A systematic review and meta-analysis revealing realistic outcomes following paediatric torsion of testes.

INTRODUCTION: Reported testicular loss rates following paediatric testicular torsion often reflect the surgical decision-making process, rather than long-term survival of the testes. OBJECTIVES: We aim to perform systematic analysis and meta-analysis to investigate testicular salvage rates and to assess predictors of long-term viability. STUDY DESIGN: Systematic review according to PRISMA guidelines was performed to investigate immediate and long-term (>12 months) testicular loss rates following torsion in the paediatric population. Literature search and study inclusion were performed by two investigators. A study quality score was derived and attributed to each study. Predictors of testicular loss were described. Proportions meta-analysis was performed with random effects modelling, and testing for heterogeneity. RESULTS: Twelve studies were includedm, 6 reporting early orchidectomy rates, and 6 reporting long-term outcomes. Study quality was generally low. DISCUSSION: The mean early testicular loss rate was 39%, whereas meta-analysis revealed late loss to approach 50%. Predictors of outcomes include prehospital symptom duration, location of presentation, transfer to a tertiary centre, social affluence and use of ultrasound prior to diagnosis or transfer. CONCLUSIONS: This study has shown a considerable late testicular loss rate, which must be relayed to families even after testicular salvage. Delay in time to presentation is consistently found to predict poor outcomes.

The Dan Young Neonatal Surgical Unit audit: an audit of admissions and mortality rates pre- and post-amalgamation.

BACKGROUND AND AIMS: (1) To record the number of Index Surgical Cases (ISCs) admitted to the Royal Hospital for Sick Children (RHSC) Yorkill in 2012-2014. (2) To investigate if the amalgamation of the neonatal intensive care unit with the Dan Young Neonatal Surgical Unit in 2008 has impacted on the number of admissions and deaths. METHOD AND RESULTS: Data were collected from the ward admission books and the BadgerNet UK database. Reason for admission and outcome was recorded. Nonsurgical cases were categorised as 'miscellaneous'.The total number of admissions per year and the number of miscellaneous cases have been increasing since the amalgamation. There were no clinically significant differences in the number of babies with each ISC. The death rate was 1.8% in 2012, 2.9% in 2013 and 2.6% 2014. CONCLUSIONS: The unit now admits more miscellaneous babies and this has caused an increase in total admissions. Although the total number of deaths has increased, the death rates as a percentage of total admissions have now plateaued after an initial rise at the time of union. There has been no significant increase in the number of ISC deaths. Thus, it appears that the amalgamation has affected survival outcomes in the short term only.

Clinical embryology: is there still a place in medical schools today?

BACKGROUND AND AIMS: Embryology remains an important tool in medicine and surgery for the management of many clinical conditions. As a subject, it is neither straightforward nor easy to learn and teach in a busy modern medical school curriculum and can be easily overlooked. The aim of this study was to assess medical students' confidence in, and attitudes towards, the learning and teaching of clinical embryology. METHOD AND RESULTS: Medical students from all years of the course were asked to complete an online questionnaire in 2014. The questionnaire focused on confidence levels in learning embryology, methods of teaching, clinical embryology and it also allowed comments. In total, 146 students completed the questionnaire. The majority of students were not confident in learning and applying embryology and were unhappy with current teaching. Despite this, they felt that embryology should be included in the medical school curriculum, in particular clinical embryology with relevant clinical scenarios. CONCLUSION: Students remain confident that embryology should remain in the medical school curriculum. Embryology should be taught at the right level, depth and through various methods, including basic concepts in the lower years of medical school and moving into clinical embryology later on. As a result, junior doctors and trainees will have a good foundation of knowledge.

Quality of life and behavioural adjustment in childhood hydrocephalus.

The aim of the paper is to describe parent and teacher reported behavioural outcomes and quality of life in childhood hydrocephalus, and to consider the implications for future service planning. A community sample of 235 school-aged children with hydrocephalus (5-16 years) were identified via a database of service users, held by the Scottish Spina Bifida Association. Parent and teacher reports of behaviour on the Strengths and Difficulties Questionnaire (SDQ), and parent reports of quality of life on the Paediatric Quality-of-Life Generic Core (PedsQL Core) and Paediatric Quality-of-Life Fatigue (PedsQL Fatigue) were obtained, as were reports of service use and satisfaction.  In total, 35% (n = 76) of parents and 86% (n = 47) of teachers who were contacted participated in the study. Parents reported behavioural difficulties in 57% and teachers in 33% of children. Quality of life was significantly reduced in comparison to published norms. Children whose parents reported unmet needs had poorer psychosocial outcomes, but families rarely accessed appropriate specialist services. In conclusion, hydrocephalus is associated with high rates of behaviour problems and markedly reduced quality of life. It is important to increase professional awareness of psychological need in this chronic neurological condition, and to increase access to appropriate psychosocial services.

The right-sided aortic arch in children with oesophageal atresia and tracheo-oesophageal fistula.

AIM: A right-sided aortic arch (RAA) occurs in around 5% of patients with oesophageal atresia and tracheo-oesophageal fistula (OA/TOF). This anatomical variation can complicate the operative management of these patients, as it is often not diagnosed preoperatively but only discovered at thoracotomy, and it remains unproven as to whether a right or left thoracotomy is the best operative approach. This retrospective study aimed to determine the prevalence of RAA in OA/TOF, review the accuracy of preoperative investigations, and investigate the best operative approach, by reviewing the literature and our own patient series. METHODS: The case notes of all infants with OA/TOF over a 15 year period (1994-2008) were retrospectively analysed to identify those with a RAA. Birth weight, gestational age, associated anomalies, preoperative investigations, surgical management, postoperative complications and long-term prognosis were all extracted. MAIN RESULTS: A total of 107 case notes of OA/TOF infants were reviewed, identifying 4 with a RAA. Preoperative echocardiography was performed in all of the 4 RAA infants, but RAA was only identified in one. All 4 infants were managed surgically via a right thoracotomy, regardless of the echocardiography result, with primary anastomosis achieved successfully in all. A laryngeal cleft repair was performed in 1 infant due to an interarytenoid cleft. Laparoscopic fundoplication was performed in 1 patient, because of severe gastro-oesophageal reflux. There were no postoperative anastomotic leaks, bleeding, or deaths in this group. CONCLUSION: In our study, the incidence of RAA in OA/TOF was 3.7%. Preoperative echocardiography identified the RAA in only 1 of 4 cases. However, echocardiography was helpful for diagnosing other cardiac anomalies, which might have potentially affected the management of these patients. Previous studies have cited the operative difficulties associated with RAA and OA/TOF. However, in this series of 4 infants, primary anastomosis was achieved via conventional right thoracotomy without complication, and with no effect on outcome or prognosis. Therefore, we conclude that, where possible, a conventional right-sided thoracotomy should be performed in OA/TOF patients with a RAA.

Colonic atresia: a clinicopathological insight into its etiology.

BACKGROUND: The pathogenesis of colonic atresia (CA) has not been discussed in detail, although the theory of a prenatal vascular insult, hypothesized from patients with small bowel atresia, has been accepted in the past. This review questions this etiology by reviewing all patients with CA treated in two institutions. MATERIAL AND METHODS: A retrospective analysis was done of the medical notes of 30 patients with CA treated in two tertiary centers of neonatal surgery in Glasgow, UK, and in Wroclaw, Poland, over a 30-year period with special emphasis on the intraoperative findings and morphology of the atretic bowel. RESULTS: Thirteen patients had CA in association with an abdominal wall defect. Eleven patients had gastroschisis. A single fibrous cord atresia was noted in 2 patients and it was located in the ascending colon and transverse colon, respectively. Four patients had type IIIa atresia affecting the ascending colon in 3 and transverse colon in one. In 5 patients an extensive defect of the intestine with two atresias of the small and large bowel was noted. In 3 of them, a separate conglomerate of the prolapsed intestine was found to be necrotic or presenting as a cystic structure. Two of these patients had an unusually narrow abdominal wall defect of less than 1 cm. Isolated CA was noted in 17 patients. A type IIIa atresia affecting the right colon was found in 14 of them. In 2 patients the ascending colonic atresia was accompanied by an extensive defect of the transverse colon. A single sigmoid colon atresia and two fibrous cord atresias of the sigmoid and transverse colon were noted in single patients respectively. CONCLUSIONS: Most cases of CA in babies with gastroschisis seem to result from bowel compression within the narrowing abdominal defect. A "two-point constriction" may lead to a wide spectrum of bowel pathologies and the morphology may depend on the viability of the intestinal segment between the atretic jejunum or ileum and the colon. Isolated CA presents with a wider spectrum in terms of anatomical types and location of the bowel pathology, but it seems that type II and IIIa atresias of the right colon may share a similar pathogenesis of temporary constriction within a closing umbilical ring.

Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia.

UNLABELLED: Primary intestinal lymphangiectasia is an uncommon congenital anomaly. It is an intrinsic abnormality of the intestinal lymphatics system. Over the years, various treatment options such as diuretics, albumin transfusions and a medium chain triglycerides (MCT) diet as well as surgical options such as resection of isolated segments and peritoneal-venous shunts have been used. An MCT diet, which is a low fat, high protein diet, is increasingly used in the management of this anomaly. AIM: The aim was to review the evidence for medium chain triglycerides as a therapeutic option in patients with primary intestinal lymphangiectasia. MATERIAL AND METHODS: A literature search was performed and individual case details were extracted. We found 55 cases, of which 3 were from our own institute. The cases were divided in 2 groups: Group A (n=27) consisted of patients treated with MCT, and Group B (n=28) consisted patients not treated with MCT. Cases were analysed for symptomatic response to MCT as well as mortality. RESULTS: 17 of 27 cases (63%) treated with MCT had complete resolution of symptoms while only 10 of 28 (35.7%) patients in group B showed complete resolution. Mortality for Group A was 1 out of 27 (3.7%), while mortality in group B was 5 of 28 (17.85%) patients. CONCLUSION: We conclude that, although an MCT diet is not completely curative in all cases, it does improve the symptoms of primary intestinal lymphangiectasia and reduces mortality. Hence it is a valid option in the paediatric age group.

Multiple atresia of the small intestine: a 20-year review.

BACKGROUND: Multiple intestinal atresia (MIA) presents with a wide spectrum of bowel pathologies. Its treatment is a challenging task since restoration of anatomical continuity of the affected intestine must be balanced against preservation of the intestine's maximal length. MATERIAL AND METHODS: A retrospective analysis of the medical notes of 26 patients with MIA treated over a 20-year period between 1986 - 2006 was undertaken with a special emphasis on the clinical and surgical perspectives. RESULTS: All 26 cases of MIA were sporadic with no familial history. The mean gestational age and birth weight were 36.1 weeks and 2781 g, respectively. Twenty-three of the infants underwent operative repair within the first days of life. Three patients with gastroschisis had a delayed diagnosis of bowel atresia. The number of atresias per patient ranged from 2 to 10. In 24 newborns atresias were confined to the small bowel, with 2 other patients having additional obstruction of the ascending colon. Various combinations of anatomical types of atresias were found, with type I and type III occurring in 19 patients each. Type II was diagnosed in 7 newborns. Surgical management of MIA consisted of one-stage restoration of bowel continuity with multiple anastomoses and/or enteroplasties in 22 patients. Four patients had an enterostomy performed at initial operation. Early and late postoperative complications requiring operative treatment occurred in 8 patients. The duration of parenteral nutrition ranged from 6 days to 20 months, exceeding 100 days in 6 children. The follow-up ranges from 3 months to 16 years. All the patients are alive and are on a full oral diet. CONCLUSION: Clinical observations of sporadic cases of MIA confined to the small bowel lend support to the hypothesis of a vascular incident etiology. One-stage restoration of intestinal continuity with preservation of maximal intestinal length should be the basic principle of any operative management of MIA. Despite a relatively high morbidity related to the primary damage of the fetal intestine, excellent results with 100 % survival rates can be obtained. After taking the differences in pathogenesis, anatomical and histological features, and the prognosis for sporadic and hereditary forms of MIA into account, these two entities should be classified separately in a modified classification of intestinal atresia.

Immunoreactive trypsin levels in neonates with meconium ileus.

Serum immunoreactive trypsin (IRT) is used as a screening test for cystic fibrosis (CF) in neonates in many countries. Variations in IRT levels are observed in healthy and cystic neonates within the first few weeks of life. Fifteen percentage of CF neonates present with meconium ileus (MI). We hypothesised that there may be differences in serum IRT levels in cystic babies with simple and complicated MI. The aim of this study was to investigate the serum levels of IRT in neonates with CF presenting with MI. IRT levels were sequentially measured in neonates (n = 29) with CF with intestinal obstruction due to simple or complicated MI. These were compared to levels obtained from non-cystic neonates/controls admitted with a variety of other intra-abdominal pathologies (n = 49) IRT levels were significantly higher in the CF-MI group than the non-cystic controls (P < 0.001). There was no statistical difference in IRT levels between the simple or complicated MI groups. In the MI group there was no statistical difference between those who required operation, no difference between the pre- and post-operative IRT levels and no significant relationship between IRT levels and birth weight or gestation. Serum IRT levels are significantly elevated in neonates with CF and MI compared with non-cystic, non-MI neonates. The results of this observational study highlight that a single raised level of IRT in a neonate should prompt the analysis for CF regardless of any underlying surgical pathology.

Interventional radiology cases from the royal hospital for sick children, glasgow: percutaneous metal biliary stent placement for palliation of intra-abdominal desmoplastic small round-cell tumour in a child.

Percutaneous placement of metal biliary stents for palliation of malignant processes affecting the porta hepatis is a well established technique in adults. We describe a case where the technique has been used successfully to treat obstructive jaundice in a ten-year-old boy suffering from intra-abdominal desmoplastic small round-cell tumour.

Congenital posterior urethral polyps in childhood. A case report.

We describe a 3-year-old boy who presented with dysuria and urinary retention. The diagnostic work-up (USS, MRI, urethrocystoscopy), suggested a polypoid lesion at the bladder base, originating from the verumontanum. Histology showed the lesion to be a fibroepithelial polyp. A rare cause of urinary retention in childhood must be considered in the differential diagnosis.

Congenital cystic mesoblastic nephroma.

With widespread use of antenatal ultrasound, an increasing number of urinary-tract lesions are being detected, and the clinician is called upon to make management decisions. Parenchymal lesions of the kidney pose a special challenge because of the associated risk of the occurrence of malignancy. A case of an antenatally-detected solid/cystic lesion of the kidney diagnosed as a congenital cystic mesoblastic nephroma following nephrectomy is presented and the issues involved in therapeutic decision-making are discussed.

Neonatal tumours: a single-centre experience.

Solid tumours are uncommon in the neonatal period. We present our experience of managing neonatal tumours in a tertiary reference centre to study the incidence, pathology and types, efficacy of treatment, and impact of antenatal diagnosis on the management in our practice in a retrospective study of case-notes and pathology reports. Eighty-three neonates with solid tumours were seen over a 45-year period (1955-1999); 62 (74%) presented at birth. Only 11 were diagnosed antenatally. Teratomas were the commonest type (n = 33, 40%) followed by neuroblastomas (NB) (14), renal (13), soft-tissue (10), hepatic (4), and miscellaneous tumours (2). Twenty-three (28%) were malignant, 50% of these being NBs. Surgery remains the mainstay of treatment. Chemotherapy has also become safer. Therapeutic complications were responsible for 50% of deaths before 1986; from 1986 onwards, there has been no therapy-related mortality. Only one-third of the recent cases were diagnosed antenatally. Counseling the family and in-utero transfer is the best option. In our limited series, there was no significant difference in management and outcome in the antenatally-diagnosed cases. The small numbers of neonatal tumours seen by individual centres underline the need for an international effort to optimise therapy and improve understanding of these tumours.

Measurement of optic nerve sheath diameter by ultrasound: a means of detecting acute raised intracranial pressure in hydrocephalus.

AIM: To evaluate the utility of measuring the optic nerve sheath diameter in children with shunted hydrocephalus, suspected of having raised intracranial pressure. METHODS: 23 children with shunted hydrocephalus were examined, six had well controlled ICP, 17 however manifested symptoms suggestive of intracranial hypertension. A clinical history was taken from all patients and their parents or carers. The shunt valve was examined clinically, and signs of raised intracranial pressure were sought. Ultrasound examination was performed in both eyes to measure the optic nerve sheath diameters 3 mm behind the globe. These measurements were compared with control data obtained from 102 children who attended the radiology department for unrelated renal ultrasound examination. RESULTS: Control data suggested that the upper limit of normal for optic nerve sheath diameter is 4.5 mm (measured 3 mm behind the globe) in patients over 1 year of age, and 4.0 mm in children less than 1 year of age. Those patients with functioning ventriculoperitoneal shunts had a mean optic nerve sheath diameter of 2.9 (SD 0.5) mm; those with raised intracranial pressure had a mean optic nerve sheath diameter of 5.6 (0.6) mm (p<0.0001). These results confirm that optic nerve sheath diameters in excess of the control data are strongly suggestive of raised intracranial pressure. CONCLUSION: The evaluation of the optic nerve sheath diameter is a simple non-invasive procedure, which is a potentially useful tool in the assessment and monitoring of children with hydrocephalus suspected of having raised intracranial pressure.

Twins with oesophageal atresia and the CHARGE association.

Twins are rarely concordant for oesophageal atresia and tracheo-oesophageal fistula. Such a case is presented and their similar associated anomalies described. The CHARGE association is considered as a possible diagnosis and the aetiological implications considered.