Long-term outcome of JJ stent insertion for primary obstructive megaureter in children.

BACKGROUND/AIM: Endoscopic stenting is an accepted treatment option for children with symptomatic or progressive primary obstructive megaureter (PROM). Here, long-term outcomes with endoscopic stenting are reviewed. METHODS: Patients with PROM treated surgically over a 12-year period were identified using a prospectively maintained departmental database. Data were analysed using Microsoft Excel 2013 and unpaired t-tests through GraphPad Software QuickCalcs. RESULTS: Fifty-seven patients with PROM were surgically managed in the study centre from 2005 to 2017. Twenty-nine of fifty-seven patients had the stent as the primary procedure, whereas the remainder had ureterostomy, re-implantation or nephrectomy. Six patients had bilateral PROM, giving a total of 35 renal units that were fully analysed. There was 7:1 male predominance, and 20 of 29 patients (69%) were diagnosed antenatally. The median age at stent insertion was 8 months (40 days-10 years); the median prestent ureteric diameter was 19 mm and the median pre-operative function on MAG3 was 44%. Cystoscopic stent insertion was feasible in all patients. The stent was left for a median of 183 days. In 9 of 35 (26%) renal units, the JJ stent was a successful sole long-term treatment, with median follow-up of 5 years and 8 months. The success rate was not different in children aged <1 year (8/22; 36%) in comparison to children aged >1 year (1/13; 8%), P = 0.1. The remaining 26 renal units required further surgical intervention: ureteric re-implantation in 25 and nephrectomy in one. Indications for further surgery were stent complications in 11 renal units and stent failure in 15 (Table 1). Complications related to the stent were noted in 14 renal units (41%), half being stent migration. Other complications included UTIs, stent encrustation and recurrent haematuria. There was no identifiable prestent parameter, whether clinical or radiological, that could predict which patients were likely to be successfully managed solely by stent insertion. Stent insertion was never successful as a definitive procedure when the distal ureteric diameter was >12 mm on the ultrasound after stent removal. DISCUSSION: Success rates with primary stenting as a sole treatment for PROM was 26%, which is less than that seen in other reports (50-66%). This may be attributed to the long-term follow-up in this study, together with the strict criteria for success. CONCLUSION: In the authors' experience, cystoscopically inserted JJ stents are of limited success as the sole treatment for PROM. In infants aged <1 year, stent insertion remains a reasonable temporising measure until the infant is old enough for a definitive procedure.

Dangerous deliveries: lessons learned during retroperitoneal specimen retrieval.

Laparoscopy is now a standard technique in pediatric surgery and urology. Unique complications have been reported during port/instrument insertion and dissection, often relating to issues of visibility or working space. Complications during specimen retrieval are currently unreported. We describe our experience of 2 serious complications occurring during attempted retrieval of a specimen through a port site at the end of the laparoscopic procedure.

Graft-versus-host disease and phimosis.

Graft-versus-host disease rarely causes genitourinary problems. We report a case of pathological phimosis in a child secondary to chronic graft-versus-host disease.

Pediatric urodynamics: baseline audit and effect on management.

OBJECTIVES: To undertake a baseline audit of our pediatric urodynamic service, identifying areas for improvement, and to determine whether clinical management was affected by urodynamic results. PATIENTS AND METHODS: All pediatric urodynamic studies during one calendar year were reviewed to determine the quality of reports that were issued and to assess any problems encountered. A postal questionnaire was sent to all referring doctors to determine whether the urodynamic report had influenced management. RESULTS: In all, 48 children attended for videocystometry, with successful tests in 39 (81%); 97% of written reports were judged to contain adequate information. In all, 33 postal questionnaires were returned (85%); in 30 (91%) the referring clinician felt that the urodynamic result had directly influenced management. CONCLUSION: The audit highlighted areas for improvement, which have been addressed. The response from the postal questionnaire showed that urodynamics directly influenced the management of children with complex urological and neurological abnormalities.

Assessment of protein gene product 9.5 as a marker of neural crest-derived precursor cells in the developing enteric nervous system.

The neurons and glial cells of the enteric nervous system (ENS) are derived from the neural crest. To study the developmental events involved in congenital abnormalities of the ENS, it is essential to identify all neural-crest cells (NCC) in the prenatal gut. The low-affinity neurotrophin receptor p75 is currently considered to be a gold-standard marker, but because it is a membrane protein, it is lost during procedures that permeabilise cells that are necessary to identify intracellular components and in apoptosis and cell-proliferation assays. We have therefore assessed the potential of the intracellular neuronal marker protein gene product (PGP) 9.5 as a label for neural-crest-derived precursor cells during gut development. Gut was taken from mouse embryos at 11.5 days post-coitum, at which time NCC had reached the proximal colon. Cellular p75 and PGP9.5 expression was determined by double-labelling immunofluorescence. The leading edge of neural-crest migration was defined as the 10 most distal p75-labelled cells. The neuronal marker PGP9.5 labelled NCC as they migrated along the gut at day 11.5. At the leading edge of migration, over 95% of p75-positive cells also expressed PGP9.5, and all PGP9.5-positive cells were also labelled for p75. PGP9.5 is expressed by at least 95% of neural-crest-derived precursor cells at the leading edge of migration along the gut. Thus, it can be used as a robust marker for developing NCC in the gut.

Time-dependent effects of endothelin-3 on enteric nervous system development in an organ culture model of Hirschsprung's disease.

BACKGROUND/PURPOSE: Terminal colonic aganglionosis (Hirschsprung disease) results from incomplete rostrocaudal colonisation of the embryonic gut by neural crest cells (NCC). Mutations in the genes encoding endothelin-3 (EDN3) or its receptor (EDNRB) have been shown to result in a similar aganglionosis. This article describes the development of an organ culture model using embryonic murine gut to determine how endothelin-3 regulates development of the enteric nervous system. METHODS: Gut explants from mice of different gestational ages were cultured for up to 3 days in the presence or absence of 5 micromol/L of the specific endothelin-B receptor antagonist BQ788. EDN3 and EDNRB mRNA expression were analysed by reverse-transcription polymerase chain reaction (RT-PCR) and whole-mount in situ hybridisation. NCC were localised using immunoreactivity for PGP 9.5, a specific neuronal marker. RESULTS: EDN3 mRNA continued to be expressed by caecal mesenchymal cells and EDNRB mRNA by the migrating NCC in culture. Embryonic day (E)11.5 explants were already colonised by NCC up to the terminal ileum. Complete colonisation occurred in organ culture over the next 72 hours (equivalent to E 14.5). Explants of E 12.5 and E 13.5 showed complete colonisation after 48 and 24 hours culture, respectively. Terminal aganglionosis resulted from treatment of E 11.5 and E 12.5 gut explants with 5 micromol/L BQ788, whereas there was no inhibitory effect on E 13.5 explants. CONCLUSIONS: An organ culture model has been developed in which NCC colonisation of embryonic gut mirrors that described in vivo. Blockade of the EDN3/EDNRB receptor pathway shows that the interaction of endothelin-3 with its receptor is only necessary for NCC colonisation at early time-points, despite the continued expression of endothelin-3 mRNA in the gut.

Ontogeny of interstitial cells of Cajal in the human intestine.

BACKGROUND/PURPOSE: Interstitial cells of Cajal (ICC) recently have been identified as intestinal pacemaker cells. Abnormalities in ICC are increasingly recognized in a number of neonatal disorders such as infantile hypertrophic pyloric stenosis, Hirschsprung's disease, and transient intestinal pseudo-obstruction. The aim of this study was to determine the fetal and postnatal differentiation and development of ICC in the human gastrointestinal tract to aid interpretation of pathological specimens. METHODS: Specimens of human gastrointestinal tract from (1) fetuses (9 to 17 weeks' gestation; n = 12), (2) premature and full-term neonates with non-gut motility-related disorders, (age 26 to 59 weeks' gestation; n = 13), and (3) children (age 4 months to 13 years; n = 7) were immunohistochemically stained with antibodies to c-kit(a marker for ICC) and protein gene product 9.5 (PGP9.5, a marker for neural tissue). RESULTS: (1) C-kit-positive ICC were present throughout the gut in all specimens including those from the earliest gestational ages. C-kit and PGP9.5 immunoreactivities were present in different cell populations. (2) The distribution of ICC varied with gestational age and with region of the gut. (3) Maturation of ICC networks continues postnatally in a region-specific manner. CONCLUSIONS: ICC are present from an early stage in human gut development. Interpretation of apparent abnormalities in ICC distribution as being of pathological significance should be tempered by the knowledge that ICC networks continue to develop postnatally and that ICC development varies throughout the gut.

Expression of endothelin 3 by mesenchymal cells of embryonic mouse caecum.

BACKGROUND: Mutations in endothelin 3 (EDN3) and endothelin B receptor (EDNRB) genes cause terminal colonic aganglionosis in mice, and mutations in these genes have also been linked to the terminal aganglionosis seen in human Hirschsprung's disease. However, details of EDN3 expression during embryogenesis are lacking, and consequently the cellular mechanism by which EDN3 regulates innervation of the terminal gut is unclear. AIMS: To localise the expression of EDN3 and EDNRB in the embryonic mouse gut. METHODS: Expression of EDN3 and EDNRB mRNA was analysed by reverse transcription polymerase chain reaction and in situ hybridisation. RESULTS: High levels of EDN3 mRNA expression were restricted to mesenchymal cells of the caecum before and after the arrival of neural crest cells. In contrast, EDNRB expression along the gut displayed a time dependent pattern similar to those of the protein tyrosine kinase ret and the neural crest cell marker PGP9.5. CONCLUSIONS: Mesenchymal cells of the caecum express high levels of EDN3 mRNA during embryogenesis and hence the production of EDN3 at the caecum is likely to act on neural crest cells as a paracrine factor necessary for subsequent innervation of the terminal gut.

Subluxation of the hip joint in multiple hereditary osteochondromatosis: report of two cases.

We report two cases of children with multiple hereditary osteochondromatosis (MHO) in whom painful restriction of hip movement developed due to intraacetabular osteochondromata. Excision of the lesions relieved pain and restored joint movement after 14 and 3 months' follow-up, respectively. Long-term follow-up of these patients is essential.

The value of QTc dispersion in assessment of cardiac risk in elective aortic aneurysm surgery.

OBJECTIVES: To determine the value of QTc dispersion in predicting cardiac risk in aortic aneurysm surgery. DESIGN: Retrospective case-control study. MATERIALS: One hundred and twenty-six patients who had abdominal aortic aneurysm surgery between May 1992 and April 1996. METHODS: Nine patients experienced a postoperative cardiac complication defined as myocardial infarction or cardiac death. Twenty-four age and sex-matched controls who had uncomplicated aortic surgery were selected at random. QTc dispersion was calculated from the preoperative 12 lead electrocardiograms. RESULTS: The mean QTc dispersion in the cardiac complication group was greater than the control group (63.1 ms1/2 vs. 50.4 ms1/2) but the difference did not approach statistical significance. CONCLUSIONS: QTc dispersion cannot be recommended as a predictor of cardiac complication following elective aneurysm repair.

Chiropractic treatment of chronic 'whiplash' injuries.

Forty-three per cent of patients will suffer long-term symptoms following 'whiplash' injury, for which no conventional treatment has proven to be effective. A retrospective study was undertaken to determine the effects of chiropractic in a group of 28 patients who had been referred with chronic 'whiplash' syndrome. The severity of patients' symptoms was assessed before and after treatment using the Gargan and Bannister (1990) classification. Twenty-six (93 per cent) patients improved following chiropractic treatment (U = 34, P < 0.001). The encouraging results from this retrospective study merit the instigation of a prospective randomized controlled trial to compare conventional with chiropractic treatment in chronic 'whiplash' injury.