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In hypospadias surgery, pre-operative hormonal therapy (PHT) is primarily used to increase penile dimensions and the vascularity of tissues available for reconstruction, but its use is non-uniform in clinical practice, with no consensus on application or utility. This review aims to summarise: (i) the penile tissue response to hormone therapy, (ii) its impact on hypospadias surgery outcomes, and (iii) the endocrinological considerations and sequelae. PHT is more often indicated for complex cases such as proximal hypospadias, hypospadias with microphallus and hypospadias reoperations. While PHT has clear effects on penile morphometry, and more recent controlled trials suggest improved surgical outcomes, the lack of consistent outcome definitions and generally inadequate follow-up periods continue to consign many of the potential long-term effects of PHT to the unknown. There is currently insufficient robust evidence to allow a clinical guideline to be constructed. The need for a well-powered multi-centre prospective randomised trial to address this question is evident but awaits a unified consensus on issues surrounding the understanding of aetiology, classification of hypospadias morphology, definition of important prognostic variables and uniform application of outcome measures. The effects of PHT may be utilised to improve outcomes in cases of proximal and severe hypospadias, which under the current paradigm represent a significant surgical challenge.

The operations involved in the repair of complete bladder exstrophy (CBE), familiarly known as the Kelly procedure, evolved over more than 100 years. Through repeated cycles of trial and error, some of the most prominent urologists in the world developed techniques that addressed each of the formidable surgical challenges presented by CBE and epispadias. A key figure is Justin H. Kelly of the Royal Children's Hospital, Melbourne, who made surgery for CBE his life's work. He took the lessons of his surgical predecessors, giants like: Friedrich Trendelenburg, Hugh Hampton Young, John Dees, and Guy Leadbetter, applied techniques for anorectal anomalies from his contemporary Alberto Pena, and saw his procedures improved by the next generation of leaders in paediatric urology that included Phillip Ransley, Peter Cuckow, Patrick Duffy, and John Gearhart. Over his long career, Mr Kelly modified and perfected his eponymous procedure patient-by-patient through a painstaking process of trial-and-error, bearing with his young patients and their families through every heart-breaking complication, and gradually creating the standard operation for children with CBE and epispadias.

Neurogenic bladder–sphincter dysfunction constitutes the major cause for morbidity in children with myelomeningocele, and the management of this condition is challenging and requires a multidisciplinary team approach. A contemporary review of the literature is presented with expert commentaries, focusing on the definition of neurogenic bladder, the natural evolution, diagnostics (highlighting investigations and their interpretation), medical and surgical treatment, prognosis, and protocols for follow-up. These aspects are illustrated with a clinical case, describing the presentation, investigation, and subsequent management of this neurogenic bladder patient.

AIM The risk of organ loss is increased in children with testicular torsion or intestinal volvulus if surgical management is not expedient. The current retrospective study aims to review the time-course from first symptom to 'knife to skin' in these conditions, to determine where delays occur and facilitate a systems approach to better manage these children. METHODS One hundred consecutive paediatric cases of scrotal exploration for presumed testicular torsion, and 100 neonatal cases presenting with possible malrotation/volvulus were analysed to evaluate the exact time-course of events from admission to surgery. RESULTS (i) Scrotal exploration: the median time from onset of symptoms to presentation was 12 h (interquartile range (IQR): 5-48 h). In children over 5 years of age, 36% (33/93) were transferred from an external district service area. (ii) Malrotation/volvulus: the median duration of symptoms prior to arrival/assessment was 12 h (IQR: 4-24 h). The median cumulative in-hospital time was over 6 h (368 min, IQR: 247-634 min). CONCLUSIONS Time to presentation contributes significantly to testicular ischaemic time. This delay to timely surgical intervention is multi-factorial, and must be addressed at a public health level. Support and training in the management of testicular torsion should be provided to all adult surgeons/trainees that may care for these children. In general, this condition is best managed at the presenting hospital whenever appropriate expertise is available. Novel pathways that streamline care may improve efficiency at an institutional level. Addressing issues of access to specialised neonatal surgery is more vexed on account of the tyranny of distance, and the pre-requisite level of surgical expertise required.

Introduction The bell-clapper deformity (BCD) predisposes to intravaginal torsion (IVT) and is classically bilateral. The precise pathological definition of what constitutes a BCD is not clear. The current study aims to clarify the specific anatomic details of this anomaly. Methods A systematic review was performed utilizing the PRISMA principles. Studies are presented chronologically based on their level of evidence. They are further divided into study types: autopsy and operative studies of acute torsion, intermittent torsion and studies of the contralateral testis in vanishing testis. Results The bell-clapper deformity is best defined by complete investment of the testis, epididymis and a length of the spermatic cord by the tunica vaginalis. Based on autopsy studies the rate of BCD in scrotal testis varied from 4.9% to 16%; with bilaterality in 66%–100%. In cases of acute IVT bilaterality was noted in 54%–100%. The most disparate results were in cases of testicular regression syndrome where contralateral BCD was noted in 0%–87% of cases. Conclusion We suggest future studies employ the strict anatomical definition above. As there is evidence of age-dependent investment of the testes, it will be important to develop age-standardized measurements of intravaginal length of spermatic cord. This critical morphometric measurement will allow a better understanding of the risk of IVT. Level of evidence Systematic review of diagnostic studies: lowest level of evidence of included manuscripts Level IV (case–control studies with a poor reference standard).

Objective: Advances in percutaneous nephrolithotomy (PCNL) toward less invasive treatment options and miniaturisation of equipment have particular application in paediatric patients. The current study aims to establish the safety and efficacy of the modified-supine position in children and a selective “tubeless” approach to paediatric PCNL.Methods: A retrospective cohort study was performed of all paediatric patients undergoing PCNL at our tertiary institution over 10 years (2010-2019).Results: Of 635 PCNL patients, 20 were paediatric (3%). Staghorn calculi (complete or partial) made up 35% of the population. The majority of procedures were performed in the modified-supine position (85%). Median procedure time was 2.1 hours, and length of stay was 2.2 days. With regards post-operative drainage, a ureteric stent was not placed in 75% of cases. The stone free rate was 65%. The median follow-up time was 8.7 years (IQR 6.7-9.9 years).Conclusion: As a result of technical and technological advances, PCNL can be considered a safe and effective procedure in children with a large stone burden, in situations of anatomic complexity or when immediate stone-free status is required. Selectively reducing post-operative drainage and utilising a modified supine position is safe, efficacious and may provide additional benefits in children.

The role of the urologist in paediatric kidney transplantation has evolved alongside advances in management for the various causes of end-stage kidney disease. Improvements in antenatal intervention and postnatal care have seen children with increasingly complex urological anomalies survive until transplant. Once solely responsible for the oversight of a child's surgical care, the paediatric urologist now works within a multidisciplinary transplant team, alongside transplant surgeons, paediatric nephrologists, transplant coordinators, psychologists, social workers, and transitional care specialists. We sought to identify available pretransplant evaluation frameworks to guide urological preparation and decision-making. Drawing from available evidence and reflecting on multi-institutional experience, we propose a streamlined approach to urologic assessment, which recognises that optimal transplant outcomes in this heterogenous cohort require lower urinary tract dysfunction to be carefully defined preoperatively.

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

BACKGROUND The aim of this study was to describe the complication profile of augmentation cystoplasty in contemporary paediatric urology as well as its effect on bladder metrics. METHODS Consecutive operative cases were retrospectively reviewed at a single institution over 20 years (1999-2019). Short- and long-term outcomes and complications following augmentation cystoplasty were defined. RESULTS Of the 71 operative cases; the most common underlying diagnoses were neurogenic bladder (34%), exstrophy-epispadias complex (30%) and posterior urethral valves (23%). The most common tissue-type utilized was ileal (58%) and ureteric (30%). Peri-operative urine leak affected nine (13%) children but reservoir perforations were less common (4%). Mean end-of-study detrusor pressure improved significantly following bladder augmentation (38-17 cmH2 O, P

Background The long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years. Methods The Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948–2017). Data included presentation, diagnostic workup, surgical management, and outcomes. Results Of 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%. Conclusions We have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable. Level of evidence IV

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 Māori, 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

There appear to be various patterns of sidedness with relation to the common urogenital malformations observed in pediatric urology. The objective of this statistical review was to synthesize this data and to assess if these patterns are significant.Eighteen urogenital conditions were investigated and for each condition the five largest studies that noted laterality were included. The sidedness of each condition was then analysed for statistical significance.Three conditions had a statistically significant higher proportion on the right side: palpable undescended testis (63%, p = 0.0002), inguinal hernia (59%, p = 0.0001) and hydrocele (60%, p = 0.003). Three conditions were significantly more common on the left side: impalpable undescended testis (59%, p = 0.0008), renal agenesis (54%, p = 0.02) and vesico-ureteric junction obstruction (71%, p 0.0001) while both pelvi-ureteric junction obstruction (62%, p = 0.09) and absent vas deferens (61%, p = 0.11) were trending towards significance.Various urogenital malformations display a predilection for one side. Proximal malformations tend to be more frequently seen on the left side, where as inguinoscrotal malformations are more frequently observed on the right. There is an increasing body of literature regarding aetiological factors for these conditions. However, our current understanding of the pathophysiology of these conditions does not completely explain this pattern of observation.

Over the last hundred years, idiopathic hypertrophic pyloric stenosis has undergone an evolution in treatment, with subsequent improvements in outcome. Initially, it was treated by physicians with antispasmodics and various alternative feeding and resuscitation modalities. The evolution of surgical approaches led to a revolution in outcome, from almost certain death to complete cure. The progression of surgical and medical treatments is reviewed in this article until, ultimately, Ramstedt's pyloromyotomy is reached. This distilled operation has remained the gold-standard procedure for over a century.

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.

Purpose Venous malformation (VM) has variable presentations. This study stratifies VM by its topographic distribution, types of tissues involved, proportion of affected patients undergoing active treatment and the types of treatment(s) administered within a closed population. Methods Consecutive VM patients were identified from the prospectively maintained vascular anomalies database 1996–2015 at our national Vascular Anomalies Centre. Demographic data of the patients and the characteristics of their VM were obtained from the database and supplemented by review of medical records. Results Among 1907 vascular anomalies patients, 316 (16.6%) had VM including 191 females and 125 males, aged 2–93 (mean, 34) years. Twelve (3.8%) patients had multiple VMs with four (1.3%) patients having a family history of VM. The lesion(s) was located in the head and neck (n = 152), lower limb (n = 80), upper limb (n = 61), trunk (n = 13), lower limb girdle (n = 11), perineal (n = 10), upper limb girdle (n = 6) and pelvic (n = 1) regions. Two anatomic sites were affected in 10 patients, and 3 sites in 6 patients. The lesion(s) affected the cutaneous/subcutaneous tissue (n = 211), muscle (n = 103), mucosa (n = 79), orbit (n = 14), parotid gland (n = 11), joint (n = 6), bone (n = 5), and intracranial vessels (n = 3). The management included observation (n = 133), surgery (n = 101), ethanol sclerotherapy (ES, n = 79) and low-dose aspirin (n = 32). Fifteen patients received both surgery and ES. Conclusions VM affects different anatomic regions and tissues. Management of VM should be individualised for each patient who may require more than one treatment modality.

Accurate knowledge of surface anatomy is fundamental to safe clinical practice. A paucity of evidence in the literature regarding thoracic surface anatomy in children was identified. The associations between surface landmarks and internal structures were meticulously analyzed by reviewing high quality computed tomography (CT) images of 77 children aged from four days to 12 years. The results confirmed that the sternal angle is an accurate surface landmark for the azygos-superior vena cava junction in a plane through to the level of upper T4 from birth to age four, and to lower T4 in older children. The concavity of the aortic arch was slightly below this plane and the tracheal and pulmonary artery bifurcations were even lower. The cardiac apex was typically at the 5th intercostal space (ICS) from birth to age four, at the 4th ICS and 5th rib in 4-12 year olds, and close to the midclavicular line at all ages. The lower border of the diaphragm was at the level of the 6th or 7th rib at the midclavicular line, the 7th ICS and 8th rib at the midaxillary line, and the 11th thoracic vertebra posteriorly. The domes of the diaphragm were generally flatter and lower in children, typically only one rib level higher than its anterior level at the midclavicular line. Diaphragm apertures were most commonly around the level of T9, T10, and T11 for the IVC, esophagus and aorta, respectively. This is the first study to provide an evidence-base for thoracic surface anatomy in children. Clin. Anat. 30:788-794, 2017. © 2017Wiley Periodicals, Inc.

Summary Fetal megacystis is variably defined and understood. The literature on fetal megacystis was systematically reviewed, focusing on prenatal diagnosis, associations and outcomes. This yielded a total of 18 primary references and eight secondary references. Fetal megacystis has an estimated first-trimester prevalence of between 1:330 and 1:1670, with a male to female ratio of 8:1. In the first trimester, megacystis is most commonly defined as a longitudinal bladder dimension of ≥7 mm. Later in pregnancy, a sagittal dimension (in mm) greater than gestational age (in weeks) + 12 is often accepted. Megacystis can be associated with a thickened bladder wall, which has been objectively defined as >3 mm. Oligohydramnios is present in approximately half of all cases. The most common underlying diagnosis is posterior urethral valves (57%), followed by urethral atresia/stenosis (7%), prune belly syndrome (4%), megacystis-microcolon-intestinal-hypoperistalsis syndrome (MMIHS) (1%), and cloacal anomalies (0.7%). Karyotype anomalies are found in 15%, and include trisomy 18, trisomy 13 and trisomy 21. Ultrasound imaging alone is often insufficient to enable a definitive diagnosis, although it may indicate that a specific diagnosis is more likely. Overall, about 50% of reported fetuses with megacystis are terminated, but this proportion varies considerably between countries and over time. Prognostic stratification is evolving, with the most important factors being oligohydramnios, gestational age at diagnosis, degree of bladder enlargement, renal hyperechogenicity, karyotype, and sex. Conclusions This review demonstrated some consensus on the ultrasound criteria for defining fetal megacystis, and illustrated the spectrum of pathologies and their relative frequencies that can cause this condition. It also underlined important associated karyotype anomalies. To progress understanding of the natural history of enlarged fetal bladders, more accurate diagnostics are required, and risk stratification needs to be refined to facilitate prenatal counseling.

The aortic valve (AV) has been used as a surrogate marker for the superior vena cava-right atrium (SVC-RA) junction during the placement of central venous catheters. There is a paucity of evidence to determine whether this is a consistent finding in children. Eighty-seven computed tomography scans of the thorax acquired at local children's hospitals from April 2010 to September 2011 were retrospectively collected. The distance between the SVC-RA junction and the AV was measured by dual consensus. The cranio-caudal level of the junction and the AV were referenced to the costal cartilages (CCs) and anterior intercostal spaces (ICSs). The results confirmed that the SVC-RA junction has a variable relationship to the AV. The junction was on average 3.1 mm superior to the AV. This distance increased with age. In the1-year-old age group, the junction was on average 1.3 mm superior to the AV (range: -6 to 11 mm). In the 1-2 years old age group: 3.5 mm (range: -8 to 15 mm). In the 3-6 years old: 3.8 mm (range: -9 to 13 mm). In the7 years old age group: 4 mm (range: -11 to 16 mm). The surface anatomy of the SVC-RA junction was variable, ranging from the second ICS to sixth CC. The SVC-RA junction has a predictable relationship to the AV, and this can be used as an adjunct marker for accurate placement of central venous catheters except in the smallest neonates. Clin. Anat. 32:778-782, 2019. © 2019 Wiley Periodicals, Inc.

Horseshoe kidneys are a common, yet enigmatic, renal malformation. This review critically appraised the literature surrounding the embryology, etiology and clinical anatomy of horseshoe kidneys. The systematic literature search produced 104 articles, and 56 primary and further secondary references. There were several etiological theories regarding horseshoe kidneys. The established view was that during ascent, the kidneys come into close apposition as they pass through an arterial fork. Another possible mechanism related to lateral flexion of the trunk or rotation of the caudal embryo; the association of asymmetrical horseshoe kidneys with a number of vertebral conditions supported this hypothesis. More recent animal models implicated the notochord and sonic hedgehog signaling. Furthermore, it has been suggested that the isthmus may be the result of ectopic mesenchymal tissue. Surgical anatomy of the horseshoe kidney is complex, due to variability in location, orientation and blood supply. Both arterial and venous anatomy is highly variable. This raised the question of whether anomalous blood supply is the cause or result of abnormal renal position. In the majority of cases, the isthmus contained functional renal parenchyma. In over 90% of cases, fusion between the kidneys occurred at the lower pole. Despite commonly being quoted as 'held back by the inferior mesenteric artery' at L3, in reality the isthmus was only found immediately inferior to this in 40% of cases.

Despite being integral to medical and surgical practice, pediatric anatomy has remained relatively neglected except for a few landmark works. Neonatal and pediatric anatomy differs structurally and functionally from adult anatomy in many ways. The aim of the current study was to reappraise common abdominal surface landmarks of important structures in infants and children. After cases with related pathologies had been excluded, computer tomography scans of 90 children were divided into three age groups and systematically analyzed. The vertebral levels of the unpaired branches of the abdominal aorta (AA) were recorded. The vertebral level and relationship to the midline of the bifurcation of the AA and the formation of the inferior vena cava were measured. The renal long axes, costal relationships, renal artery vertebral levels, and hilar vertebral levels were measured. The splenic long axis and relationship to the mid-axillary line were also measured. The renal length was disproportionately large in the youngest age group and increased less with age (7.12 cm, 7.85 cm, 8.86 cm). The renal artery was consistently found around L1; the left kidney was related to the 11th and 12th ribs posteriorly, the right kidney only to the 12th rib. The AA bifurcated to the right of the midline in 10% of children. The unpaired visceral branches of the aorta were commonly found at T12 (celiac artery), L1 (superior mesenteric artery), and L3 (inferior mesenteric artery). The current study provides age-standardized surface landmarks and measurements for major abdominal vascular structures and solid organs in normal children. The clinical applications of these data are multiple and diverse.

Pediatric emergency physicians, pediatric critical care specialists, and pediatric surgeons perform central venous catheterization in many clinical settings. Complications of the procedure are not uncommon and can be fatal. Despite the frequency of application, the evidence-base describing the surface landmarks involved is missing. The aim of the current study was to critically investigate the surface markings of the central venous system in children. The superior vena cava/right atrial (SVC/RA) junction, superior vena cava (SVC) formation, and brachiocephalic vein (BCV) formation were examined independently by two investigators. Three hundred computed tomography (CT) scans collected across multiple centers were categorized by age group into: 0-3 years, 4-7 years, and 8-11 years. Scans with pathology that distorted or obscured the regional anatomy were excluded. The BCV formation was commonly found behind the ipsilateral medial clavicular head throughout childhood. This contrasts with the variable levels of SVC formation, SVC length, and SVC/RA junction. In the youngest group, SVC formation was most commonly at the second costal cartilage (CC), but moved to the first CC/first intercostal space (ICS) as the child grew. The SVC/RA junction was at the fourth CC in the youngest group and moved to the third CC/third ICS as the child grew. This study demonstrates the variable anatomy of SVC formation and the SVC/RA junction with respect to rib level. This variability underscores the unreliability of surface anatomical landmarks of the SVC/RA junction as a guide to catheter tip position.

The inguinoscrotal region is one of the most common areas operated on in pediatric surgery. Despite this, the surface anatomy of the pediatric inguinal canal is variably defined. The aim of the current systematic review is to evaluate the development and surface anatomy of the pediatric inguinal canal. A systematic review of inguinal canal anatomy in children was conducted using the electronic databases: Medline, PubMed, Scopus, and Google Scholar. Relevant anatomical measurements and relationships were reviewed. The anatomical structures forming the walls of the inguinal canal were identified in fetuses as early as 8-10 weeks gestation. No studies addressed the developmental basis of this early defect in the lower anterior abdominal. Later gonadal development and descent has a defined role. In vivo measurements of children carried out during open surgery are inconsistent. Some studies showed rapid growth velocity of the length of the inguinal canal up to 2 years of age (with height and growth of the bony pelvis) before plateauing, while others suggested no increase in canal length prior to 10 years of age. The position of the deep inguinal ring was equally unclear; some studies suggested this was medial to the midpoint of the inguinal ligament. No studies described the position of the superficial ring, challenging the assumption that the rings are superimposed in the neonate. The dearth of studies analyzing pediatric inguinal anatomy means that changes in the position of the rings with respect to the lengthening of the canal remain unclear.

The duodenojejunal flexure (DJF) is an important surgical landmark that enables the pediatric surgeon to establish whether normal intestinal rotation has occurred. The degree of variation in the position of the DJF has not been studied in the pediatric population, and there have been only limited studies on adults. The aim of the present study was to determine the position and relationships of the DJF in infants and children utilizing cross-sectional imaging. Computer tomography scans of 120 children were divided into three age groups and systematically analyzed. The DJF position was measured in relation to the vertebral body level, midline, anterior-posterior distance from the vertebral body, transpyloric plane, and mesenteric vessels. The position of the third part of the duodenum and the length of the mesenteric root were also determined. There was considerable variation in the DJF position with respect to the above landmarks in all three age groups. The vertebral body level of the DJF was centered on L1, but ranged between T11 and L3. In 3% of children with normal rotation the SMA/SMV relationship was abnormal. The third part of the duodenum was consistently found to be retromesenteric. The length of the mesenteric root ranged from 7 to 22 cm, and generally lengthened with increasing age. Owing to its variable position in infants and children, the DJF on its own may not be a reliable landmark for establishing normal intestinal rotation. Assessing for normal rotation is multifaceted and further comparative studies are required to characterize the anatomical features of normal and abnormal rotation.

Accurate knowledge of surface anatomy is fundamental to safe clinical practice. A paucity of evidence in the literature regarding thoracic surface anatomy in children was identified. The associations between surface landmarks and internal structures were meticulously analyzed by reviewing high quality computed tomography (CT) images of 77 children aged from four days to 12 years. The results confirmed that the sternal angle is an accurate surface landmark for the azygos-superior vena cava junction in a plane through to the level of upper T4 from birth to age four, and to lower T4 in older children. The concavity of the aortic arch was slightly below this plane and the tracheal and pulmonary artery bifurcations were even lower. The cardiac apex was typically at the 5

Background Fetal megacystis is a sonographic feature that may be indicative of several underlying pathologies. Despite advances in diagnosis and management, the overall prognosis of affected fetuses remains poor and about 50% of such pregnancies are terminated. Aims To define the frequency, management, survival and renal outcomes of fetal megacystis over nine years at Wellington Hospital, New Zealand. Materials and Methods A nine-year retrospective review of fetuses with an antenatal diagnosis of megacystis was undertaken. Results Sixteen cases were identified (nine live births, five terminations and two perinatal deaths). This gives an observed frequency of one per 940 fetuses scanned. Two-thirds of the live births have survived and been followed for a mean of 5.3 years. None have required renal dialysis or transplantation to date. Conclusions The current series contributes to our knowledge of fetal megacystis and helps to inform antenatal counselling. Improved prognostic criteria are urgently required to accurately differentiate between fetuses with favourable versus poor outcomes.

Summary The anatomy of the sacral hiatus and caudal canal is prone to significant variation, yet studies assessing this in the pediatric population remain limited. Awareness of the possible anatomical variations is critical to the safety and success of caudal epidural blocks, particularly when image guidance is not employed. This systematic review analyzes the available evidence on the clinical anatomy of the caudal canal in pediatric patients, emphasizing surface anatomy and internal anatomical variations. A literature search using three electronic databases and standard pediatric and anatomy reference texts was conducted yielding 24 primary and seven secondary English-language sources. Appreciating that our current landmark-guided approaches to the caudal canal are not well studied in the pediatric population is important for both clinicians and researchers.

Summary Objective The aim of this study is to evaluate the efficacy of primary cystoscopic transurethral incision (CTUI) in the management of paediatric ureteroceles. The secondary aim is to compare the efficacy of CTUI between simplex and duplex systems. Patients and methods This is a retrospective review of consecutive paediatric patients requiring surgical intervention for ureterocele. Data collected for analysis included demographics, diagnostic, pre-operative investigations, operative interventions and postoperative variables. Results Over a 19-year period, 79 consecutive cases were identified, and 42 were male (53.2%). The mean follow-up was 6.7 years. Seventy-three (92.4%) cases underwent primary CTUI; 50 of these cases (68.5%) required no further procedures during the study period. Sixty-one cases were treated by endoscopic intervention alone (77.2%). Forty-one (51.9%) cases had a simplex system, and 38 (48.1%), a duplex system. There was no statistically significant difference in the efficacy of primary CTUI in simplex vs duplex systems. Of the 20 patients who had pre-operative and postoperative micturating cystourethrograms performed, seven (35%) developed de-novo postoperative vesicoureteric reflux after CTUI. Conclusion Primary CTUI is a safe, minimally invasive procedure that is definitive in the majority of children presenting with a ureterocele that requires intervention. There was no difference in success of primary CTUI between simplex or duplex systems. Download : Download high-res image (302KB) Download : Download full-size image Summary Fig .

The ex utero intrapartum treatment (EXIT) procedure is a term given to a technique that can transform a potentially fatal neonatal emergency to a controlled intervention with an improved outcome. It has revolutionised the care of prenatally diagnosed congenital malformations in which severe upper airway obstruction is anticipated. An extended period of utero-placental circulation can be utilised to avoid profound cardiopulmonary compromise. Its therapeutic applications have been broadened to include fetuses with congenital diaphragmatic hernia after tracheal plugging, high-risk intrathoracic masses, severe cardiac malformations and conjoined twins. It requires the co-ordination of a highly skilled and experienced multidisciplinary team. The recent enthusiasm for the EXIT procedure needs to be balanced against maternal morbidity. Specific indications and guidelines are likely to be refined as a consequence of ongoing advances in fetal intervention and antenatal imaging.

Cervical teratomas are rare but life-threatening neonatal tumors and management of the fetus with a cervical teratoma that threatens the airway remains a clinical challenge. This has been revolutionized by advances in fetal imaging and management of the airway at delivery including the use of Ex-utero Intrapartum Treatments (EXIT procedures). We present a retrospective case series of three neonates managed over a 12-month period. Following pre-natal fetal MRI and a multi-disciplinary management approach, two newborns were managed by prompt post-natal endotracheal intubation while an EXIT procedure was required in one. All three underwent surgical resection in the first few days of life. A decision regarding the best means by which to manage the airway in fetal cervical teratoma requires fetal MRI and a multi-disciplinary team approach to determine whether EXIT, or a safer approach from a maternal perspective can be employed. We also recommend routine endotracheal intubation at birth, due to the risk of spontaneous intra-tumoral hemorrhage. The need for surgery should be planned early, as rapid growth of the tumor can threaten the viability of the overlying skin and surrounding structures.

his case highlights a rare but important presentation of aorto-oesophageal fistula caused by a concealed foreign body. Primary prevention strategies are needed to address the danger associated with button batteries.

A 2-year-old boy was born with oesophageal atresia and a low anorectal malformation (perineal fistula). There were two urethral orifices: one pinpoint epispadiac and one penoscrotal (figure 1A,B). The corpora cavernosa were hypoplastic and flaccid. He passed urine predominantly through the penoscrotal meatus. His parents noted that his penis ‘grew bigger’ during micturition. Figure 1 (A) The hypospadiac urethral meatus at the penoscrotal junction. (B) The dorsal epispadiac urethral meatus and …

Pediatric emergency physicians, pediatric critical care specialists, and pediatric surgeons perform central venous catheterization in many clinical settings. Complications of the procedure are not uncommon and can be fatal. Despite the frequency of application, the evidence-base describing the surface landmarks involved is missing. The aim of the current study was to critically investigate the surface markings of the central venous system in children. The superior vena cava/right atrial (SVC/RA) junction, superior vena cava (SVC) formation, and brachiocephalic vein (BCV) formation were examined independently by two investigators. Three hundred computed tomography (CT) scans collected across multiple centers were categorized by age group into: 0-3 years, 4-7 years, and 8-11 years. Scans with pathology that distorted or obscured the regional anatomy were excluded. The BCV formation was commonly found behind the ipsilateral medial clavicular head throughout childhood. This contrasts with the variable levels of SVC formation, SVC length, and SVC/RA junction. In the youngest group, SVC formation was most commonly at the second costal cartilage (CC), but moved to the first CC/first intercostal space (ICS) as the child grew. The SVC/RA junction was at the fourth CC in the youngest group and moved to the third CC/third ICS as the child grew. This study demonstrates the variable anatomy of SVC formation and the SVC/RA junction with respect to rib level. This variability underscores the unreliability of surface anatomical landmarks of the SVC/RA junction as a guide to catheter tip position.

The inguinoscrotal region is one of the most common areas operated on in pediatric surgery. Despite this, the surface anatomy of the pediatric inguinal canal is variably defined. The aim of the current systematic review is to evaluate the development and surface anatomy of the pediatric inguinal canal. A systematic review of inguinal canal anatomy in children was conducted using the electronic databases: Medline, PubMed, Scopus, and Google Scholar. Relevant anatomical measurements and relationships were reviewed. The anatomical structures forming the walls of the inguinal canal were identified in fetuses as early as 8-10 weeks gestation. No studies addressed the developmental basis of this early defect in the lower anterior abdominal. Later gonadal development and descent has a defined role. In vivo measurements of children carried out during open surgery are inconsistent. Some studies showed rapid growth velocity of the length of the inguinal canal up to 2 years of age (with height and growth of the bony pelvis) before plateauing, while others suggested no increase in canal length prior to 10 years of age. The position of the deep inguinal ring was equally unclear; some studies suggested this was medial to the midpoint of the inguinal ligament. No studies described the position of the superficial ring, challenging the assumption that the rings are superimposed in the neonate. The dearth of studies analyzing pediatric inguinal anatomy means that changes in the position of the rings with respect to the lengthening of the canal remain unclear.

The duodenojejunal flexure (DJF) is an important surgical landmark that enables the pediatric surgeon to establish whether normal intestinal rotation has occurred. The degree of variation in the position of the DJF has not been studied in the pediatric population, and there have been only limited studies on adults. The aim of the present study was to determine the position and relationships of the DJF in infants and children utilizing cross-sectional imaging. Computer tomography scans of 120 children were divided into three age groups and systematically analyzed. The DJF position was measured in relation to the vertebral body level, midline, anterior-posterior distance from the vertebral body, transpyloric plane, and mesenteric vessels. The position of the third part of the duodenum and the length of the mesenteric root were also determined. There was considerable variation in the DJF position with respect to the above landmarks in all three age groups. The vertebral body level of the DJF was centered on L1, but ranged between T11 and L3. In 3% of children with normal rotation the SMA/SMV relationship was abnormal. The third part of the duodenum was consistently found to be retromesenteric. The length of the mesenteric root ranged from 7 to 22 cm, and generally lengthened with increasing age. Owing to its variable position in infants and children, the DJF on its own may not be a reliable landmark for establishing normal intestinal rotation. Assessing for normal rotation is multifaceted and further comparative studies are required to characterize the anatomical features of normal and abnormal rotation.