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.

Periprosthetic fractures of the femur in primary total hip arthroplasty: a New Zealand Joint Registry analysis.

BACKGROUND: Periprosthetic femoral fractures around total hip arthroplasty (THA) are increasing annually, and are a major cause for revision surgery in the New Zealand Joint Registry (NZJR). The aim of this study was to determine the timing and risk factors for periprosthetic femoral fractures in THA requiring revision surgery in the NZJR. METHODS: All patients with a primary THA who sustained a periprosthetic femoral fracture requiring revision THA recorded in the NZJR were reviewed to determine age, gender, body mass index, American Society of Anesthesiologists (ASA) rating and femoral stem fixation (cemented or uncemented). Revision rates were calculated per 100 component years (cy), and risk factors determined in the first 90 days, and beyond. RESULTS: Between 1999 and 2017, there were 713 revision THA for periprosthetic femoral fractures. Early revision THA was significantly associated with ASA score and body mass index ≥40. There was no gender difference (P = 0.274). The revision rate in uncemented femoral stems (1.12/100 cy) was significantly higher (p < 0.001) than cemented stems (0.16/100 cy). After 90 days, the revision rate was significantly higher with increasing age (P < 0.001), males (P < 0.001) and higher ASA score (P < 0.001). The difference between cemented (0.07/100 cy) and uncemented (0.05/100 cy) stems did not reach statistical significance (P = 0.108). CONCLUSION: The rate of revision THA for periprosthetic femoral fracture is higher in uncemented femoral stems in the first 90 days, and is significantly associated with ASA score and morbid obesity. After 90 days, the rate is lower for uncemented femoral stems, but did not reach statistical significance.

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.

State targeted response to the opioid Crisis grants (opioid STR) program: Preliminary findings from two case studies and the national cross-site evaluation.

Opioid misuse is a national health crisis that requires sustained treatment, prevention, and recovery efforts. This study evaluates the innovative treatment approaches that two states - Kentucky and Missouri - implemented in their states using State Targeted Response to the Opioid Crisis Grant (Opioid STR) program funds from the Substance Abuse Mental Health and Services Administration (SAMHSA), as well as preliminary findings from the Opioid STR national, cross-site evaluation that is funded and managed by SAMHSA. The Kentucky approach discusses the Emergency Department (ED) bridge model, which links patients discharged from EDs to appropriate professional treatment and recovery services. Missouri implemented the Medication First (MedFirst) model, an evidence-based treatment for individuals with opioid use disorder (OUD). These states highlight novel approaches likely being implemented throughout the country to combat the opioid epidemic. Findings from the case studies and supported by the national evaluation indicate that key factors to successful program implementation - supportive state policies, partnerships and collaborations, and sustainability - facilitated the implementation of planned interventions. The novel approaches discussed combined with care across the continuum (prevention, treatment and recovery) and continued federal support is likely to have an impact on reducing opioid misuse across the U.S.

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).

Risk factors for periprosthetic femoral fractures around total hip arthroplasty: a systematic review and meta-analysis.

BACKGROUND: Periprosthetic fractures are becoming increasingly common, often leading to poor patient outcomes. The aim of this study was to identify risk factors associated with periprosthetic femoral fractures (PPFFx). METHODS: Two independent reviewers conducted a systematic review of the databases MEDLINE, Embase and Cochrane Library according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, from the earliest available date to December 2017. We included all clinical articles reporting more than 100 post-operative PPFFx around total hip arthroplasty (THA). Outcomes included demographics, fracture type, risk factors and time to fracture. RESULTS: A total of 12 868 PPFFx were reported across 18 eligible studies; 64% occurred following primary THA and 36% occurred after revision THA, and 66% of all fractures were in females. Vancouver B2 fractures were the most common type (39%). We found the odds of sustaining a PPFFx is significantly lower after a primary THA compared to revision THA (odds ratio 0.31, 95% confidence interval 0.24-0.40, P < 0.00001). The incidence of PPFFx is 2.96 per 1000 person-years following primary THA compared to 9.08 per 1000 person-years following revision THA (odds ratio 0.33, 95% confidence interval 0.27-0.40). The time to fracture was 6.03 years following primary THA and 4.08 years following revision THA. Gender and cementation did not significantly affect the odds of fracture. CONCLUSION: The odds of sustaining a PPFFx following revision THA is three times greater compared to primary THA. Other risk factors including gender and cementation did not affect the odds of fracture.

Pectoralis Major Rupture in an Active Female.

Pectoralis major rupture was historically a rare occurrence, but the incidence is increasing. Most cases occur from indirect trauma in active men aged 20 to 40 years, especially during bench press. Pectoralis major rupture has never been reported in the literature in a woman in this age group. We report the first case of pectoralis major rupture in a young, active woman who underwent successful surgical fixation.

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.