Diagnosis and therapy of ultrashort Hirschsprung's disease.

BACKGROUND: Although ultrashort Hirschsprung's disease (UHD) was enzyme-histochemically characterised about 35 years ago, its existence is still often ignored. The aim of this study is to summarise the clinical diagnostic, incidence, gender ratio, morphological characteristics, and therapy over 15 years. METHODOLOGY: The reliable diagnosis of suspected UHD requires a minimal enema of contrast medium to exclude Hirschsprung's disease (HD). In UHD during pressing or crying no reflux of contrast medium is observed. Final proof of UHD is an enzyme-histochemical biopsy examination of distal rectal mucosa. The biopsies must demonstrate submucosa and be taken from the dentate line and 1 cm, 2 cm, 4 cm and 6 cm above the dentate line. The cryostat sections must be cut 15 microm thick; this thickness is reduced to 4.5 microm by the thawing, spreading and drying of the sections on microscope slides. A reliable diagnosis of UHD needs an enzyme-histochemical acetylcholinesterase reaction of native sections of rectal mucosa. RESULTS: UHD develops with first symptoms of chronic constipation in the second half of the first year of life. The chronic constipation proves to be therapy resistant. In HD constipation occurs in the first weeks of life or after weaning. In contrast to HD, no nerve fibres with increased AChE activity are observed in the lamina propria mucosa. Nets of nerve fibres with increased AChE activity can be found only in the muscularis mucosa and the musculus corrugator cutis ani (MCCA). The therapy of choice has proven to be a partial myectomy of the distal internal sphincter if dilatation of the internal sphincter was ineffective. UHD is either limited to the anal ring, or extends 3 - 4 cm into the distal rectum. Over the past 15 years, UHD had in our series an incidence of 13.4 % of all aganglionoses. The gender ratio of girls to boys was 1 : 2. CONCLUSION: UHD is reliably diagnosed by an AChE reaction in native biopsy sections from the anocutaneous transitional zone and, potentially, from 3 - 4 cm above the pectinate line. As UHD is always accompanied by aganglionosis of the distal internal sphincter, an increase in AChE activity is observed in the nerve fibres of the MCCA. The therapy of choice is a partial myectomy of the distal internal sphincter.

[Diagnosis and primary surgical therapy of anorectal abnormalities with regard to postoperative incontinence]. / Diagnose and primäre operative Therapie von anorektalen Fehlbildungen im Hinblick auf die postoperative Inkontinenz.

Aspects relating to diagnosis of anorectal agenesis are covered in this paper, with reference being made to the author's patients at the Cologne Department of Paediatric Surgery. Accurate preoperative diagnosis of both the type of malformation relative to anatomic pelvic floor structures and of possible concomitant malformations is considered to be the key to subsequent optimal continence. Proper choice of an anatomy-correlated, individual surgical approach is possible only on the basis of accurate analysis of the malformation concerned and its correct assignment and classification according to Wingspread or Rehbein. Optimum continence has proved to depend also on involvement of a surgeon with profound experience in and with all forms of anorectal malformations as well as on subtle approach accompanied by uninterrupted electrostimulation to identify muscular structures. Yet, even with all those prerequisites optimally satisfied, about 25 percent of all patients with severe anorectal malformations must be expected not to achieve continence. This may be attributable to one or several of the following causes: The muscular structures applied may be too hypoplastic and thus may fail to develop sufficient sphincter functionality. Postoperative management may be insufficiently careful and cause atrophy of muscle equivalents restored in the first place. Continence may be difficult or even impossible to achieve for concomitant sacral or urogenital malformations. Application of colostomy should be avoided in any case, and advantage should be taken, first of all, of all possible ways and means described in this paper for restoration of sphincter action.

[Electrostimulation of the neurogenic bladder (author's transl)]. / Elektrostimulation der neurogenen Blase.

Six children with neurogenic disorders of micturition are stimulated by Katona's method. The evaluation of the clinical and cystometric results carried out every 4 weeks shows the contradictoriness of this method. The clinical results show an unequivocal improvement in micturition behavior, whereas checking the objective cystomanometric results only suggests an increasing tonicity of the detrusor and suppression of the uninhibited detrusor contractions. The urgency for carrying out cystomanometric measurements during Katona's electrostimulation therapy is pointed out.