Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: a population-based study.

OBJECTIVE: To assess bowel function and gastrointestinal quality of life among adults with operated Hirschsprung's disease (HD). SUMMARY BACKGROUND DATA: Outcomes of HD extending to adulthood are unclear; bowel function and quality of life may deteriorate by aging. METHODS: Bowel function and gastrointestinal quality of life were cross-sectionally assessed in a population-based manner among adults operated for HD during childhood between 1950 and 1986. Patients were interviewed during their outpatient visit. Controls matched for age and sex were randomly chosen from the Population Register Centre of Finland. RESULTS: Ninety-two (64%) patients representative for the entire study population responded. The mean age of patients (79% male) was 43 (interquartile range [IQR], 35-48) years. Most (78%) had undergone Duhamel operation, and 94% had aganglionosis confined to the rectosigmoid. The mean overall bowel function score was decreased among patients (17.1 ± 2.8 vs 19.1 ± 1.2; P < 0.0001). They reported increased incidence of inability to hold back defecation (40% vs 17%), fecal soiling (48% vs 22%), constipation (30% vs 9%) and social problems related to bowel function (29% vs 11%; P < 0.05 for all). Gastrointestinal quality of life was only marginally lower among patients (121 ± 15.3 vs 125 ± 13.1; P = 0.0578) mainly because of significantly lower scores in questions assessing disease-specific factors such as bowel function and continence. Age was the only predictor of poor bowel function (OR 1.07, 95% CI 1.00-1.14, P = 0.049), which weakly predicted gastrointestinal quality of life (OR 0.81, 95% CI 0.66-1.01, P = 0.055). CONCLUSIONS: Although bowel function deteriorates with increasing age after operated HD, it is associated with only slightly decreased gastrointestinal quality of life.

Anorectal manometry with reference to operative rectal biopsy for the diagnosis/exclusion of Hirschprung's disease in children under 1 year of age.

AIMS: To establish the accuracy of anorectal manometry (ARM) with reference to operative rectal biopsy in the investigation of children under 1 year of age with defaecation difficulties. MATERIALS AND METHODS: The records of all infants who underwent ARM and operative rectal biopsy at our centre between 1994 and 2007 were reviewed. ARM was performed by a consultant paediatric surgeon in each and under ketamine anaesthesia, as operative rectal biopsy was taken. RESULTS: There were 81 patients (49 males, 32 females). The median age at investigation was 2 months (range 0.1-11 months). Thirty-three patients (41%) had Hirschprung's disease. No patient with a normal recto-anal inhibitory reflex (RAIR) had Hirschprung's disease (100% negative predictive value). No patient with Hirschprung's disease exhibited a reflex (100% sensitivity). The specificity and positive predictive value of ARM for Hirschprung's disease were 83% and 80%, respectively. Bowel habit normalised in 88% of patients with a reflex within 1 year. CONCLUSIONS: In children under 1 year of age, Hirschprung's disease is very unlikely in the presence of a RAIR. The specificity and positive predictive value of ARM for the diagnosis of Hirschprung's disease are inferior to those of rectal suction biopsy, and therefore, it cannot be recommended for use as a sole diagnostic tool for this disease. ARM may be a useful investigation in patients where the histologic specimen is inadequate and/or functional constipation is the most likely diagnosis. If RAIR is present, rectal biopsy may not be required.

Successful resurfacing of scars from previous deliberate self-harm using Integra dermal matrix substitute.

BACKGROUND: There are currently limited treatment options available to modify the appearance of scarring due to deliberate self-harm (DSH). The authors present two cases of Integra dermal matrix use for resurfacing scars due to past DSH. METHODS: By excising the scars and resurfacing with Integra, we present and describe a novel technique for treating the visible stigmata of previous DSH. RESULTS: No complications occurred, and patients noted improved appearance, skin quality and self-esteem following surgery. Both patients would recommend this treatment to others. CONCLUSIONS: Integra dermal matrix substitute may be used successfully to treat scarring from previous DSH.

Segmental stem fracture of a cemented femoral prosthesis.

We report an unusual case of segmental stem fracture in 4 places of a cemented femoral prosthesis in a 43-year-old man 4 years after total hip arthroplasty. To our knowledge, this is the first reported case of segmental stem fracture. Detailed analysis of the prosthesis was performed at an independent testing laboratory. Initiation of all the cracks occurred on the medial aspect on the stem where the manufacturer's identification lettering was present, and the fractures occurred sequentially along the stem from distal to proximal. Laser etching is likely to have caused the microstructural alterations found on the surface of the stem, which, in turn, predisposed to fatigue cracking. Proximal debonding of the cement-bone interface observed in prefracture radiographs suggests that cantilever-bending stresses represented the initial mode of failure.

Bilateral ovarian metastases from carcinoma of the gallbladder.

BACKGROUND: Metastasis of carcinoma of the gallbladder to other intra-abdominal organs is recognised, but is rare. Ovarian metastases can mimic the clinical and morphological appearances of primary ovarian tumours, making the diagnosis difficult particularly when the primary source is not apparent. CASE: A patient presented with abdominal pain and was found to have gallstones and bilateral ovarian masses. Tumour markers were all normal. The patient developed jaundice and was subsequently operated on for the gallstones and ovarian masses. Histology showed these to be metastases from a gallbladder carcinoma. CONCLUSION: Presentation of ovarian metastases can cause diagnostic difficulty. Careful evaluation of the digestive tract should be considered in patients presenting with ovarian masses of uncertain origin.