Is pre-operative imaging of external angular dermoid cysts in children necessary?

AIM: To investigate the frequency and modality of pre-operative imaging in children with an external angular dermoid cyst and whether this influenced subsequent management. METHODS: A retrospective study was undertaken to assess the presentation, investigation, treatment and outcomes in children aged less than 16 years with an external angular dermoid cyst. All children who underwent external angular dermoid cyst excision between January 2008 and April 2021 at a regional children's hospital were analysed. RESULTS: Sixty-one patients (32 boys) were identified. Fifty-four were managed by paediatric surgeons and seven by ophthalmic surgeons. Pre-operative imaging was performed in 19 (31%) patients, including plain radiographs (3), ultrasound scan (14), computerised tomography scan (1), and magnetic resonance imaging scan (2). None of these investigations showed evidence of an intracranial or orbital extension of the cyst or changed management. All cysts were excised under day-case general anaesthesia. There was one minor postoperative complication and two recurrences. CONCLUSIONS: External angular dermoid cysts rarely communicate through the bone with the orbit or anterior cranial fossa. Routine pre-operative imaging is unnecessary unless there are clinical features such as an atypical location (e.g. temporal), eye signs (e.g. strabismus, proptosis and globe displacement) or a draining sinus.

Esophageal length in children: Reference data and a novel predictive equation.

Minimal information exists on the length of the child's esophagus, namely the distance from the cricopharyngeus to the esophagogastric junction (EGJ). We aimed to investigate the relationship between esophageal length (EL) and the age, height, and weight of the child. Children undergoing upper gastrointestinal endoscopy between February 2019 and May 2021 at our institution were prospectively audited. Children with anatomical esophageal disorders were excluded. Endoscopic distances from the incisors to the cricopharyngeus and EGJ were obtained, and novel predictive equations derived to predict EL. Intra-observer agreement for endoscopic measurements showed an intra-class correlation coefficient of 0.99. A total of 290 children aged 0.4-17.3 years were included in the analysis; they were divided into a model development cohort (n = 261) and a model validation cohort (n = 29). Measured EL correlated best with height (r = 0.92) as compared to age (r = 0.90) or weight (r = 0.83). The optimal equation for predicting EL was 0.156 × height (cm) - 1.336 (adjusted R2  = 0.841); this had a success rate of 76% in the validation cohort. The optimal equation for predicting distance from incisors to EGJ was 0.199 × height (cm) + 6.470 (adjusted R2  = 0.889); this had a success rate of 69% in the validation cohort. This is the first study to report a predictive equation for estimating esophageal length in children. Accurate prediction of esophageal length may assist with clinical esophageal procedures in children such as nasogastric and pH probe placement.

Epigastric Hernias in Children: A Personal Series and Systematic Review of the Literature.

Epigastric hernias are relatively uncommon in children, and there is a paucity of literature on their incidence, presenting features, natural history, and surgical outcomes. A systematic review was conducted according to PRISMA guidelines. Articles describing the incidence, outcome, and interventions for pediatric epigastric hernias, both open and laparoscopic, were analyzed. Eight relevant articles published between 1975 and 2019 were included in the analysis. Of 81 children, 58% were females, 35% were symptomatic and 8% were multiple. All hernias contained preperitoneal fat only and were repaired using standard open surgery or laparoscopic techniques. No recurrences were recorded. In a personal series of 37 hernias in 36 children of median age 4 years, there were no recurrences; however, this series included two children with a recurrent or persistent epigastric hernia after surgery by others. Epigastric hernias in children are relatively uncommon. They typically contain only preperitoneal fat but more than a third are symptomatic. Standard open repair can be undertaken with minimal morbidity. Laparoscopic repair takes longer and provides a marginal cosmetic benefit.

Intestinal malrotation in extremely premature infants: a potential trap.

INTRODUCTION: Intestinal malrotation is life-threatening and often presents during infancy with bilious vomiting. The prevalence and presentation among extremely premature infants are unknown. METHODS: We retrospectively reviewed all infants born at less than 28 weeks' gestation diagnosed with symptomatic intestinal malrotation in a tertiary neonatal intensive care unit over a 10-year period (2010-2020). RESULTS: Seven of 514 (1.4%) extremely premature infants developed symptomatic intestinal malrotation during this period. All were non-syndromic. In comparison, the prevalence of symptomatic intestinal malrotation in 7382 infants ≥ 28 weeks' gestation admitted during the same period was 0.2%. Intestinal malrotation was confirmed at laparotomy in all extremely premature infants and six of seven had midgut volvulus. All but one presented with marked abdominal distension; none had bilious vomiting and only three had bilious gastric aspirates. A subacute onset with non-specific features such as recurrent apnoea and bradycardia, feed intolerance, and intermittent abdominal distension was common. All infants underwent a Ladd procedure. Two required extensive bowel resection resulting in short gut syndrome and three underwent further surgery for adhesive small bowel obstruction. One patient died at 10 months of age from respiratory failure but the others were well 1-3 years later. CONCLUSIONS: Symptomatic intestinal malrotation in extremely premature infants has a relatively high prevalence. It may present with marked abdominal distension without bilious vomiting, demanding a high index of suspicion. An atypical presentation, potential alternative abdominal pathologies, coexisting comorbidities, and concerns about survival in these fragile babies may deter the surgeon despite the opportunity of a good outcome.

A self-report questionnaire for pregnancy-related symphyseal pain.

BACKGROUND: Symphyseal pain (SP) experienced during pregnancy is a common condition that can negatively influence function and wellbeing. Despite its adverse impact on quality of life, standardised diagnostic criteria for SP as a distinct type of pelvic girdle pain (PGP) are lacking. OBJECTIVES: To develop a reliable self-administered instrument that could differentiate SP from posterior PGP in pregnant women, and ultimately be used for epidemiological or clinical purposes. METHOD: Qualitative data from 17 women (four focus groups) were used to develop a questionnaire. The questionnaire was tested against physical therapy diagnoses based on clinical assessment in 122 pregnant women with SP (n = 41), posterior PGP (n = 41) or no PGP (n = 40); 30 women repeated the questionnaire a day later to assess reliability. Multinomial logistic regression models were used to assess the performance of candidate items in distinguishing between the groups. RESULTS/FINDINGS: The single questionnaire item relating to location of worst pain (diagrammatic form) is useful for differentiating SP from posterior PGP and individuals with no PGP. The worst pain location question with the addition of the Pelvic Girdle Questionnaire provides a measure of "SP with impact", and is the best combination for distinguishing SP and posterior PGP. Test-retest reliability scores were excellent. CONCLUSION: These findings provide new opportunities for diagnosing pregnancy-related SP, and highlight questionnaire items which best differentiate SP from posterior PGP. These items could be used in future epidemiological research, and in clinical settings as a quick, effective screening tool.

Contemporary management of Hirschsprung disease in New Zealand.

BACKGROUND: The aim of this study was to report the contemporary management of Hirschsprung disease (HD) in New Zealand. METHODS: We undertook a national multi-centre retrospective review of all newly diagnosed cases of HD during a 16-year period (2000-2015). Demographics, genetic and syndromic associations, family history, radiology and histology results and surgical interventions were analysed. RESULTS: A total of 246 cases (males:females 4:1) were identified, an incidence of 1:3870 live births. Short-segment disease was present in 81.7%, long-segment disease in 8.5%, total colonic aganglionosis in 6.5% and unknown in 3.3%. HD was diagnosed by 4 weeks' corrected gestational age in 67%. Thirty cases (12%) also had Trisomy 21. Fifty-three (21.5%) patients required a repeat rectal biopsy for definitive diagnosis. A contrast enema was performed in 55% and identified the transition zone with 69% accuracy. Primary pull-through surgery was undertaken in 59% (65% of short-segment cases) at a median age of 27 days; others were initially managed by a defunctioning stoma. The commonest definitive procedure was a Soave-Boley endorectal pull-through (79%) (or similar variant). During a median follow-up of 7.4 years, six (2.5%) survivors underwent a redo pull-through, 13 (5.5%) an appendicostomy, 16 (6.8%) a defunctioning stoma and 10 never had a definitive procedure. Total colonic aganglionosis was significantly more likely to be fatal (12.5% versus 0.5%, P < 0.0005) or associated with a permanent end stoma (27.5% versus 4.5%, P < 0.0005). CONCLUSIONS: Most New Zealand born infants with short-segment HD are currently managed by primary pull-through, usually in the first months of life.

Scoliosis after thoracotomy repair of esophageal atresia: a systematic review.

Standard surgical repair of esophageal atresia/tracheoesophageal fistula (EA/TEF) is via a right posterolateral thoracotomy. A recognized complication is the later development of scoliosis. The prevalence and pathogenesis of secondary scoliosis are poorly understood. We, therefore, conducted a systematic review on this topic. All English language articles reporting incidence, outcomes and/or interventions for scoliosis in children after EA repair via thoracotomy were identified. Fourteen relevant articles published between 1969 and 2019 reporting 1338 children were included in the analysis. The aggregate prevalence of scoliosis among 937 children without congenital vertebral anomalies was 13%, but this figure varied widely between studies. Severity of scoliosis was documented in 181 children; eight children had a Cobb angle > 40° and 10 had undergone spinal surgery. The spinal curvature in affected individuals was dominantly or exclusively convex to the left. In conclusion, the reported prevalence of scoliosis varies widely but on average affects about one in eight children after open repair of EA/TEF. Most cases are mild and do not require intervention. It is currently uncertain whether secondary scoliosis is preventable by using meticulous thoracotomy techniques or thoracoscopic repair.Level of evidence IV.

Gastric heterotopic pancreas in children: A prospective endoscopic study.

PURPOSE: To document the prevalence and variable appearance of gastric heterotopic pancreas (HP) in children undergoing upper gastrointestinal (GI) endoscopy. METHODS: A prospective 4-year study of children undergoing flexible upper GI endoscopy in a single institution. RESULTS: A total of 607 upper GI endoscopies were performed in 478 pediatric patients during the 4-year period. Eleven children (6 girls, 5 boys) aged 2.9 to 16.9 years had endoscopic features typical of gastric HP. All but one lesion was located in the gastric antrum and most appeared as an umbilicated submucosal nodule measuring 1-2 cm in diameter. Five of 13 children with repaired esophageal atresia (EA) and two of nine children with trisomy 21 had gastric HP. The prevalence of endoscopically visualized gastric HP in children without a history of EA or trisomy 21 was 1.1%. CONCLUSIONS: Gastric HP is present in about 1% of pediatric upper GI endoscopies. It is significantly more common in patients with EA and may also be associated with trisomy 21. Gastric HP typically appears as a single 1-2 cm antral submucosal nodule, usually with a central pit. Recognition of this lesion is important to avoid misdiagnosis and inappropriate treatment. LEVEL OF EVIDENCE: Level II (diagnostic).

The 3-D muscular structure of the human pancreaticobiliary junction

The musculature of the human pancreaticobiliary junction (PBJ) is implicated in several pathologies and is of significance to clinicians who perform endoscopic retrograde cholangiopancreatography (ERCP). This study sought to describe the musculature of the human PBJ by generating a three-dimensional reconstruction of histologic sections. A single pancreaticoduodenal specimen was removed en bloc from an embalmed cadaver with no pancreaticoduodenal disease. Sections were stained with Masson's trichrome and the staining pattern of muscle fibers was used to generate information regarding their location and orientation. The outline of groups of muscle fibers taken from photomicrographs of alternate thin serial sections were highlighted based upon their orientation (circular or longitudinal) and location (duodenal or papillary). Data point co-ordinates were used to create a 3-D image reconstruction.A total of 91 composite serial cross-sections were reconstructed. Circular and longitudinal muscle fibers formed a completely investing muscle layer around both the bile and pancreatic ducts, and there was a clear distinction between the intrinsic muscles of the PBJ and those of the duodenal wall. Circular fibers were particularly dense distal to the confluence of the ducts. Longitudinal fibers were incompletely distributed around the pancreaticobiliary sphincter and did not extend to the tip of the major duodenal papilla. This model supports the well-established concept of an intrinsic pancreaticobiliary sphincter composed of circular and longitudinal muscle fibers, distinct from the surrounding duodenal muscle. Targeting the distal end of the PBJ during ERCP would only partially disrupt this muscular sphincter mechanism

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Outcomes of infants born at 23 and 24 weeks' gestation with gut perforation.

PURPOSE: The provision of neonatal intensive care to infants born at 23 or 24 weeks' gestation poses medical, surgical and ethical challenges. Gastrointestinal perforation is a well-recognized complication of preterm birth, occurring most often as a result of necrotizing enterocolitis (NEC) or spontaneous intestinal perforation (SIP). Given the risk of morbidity and mortality in these 'periviable' infants, this complication may prompt transition from active management to palliative care. In our institution, the surgical care of periviable infants with gut perforation has not been dictated by gestational age. This study reports our outcomes. METHODS: A retrospective cohort analysis of integrated neonatal medical and surgical care of all infants born between 23+0 and 24+6 weeks' gestation admitted to a tertiary level neonatal intensive care unit (NICU) during a 16 year period (2002-2017). RESULTS: A total of 198 periviable neonates (73 born at 23 weeks' gestation and 125 born at 24 weeks) were admitted during the 16-year period; most were inborn with only 26 retrieved from regional centers. Twenty-six of these infants developed gut perforation: 14 SIP, 8 NEC, 3 esophageal perforation and one after reduction of an incarcerated inguinal hernia. Twelve (46%) periviable infants with gut perforation survived to discharge home, seven of whom had no/mild disability at 2-3 years corrected gestational age. Of the 198 periviable infants admitted to NICU, 116 (58%) were alive at a corrected gestational age of 2-3 years and 29 of the 56 (51%) assessed had mild or no disability. CONCLUSIONS: In the setting of combined medical and surgical care in a tertiary level NICU almost half of all periviable infants with a gut perforation survived, many with no/mild disability at 2-3 years corrected gestational age. Rigid protocols that rely on gestational age alone to guide treatment are not appropriate. These results support the contention that, when possible, extremely preterm infants should be born and cared for in units with combined medical and surgical expertise. LEVEL OF EVIDENCE: Level III cohort study.

Ethnic variations in the childhood prevalence of Hirschsprung disease in New Zealand.

BACKGROUND: Epidemiological studies have suggested that there may be ethnic variations in the prevalence of Hirschsprung disease (HD) but no study has systematically investigated this issue or potential ethnic variations in the extent of aganglionosis in HD. This study aimed to investigate this in a childhood population in New Zealand. METHODS: A multicentre national retrospective review was undertaken of all newly diagnosed cases of HD at each of the four paediatric surgical centres in New Zealand over a 16-year period (January 2000 to December 2015). Original histological, radiological and operative reports were obtained and analysed. Self-identified ethnicity was recorded from admission documents. Birth statistics were obtained from Statistics New Zealand. RESULTS: A total of 246 cases of HD were identified. The prevalence of HD was 1:3790 live births for European, 1:6610 among Maori, 1:1834 among Pacific Peoples, 1:3847 among Asian and 1:5694 among Middle Eastern. The prevalence of HD was statistically significantly greater in Pacific Peoples (P < 0.0005). The proportion of children with long-segment HD was also significantly greater in Pacific and Asian populations than others (P = 0.04). These findings were not due to differences in the proportion of familial cases of HD among the different populations. CONCLUSIONS: The prevalence and phenotype of HD varies significantly between different ethnic groups within New Zealand. This may well be related to variations in the frequencies of HD-associated gene mutations within these populations.

Thrombocytosis and portal vein thrombosis after splenectomy for paediatric haemolytic disorders: How should they be managed?

Splenectomy is indicated in selected children with haemolytic anaemia. Postoperatively, thrombocytosis occurs in at least 80% and is one of the factors implicated in the development of acute portal venous thrombosis after splenectomy in adults. A literature review shows that children are also at risk of this complication, but the incidence is low. The risk is greatest in those with particularly large spleens. Laparoscopic splenectomy does not reduce the risk of this complication. Early detection and anticoagulation usually leads to successful resolution of the thrombosis and can mitigate the risk of developing cavernous transformation of the portal vein and chronic portal hypertension. Any child with severe or unexplained postoperative abdominal pain, fever and/or vomiting after splenectomy demands urgent abdominal imaging to exclude this complication. In asymptomatic individuals, a routine Doppler ultrasound scan 1 week postoperatively is advisable if they had a particularly large spleen, longer than usual duration of surgery and/or have a marked postoperative thrombocytosis. There is no evidence for routine administration of antiplatelet drugs and/or subcutaneous heparin prophylaxis in children after splenectomy, including those who develop postoperative thrombocytosis, but children with particularly large spleens may be a subset that benefit.

Preoperative laryngotracheobronchoscopy in infants with esophageal atresia: why is it not routine?

The value of laryngotracheobronchoscopy (LTB) immediately prior to repair of esophageal atresia with or without tracheo-esophageal fistula is contentious. Currently, there is a wide range of opinion on the utility of this investigation which is reflected by huge variation in clinical practice. This review is a critical analysis of the arguments for and against performing routine LTB prior to esophageal atresia repair. Reserving LTB for selected cases only is potentially disadvantageous since it limits the surgeon's and anesthetist's familiarity with the procedure, equipment, and range of potential findings. There is sufficient evidence to suggest that routine preoperative LTB becomes the standard of care.

Acute appendicitis.

Acute appendicitis is the most common reason for abdominal surgery in children. Luminal obstruction of the appendix progresses to suppurative inflammation and perforation, which causes generalised peritonitis or an appendix mass/abscess. Classical features include periumbilical pain that migrates to the right iliac fossa, anorexia, fever, and tenderness and guarding in the right iliac fossa. Atypical presentations are particularly common in preschool children. A clinical diagnosis is possible in most cases, after a period of active observation if necessary; inflammatory markers and an ultrasound scan are useful investigations when the diagnosis is uncertain. Treatment is by appendicectomy after appropriate fluid resuscitation, analgesia and intravenous antibiotics. Laparoscopic appendicectomy is better than open appendicectomy in most cases because it is associated with less postoperative pain and a shorter hospital stay, but recovery after acute appendicitis is mostly dictated by whether the appendix was perforated or not. Management of the appendix mass remains controversial and not all affected children need an interval appendicectomy. This article discusses tips and pitfalls in diagnosis and addresses many of the controversies that surround the management of this condition.