Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of kidney tumor that has only recently been described, with less than eighty cases in the literature. This was only recognized as a specific entity in the World Health Organization 2004 classification of Renal Cell Carcinoma (RCC). MTSCCs are polymorphic renal neoplasms characterized by small, elongated tubules lined by cuboidal cells with cords of spindled cells separated by pale mucinous stroma. We report the case of a 57 year old lady who had an incidental finding of a mass in her right kidney. The radiological features were consistent with a RCC and following a multidisciplinary team discussion she underwent a laparoscopic radical nephrectomy. Macroscopic examination revealed a well circumscribed 6.5 × 6 × 6.5 cm right lower pole mass. Histologically it was composed of elongated tubules, small tubules and papillary structures with a necrotic centre. The cells demonstrated cuboidal and spindle cell morphology. Histological grade was Fuhrman grade 2. The majority of MTSCCs are indolent, and there are only two reports of distant metastases which responded favorably to adjuvant sunitinib. To date there is no international consensus on long term surveillance of these patients. Due of the favorable prognosis with this type of tumor, MTSCC must be differentiated from papillary renal cell carcinoma to avoid administration of excessive adjuvant treatment to patients.

Since its first clinical application in 1984, the endoscopic subureteral injection of bulking agents has become an alternative to long-term antibiotic prophylaxis and open surgical intervention in the treatment of VUR in children. The 15 min day care endoscopic procedure has gained worldwide popularity in the management of VUR in children. Over the years, multiple studies have demonstrated safety and long-term efficacy of this minimally invasive outpatient procedure. Nowadays almost 90% of the surgical treatment of VUR in Sweden is done by endoscopic procedure. In the current article, our aim was to review how the endoscopic treatment of VUR developed.

The need for paediatric urological care in low- and middle-income countries in sub-Saharan Africa (SSA) is enormous due to a burgeoning paediatric-aged population and a disproportionate burden of congenital malformations. There are formidable challenges in the provision of a skilled workforce and appropriate infrastructure, resulting in a huge unmet need with consequent effects on the long-term health and prosperity of the population. Constraints of funding, geography, culture, surgical and anaesthetic skills, and instrumentation means that many conditions present late and with complications that could have been avoided by an earlier attendance. It also means that the management of congenital malformations, e.g., bladder exstrophy and congenital obstructive posterior urethral membrane, differ substantially from that seen in the developed world, with the outlook for children with renal failure being particularly bleak. Collaborations between paediatric urologists from high- and low-income countries are beginning to help with the development of a surgical infrastructure customised to paediatric care, and with the training of specific paediatric urological knowledge and skills. These collaborations, whilst welcome, still require substantial expansion to achieve more equitable access to appropriate paediatric urological care for children in SSA. Future efforts have to focus on the creation of sustainable and equal partnerships between urologists from low- and high-income healthcare environments, with an emphasis on providing sustainable management, appropriate to local need and available resources. The provision of shared learning, utilising the benefits of global digital communication, will improve mutual understanding of needs in a resource-poor environment and the involvement of trainees from both income settings can help perpetuate long-term collaborations.

BACKGROUND:The repair of rectoperineal fistulae can pose a significant challenge to the pediatric surgeon given the proximity of the fistula to the urethra in males and vagina in females. In these children, a simple cutback procedure may leave the neoanus in a position anterior to the center of the sphincter, which theoretically could impair future continence. We devised an adaptation of the cutback anoplasty which we call the posterior rectal advancement anoplasty (PRAA) to treat patients with a rectoperineal fistula that is both narrow in lumen and located within, but at the anterior-most limit of the sphincter complex.MATERIAL AND METHODS:Patient selection, operative steps, and perioperative care of patients undergoing PRAA are detailed.RESULTS:10 children (6 males, 4 females) underwent PRAA. There were no vaginal wall or urethral injuries. At 6 months postoperatively, all patients were passing stool spontaneously. No patients required dilation of the anoplasty in the postoperative period and there were no anal strictures identified.CONCLUSIONS:A modification of the cutback anoplasty can be performed in patients with a perineal fistula and the distal fistula tract within the sphincter complex. We have demonstrated that this can be performed safely and obviates the need for an anterior rectal wall dissection, thus eliminating the risk of injury to urethra or vagina.LEVEL OF EVIDENCE:IV.

A wide spectrum of vascular anomalies exists and the International Society for the Study of Vascular Anomalies (ISSVA) have classified all known lesions as either vascular tumors (benign, borderline, malignant) or vascular malformations (capillary, venous, arteriovenous, combined, syndromic of vessel specific). Infantile hemangiomasInfantile hemangioma (IH) are both the most common vascular tumor and vascular anomaly. They usually only appear in the first few weeks of life and undergo a characteristic proliferation phase from about 3 months of life with a plateau phase around a year of age followed by an involution phase, which takes many years. Propranolol has proven a very successful medical therapy at managing IH complicated by ulceration or functional impairment, e.g., paraglottic, periocular. Congenital hemangiomas are fully grown at birth and exhibit either rapid involuting or non-involuting behavior, the latter of which require surgical resection if complicated by pain, ulceration, or cosmetic/functional impairment, although some can be managed with pulsed-dye laser therapy. Kaposiform hemangioendotheliomas are borderline tumors which invade locally but do not metastasize. They can be associated with a potentially fatal constellation of severe thrombocytopenia and coagulopathy known as Kasabach-Merritt phenomenonKasabach-Merritt phenomenon and vincristine is now the first-line medical therapy for unresectable lesions, which constitutes most lesions. Any child with ≥5 cutaneous hemangiomas should be screened for hepatic hemangiomas, which, if large (multifocal or diffuse) can give rise to high-output cardiac failure or consumptive hypothyroidism. Capillary malformations (port-wine stains) are the most common form of vascular anomaly. Large (segmental) capillary malformations may be associated with underlying spinal or CNS abnormalities and they may form part of a wider disorder, such as capillary malformation-arteriovenous malformation syndrome. Venous malformations are largely sporadic but challenging to treat. They may be complicated by thrombosis, pain, and expansion, especially peripubertally. Compressive garment therapy is the basic mainstay of treatment, with sclerotherapy being the first-line treatment for complicated lesions. Arteriovenous malformations may occur in isolation or as part of a wider overgrowth disorder, such as Parkes Weber syndrome. They follow a well described life cycle, and when they complicate with ulceration, pain, and expansion, treatment with embolization with or without surgical resection is often necessary. With many vascular malformations and some vascular tumors, treatment is often with the intent of symptom control and functional preservation rather than curative intent. Vascular anomalies are best managed in a center with a specialized multidisciplinary team with dermatology, surgery, interventional radiology, and appropriate adjunct specialists. A range of antiangiogenic pharmacotherapies are becoming available which may have a role in the management of patients with vascular malformation-associated overgrowth disorders such as Klippel-Trenaunay syndrome and CLOVES syndrome.

Gastric volvulus (GV) is a rare surgical emergency, defined by the abnormal rotation of a part of the stomach around another part, leading to obstruction and, in some cases, tissue ischemia and necrosis. Anatomically, most cases are either organoaxial volvulus, occurring along the stomach’s longitudinal axis from the gastroesophageal junction to the pylorus, or mesenteroaxial volvulus, occurring perpendicular to the longitudinal axis, such that the pylorus and antrum come to lie above the gastroesophageal junction. Associated contributory diaphragmatic anomalies are common, especially in neonates, while splenic anomalies are also frequent. GV may present as an acute or chronic condition. The mode of presentation varies depending on age. Neonates and infants may present with respiratory distress, non-bilious vomiting, excessive salivation, and regurgitation of feeds. Older children may present with Borchardt’s triad of non-productive retching, localized epigastric swelling, and failure to pass a nasogastric tube. Diagnosis relies on index of suspicion and is aided by plain radiography of the chest and abdomen and upper gastrointestinal contrast series to clarify the anatomical orientation of the stomach. Suggestive plain radiography findings include spherically distended stomach with two air-fluid levels or a double retrocardiac air-fluid level, as is the case with intrathoracic GV. In acute GV, surgery is the management of choice and involves correction of any contributory anatomical abnormalities such as diaphragmatic hernia as well as fixation of the stomach to the anterior abdominal wall with a gastrostomy and/or anterior gastropexy. Minimally invasive approaches are being more frequently employed to achieve these goals.

BACKGROUND:Despite increased utilization of robotic-assisted surgery in the pediatric population during the past decade, reports of comparative analysis between robotic surgery and laparoscopic surgery are lacking. Our aim was to evaluate outcomes between pediatric robotic-assisted cholecystectomy (RC) and laparoscopic cholecystectomy (LC). METHODS:A single institution retrospective analysis of 299 patients undergoing either RC or LC, between January 2015 and December 2018 was performed. Demographic data as well as clinical characteristics and related outcomes were abstracted and compared using univariate analysis. Related hospital costs were estimated using a charge to cost methodology. RESULTS:The median age of the cohort was 15.5 years (IQR 14.0-17.0); 76% females and 70% white, with 74% (n = 220) undergoing LC and 26% (n = 79) undergoing RC. The majority of RC were performed using single-site technique and RC proportion increased with time (10% in 2015 vs. 41% in 2018, p<0.001). The majority of RC were more commonly attributed to patients with nonacute indications for cholecystectomy compared to acute clinical indications (87% vs. 13%). Median operative time was 98 min vs. 79 min for RC and LC respectively (p<0.001). Median postoperative LOS was similar between groups (22 h). There were no significant differences in postoperative complication, in-hospital opioid utilization and 30-day readmissions. Average total hospital costs for RC were $15,519 compared to $11,197 for LC. CONCLUSIONS:Pediatric robotic-assisted cholecystectomy is feasible with similar outcomes compared to laparoscopic cholecystectomy. However, it is associated with longer operative times and higher costs. The single-site RC technique may provide a potential cosmetic benefit.

To compare the quality of life (QoL) in children with spina bifida with a control group of their peers using a validated questionnaire, the Neurogenic Bowel Dysfunction Score (NBDS). The NBDS questionnaire was prospectively distributed to children attending a multi-disciplinary Spina Bifida clinic and healthy controls attending pediatric urology clinics. A score (out of 41) was assigned to each child based on their responses to the validated questionnaire. A lower score indicates better bowel function-related quality of life. SPSS software (v.25) was used for all statistical analysis. There were 98 respondents to the questionnaire, 48 children with spina bifida and 50 controls. The average age of respondents was 7.88 years (3–16 years). Of those with Spina Bifida, 33 (69%) were on retrograde rectal irrigations, [19 (58%) Peristeen® system, 11 (33%) tube rectal irrigations, and 3 (9%) Willis system], 6 (12%) were on laxatives, and 9 (19%) were on no treatment. The median NBDS for Spina Bifida patients was significantly higher 13.5 (2–32) compared to the control group 2 (0–26, p < 0.001). Amongst Spina Bifida patients, there was no difference in quality of life between the modalities of bowel management (p = 0.203). Despite active bowel management, children with spina bifida report a worse quality of life compared to the control group. In those with spina bifida, the lack of a difference between various bowel management strategies, including no treatment, indicates the need for a longitudinal study to evaluate the basis for this unexpected finding.

Background/purpose The pathogenesis of Hirschsprung's disease-associated enterocolitis (HAEC) is unclear. Caveolin-1 (Cav-1) regulates the functions of different nitric oxide synthase (NOS) isoforms, which play critical roles in inflammation and intestinal epithelial barrier function. We designed this study to investigate the hypothesis that Cav-1 expression is altered in the bowel of patients with Hirschsprung's disease (HSCR). Methods HSCR tissue specimens (n = 10) were collected at the time of pull-through surgery and control samples were obtained at the time of colostomy closure in patients with imperforate anus (n = 10). qRT-PCR analysis was undertaken to quantify Cav-1 gene expression, and Western blot analysis was undertaken to determine Cav-1 protein quantification. Immunolabelling of Cav-1 proteins was visualized using confocal microscopy. Results qRT-PCR and Western blot analysis revealed that Cav-1 was significantly downregulated in the aganglionic and ganglionic colon of patients with HSCR compared to controls (p < 0.01). Confocal microscopy revealed a markedly decreased expression of Cav-1 in colonic epithelium of aganglionic and ganglionic bowel of patients with HSCR compared to controls. Conclusion To our knowledge, this is the first report of significantly decreased Cav-1 expression in patients with HSCR. Decreased expression of Cav-1 in the bowel of HSCR may increase susceptibility to HAEC in HSCR.

Purpose: The pathophysiology of Hirschsprung's associated enterocolitis (HAEC) is not understood. Abnormal intestinal motility and altered intestinal epithelial barrier function have been suggested to play a key role in the causation of HAEC. Protease-activated receptors (PARs) 1 and 2, have been implicated in inflammatory reactions, intestinal permeability and modulation of motility in the gut. Methods: We investigated PAR-1 and PAR-2 protein expression in aganglionic and ganglionic regions of patients with Hirschsprung's Disease (HSCR) (n = 10) versus normal control colon (n = 10). Protein distribution was assessed by using immunofluorescence and confocal microscopy. Gene and protein expression were quantified using quantitative real-time polymerase chain reaction (qPCR), western blot analysis, and densitometry. Results: qPCR and Western blot analysis revealed that PAR-1 and PAR-2 expression was significantly increased in ganglionic and aganglionic bowel in HSCR compared to controls (p < 0.003). Confocal microscopy revealed strong PAR-1 and PAR-2 expression in smooth muscles, interstitial cells of Cajal (ICCs), platelet-derived growth factor-alpha receptor-positive (PDGFR alpha(+)) cells, enteric neurons and epithelium in the ganglionic and aganglionic bowel compared to controls. Conclusion: Increased PAR-1 and PAR-2 expression in the colon of patients with HSCR suggests that excessive local release of PAR activating proteases may trigger inflammatory responses leading to HAEC. (c) 2019 Elsevier Inc. All rights reserved.