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D C Crabbe

Publications and source records attributed to D C Crabbe.

14 recordsLinked to original sources
PubMed2000

Hirschsprung's disease: problems with transition-zone pull-through.

BACKGROUND/PURPOSE: It is generally accepted that if surgery for Hirschsprung's disease is to be successful, ganglionic bowel must be anastomosed to the lower rectum or anal canal. Above the aganglionic distal bowel lies a transition zone (TZ) where more subtle abnormalities of innervation are apparent. The significance of this transition zone in respect to the functional outcome of surgery has received little attention. The aim of this study was to identify the incidence of transition zone pull-through (TZPT) in a cohort of children who underwent surgery for Hirschsprung's disease, to identify the reasons why TZPTs occurred, and to identify the functional consequences. The authors report the long-term outcome of these children with emphasis on bowel function and the results of subsequent surgery. METHODS: A Retrospective study was conducted of children treated at a single institution from 1979 through 1994. TZPT patients were subject to detailed review of surgical records and histopathologic material. RESULTS: Thirteen children were identified with a TZPT. In 12 cases, histopathologic errors contributed to the TZPT: in 5 cases this was caused by single point biopsies missing an asymmetrical TZ, whereas in 7 cases the histopathologic features of the TZ were not recognized. In 1 case the TZPT was caused by surgical error. As a consequence of the TZPT 7 children underwent repeat pull-through. One child is fully continent, one has daytime fecal continence, and 2 others are incontinent. Two children have permanent stomas. One child is clean with antegrade colonic washouts. Repeat pull-throughs were not attempted in 6 children. Two children have achieved full continence, 2 have permanent stomas, 1 is clean with antegrade colonic washouts, and 1 child receives regular suppositories. CONCLUSIONS: Transition zone pull-throughs occurred because of a combination of surgical and histopathologic errors. The transition zone may follow an asymmetric course around the circumference of the bowel and may be missed if single-point extramucosal biopsy specimens are taken. Recognition of the subtle histologic features of the transition zone requires an experienced pathologist. The functional consequences of a TZPT are severe, with symptoms of constipation, diarrhea, and incontinence. The results of revisional pull-through were disappointing. Serious consideration should be given to alternative procedures such as the antegrade continence enema operation.

Child↗
PubMed1998

Posterior sagittal proctectomy.

Rectal excision for non-malignant conditions using a posterior sagittal approach is described in three patients. The technique allows excellent exposure of the rectum, meticulous haemostasis, minimal risk of pelvic nerve injury and accurate reconstruction of the pelvic floor without the need for drainage.

Adenomatous Polyposis Coli↗
PubMed1997

Separation of thoraco-omphalopagus conjoined twins with complex hepato-biliary anatomy.

This report describes separation of thoraco-omphalopagus conjoined twins and management of the hepatobiliary complications that ensued. The livers were fused and, although both infants had gallbladders, the extrahepatic biliary trees were fused. After separation, obstructive jaundice developed in one twin and she required a biliary diversion. The other twin had an external biliary fistula that only closed after resection of a segment of liver with no connection to the ipsilateral biliary tree. Closure of the abdominal wall defects with biodegradable patches proved very satisfactory and eliminated the problems with persistent wound sinuses related to nonabsorbable mesh.

Abdomen↗
PubMed1996

Management of the isolated congenital tracheo-oesophageal fistula.

Over a ten-year period sixteen infants with isolated congenital tracheo-oesophageal fistulas (TOF) were treated. All infants were symptomatic from birth although the diagnosis was only established in the neonatal period in eleven cases. Tube oesophagography was the diagnostic investigation of choice. Bronchoscopic cannulation of the fistula greatly assisted division, which was achieved through a cervical incision in all cases. There were two early recurrent fistulas both of which closed spontaneously. Persistent oesophageal symptoms were common. Seven of the patients (43%) experienced dysphagia, three of whom developed oesophageal strictures requiring repeated dilation.

Deglutition Disorders↗
PubMed1996

Familial hiatus hernia and gastro-oesophageal reflux.

This report describes a family in which the father and three children had a sliding hiatus hernia. In each case there was gastro-oesophageal reflux from the neonatal period which proved unresponsive to medical therapy, ultimately requiring anti-reflux surgery. The trait in this family supports an autosomal dominant genetic component to the infant hiatus hernia.

Chromosome Aberrations↗
PubMed1993

Malignant rhabdoid tumor. A study with two established cell lines.

BACKGROUND: Malignant rhabdoid tumor (MRT), originally described as a rare renal sarcoma in childhood, has been known to express phenotypic diversity. In this study, unique characteristics of the MRT cells were investigated by using established cell lines. METHODS: Immunocytochemical, ultrastructural, cytogenetic, and molecular (by polymerase chain reaction, PCR) analyses were done for two MRT cell lines, one of renal and one of extrarenal origin, before and after differentiation-induction with either 12-O-tetradecanoyl phorbol-13-acetate (TPA) or transretinoic acid (RA). RESULTS: The proliferating cells in the original tumor tissues as well as in the established cell lines demonstrated neural, epithelial, and mesenchymal markers morphologically. Both cell lines had karyotypic abnormalities including chromosome 22q11.2. The cell line from the extrarenal MRT, Tm87-16, demonstrated distinct morphologic changes with neuroblastic differentiation and produced numerous neuritic processes after treatment with either TPA or RA. The cell line from the renal MRT, STM91-01, suggested schwannian differentiation but did not change morphologically after chemical induction. Both cell lines expressed c-myc, but did not express N-myc, MyoD1, tyrosine hydroxylase, or neural cell adhesion molecule (N-CAM). With PCR and immunocytochemical study, a high level of chromogranin expression was detected by the cells of Tm87-16 only after TPA induced differentiation. CONCLUSIONS: MRT cells demonstrated diverse phenotype of neuro-ecto-mesenchymal differentiation. The results of this study suggest that MRT may be derived from a primitive pluripotential cell, such as neural crest or equivalent. MRT, therefore, might be categorized as one of the subsets of primitive neuroectodermal tumor.

Gene Expression Regulation, Neoplastic↗
PubMed1992

Use of 99mtechnetium-dimercaptosuccinic acid to study patterns of renal damage associated with prenatally detected vesicoureteral reflux.

Static isotope imaging with 99mtechnetium-dimercaptosuccinic acid was performed at a mean age of 34 days in 32 children (50 kidneys) whose vesicoureteral reflux had been identified as a result of prenatal ultrasound scanning. Three patterns of isotope uptake were observed: 1) noninfected primary vesicoureteral reflux (15 children, 24 kidneys), 2) noninfected secondary (obstructed) vesicoureteral reflux (9 patients, 11 kidneys) and 3) infected primary reflux (8 infants, 15 kidneys). In 20 pattern 1 kidneys (83%) renal morphology and differential isotope were normal. In the 4 kidneys (17%) that showed evidence of impaired function this took the form of global parenchymal loss, that is small kidneys rather than focal scarring. In pattern 2 the combination of fetal vesicoureteral reflux and obstruction was a potent cause of renal damage with total or near total loss of function in 7 of 9 refluxing units associated with posterior urethral valves and in 2 kidneys with secondary ureteropelvic junction obstruction. Appearances of focal scarring were confined in pattern 3 and were found in 4 kidneys (27%). This overall incidence of detectable renal damage was lower than expected. Even when infection occurs, prenatal diagnosis may lessen the risk of scarring by enabling treatment to be instituted promptly. The findings suggest that uncomplicated primary vesicoureteral reflux is a relatively benign insult to the fetal kidney and that reflux nephropathy found in children presenting clinically is the result of infected vesicoureteral reflux in postnatal life. Any comparison of published studies will prove difficult until there is a more standardized approach to imaging technique and patient selection.

Female↗
PubMed1992

Rapid detection of MYCN gene amplification in neuroblastomas using the polymerase chain reaction.

We have used the polymerase chain reaction (PCR) to detect amplification of the MYCN oncogene in neuroblastoma cell lines and to distinguish primary tumors with a single copy from those with MYCN amplification using DNA extracted from frozen sections. Two primer pairs were used to co-amplify a 428-bp fragment of the MYCN oncogene along with a 268-bp fragment of the beta-globin gene (a single-copy reference standard). After 30 cycles of PCR, the products were resolved by agarose gel electrophoresis. MYCN gene amplification was identified by visual comparison of the relative intensities of MYCN and beta-globin PCR product bands on the ethidium bromide-stained gel. This semiquantitative approach, while inadequate for precise determination of copy number, provided a simple, rapid, nonisotopic method for differentiating tumors with MYCN amplification from those with a single copy. Seventy-four primary tumors were classified as amplified or nonamplified by semiquantitative PCR. Twenty-two of 23 tumors known to carry MYCN gene amplification by Southern analysis were correctly identified by PCR. The single false-negative result was due to a sampling error: DNA was extracted from a block of tissue containing small foci of tumor surrounded by normal tissue. Fifty-one of 51 tumors with a single copy of MYCN were also correctly identified by PCR. We conclude that semiquantitative PCR is a reliable, non-isotopic alternative to Southern blotting for detection of MYCN gene amplification that can be performed rapidly on DNA extracted from frozen sections.

Base Sequence↗
PubMed1990

Gastrointestinal complications of the haemolytic uraemic syndrome.

The haemolytic uraemic syndrome (HUS) is a well recognized cause of acute renal failure in children. Gastrointestinal involvement is common, and may result in rectal bleeding which can be an important presenting symptom. Previous publications have stressed the importance of correct diagnosis to avoid unnecessary surgery. Occasionally serious gastrointestinal complications do occur. We present five children with life-threatening gastrointestinal complications of the HUS and discuss the indications for laparotomy.

Child, Preschool↗