Ileocaecal valve: how important is it?

PURPOSE: Our aim was to investigate the importance of the ileocaecal valve and its reconstruction in patients that are not suffering from short bowel syndrome and Crohn's disease. METHODS: Casenotes of 99 children with hemicolectomy and 24 children with terminal ileal resection were reviewed and sorted into three groups. Group 1: ileocaecal valve resection (limited hemicolectomy), Group 2: hemicolectomy, Group 3: terminal ileal resection between 10 and 25 cm. Patients with Crohn's, short bowel syndrome and incomplete follow-up were excluded. RESULTS: Chronic diarrhoea was documented in 7/26 cases (27%) in Group 1, 6/23 patients (26%) in Group 2, and none of the 13 patients had diarrhoea in Group 3. Pearson Chi-square test showed significant difference between Group 1 and Group 3 (p = 0.038) and between Group 2 and Group 3 (p = 0.043). But there was no significant difference between Group 1 and Group 2 (p = 0.947). CONCLUSION: Chronic diarrhoea is a significant complication after limited hemicolectomy not only in Crohn's disease and short bowel syndrome. This is likely to originate from the loss of the ileocaecal valve itself rather than the loss of the ileal or colonic segment. Our results justify attempts to reconstruct the ileocaecal valve.

Wall or machine suction rectal biopsy for Hirschsprung's disease: a simple modified technique can improve the adequacy of biopsy.

Suction rectal biopsy (SRB) may not include sufficient submucosa for histological diagnosis of Hirschsprung's disease (HD) and a repeat biopsy is required. Substitution of the conventional syringe for sustained suction from a machine or wall suction unit could provide a more consistently adequate tissue biopsy. This study was conducted to compare the adequacy of SRB specimens using a conventional method of syringe suction (SSRB) with those taken by wall or machine suction (WSRB). Hospital records of patients who had a SRB between 2002 and 2004 were studied retrospectively. The biopsy was considered inadequate if there was insufficient submucosa and a repeat specimen was requested. SSRB were taken using the conventional SSRB technique. WSRB were taken with the same Noblett forceps but with suction from wall suction or from a portable suction machine. Ninety-five infants had 103 SRB, 24 had WSRB and all (100%) were adequate for histopathological diagnosis or exclusion of HD. Seventy nine conventional SSRB were undertaken in 71 infants of which 64 (81.02%) were adequate. The difference in adequacy between WSRB and SSRB was statistically significant (P = 0.02). There were no instances of haemorrhage or bowel perforation in either subgroup. WSRB with the Noblett biopsy forceps is more likely to produce an adequate specimen and to reduce the incidence of repeat biopsies.

NOD2/CARD15, TLR4 and CD14 mutations in Scottish and Irish Crohn's disease patients: evidence for genetic heterogeneity within Europe?

NOD2/caspase recruitment domain (CARD)15 variants are identified in up to 50% of Crohn's disease (CD) patients. Functional variants of toll-like receptor-4 (TLR4) and CD14 genes may also be relevant to disease pathophysiology. We aimed to assess the contribution of NOD2/CARD15, TLR4 and CD14 variants in Scottish and Irish CD patients. In all, 612 patients with well-characterised inflammatory bowel disease (252 Scottish CD, 247 Scottish UC, 113 Irish CD) and 304 controls were genotyped for variants of NOD2/CARD15 (1007fsinsC, G908R, R702W, P268S), TLR4 (A299G) and CD14 (T-159C). Genotype-phenotype analyses were performed. Variant 1007fsinsC (P=0.003) and G908R (P=0.008) but not R702W (P=0.269) alleles were more prevalent in Scottish CD (4.7, 1.8 and 7.1%, respectively) than Scottish control (2.3, 0.3 and 5.4%). CD allelic frequencies were lower than the series from Europe (P<0.00001) and North America (P<0.00001) but not Scandinavia (P<0.7). Associations were identified with age at diagnosis (P=0.002), ileal disease (P<0.02), penetrating disease (P=0.04) and inflammatory joint disease (P<0.02). TLR4 and CD14 variants did not differ between CD and controls. In conclusion, we present compelling evidence for genetic heterogeneity within Europe. These NOD2/CARD15 variants have a minor contribution in Scottish and Irish CD patients, consistent with an emerging pattern from Northern Europe.

Genetics of inflammatory bowel disease: scientific and clinical implications.

Considerable progress has been made in the last decade in studies of the genetics of the inflammatory bowel diseases, Crohn's disease and ulcerative colitis. Epidemiological data, notably concordance rates in twin pairs and sibling pairs, have provided strong evidence for the importance of the genetic contribution, particularly in Crohn's disease. These observations provided the catalyst for laboratory-based studies of the molecular genetics of Crohn's disease and ulcerative colitis around the world. The complementary strategies of genome-wide scanning and candidate gene-directed studies have led to the identification of a number of genetic markers which appear to predict disease susceptibility and behaviour. The identification of the IBD1 gene on chromosome 16 as NOD-2 is unquestionably an important scientific discovery. Although many issues with respect to gene function and expression remain to be resolved there is great optimism that important clinical applications will directly result.

Unusual case of tracheal agenesis.

We describe the clinical presentation and pathological features of an unusual case of tracheal agenesis. The axial derivatives of the primitive foregut between the larynx and stomach were represented by a single structure featuring sequential segmentation into regions showing exclusively tracheal or esophageal differentiation in a pattern that is not easily classified by existing nosologic systems nor explained by the conventional hypothesis of dysontogenesis.

Laparoscopy for the impalpable testis.

BACKGROUND: Use of laparoscopy in the management of the impalpable testis remains controversial. Localization of the testis may help plan or obviate the need for groin exploration. This study reviews the need for inguinal exploration with respect to laparoscopic findings, particularly of vas and vessels entering a closed deep inguinal ring. METHOD: Case notes of boys undergoing laparoscopy for undescended testes were reviewed retrospectively. RESULTS: Of 86 impalpable testes, 32 were intra-abdominal and ten were absent with intra-abdominal blind ending vas and vessels. In 17 instances the vas and vessels entered an open internal ring and in 26 a closed internal ring. In one boy neither vas, vessels nor testis were visualized. Of the 26 impalpable testes with a closed internal ring, excision of testicular remnants in 18 revealed no histological testicular parenchyma, one boy had bilateral perineal ectopic testes missed clinically and six were not explored. CONCLUSION: The laparoscopic finding of vas and vessels entering a closed deep inguinal ring should prompt a careful examination for an ectopic testis. If a palpable testis can be ruled out, inguinal exploration is not necessary, as viable testicular parenchyma is rarely found. Laparoscopy would have avoided negative exploration in 42 per cent of impalpable testes in this series.

Detrusorectomy with Mitrofanoff stoma.

The clinical and social benefits derived from bladder augmentation are well known. Most procedures involve the implantation of gastric or intestinal segments into the bladder. These have problems of the acid-haematuria syndrome, mucous production, urinary infection, stone formation and metabolic disturbances. Lang and colleagues presented their experiences in 1994 with detrusorectomy alone as an alternative procedure. We have performed detrusorectomy with Mitrofanoff stoma on 9 patients, protecting the denuded bladder mucosa with omentum. This represents 50% of the bladder reconstructions managed over an 18-month period. Follow-up urodynamics studies have demonstrated a compliant low-pressure system of good capacity in all but one case. Apart from this failure, all the rest are continent. One boy suffered a ruptured bladder due to rough play and another suffered excruciating bladder pain on drainage. He was managed by the instillation of bupivicaine (4 mg/kg) twice daily. Successful management of cases depends on intensive support of the outreach nursing service.

Oesophageal atresia: at-risk groups for the 1990s.

The authors analyzed the outcome for 357 infants with oesophageal atresia and 15 with H-type tracheoesophageal fistula treated from 1980 through 1992. Survival according to Waterston risk categories was 99% for group A, 95% for group B, and 71% for group C. Presently, with optimal management, virtually all infants in groups A and B should survive. When examining the risk factors in the infants who died, two criteria were found to be important predictors of outcome: birth weight of less than 1,500 g and the presence of major congenital cardiac disease. A new classification for predicting outcome in oesophageal atresia is proposed: group I: birth weight > or = 1,500 g, without major cardiac disease, survival 97% (283 of 293); group II: birth weight < 1,500 g, or major cardiac disease, survival 59% (41 of 70); and group III: birth weight < 1,500 g, and major cardiac disease, survival 22% (2 of 9).

Plasma interleukin-6 and tumour necrosis factor levels as predictors of disease severity and outcome in necrotizing enterocolitis.

Plasma tumour necrosis factor (TNF) and interleukin-6 (IL-6) levels of 18 infants with necrotizing enterocolitis (NEC) were measured at the time of diagnosis or on transfer to a paediatric surgical unit. Whereas TNF levels were similar for infants managed medically (stage II, n = 8) and surgically (stage III, n = 7), IL-6 levels were significantly higher in stage III cases (mean, 3,127 pg/mL [95% CI, 1,809 to 4,445 pg/mL]) than in stage II (mean, 127 pg/mL [95% CI, 10 to 329 pg/mL]; P = .001). Neither TNF nor IL-6 level predicted eventual outcome. Plasma IL-6 may be useful as an indication for operation in NEC.

Plasma cytokine levels in necrotizing enterocolitis.

Plasma concentrations of tumour necrosis factor (TNF) and interleukin-6 (IL-6) were measured by ELISA in samples taken from 24 infants with necrotizing enterocolitis (NEC) between 0 and 306 h from diagnosis. TNF was detected (> 10 pg/ml) in 71% samples with a mean of 48 pg/ml (95% CI 42 to 55 pg/ml) and did not vary with either time from diagnosis or severity of disease. IL-6 was raised during the first 48 h with a significant difference between stage II (mean 127 pg/ml, 95% CI 10 to 329 pg/ml) and stage III (mean 3127 pg/ml, 95% CI 1809 to 4445 pg/ml, p = 0.001). Postoperative plasma IL-6 concentration fell to similar levels seen in stage II (mean 150 pg/ml, 95% CI 37 to 283 pg/ml, p = 0.79). We conclude that plasma concentration of IL-6 rather than TNF reflects the clinical severity of necrotizing enterocolitis and that the relative level of these cytokines has important implications for the use of anti-cytokine therapy in NEC.

Necrotizing enterocolitis--multisystem organ failure of the newborn?

Evidence of failure of organ system other than the gastrointestinal tract was reviewed in a series of 46 infants treated for necrotizing enterocolitis (NEC) at a regional combined medical and surgical neonatal unit. Respiratory failure was identified in 42 infants (91%), renal failure in 39 (85%), cardiovascular failure in 15 (33%) and hepatic failure in 7 (15%). The number of systems involved correlated with both severity of disease and outcome. Onset of failure in the above systems was closely related to the diagnosis of NEC and 29 cases predated the clinical onset. We conclude that NEC is part of a syndrome of multisystem failure and that this has important implications for the treatment of NEC.

Follow-up after inguinal herniotomy or surgery for hydrocele in boys.

A retrospective review of 556 boys who had undergone inguinal herniotomy or surgery for hydrocele was carried out to assess the value of early routine postoperative follow-up. Of 386 children given an appointment, 319 (82.6 per cent) attended. The testis was no longer palpable in the normal scrotal position in 12 boys; eight were normal on further review and four required orchidopexy. Six hydroceles, all of which resolved, and four contralateral hernias were detected. Nineteen boys (4.9 per cent) were referred later with recurrent hernia (two patients), ipsilateral high testis (two), or contralateral hernia or hydrocele (15). Of 170 children not given a routine appointment, ten (5.9 per cent) were subsequently referred back with a transient hydrocele (two) or a contralateral hernia (eight). After a total of 440 appointments, eight boys (1.8 per cent) were found to have a significant abnormality. The rate of referral back to hospital was similar in both groups. Early discharge would make more efficient use of outpatient resources without significantly affecting overall outcome.

Circumumbilical versus transumbilical abdominal incision.

One hundred and fifteen patients were entered into a prospective randomized trial to establish whether midline abdominal incisions through the umbilicus produced any differences in the incidence of wound infection or failure when compared with the more conventional midline incision skirting around the umbilicus. No significant difference was shown between wound infection rates in 58 transumbilical and 51 circumumbilical incisions. Follow-up of surviving patients for at least one year showed no difference in incisional hernia rate between the two groups of patients. No problems were encountered in making the incisions through the umbilicus, but skirting the umbilicus was felt to cause problems with slewing of the scalpel blade, and asymmetry of the scar in 13 circumumbilical incisions. This study has shown that the practice of avoiding the umbilicus in midline abdominal surgery serves no useful purpose.