Anorectal manometry with and without ketamine for evaluation of defecation disorders in children.

INTRODUCTION: Anorectal manometry (ARCM) provides valuable information in children with chronic constipation and fecal incontinence but may not be tolerated in the awake child. This study aimed to evaluate the effect of ketamine anesthesia on the assessment of anorectal function by manometry and to evaluate defecation dynamics and anal sphincter resting pressure in the context of pathophysiology of chronic functional (idiopathic) constipation and soiling in children. METHODS: This was a prospective study of children who were investigated for symptoms of chronic constipation and soiling between April 2001 and April 2004. We studied 52 consecutive children who had awake ARCM, biofeedback training and endosonography (awake group) and 64 children who had ketamine anesthesia for ARCM and endosonography (ketamine group). We age matched 31 children who had awake anorectal studies with 27 who had ketamine anesthesia. RESULTS: The children in awake and ketamine groups were comparable for age, duration of bowel symptoms and duration of laxative treatments. ARCM profile was comparable between the awake and the ketamine groups with regard to anal sphincter resting pressure, rectal capacity, amplitude of rectal contractions, frequency of rectal and IAS contractions and functional length of anal canal. Of 52 children who had awake ARCM, dyssynergia of the EAS muscles was observed in 22 (42%) and median squeeze pressure was 87mm Hg (range 25-134). The anal sphincter resting pressure was non-obstructive and comparable to healthy normal children. Rectoanal inhibitory reflex was seen in all children excluding diagnosis of Hirschsprung disease. CONCLUSIONS: Ketamine anesthesia does not affect quantitative or qualitative measurements of autonomic anorectal function and can be used reliably in children who will not tolerate the manometry while awake. Paradoxical contraction of the EAS can only be evaluated in the awake children and should be investigated further as the underlying cause of obstructive defecation in patients with chronic functional constipation and soiling.

Clinical and psychosocial functioning in adolescents and young adults with anorectal malformations and chronic idiopathic constipation.

BACKGROUND: Faecal incontinence (FI) and constipation occur following corrective surgery for anorectal malformations (ARMs) and in children or adults with chronic constipation without a structural birth anomaly (chronic idiopathic constipation, CIC). Such symptoms may have profound effects on quality of life (QoL). This study systematically determined the burden of FI and constipation in these patients in adolescence and early adulthood, and their effect on QoL and psychosocial functioning in comparison with controls. METHODS: Patients with ARMs or CIC were compared with age- and sex-matched controls who had undergone appendicectomy more than 1 year previously and had no ongoing gastrointestinal symptoms. Constipation and FI were evaluated using validated Knowles-Eccersley-Scott Symptom (KESS) and Vaizey scores respectively. Standardized QoL and psychometric tests were performed in all groups. RESULTS: The study included 49 patients with ARMs (30 male, aged 11-28 years), 45 with CIC (32 male, aged 11-30 years) and 39 controls (21 male, aged 11-30 years). The frequency of severe constipation among patients with ARMs was approximately half that seen in the CIC group (19 of 49 versus 31 of 45); however, frequencies of incontinence were similar (22 of 49 versus 21 of 45) (P < 0·001 versus controls for both symptoms). Physical and mental well-being were significantly reduced in both ARM and CIC groups compared with controls (P = 0·001 and P = 0·015 respectively), with generally worse scores among patients with CIC. Both were predicted by gastrointestinal symptom burden (P < 0·001). There were no statistically significant differences in state or trait psychiatric morbidity between groups. CONCLUSION: FI and constipation are major determinants of poor QoL in adolescents and young adults with ARMs and in those with CIC.

Combined vertical reduction rectoplasty and sacral nerve stimulation for rectal evacuatory dysfunction and faecal incontinence associated with previous anorectal malformation.

Functional outcomes following surgery for anorectal malformation are variable, with many children experiencing persisting anorectal dysfunction. We describe a 34-year-old female with previous vestibular fistula who experienced lifelong rectal evacuatory dysfunction and faecal incontinence; she was treated in a two stage process producing efficient defecation and almost total continence.

Importance of extrasphincteric mechanisms in the pathophysiology of faecal incontinence in adults with a history of anorectal anomaly.

BACKGROUND: Although surgery for congenital anorectal anomalies (ARAs) aims to preserve anorectal function, faecal incontinence and constipation often result. Apart from the anal sphincters, continence is dependent on multiple anatomical and physiological factors. The aim of this study was to evaluate adults with a history of ARA to determine the role of such factors in functional outcome. METHODS: The study included 20 consecutive adult patients with faecal incontinence who had undergone anorectal surgery as infants. Comprehensive testing included anal manometry, endoanal ultrasonography, tests of pudendal nerve function and rectal sensory function, evacuation proctography and colonic transit studies. RESULTS: Anal resting tone and squeeze increments were both attenuated in 15 of 19 patients. Integrity of the internal and external anal sphincters was compromised in 16 and 15 of 18 patients respectively. Eleven of 13 had evidence of pudendal neuropathy. Rectal sensation was abnormal in 14 of 18 patients, of whom ten were hypersensitive and four hyposensitive. Rectal evacuation was abnormal in nine of 14. Colonic transit was delayed in five of eight patients with constipation. CONCLUSION: Faecal incontinence in adult patients with ARA is related to various pathophysiologies. Structural integrity of the anal sphincters is a major factor, but extrasphincteric mechanisms, notably rectal sensory function, may be as important.

Evaluation of outcome of anorectal anomaly in childhood: the role of anorectal manometry and endosonography.

The aim of this study was to evaluate role of anorectal manometry (ARM) and anal endosonography (ES) in assessment of the internal anal sphincter (IAS) quality on continence outcome following repair of anorectal anomalies (ARA). We devised a scoring system to evaluate the quality of the IAS based on ARM and ES and correlated the scores with clinical outcome, using a modified Wingfield score (MWS) for faecal continence. We also assessed the implication of megarectum and neuropathy on faecal continence. Of 54 children studied, 34 had high ARA and 20 had low ARA. Children with high ARA had poor sphincters on ES and ARM, and also poor faecal continence compared to those with low ARA. The presence of megarectum and neuropathy was associated with uniformly poor outcome irrespective of the IAS quality. The correlations between MWS on one hand, and ES and ARM scores for IAS on the other hand were weak in the whole study group, ES r = 0.27, P < 0.04, and ARM r = 0.39, P < 0.004. However, the correlations were strong in those who had isolated ARA without megarectum or neuropathy, ES r = 0.51, P < 0.02 and ARM r = 0.55, P < 0.01, respectively. In conclusion, the ARM and ES are valuable in evaluation of continence outcome in children after surgery for ARA and those with good quality IAS had better faecal continence. The IAS is a vital component in functional outcome in absence of neuropathy and megarectum.

Investigations for incontinence and constipation after surgery for Hirschsprung's disease in children.

Surgery for Hirschsprung's disease is associated with high rate of morbidity, in the form of either constipation or incontinence or a combination of the two. This study investigates the mechanisms responsible for incontinence and/or constipation following the pull-through operation for Hirschsprung's disease. There were 19 children (15 boys and 4 girls), who at the time of study; 16 had undergone Duhamel, 1 Rehbein, and 2 Soave operation. We classified patients according to their symptoms into 3 groups: Group A was incontinent of faeces; Group B was constipated and incontinent of faeces, and Group C was constipated only. The median age at referral was 6 years, and the median period after operation was 5 years. All patients were investigated by intestinal transit study, endoanal sonography and anorectal manometry. Group A had normal or rapid transit study, as opposed to Groups B and C, who had delayed-transit study. On endoanal sonography, all children had an intact internal and an external anal sphincter, below the level of pull-through operation. The anorectal manometry showed a significantly lower resting anal pressure in the incontinent Group A as compared to the constipated children with or without incontinence in Group B or C (38 mmHg versus 57 or 66 mmHg respectively). The rectal pressure was also significantly higher in children in Group A as compared to those in Group B or C (71 mmHg versus 42 or 36 mmHg). The ratio of rectal/anal pressure was higher in incontinent children in Group A, as compared to constipated children in Group B or C. Therefore, constipation can be caused by high anal resting pressure and a weak rectal peristalsis, while faecal incontinence can be secondary to poor compliance and elevated rectal pressure in the presence of normal or low anal sphincter resting pressure. Aperients are the mainstay of treatment of constipation, however, children with incontinence are more difficult to treat. We did not attempt to define the pattern of nerve plexus because of poor results of revision operation for residual hypoganglionic segment and intestinal neuronal dysplasia. Treatment of these children can become more rational, if furnished with detailed functional studies. We advocate investigation of the anorectal function at an early stage in symptomatic children after surgery for Hirschsprung's disease, and less invasive treatment should be considered before embarking on major surgery.

Evaluation of dysfunction following reconstruction of an anorectal anomaly.

To evaluate the utility of anorectal manometry (ARM) and magnetic resonance imaging (MRI) with an endocoil in the assessment of dysfunction in children with repaired anorectal anomalies (ARA), 15 patients aged 1 to 15 years with repaired ARAs and chronic faecal incontinence or constipation were prospectively recruited. They underwent clinical assessment using a modified Wingfield score (MWS). ARM and MRI with an endocoil and conventional external coil were carried out. The results of ARM alone, MRI alone, and a combination of ARM and MRI were correlated with the MWS. Manometric internal anal sphincter (IAS) scores determined from sphincter length and activity correlated with MWS (r = 0.56, P = 0.02); manometric scores of rectal peristaltic activity did not. Overall manometric score (IAS and rectal scores combined) showed a correlation with MWS (r = 0.55, P = 0.02). Endoanal MRI sphincter scores did not correlate with MWS, but the presence of a megarectum on MRI did (r = 0.44, P = 0.05). Overall MRI score did not correlate with MWS. Minor neurosacral anomalies were shown on MRI in 3 children who had poor functional scores. Combined manometric and MRI scores showed a correlation with MWS (r = 0.58, P = 0.01). ARM and MRI are potentially useful in the assessment of dysfunction of children with repaired ARAs. Both modalities require refinement and further assessment in the context of directing management.

Bladder and kidney function after cure of pelvic rhabdomyosarcoma in childhood.

Eleven survivors of pelvic rhabdomyosarcoma underwent bladder function studies and upper urinary tract evaluation at a mean of 6.6 years after completion of therapy, which included a conservative, bladder-sparing surgical policy. Primary tumour sites were: bladder base/prostate, 6; bladder dome, 1; vagina, 2; and pelvic side wall, 2. Seven children (five bladder base/prostate, one vagina and one pelvic side wall tumours) had received irradiation to the pelvis with external beam alone, brachytherapy or both. All seven of these patients had markedly reduced functional bladder capacity (11-48% of mean expected value for age) and abnormal voiding patterns, though bladder compliance was not reduced and bladder emptying was almost complete in five cases. Four of these children also had upper tract dilatation and two required reconstructive bladder surgery because of severe bilateral hydronephrosis. By contrast, each of four children treated without radiotherapy had a normal functional bladder capacity and a normal voiding pattern. all survivors of pelvic rhabdomyosarcoma, especially those who have received radiotherapy, should be carefully monitored for dysfunction of both lower and upper urinary tracts. The frequency-volume voiding chart is a sensitive and easily accomplished method of assessing bladder function in these patients.

Conservative surgery in multimodal therapy for pelvic rhabdomyosarcoma in children.

Twenty-six previously untreated children, median age 3.4 years, with pelvic rhabdomyosarcoma (RMS) were seen between 1983 and 1988. Fourteen were girls. The planned strategy was to conserve pelvic organs, especially the bladder, by using primary chemotherapy, conservative surgery and, in most cases, radiotherapy. With a median follow-up of 71 months (range 34-103 months) overall survival was 73%, with no treatment-related death. The bladder salvage rate of 88% in survivors with bladder base/prostate primaries was much higher than that reported by the United States Intergroup Rhabdomyosarcoma Studies (IRS), though many of the preserved bladders did not function normally. We identified problems with both radiological and histological off-treatment monitoring. The overall accuracy of computerised tomographic (CT) scanning for prediction of tumour recurrence was only 81%, and endoscopic biopsies proved misleading in four of the ten bladder base/prostate patients monitored by serial cystoscopy. We conclude that a higher cure rate can be achieved by using intensive chemotherapy/radiotherapy and conservative surgery to treat children with pelvic RMS. Factors that might contribute to our favourable bladder salvage results, compared with those of the IRS, include (a) the fact that one of two specialist surgeons monitored and operated on all these patients and (b) our increasing awareness, during the study, that post-chemotherapy/radiotherapy histopathology and pelvic CT scan appearances may be misleading. Referral to paediatric centres with special experience of pelvic RMS may help raise the rate of bladder salvage in these children.

Papillary cystic cancer of the pancreas: diagnostic difficulties.

Pancreatic tumors rarely present in childhood. Diagnostic difficulty and inappropriate treatment may result especially when the lesions have a cystic component. Two cases of papillary cystic carcinoma of the pancreas in teenage girls are presented. Optimal treatment of such lesions in the head of pancreas consists of partial pancreaticoduodenectomy preserving the pylorus when possible.

Brown bowel syndrome: a late complication of intestinal atresia.

Two children, aged 11 years, who originally had jejunal atresia corrected in the neonatal period, developed massive dilatation of the proximal small intestine. This resulted in circular muscular hypertrophy with lipofuscin deposits giving the typical appearance of "brown bowel." The condition was associated with malnutrition and vitamin E deficiency. Because of relatively short bowel, the condition was treated by limited resection and extensive tapering of the dilated segment, end-to-end reanastomosis, vitamin E supplementation, and intensive nutritional support.

Intussusception and duodenal stenosis: an infrequent but expected association.

Two boys with intussusception, intestinal malrotation and duodenal stenosis are reported. Because intussusception is associated with malrotation, which is in turn associated with duodenal stenosis, this combination of abnormalities may be expected in up to 3% of children with intussusception.

Pelviureteric junction obstruction and recurrent abdominal pain in childhood.

Three cases of recurrent abdominal pain due to pelviureteric junction obstruction in childhood are presented. Ultrasound is useful in confirming the diagnosis and pyeloplasty is recommended.

Cervical repair of esophageal atresia.

This report describes three neonates with Type IIIb3 esophageal atresia treated by primary repair utilizing a combined thoracic and cervical approach.

Protein and energy metabolism with biosynthetic human growth hormone after gastrointestinal surgery.

The effect of biosynthetic human growth hormone (BSHGH) on postoperative protein and energy metabolism has been studied in patients who had major gastrointestinal surgery. Seven patients received placebo and seven patients received BSHGH, 0.1 mg/kg/24 h, for the first six postoperative days. Mean total nitrogen excretion was significantly lower with BSHGH (31.5 +/- 2.4 g N) (2287 +/- 160 mmol) than with placebo (42.7 +/- 3.1 g N) (3049 +/- 219 mmol) over the 6-day study period. The mean daily measured energy expenditure over days 3-6 was higher with BSHGH (31.3 +/- 1.8 kcal/kg LBM/24 h) (131 +/- 7 kJ/kg LBM/24 h) than with placebo (27.6 +/- 0.8 kcal/kg LBM/24 h) (114 +/- 2 kJ/kg LBM/24 h). Fat oxidation with BSHGH (2.05 +/- 0.26 mg/kg LBM/24 h) was greater than with placebo (1.5 +/- 0.17 mg/kg LBM/24 h) and protein oxidation was less with BSHGH (0.68 +/- 0.07 g/kg LBM/24 h) than with placebo (0.9 +/- 0.09 g/kg LBM/24 h) on days 1-6. Postoperative nitrogen turnover (BSHGH 943 +/- 174 mg N/kg LBM/24 h, placebo 557 +/- 50 mg N/kg LBM/24 h) (BSHGH 67 +/- 13 mmol/kg LBM/24 h, placebo 40 +/- 4 mmol/kg LBM/24 h), protein synthesis (BSHGH 5.31 +/- 1.09 g prot/kg LBM/24 h, placebo 2.54 +/- 0.33 g prot/kg LBM/24 h) and protein breakdown (BSHGH 5.90 +/- 1.09 g prot/kg LBM/24 h, placebo 3.48 +/- 0.31 g prot/kg LBM/24 h) were greater with BSHGH. On the first postoperative day serum insulin and blood glucose levels were higher with BSHGH than with placebo, and on days 4 and 7 serum somatomedin-C levels were significantly elevated. This study shows that BSHGH alters postoperative protein and energy metabolism by reducing protein oxidation and increasing fat oxidation with raised rates of whole body nitrogen turnover.

Elevated rates of whole body protein metabolism in patients with disseminated malignancy in the immediate postoperative period.

Protein metabolism after surgery in the presence of disseminated malignancy has been investigated and compared with results obtained from patients undergoing similar surgical procedures for benign disease and localized malignancy. Whole body nitrogen turnover, measured by primed continuous infusion of 15N glycine, was highest with disseminated malignancy. Similarly rates of whole body protein synthesis and breakdown, calculated from turnover and nitrogen excretion (nitrogen intake being zero), were elevated in the presence of disseminated malignant disease. The increased rates of protein metabolism may represent adaptation to the demands of inevitably growing malignant tissue.