The Irish Experience with Sacrococcygeal Teratomas: Are Type IV Lesions More Common than We Think?

INTRODUCTION: Sacrococcygeal teratomas (SCTs) are rare tumors occurring in approximately 1 in 35,000 to 40,000 live births. The Altman classification is used to describe SCTs. There are four types, with type 1 predominantly external through to type IV which is a presacral, completely internal mass. As far as the authors are aware, this is the first study to focus on type IV SCT lesions. MATERIALS AND METHODS: Using ICD-10-AM (The International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Australian Modification) codes, we identified all patients in the Republic of Ireland with a diagnosis of SCT from 2004 to 2020. The following information was obtained for each patient: gender, time of diagnosis, clinical presentation, method of diagnosis, Altman classification, biomarkers, age at operation, surgical technique, pathology, recurrence, and age at most recent follow-up. RESULTS: There were 29 patients in total; 23 females (79%) and 6 males (21%). In total, 16 (55%) were diagnosed antenatally, 4 (14%) at less than 1 month, 4 (14%) less than 1 year, 3 (10%) age 2 to 4 years, and 2 (7%) were aged 5 to 12 years. In addition, 22 (76%) were mature teratomas, 2 (7%) immature teratomas, and 5 (17%) were malignant tumors. There were 6 (21%) type I lesions, 9 (30%) type II, 6 (21%) type III, and 8 (28%) type IV lesions. CONCLUSION: In Altman's original 1974 study, type IV lesions were present in 10% of cases. As a result, this is the most frequently quoted figure. Type IV lesions were present in 28% of cases in our study. We propose that type IV lesions may be more common than the current literature suggests and consequently a higher index of suspicion of their presence should be entertained.

Colectomies in children with inflammatory bowel disease: a national referral centre experience.

OBJECTIVES: Few studies have reported on the utility and efficacy of laparoscopic colectomy in the paediatric population. We aim to compare the characteristics and outcomes of children with inflammatory bowel disease (IBD) who underwent open vs laparoscopic treatment at our centre. METHODS: A 10-year retrospective review was performed. Collected data included demographics, indication for surgery, operative characteristics, histopathology results, post-operative course and peri-operative complications. RESULTS: A total of 58 patients underwent subtotal colectomy for IBD, with 38 laparoscopic procedures. The cohort included 33 males and 25 females with a mean age at surgery of 12.9 ± 3.3 years. The pre-operative diagnosis was ulcerative colitis in n = 41, Crohn's disease in n = 5 and indeterminate colitis in n = 11. There was an 84.2% concordance between the pre-operative and the post-operative histological diagnoses. Overall, 34 (58.6%) patients had complications, of which 19 (32.7%) patients required return to theatre. The complication rate was significantly smaller for laparoscopic compared to open procedures (42.1% vs 75%) as well as for elective compared with emergency colectomies (38.4% vs 75%). Four patients (10.5%) required conversion to open approach. CONCLUSIONS: Laparoscopic approach is feasible and safe in most paediatric patients with IBD and has a lower complication rate and better recovery than open procedures, especially when performed in an elective setting.

Smartphones in clinical practice: doctors' experience at two Dublin paediatric teaching hospitals.

INTRODUCTION: We conducted a study at two main centres for paediatric surgery in the Republic of Ireland, Temple Street Children's University Hospital (TSCUH) and Our Lady's Children's Hospital Crumlin (OLCHC). AIM: The aim of the study was to assess doctors' opinions, frequency and methods of smartphone use in clinical practice. METHODS: Doctors (n = 265) at the two hospitals were invited to complete a 12-item survey. Descriptive statistics were calculated accordingly. RESULTS: Almost 50% (132/256) responded; 98.4% owned a smartphone and 76% owned an iPhone. Seventy-nine percent used medical apps and 59% used the OLCHC formulary. Ninety-seven percent used smartphones for inter-team communication with 73.5% (n = 97) using WhatsApp. Smartphone camera was used by 57% for clinical photos and 41% for photos of radiological imaging. Only 42% reported obtaining consent for smartphone camera use from patients or guardians. Ninety-two percent either 'strongly agreed' or 'agreed' that smartphones positively impact their practice. CONCLUSION: Smartphone usage is universal among healthcare professionals and their influence is growing in patient care.

Incidence and outcome of retained Port-A-Cath fragments during removal.

PURPOSE: Port-A-Cath devices are frequently used for long-term venous access. We postulate that long-term use predisposes them to getting stuck and retained in a central vein at the time of removal. We aim to report the incidence and outcome of this complication. METHODS: Between January 2006 and July 2016, a retrospective review of all Port-A-Cath removals that were performed at our centre was conducted. At the time of removal, catheters that could not be removed from the vein were considered retained. RESULTS: During the study period, 107 children had 174 episodes of silicone Sitimplant (Vygon, Ecouen, France) Port-A-Cath insertions. These children required 135 removal episodes and there were 3 (2.2%) instances whereby the catheter fragment was retained. These episodes of retained catheters only occurred in children with factor VIII deficiency (4.1% incidence in this cohort). For each episode of catheter insertion and removal, the catheters had been left in situ for a mean duration of 43 months in children with factor deficiency and no retained fragments, and the mean duration was 91 months in children with factor deficiency and retained catheter fragments (p = 0.0011). CONCLUSIONS: Port-A-Caths that are retained after attempted removal is a complication encountered predominantly in catheters that have been in use for a prolonged duration. Furthermore, factor replacement therapy in haemophiliacs may be a risk factor for this complication.

Abdominal radiography is not necessary in children with intussusception.

BACKGROUND: Children with intussusception require rapid and accurate diagnosis to enable timely intervention for satisfactory outcome. Ultrasonography is the recommended standard diagnostic modality; however, abdominal radiography (AR) is still used as an initial investigation. The aim of this study was to investigate the benefit of AR in intussusception by determining diagnostic accuracy and analysing correlation of AR findings with outcome. METHODS: Index cases of intussusception presenting over 15 years (1998-2013) were analysed. Those who had AR performed were allocated into groups with positive or normal findings. Outcome of pneumatic reduction of intussusception (PRI) between these groups was compared. RESULTS: Six hundred and forty-four cases of intussusception treated with PRI were identified, 412 (64 %) had AR performed and 232 (36 %) did not. 303 (74 %) radiographs had positive findings and 109 (26 %) were normal. The success rate of PRI did not differ between AR positive (82 %) and AR normal (84 %). Occult pneumoperitoneum was not detected in any patient by AR in our cohort. CONCLUSION: AR is not recommended for the diagnosis of intussusception in children, for the prediction of the outcome of PRI or for the detection of occult pneumoperitoneum. AR should always be performed when clinical peritonism is present but is not otherwise necessary in children with suspected or confirmed intussusception.

Oesophageal inflammatory paediatric chylothorax.

Paediatric chylothoraces are rare, particularly outside the operative setting. Cases of spontaneous chylothorax are often demanding diagnostically and frequently associated with patient morbidity. We present a challenging case of paediatric chylothorax associated with inflammatory oesophageal perforation likely related to foreign body ingestion.

Publication patterns on neonatal surgery over 65 years.

BACKGROUND/PURPOSE: Staying abreast of the literature in a given speciality is difficult. The aim of this study is to analyze the publication patterns of the neonatal surgical literature over the last six decades. MATERIALS: A search strategy for the Web of Science database was designed using MeSH defined terms for 10 index neonatal surgical conditions, with output analyzed over two time-periods. RESULTS: There were 6215 and 6144 publications for periods 1 (1945-1994) and 2 (1995-2010), respectively. There were 24 and 546 articles published in 1945 and 2010, respectively. The mean citation counts of the top 50 publications are 228 and 156 for periods 1 and 2, respectively. There were 6 and 11 authors with two or more publications in the top 50 list in periods 1 and 2, respectively. Three of the pediatric surgery journals cumulatively have published 30.9% of the total articles. CONCLUSIONS: Publication patterns for neonatal surgical conditions have changed significantly over time. The majority of articles are published outside of pediatric surgical journals. Pediatric surgeons should not limit their reading to these journals.

The top 100 cited articles in urology: An update.

BACKGROUND: In this paper, we identify and analyze the top 100 cited articles in urology since 1965 and assess changes in the top 100 since 2007. METHODS: We selected highest impact journals in both urological and general medicine journals from the 2011 edition of Journal Citation Reports: Science edition. We identified and analyzed the 100 most cited articles using the Science Citation Index Expanded (1965-present). RESULTS: The top 100 articles were cited a mean of 892 times (range: 529-2088) and published between 1966 and 2009, with 21 published since 2000. In 2012, 19 new articles appeared in the updated top 100 cited articles. Also, 16 journals were represented, led by the New England Journal of Medicine (n=36), the Journal of Urology (n=16) and the Lancet (n=12). In total, 81 articles were published from North America (USA=77, Canada=4). From the United States, the following institutes were among the top 5 represented: Johns Hopkins University (n=12), Harvard University, Memorial Sloan Kettering Cancer Centre, National Institute of Health and Washington University (all 5). Only one institute outside the United States published more than one article in the top 100 (Institut Gustave Roussy, France). Nine urologists were first authors of 2 or more articles. Oncology (n=54) and transplantation (n=22) were the most common subspecialties represented. CONCLUSION: It is important to acknowledge the top cited articles as they mark key topics and advances in urology. There has been a 19% change in the top 100 cited articles in the past 5 years. Oncology and transplantation remain the most highly cited topics.

Scrotal exploration for acute scrotal pain: a 10-year experience in two tertiary referral paediatric units.

OBJECTIVE: Acute scrotal pain is a common presentation to the paediatric emergency department. Testicular torsion is one of the most common causes of acute scrotal pain. Testicular torsion is a surgical emergency requiring immediate surgical exploration to prevent permanent testicular damage or loss. The aim of this study was to determine the surgical outcome of all scrotal explorations and to assess the use of colour Doppler ultrasound (CDUS) in the assessment of acute scrotal pain in two tertiary referral paediatric units. MATERIAL AND METHODS: A retrospective review of a prospectively maintained database was carried out for all scrotal explorations between 1999 and 2010. RESULTS: In total, 155 scrotal explorations were carried out for acute scrotal pain. The mean age was 9.1 years (range 0-15 years). The pathology in 46.5% (n = 72) was testicular torsion, 30.3% (n = 47) were torsion of a testicular appendage, 16.1% (n = 25) were epididymitis, 3.3% (n = 5) had no obvious pathology identified and other pathology accounted for 4%. There was a significant difference in age of presentation between those with testicular torsion and those with torsion of a testicular appendage (9 vs 10 years, p = 0.0074). CDUS was performed by a trained radiologist on 40 patients. Overall sensitivity, specificity, positive predictive value and negative predictive value for CDUS predicting testicular torsion were 96.9%, 88.9%, 96.9% and 89%, respectively. Overall, 36 patients (23%) with acute scrotal pain (50% of patients in the group with confirmed testicular torsion at exploration) required orchidectomy. CONCLUSION: This study supports the practice of immediate surgical exploration with a clinical suspicion of testicular torsion in a paediatric population.

Epidermolysis bullosa: management of esophageal strictures and enteric access by gastrostomy.

Epidermolysis bullosa (EB) is a spectrum of rare, inherited, blistering skin disorders, primarily affecting the skin and pharyngoesophageal mucosa. EB affects approximately 2 to 4 per 100,000 children each year. Blistering and scarring occur in response to even the most minor trauma. In this article, the authors outline the potential management options for patients with EB complicated by feeding difficulties secondary to esophageal strictures as well as those with nutritional deficiencies requiring a gastrostomy tube for supplemental feeding.

Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience.

Esophageal atresia/tracheoesophageal fistula (EA/TEF) repair using an open muscle-sparing thoracotomy has been the standard approach used in our institution. Whereas perioperative mortality is now very uncommon, short- and long-term morbidity is very common in these patients. However, the complexity of the esophageal anatomy and significant comorbidities appear to be important contributors to significant complications in these patients. At least 30% of the EA/TEF patients required esophageal dilatations for anastomotic stricture; this increased to 50% for patients with pure EA. Gastroesophageal reflux requiring an antireflux procedure was performed 23% of the time for EA/TEF and 30% for EA patients. In addition, there were a few complications, such as winging of the scapula and scoliosis, that were attributed in part to the utilization of a nonmuscle-sparing thoracotomy. The standard muscle-sparing thoracotomy remains a very versatile and useful approach to repairing esophageal atresia, and it is the standard for repairing more complex anatomical variants. The self-reported long-term quality of life in these patients is very good, except for a few individuals with protracted feeding disorders and severe dysphagia.

Transhepatic central venous catheter for long-term access in paediatric patients.

Vascular access in paediatric patients with chronic and/or life-threatening illness is crucial to survival. Access is frequently lost in this group because of thrombosis, infection, or displacement, and vascular options can quickly be exhausted. The last resort access procedure is generally a direct atrial catheter inserted via a thoracotomy. A viable alternative is the percutaneous transhepatic Broviac catheter (Bard Access Systems, Salt Lake City, UT). We retrospectively reviewed the charts of 5 patients who underwent percutaneous transhepatic Broviac insertion for long-term access over a 4-year period in a single institution. Four of the patients (80%) had a significant cardiac abnormality, with 1 patient requiring long-term parenteral nutrition after complicated necrotizing enterocolitis. All patients had significant caval thrombosis, which precluded them having placement of a standard percutaneous or openly placed central catheter. Of the 5 patients, 2 (40%) died of cardiac-related illnesses. Of the 3 surviving patients, 2 had functioning catheters electively removed because they were no longer required. One catheter was removed at thoracotomy for right atrial perforation because of catheter erosion. Vascular access in paediatric patients with chronic and/or life-threatening illness is crucial to survival. Transhepatic central venous catheters are a feasible, reliable, and relatively easily placed form of central access in patients with multiple venous thromboses requiring long-term access. This route should be considered in paediatric patients requiring central access in preference to a thoracotomy.

Alpha glutathione S-transferase: a potential marker of ischemia-reperfusion injury of the intestine after cardiac surgery?

BACKGROUND: The aim of the study was to assess the utility of alpha glutathione S-transferase (alphaGST) as a potential marker of intestinal ischemia-reperfusion injury in children after cardiac surgery. METHODS: Twenty-six patients undergoing cardiac surgery were enrolled in this longitudinal experimental study. Blood samples were drawn for analysis at specified time points during surgery and analyzed for alphaGST levels. Clinical indices of splanchnic morbidity were assessed up to discharge from hospital. Results were analyzed using Mann-Whitney tests and linear mixed effects models. RESULTS: Two groups were identified. Group 1 (n = 16) showed no intestinal morbidity and group 2 (n = 10) had signs of intestinal morbidity. Statistical differences were shown between the 2 groups with respect to time with aortic cross-clamp (ACC) in situ, time on cardiac bypass, duration of operation, time to enteral feeding and full feeding, time on mechanical ventilation, and time in the intensive care unit postoperatively. The serum concentration of alphaGST was significantly higher for group 2 and this rise was greatest after removal of the ACC. CONCLUSIONS: AlphaGST showed significant elevation in patients with prolonged bypass times and ACC times. These patients also displayed signs of intestinal morbidity, suggesting that this marker may be useful in screening patients at risk for intestinal pathology. This rise in alphaGST was associated with a prolonged ischemia time, and was greatest after the cross-clamp was released, suggesting that it is a postischemic reperfusion phenomenon leading to its elevation. A low alphaGST level appears to exclude significant intestinal ischemia.

Notable sequential alterations in notochord volume during development in the Adriamycin rat model.

BACKGROUND/PURPOSE: The Adriamycin rat model (ARM) is a well-established model of the vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb association. An important finding in the ARM is that Adriamycin induces abnormal notochord morphology in the region of the foregut. Having recently demonstrated notochord hypertrophy in ARM embryos, the authors designed this study to assess notochord volume sequentially from gestational days 10 to 14 (E10-E14) to test the hypothesis that notochord hypertrophy occurs maximally soon after Adriamycin administration. METHODS: Adriamycin (1.75 mg/kg) was administered intraperitoneally to pregnant rats on E7, E8, and E9. Control animals were given saline. Embryos were recovered at E10, E11, E12, E13, and E14 and embedded in paraffin. Quantitative morphology using the Cavalieri technique was performed on hematoxylin and eosin-stained transverse serial sections to determine total embryo and total notochord volume. RESULTS: The percentage volume of notochord per embryo was significantly increased (P < .05) in Adriamycin-treated embryos at all gestational time frames from E10 to E14 when compared with equivalent controls. This increased volume of notochord was found to be maximal at E11. CONCLUSIONS: These data support the authors' previous finding that Adriamycin induces notochord hypertrophy and suggest that notochord volume is increased relative to embryo volume soon after Adriamycin administration and is maximal on E11. The abnormal increase in notochord volume during the critical phase of development may interfere with organogenesis, resulting in the vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb association.