of children undergoing laparoscopic surgery for impalpable testes between May 1996 and March 2018 was reviewed. Data were collected from case and operative records. The primary outcomes of interest were testicular atrophy or re-ascent. Regression analysis was conducted to identify factors predictive of successful outcome. Data was expressed as median (IQR). RESULTS: Of 279 patients (300 testes) undergoing laparoscopy for impalpable testis, 114 patients (128 testes) underwent LFSO. Eighty-five patients (96 testes) had adequate follow-up available (53 left; 43 right). Age at first stage was 19 (IQR 13-36) months. Fifteen children had relevant co-morbidities. Time between procedures was 7 (IQR 6-8) months. Longest follow-up available was 12.5 months (IQR 6.8-19.8). Atrophy occurred in 8 testes (8.3%), and ascent occurred in 6 (6.3%). No factors were significantly predictive of success, although a trend towards atrophy was seen amongst testes undergoing gubernacular division compared with a gubernaculum-sparing technique (p = 0.06; OR 3; 95% CI 0.97-9.3). CONCLUSION: A successful outcome was seen amongst 82 of 96 testes (85%) undergoing LFSO, similar to previous reports. No factors were identified that significantly predicted outcome. Number of adverse outcomes was limited (hence possibility of type II error), and therefore preservation of the gubernaculum may reduce risk of testicular atrophy. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: Level IV.

Spuriously raised serum amylase in a five-year-old girl: a note of caution in paediatric blood sampling.

INTRODUCTION: Capillary (finger prick) blood sampling is commonplace in paediatric practice but this method is prone to produce spurious laboratory results. CASE PRESENTATION: A five-year-old girl presented with abdominal pain, epigastric tenderness, tachycardia and reduced oxygen saturation. A venous blood sample haemolysed, and serum amylase on a finger prick sample was reported as 2831 units/L. The working diagnosis was acute pancreatitis and respiratory tract infection. A repeat amylase 9 h later was within the normal range. The patient was known to bite her fingers and the possibility of salivary contamination was considered. Serum isoenzyme analysis confirmed presence of high salivary amylase levels with no pancreatic amylase detected. A viral respiratory tract infection and buried gastrostomy bumper were eventually thought to account for the patient's presentation. CONCLUSION: Increased awareness of the potential for salivary contamination of serum amylase in finger prick samples may prevent misdiagnoses of pancreatitis.

Quantification of urinary sodium concentrations in term infants.

BACKGROUND: Infants with gastrointestinal conditions and poor weight gain are administered sodium supplementation based on urinary sodium concentrations. However, the reference range of urinary electrolytes is unknown. The aim of this study was to ascertain the normal values of urinary electrolytes in healthy, term infants. Secondary aims were to establish the relationship between urinary electrolytes with weight velocity and feeding practices. METHODS: Healthy, term (≥37 weeks' gestation) infants were recruited. Parental questionnaires were completed before discharge and at six weeks. Electrolytes were quantified from a urine sample at six weeks. t-Tests and Mann-Whitney U tests were conducted for parametric and non-parametric electrolytes, respectively. RESULTS: A total of 200 infants were recruited before discharge. Twenty-nine follow-up questionnaires and urine samples were returned (nine female; mean gestational age 39 + 6 weeks [SD 9.9 days]; mean birthweight 3350 g [SD 483 g]; 17 breastfed, nine formula and three mixed; mean change in Z score for weight -0.914 [SD 0.814]). Majority (25/29) of infants had urinary sodium <20 mmo/L. Change in Z score for weight was similar between infants with sodium <20 mmol/L and >20 mmol/L ( P = 0.78). All exclusively breastfed infants had sodium <20 mmol/L, however, not statistically dissimilar to formula-fed infants ( P = 0.27). CONCLUSION: Most term infants in this study had urinary sodium values <20 mmol/L with no identified relationship to weight velocity. Lower concentrations of sodium could be not quantified reliably because of the limitations of the analytical method that were used. More evidence is required to identify candidates for sodium supplementation.

Major complications of jejunal feeding in children.

AIM OF THE STUDY: The aim of the study was to identify major gastrointestinal complications associated with direct jejunal feeding. We hypothesized that jejunal feeding may cause life-threatening surgical complications in a minority of patients. METHODS: All patients undergoing jejunal feeding between 1/2008 and 1/2018 at a pediatric surgical unit were identified retrospectively. Data sought from records included demographics, comorbidities, indications, feeding strategies, adverse events, and follow-up. Major surgical complications were defined by Clavien-Dindo grade ≥ IIIb and involving the GI tract (excluding changes of jejunal tube). MAIN RESULTS: 197 patients were identified (110 female). Median age (IQR) at initiation of jejunal feeding months was 5.6 (6-164) months. 122 were neurologically impaired. The most frequent indications were: GERD/gastroparesis (n = 114), prophylaxis/treatment of Superior Mesenteric Artery (SMA) syndrome (N.B. our center is a national spinal deformity unit) (n = 47), congenital anomalies of aerodigestive anatomy (n = 17), and malignancy (n = 7). 125 patients were managed with nasojejunal feeding alone: gastrojejunal tube (n = 51) and via Roux-en-Y jejunostomy (n = 21). There were 14 significant gastrointestinal complications (n = 11 grade > IIIb) identified among 12 patients, of whom 8 required bowel resections, and 2 died as a result: nonmechanical bowel ischemia (n = 7), intussusception (n = 4), and volvulus (n = 3). CONCLUSION: This series highlights the major complications of jejunal feeding, including a significant yet underreported risk of gut compromise. Patients undergoing jejunal feeding had a 6.1% risk of developing major surgical complications (of note, 3.6% developed bowel ischemia of unknown etiology). Susceptible children were comorbid, fragile, and neurologically impaired. These findings should influence parental discussions and informed consent before embarking upon jejunal feeding. LEVEL OF EVIDENCE: Level IV prognosis study.

George Macaulay: A short biography and his place in the history of congenital diaphragmatic hernia.

George Macaulay (1716-1766) was a Scot admired for his work as an obstetric physician, philanthropist, author, and feminist, but whom history has largely forgotten. In a time rampant with misogyny, Macaulay empathized with women, discouraged sexism within institutions, and contributed to the training of midwives. He spent much of his career working at the British Lying-in Hospital in London. Perhaps most importantly, he worked tirelessly for this hospital, contributed to various medical innovations, and reported several medical cases throughout his career, including that of congenital diaphragmatic hernia, almost a century before the anatomist whose eponymous name it bears. The purpose of this article is to reappraise the life, achievements, and specifically, the role of George Macaulay in our understanding of CDH.

Minimally invasive surgery for superior mesenteric artery syndrome: a case report and literature review.

INTRODUCTION: Superior mesenteric artery syndrome is a rare condition characterised by nausea, vomiting, postprandial pain, anorexia and early satiety. Conservative management is tried initially, but if this fails, surgery is indicated. There are few reports in the literature concerning superior mesenteric artery syndrome in children, and fewer still managed surgically by minimally invasive means. CASE PRESENTATION: A 12-year-old girl presented with weight loss, early satiety and vomiting after corrective scoliosis surgery. After upper gastrointestinal endoscopy, contrast study and computed tomography imaging, a diagnosis of superior mesenteric artery syndrome was made. Conservative management by nasojejunal feeding failed; therefore, a laparoscopic duodeno-jejunostomy was undertaken. At follow-up, her symptoms had improved. CONCLUSION: This report describes the youngest child to undergo laparoscopic duodeno-jejunostomy for superior mesenteric artery syndrome. Laparoscopic duodeno-jejunostomy appears to be the most widely employed and reliable minimally invasive approach to superior mesenteric artery syndrome with a high success rate and acceptably low complication rate.

Is Screening of Intestinal Foregut Anatomy in Heterotaxy Patients Really Necessary?: A Systematic Review in Search of the Evidence.

OBJECTIVE: (1) Is screening of intestinal rotational anatomy obligatory in "asymptomatic" patients with heterotaxy? (2) Does detection of an anomaly warrant surgical correction? SUMMARY OF BACKGROUND DATA: Heterotaxy is an abnormal arrangement of thoraco-abdominal viscera across a left-to-right axis. Intestinal rotational anomalies are frequent among patients with heterotaxy, but debate exists as to whether they are benign in nature, requiring careful observation alone, or if surgical correction is warranted to prevent obstruction or midgut volvulus. METHODS: A systematic review [according to PRISMA guidelines] was conducted using CINAHL, EMBASE, Medline, and Cochrane Databases. Article quality was assessed using MINORS criteria. Conference proceedings and unpublished data were screened additionally. RESULTS: Nineteen studies met the eligibility criteria but reporting was adequate for 9. All were observational studies. These included a total of 414 patients managed expectantly, that is, "asymptomatic patients" in whom no intestinal rotation screening was undertaken (group A), 191 cases in whom screening was performed routinely (group B), and 92 patients considered "symptomatic" of potential rotational anomalies and therefore underwent imaging or laparotomy (group C). In group A, 1 patient developed symptoms attributable to malrotation in whom laparotomy confirmed the diagnosis (0.24%). Among groups B and C, 151 had Ladd's operations (53%) and 14 cases of malrotation with obstruction or volvulus were described (4.9%), of which 2 "symptomatic patients" died before laparotomy. Overall surgical complication rate was 17% with 30-day mortality rate of 2.6% to 4.6%. CONCLUSION: The evidence base for screening "asymptomatic" patients is weak especially considering the life-limiting comorbidities.

Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre.

BACKGROUND: The association of heterotaxy with intestinal rotation anomalies is well described. However debate exists with regard optimal management notably should 'asymptomatic' bowel rotation anomalies undergo operation? The present study therefore sought to determine: (1) the risk(s) of volvulus in patients diagnosed with heterotaxy and (2) define morbidity associated with operation for 'asymptomatic' anomalies in a fragile patient cohort with co-existent congenital heart disease. METHODS: Medical case record reviews of ALL heterotaxy patients born during January 1993-December 2013 and attending a UK paediatric centre were analyzed. RESULTS: Of a total of 92 patients, 16 (17.4%) cases underwent foregut imaging studies. Three examinations were performed in 'symptomatic' patients. Twelve studies reported 'abnormal anatomy' with only five patients undergoing surgical correction. No complication(s) were recorded after Ladd's operation to correct defects. A single fatality occurred within 30 days postoperatively from cardiac failure. In 87 patients in whom Ladd's operation was not undertaken, no single patient developed intestinal volvulus (median length of follow-up 27.2 months, total 446.1 person years). CONCLUSION: This study strongly supports a 'watchful waiting' policy for heterotaxy patients. Many children who ultimately die from heart disease may avoid unnecessary abdominal surgery.

Chemical stability of heparin, isopropanol, and ethanol line lock solutions.

BACKGROUND: Ethanol line locks are used in the US to prevent catheter associated bloodstream infections. Heparin precipitates in solution with ethanol. However, isopropanol may reduce precipitate formation. We aimed to determine the chemical stability of heparin, isopropanol, and ethanol line lock for a 10 day period at 2-8°C and 25°C. METHODS: Forty samples were prepared for analysis. Each sample was prepared identically using a 5 ml syringe capped with a Combi-stopper: 1 ml 70% isopropanol, 1 ml 70% ethanol, and 1 ml heparin sodium 10 IU/ml. Twenty syringes were stored at 2-8°C and 20 at 25°C. Analysis was carried out on days 1, 3, 6, 8, and 10 with a single syringe from each condition being tested in duplicate. Samples were assessed visually. Sub-visible particle count analysis was carried out using a CLIMET particle counting system. Heparin concentration was analysed using an anti-Xa assay. Ethanol and isopropanol concentrations were analysed by gas chromatography. RESULTS: Samples remained clear and colourless throughout the study. Sub-visible particle counts remained within limits specified in British Pharmacopoeia 2013 when stored at 2-8°C and 25°C, 60% humidity for up to 10 days. There was no significant change in ethanol or isopropanol concentration during the study. However, heparin activity fell by >10% after 1 day storage and to 65% of original activity after 10 days. CONCLUSIONS: This study shows that addition of isopropanol to heparin and ethanol prevents precipitation. However, this solution shows a progressive decline in heparin activity over time making it unsuitable for extended shelf life.