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Publications (31)
An 11-year-old girl presented to the hospital with vomiting, left upper-quadrant pain and blood in the stool. An ultrasound scan showed no blood flow in the coeliac axis or the splenic artery. A contrast-enhanced CT scan then demonstrated no flow beyond the coeliac axis origin, a large clot in the distal superior mesenteric artery (SMA), a large sp...
The Lima constrictor was described in 1996 as a less complex and less expensive alternative to the artificial urinary sphincter for use in cases of pediatric neuropathic sphincter incontinence. The device provides a fixed periurethral resistance which creates continence, yet allows urethral catheterization without the need to deflate the cuff. We r...
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...
Lower urinary tract symptoms are common in pediatric patients. To our knowledge no validated instruments properly designed to screen lower urinary tract symptoms in the pediatric population have been published to date. In the International Consultation on Incontinence Questionnaire Committee the psychometric properties of a screening questionnaire...
Disorders of sex development (DSD) occur in 1–2/10,000 live births, with a specific molecular diagnosis only possible in 20% of cases. Presentation is usually at birth, and gender assignment must be avoided before review by an expert multidisciplinary team. Initial investigations allow a working diagnosis to be made within 48 hours. In 46,XY DSD, s...
We present the case of an 11-year-old boy diagnosed with an Effmann Type II A1 urethral duplication after routine circumcision for balanitis xerotica obliterans (BXO). We discuss the pathophysiology, investigation and management both of BXO and urethral duplication.
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.
Rectal duplication cysts are rare, comprising <5% of all gastrointestinal duplications. Early excision is the treatment of choice and a number of surgical approaches have been described. We present a 3-week-old infant with a 3 cm cyst that was excised using a previously unreported combined abdominal and endoanal approach.
Children with neurological and non-neurological lower urinary tract dysfunction normally undergo video urodynamics. One reason is to try to diagnose 'dangerous' bladders. Currently, bladder compliance is used to predict 'dangerous' bladders; however, in children there are no standardized methods of measurement and thus no 'cut-off' values. Complian...
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.
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...
Surgery is indicated in very few children with intractable functional constipation. A number of operations have been described with unpredictable outcome and significant morbidity. The authors present a series of 10 children who underwent a Hartmann procedure with end colostomy formation.
Preoperative management, in addition to maximum conservative...
Mutations in the endothelin-3 (ET-3) and endothelin-B receptor (EDNR-B) genes cause terminal colonic aganglionosis in mice and are linked to Hirschsprung's disease. These experiments are designed to determine if the development of terminal enteric ganglia depends on changes in proliferation, apoptosis, or differentiation of enteric neural crest (NC...
The aganglionosis in a variable length of the distal gut found in Hirschsprung's disease results from the abnormal prenatal development of neural crest-derived stem cells of the enteric nervous system. The cytokine endothelin-3 is necessary for successful colonization of the distal gut, but the location of this interaction with neural crest-derived...
Background/Purpose: The aganglionosis in a variable length of the distal gut found in Hirschsprung's disease results from the abnormal prenatal development of neural crest-derived stem cells of the enteric nervous system. The cytokine endothelin-3 is necessary for successful colonization of the distal gut, but the location of this interaction with...
Definitive treatment of Hirschsprung's disease involves ‘pull-through’ surgery, either as a primary procedure or following formation of a preliminary colostomy. Regular follow-up is essential postoperatively as children often have problems with stool frequency, peri-anal excoriation, constipation or even recurrent episodes of enterocolitis. In the...
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 m...
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 u...
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 p...
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...
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.
To determine the value of QTc dispersion in predicting cardiac risk in aortic aneurysm surgery.
Retrospective case-control study.
One hundred and twenty-six patients who had abdominal aortic aneurysm surgery between May 1992 and April 1996.
Nine patients experienced a postoperative cardiac complication defined as myocardial infarction or cardiac de...
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' symptom...
This paper reviews the presentation and management for congenital intrathoracic stomach (CIS). The features that distinguish CIS from congenital short oesophagus and acquired oesophageal shortening are emphasised. The study is based on a retrospective review of six patients treated between 1988 and 1994. The four youngest presented with vomiting an...
