Does anorectal manometry predict clinical outcome after laparoscopic ACE procedures in children with spina bifida?

PURPOSE: The laparoscopic antegrade continence enema (LACE) procedure is used for the treatment of faecal incontinence in children with spina bifida. The purpose of this study was to relate the outcome of the LACE procedure to anorectal function, as determined by anorectal manometry. METHOD: Eleven children with spina bifida who had the LACE procedure underwent anorectal manometry (ARM) to document their anorectal function, and its relationship to the level of continence obtained following surgery. RESULTS: There was a consistently high level of functional continence achieved following surgery despite wide variability in the parameters of anorectal manometry. There was no demonstrable correlation between the outcome of the LACE procedure and anorectal function as assessed by manometry. CONCLUSION: Anorectal function as determined by manometry failed to predict outcome after the laparoscopic ACE procedure in children with spina bifida.

Iliopsoas haemophiliac pseudotumours with bowel fistulation.

Two cases of iliopsoas haemophilic pseudotumours are presented. In one patient a fistula developed between a pseudotumour and the large bowel. This resulted in an abscess involving the pseudotumour and adjacent tissues. It resolved after 5 years of therapy involving percutaneous drainage and closure of the fistula. The second patient had a massive pseudotumour that had obstructed both ureters. Later he suffered a fatal mixed Gram negative septicaemia probably related to erosion into the colon.

Comparison of results of laparoscopic and open antegrade continence enema procedures.

Several modifications of the Malone antegrade continence enema (ACE) procedure have now been reported. In this study we have compared the results of our experience with the laparoscopic appendicostomy (LACE procedure) with the published results of previously described open ACE procedures. Children who had the LACE procedure at our institutions were reviewed. Intra- and postoperative problems were identified by review of hospital case notes and from a questionnaire completed by the children's caregivers. These results were compared with the reported results of the original Malone procedure, the Peña modification, and the open appendicostomy as described by Wilcox. Since 1994, 30 children have had LACE procedures performed at our institutions. Two have required conversion to an open procedure because of difficulty locating the appendix. The stoma is being used for regular antegrade colonic washouts in 29 of the 30, compared with 19 of 31 in Malone's series and 16 of 20 in Peña's. Improvement in soiling has been achieved in 27 (90%) of our patients, 15 of whom are completely clean, a rate similar to that of the other types of procedure (61%-78%). Stenosis of the stoma has occurred in 8 children (27%), compared with rates of 10%-33% in other series. Stomal leakage has been troublesome for 2 (6.7%), compared with leak rates between 5.6% and 15% in other series. The LACE procedure is technically the simplest. The laparoscopic approach to the ACE procedure appears to be a simpler and safe alternative to previously described methods. It causes minimal morbidity, has a high long-term viability rate, and resulted in improved control of soiling in 90% of the children with faecal incontinence in whom it has been employed.

Early experience with the laparoscopic ace procedure.

BACKGROUND: In 1990 Malone et al. introduced the reversed appendicocaecostomy as a technique to enable an antegrade continence enema (ACE) to be administered. The aim is to achieve colonic emptying and thus prevent soiling in children with faecal incontinence. The procedure has been modified by suturing the opened appendix directly to the skin as a stoma, and can now also be performed laparoscopically. METHODS: All children undergoing laparoscopic ACE procedures at Christchurch Hospital, Christchurch, New Zealand and Princess Margaret Hospital, Perth, Australia, were reviewed. Intra-operative and postoperative problems were identified retrospectively and questionnaires were completed by the children's caregivers detailing their experience with managing their children following the ACE procedure. RESULTS: Thirty children have had a laparoscopic ACE procedure performed since 1994. Two required conversion to an open procedure because of difficulty locating the appendix. Twenty-nine children are currently using the stoma for regular antegrade colonic washouts. Improvement in continence was seen in 27 children, 15 of whom have been completely continent. Stenosis of the stoma occurred in eight children, two of whom required operative revision of the stoma; the others have been managed by daily dilatation, either with the irrigation catheter or a small perspex dilator. More recently, spatulating the appendix tip has reduced the incidence of stenosis. Two children have had troublesome leaks. CONCLUSIONS: The laparoscopic approach to the ACE procedure results in an improved level of continence in this difficult group of children with faecal incontinence. It is a simpler alternative to previously described methods and causes minimal morbidity. Results to date suggest a satisfactory outcome for both the children and their families.

Congenital caecal cystic duplication presenting with caecal volvulus in an elderly woman.

Congenital caecal cystic duplication of the gastrointestinal tract usually present during infancy or young childhood. We have identified a patient who presented at 91 years of life with a caecal volvulus caused by a large communicating caecal cystic duplication. Review of the literature shows that caecal cystic duplications become symptomatic early when they cause complications, but until this report, volvulus was not a recognised presentation of caecal cystic duplication.

Down-regulation of nuclear aryl hydrocarbon receptor DNA-binding and transactivation functions: requirement for a labile or inducible factor.

Aryl hydrocarbons (AHs) such as 2,3,7,8-tetrachlorodibenzo-p-dioxin and benzo[a]pyrene activate the sequence-specific DNA-binding activity of the AH receptor. In the rat hepatocyte-derived cell line LCS7, DNA-binding activity peaked after 30 min and was then down-regulated, reaching negligible levels by 2 h. Down-regulation could be blocked, and DNA-binding activity maintained at maximum for many hours by inhibiting protein or RNA synthesis, implying that down-regulation is a mediated process requiring a labile or inducible protein. CYP1A1 transcription and in vivo DNA-protein interactions at xenobiotic response elements were down-regulated in parallel with DNA-binding activity in nuclear extracts, and these changes could also be blocked by inhibitors of protein synthesis. The correlation between AH receptor DNA-binding activity, intensity of in vivo footprints at xenobiotic response elements, and CYP1A1 transcription rate implies that down-regulation of AH receptor DNA-binding activity is important in regulating CYP1A1 transcription and that receptor is required continuously to maintain transcription. This correlation extends to the murine hepatoma cell line Hepa-1c1c7, in which slower kinetics of activation and down-regulation of CYP1A1 transcription paralleled slower activation and down-regulation of AH receptor DNA-binding activity. The difference in kinetics between cell lines also implies that AH receptor DNA-binding activity is modulated by a mechanism that may be influenced by cell-specific regulatory pathways. The above observations in conjunction with mixing experiments and comparisons of cytoplasmic and nuclear extracts indicate that down-regulation of AH receptor DNA-binding activity is probably due either to degradation or to conversion of the receptor to form that is inactive in both DNA binding and transactivation.

Aryl hydrocarbon-induced interactions at multiple DNA elements of diverse sequence--a multicomponent mechanism for activation of cytochrome P4501A1 (CYP1A1) gene transcription.

In vivo footprinting experiments, augmented with gel shift and transfection analyses suggest that activation of the CYP1A1 gene by aryl hydrocarbons may be a multicomponent process. During the first 30 minutes of exposure to aryl hydrocarbon carcinogens and environmental contaminants, in vivo footprints appear at nine distinct sites within a 281 bp region centered 950 bp upstream of the CYP1A1 transcription start site. Six of these sites are unrelated in sequence to the three xenobiotic response elements (XREs) within this region, at which the aryl hydrocarbon (AH) receptor is known to bind. These six display a variety of footprint patterns, are diverse in sequence and range in G-C content from 60 to 75%. This diversity suggests that multiple nuclear factors may be responsible for these six in vivo footprints. These observations are consistent with competition gel shift experiments showing that the nuclear factors binding at two of these sites are different from each other, as well as from the AH receptor. Gel shifts also indicate that the sequence-specific factors binding at these sites are expressed constitutively. This is consistent with a model in which in vivo footprints are induced at these six sites, not through direct activation or de novo synthesis of DNA-binding factors, but through a two phase mechanism in which binding of the nuclear AH receptor complex to XREs facilitates the binding of constitutive factors at these sites. This facilitation could be mediated either through specific protein-protein interactions or through alterations in chromatin structure that make these sites accessible to constitutive nuclear factors. A function for the sequences at which aryl hydrocarbons induce in vivo footprints is suggested by transfection experiments showing that one of these sequences cooperates with a weak XRE to confer on a reporter gene responsiveness to aryl hydrocarbons.

Segmental phenolization of ingrowing toenails: a randomized controlled study.

One hundred and three patients, on whom 107 procedures for ingrowing toenails were performed, were randomly allocated into one of two treatment groups: segmental or angular phenolization and wedge excision. There were 53 wedge excisions and 54 segmental phenolizations. Post-treatment discomfort was assessed on a linear analogue scale of 10 cm. There was no difference between the two groups one week after treatment. Over a mean follow-up period of 14 months a total of 20 nail spikes occurred, 4 in the phenolized group and 16 in the wedge excision group, this being a significant difference at the one per cent level.