Composite Functional and Quality of Life Outcomes in Adults Operated for Cloacal Malformation.

INTRODUCTION: Composite long-term outcomes of cloacal malformations remain unclear. We aimed to evaluate bowel and bladder control, sexual function, reproductive health, and quality of life in adulthood. METHODS: A Nordic multi-center cross-sectional observational study evaluating the outcome of adult patients with cloacal malformations was performed. Patient characteristics were retrieved from case records. Established questionnaires were sent to the patients to evaluate bowel- bladder- and sexual function and quality of life. RESULTS: Thirty-four of 48 (70%) eligible patients with median age 28 years (18-45) responded. Eight (24%) patients had a common channel >3 cm. Imaging-confirmed spinal and sacral abnormalities were present in 4 and 17 patients respectively. 85% (n = 29/34, four patients with permanent urinary diversions after bladder neck closure excluded) reported no urinary leakage without physical activity or urge to urinate. Three patients had a permanent enterostomy. 42 % (n = 11/26) reported acceptable bowel function according to Bowel Function Score (with eventual ongoing bowel management). The median Profile of Female Sexual Function Score was below normative values. Quality of life was comparable to the reference population, but thirty percent scored within the distress domains. Eight patients (24%) had been pregnant (15 pregnancies), resulting in seven live births, but the need of assisted reproduction techniques (40%, n = 6/15) and miscarriage (40%, n = 6/15) was common. CONCLUSIONS: Adequate spontaneous bowel control was rare, while most patients were dry for urine without additional procedures. Cloacal malformation also have a negative impact on sexual function health related quality of life and reproductive health. Long-term follow-up is crucial, not only regarding bowel and bladder function, but also for sexual and reproductive function, which may be important negative factors for health-related quality of life. LEVEL OF EVIDENCE: Level IV.

Location of TEF at the carina as an indicator of long-gap C-type esophageal atresia.

We analyzed retrospectively the outcomes in long gap Gross type C esophageal atresia. We hypothesized that outcomes in type C (long gap) atresia differ from type C (normal gap) and be similar with outcomes in Gross type A and B esophageal atresia. Location of the distal tracheoesophageal fistula (TEF) at the carina was chosen as the hallmark of type C atresia (long gap). We compared the type of esophageal repair, major reoperations for anastomotic complications and gastroesophageal reflux, and long-term mucosal changes between type C (normal gap), type C (long gap), and type A/B. We analyzed the hospital charts of 247 successive patients from 1984 to 2014 who either underwent repair of esophageal atresia in our institution (n = 232) or were referred from elsewhere because of anastomotic complications (n = 15). Among the 232 patients of our institution, 181 had type C and 21 type A or B esophageal atresia. Twenty-two (12%) of type C patients had TEF at the carina and were considered as type C (long gap). The referred patients included a disproportionately high number (42%) of patients with type C (long gap). We attempted primary anastomosis in 98% of patients with type C (normal gap), in 95% with type C (long gap), and 53% with type A/B underwent delayed primary anastomosis. Leakage after primary anastomosis occurred in 40% of patients with type A/B and in 23% with type C (long gap) compared with 6% in patients with type C (normal gap) (P < 0.05). Recalcitrant anastomotic stricture that eventually required esophageal resection occurred in 30% of patients with type A/B and in 18% with type C (long gap) compared with 3% in patients with type C (normal gap) (P < 0.05). The overall rate of major reoperations for anastomotic complications after primary anastomosis, type A/B (36%), type C (long gap) (27%), and antireflux surgery, type A/B (100%) and type C (long gap) (61%) were higher than in type C (normal gap), (9% and 24%), (P < 0.05 in both). Ten (47%) patients with type A/B esophageal atresia (primary anastomosis not possible n = 10), three (14%) with type C (long gap) (primary anastomosis not possible n = 1, significant loss of esophageal length after complications n = 2) and two (1%) with type C (normal gap) (significant loss of esophageal length after complications n = 2) underwent esophageal reconstruction. Endoscopic follow-up, median length 7.0 (IQR: 3.0-14) years, disclosed gastric metaplasia in 31% and 33% of patients with type A/B and type C (long gap) compared with 11% in type C (normal gap) (P < 0.05). Intestinal metaplasia was found in one patient type C (normal gap) (0.7%) and one with type C (long gap) (5.6%), (P = 0.21), only. The outcomes of type C (long gap) esophageal atresia are associated with more frequent complications, gastroesophageal reflux and esophageal mucosal changes than outcomes in type C (normal gap). Outcomes in type C (long gap) esophageal atresia resemble those in type A/B. The percentage of patients who remain with their native esophagus is, however, higher in type C (long gap) atresia (86%) than in type A/B (53%).

Surgical treatment of neuroblastoma: twenty-three years of experience at a single institution.

PURPOSE: Neuroblastoma (NB) is treated with surgery, chemotherapy and radiotherapy. We assessed the effects of surgical resection on the outcome over a 23-year period at our institution. METHODS: 85 children were included with a median age at diagnosis of 2.0 (range 0.1-15) years. We assessed the correlation of the complete surgical resection (CR) rate, metastases, NMYC amplification (NMYCA) and chemotherapeutic response with the 5-year overall survival (OS). RESULTS: The INSS stage of NB was 1 in 11 (13 %) patients, 2 in 10 (11 %), 3 in 13 (17 %), 4 in 46 (53 %) and 4S in five patients (6 %). Fifty-two (61 %) patients had high-risk NB and 22 (26 %) had NMYCA. The resection was complete in 72 (85 %) patients, incomplete (ICR) in seven (8 %) and six (7 %) patients did not undergo surgery. Fifty-five patients were administered neoadjuvant and 61 were administered adjuvant chemotherapy (high-dose, n = 50). The OS (5 year) was 68 %: stage 1 (100 %), 2 (90 %), 3 (77 %), 4 (52 %), 4S (80 %) and high-risk NB (52 %). The OS in high-risk NB patients was correlated with a good chemotherapeutic response of the primary tumour, with a RR for mortality = 0.3 (95 % CI 0.1-0.7; p = 0.01), but not with the CR, which had an RR = 0.9 (95 % CI 0.3-2.4; p = 0.84). CONCLUSIONS: The OS in high-risk NB patients was related to a good histological chemotherapeutic response, but not with complete excision of the primary tumour.

Effect of onabotulinumtoxinA treatment on symptoms and urodynamic findings in pediatric neurogenic bladder.

OBJECTIVE: To evaluate clinical and urodynamic efficiency of onabotulinumtoxinA (Botox) treatment in pediatric patients with urinary incontinence due to neurogenic overactive bladder. PATIENTS AND METHODS: Seventeen patients aged from 6 to 17 years (median 11 years) were treated with Botox injections. Clinical response to incontinence, duration of the response, and urodynamic results before and 1-3 months after treatment were evaluated. RESULTS: Mean incontinence frequency decreased significantly (p = 0.036); six of 17 patients had >90% reduction, and a further three patients had a 50-90% reduction in incontinence episodes. Median duration of the response was 15 months (range 3-42 months). Mean bladder volume changed from 380 ± 148 ml to 453 ± 147 (p = 0.078), maximal detrusor pressure decreased from 45 ± 31 cmH2O to 32 ± 21 cmH2O (p = 0.030), and the number of patients with detrusor contractions during filling decreased from 12 to three (p = 0.005) after the treatment. The patients with poor bladder compliance had either no response or a short duration of response. At follow-up eight patients had undergone bladder augmentation because of persistent incontinence. CONCLUSIONS: About one third of pediatric patients with neurogenic bladder had a good response to Botox treatment. In many patients, the clinical response was longer than expected. The patients who initially had poor bladder compliance had a poor response to the treatment.

Long-term outcome of esophageal anastomosis.

After repair of esophageal atresia (EA) in a newborn, esophageal dysmotility presenting as dysphagia and symptomatic gastroesophageal reflux are common. Significant esophageal morbidity associated with EA extends into adulthood. In adulthood approximately one-fifth of the patients have developed epithelial metaplastic changes, one-third of these have intestinal metaplasia (Barrett esophagus). Surgical complications, increasing age, and impaired esophageal motility predict the development of epithelial metaplasia after repair of EA. To date, worldwide, eight cases of esophageal cancer have been reported in young adults treated for EA. Incidence of esophageal cancer after EA repair is very much likely to increase in the future. Life-long endoscopic follow-up is warranted in patients with EA.

Dynamic defecography in the diagnosis of paediatric rectal prolapse and related disorders.

BACKGROUND AND AIM: Paediatric rectal prolapse (RP) is rarely a diagnostic problem and resolves often spontaneously. We studied whether the assessment of recurrent RP (RP), postoperative relapsed RP (RRP) or anorectal discomfort without RP (ARD) benefits from dynamic defecography (DD) and describe DD findings in relation with outcome. PATIENTS AND METHODS: Fifteen patients (7 males), median age of 10 (range 3.7-15) years, underwent 19 DD with a synchronic small bowel contrast study. Indications for DD were RP (n = 11), RD (n = 3) and RRP (n = 1). Three patients had solitary rectal ulcer and one juvenile rectal polyps (n = 1). Three patients underwent a total of four postoperative DD because of suspected relapse. RESULTS: In 11 patients with a clinically diagnosed prolapse DD displayed a simple prolapse (n = 3), prolapse with enterocele (n = 1), prolapse with small bowel interposition (n = 1), rectal intussusception with anterior rectocele (n = 1) and rectal intussusception (n = 1) and no pathology (n = 4) (37 %). In four patients with ARD DD displayed rectal prolapse originating from sigmoid intussusception (n = 1), enterocele (n = 1) and anterior rectocele (n = 1) and no pathology in one. Median follow-up was 8.1(range 3.0-44) months. Ten patients underwent surgery. Three patients with RP underwent simple laparoscopic rectopexy, five with RP or RD with enterocele or anterior rectocele had rectopexy with anterior peritoneoplasty and two (RP n = 1, RRP n = 1) with sigmoid intussusception had sigmoid resection with rectopexy. Two symptomatic patients (RP, negative DD) are scheduled for rectopexy. Three patients PPRP (n = 2) RD (n = 1) had spontaneous cure. Postoperative DD confirmed relapsed RP in one patient. CONCLUSION: In patients, RP and associated disorders' DD can disclose significant pathology (enterocele, rectocele or sigmoid intussusception) and thereby guide surgical treatment, and should be included in the pre-treatment assessment.

Magnetic resonance enterography guiding treatment in children with Crohn's jejunoileitis.

AIM: To determine the usefulness of magnetic resonance enterography (MRE) in treatment for paediatric patients with Crohn's disease. METHODS: To evaluate small bowel involvement, 45 children with Crohn's disease were scheduled for MRE. Two radiologists blinded to the patient data independently re-evaluated the images. Findings in images were compared to macroscopic findings at surgery or endoscopy. RESULTS: The terminal ileum was visualized in all with a completed procedure (43/45). The treatment remained conservative in 74% after imaging. In all 13 patients who underwent ileocolonoscopy within 3 months of MRE, the MRE findings were comparable with the macroscopic findings or revealed a more extensive disease. Bowel resection was performed in 26% after imaging. The macroscopic findings in the bowel corresponded to the MRE findings in 73%. In three MRE suggested a more severe disease than was verified intraoperatively. CONCLUSIONS: Magnetic resonance enterography identifies disease involvement in the small bowel in young patients with Crohn's disease and may guide decisions on the need for intestinal surgery or adjustment of medication.

Surgery of biliary atresia.

Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy of unclear etiology affecting varying degrees of both extra- and intrahepatic biliary tree resulting in obstructive bile flow and cholestasis in neonates. The diagnostic work-up is designed to diagnose or rule out BA without any unnecessary delay. Kasai portoenterostomy is a palliative operation performed to establish bile drainage from microscopic bile ductules that remain in the porta hepatis. It is advantageous to perform portoenterostomy as early after birth as possible for better chances of success. Actuarial native liver 5-year survival rates range from 30% to 60% after portoenterostomy, and about 20% of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Portoenterostomy remains as the first line operative treatment in BA while liver transplantation serves as a salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow.

Repaired oesophageal atresia: respiratory morbidity and pulmonary function in adults.

Although after oesophageal atresia (OA) repair in infancy, respiratory problems are common, their natural history remains unclear. We assessed morbidity, pulmonary function (PF), and bronchial hyperresponsiveness (BHR) in adults with repaired OA respiratory. 588 patients who underwent surgery for OA during 1947-1985 were identified and those 262 who were alive and had their native oesophagus were included. Respiratory symptoms and respiratory symptom-related quality of life (RSRQoL) were assessed by questionnaire and interview, and the patients underwent spirometry, a histamine challenge test, and an exhaled nitric oxide test. For the questionnaires, we added 287 carefully matched general population-derived controls. Among the 101 (58 male) patients, median age 36 yrs (range 22-56 yrs), respiratory morbidity was significantly increased compared to controls. Patients had more respiratory symptoms and infections, as well as asthma and allergies, and more often impaired RSRQoL (p<0.001 for all). PF tests revealed restrictive ventilatory defect in 21 (21%) patients, obstructive ventilatory defect in 21 (21%) patients, and both in 36 (36%) patients. A total of 41 (41%) had BHR, and in 15 (15%), it was consistent with asthma. The most significant risk factors for restrictive ventilatory defect were thoracotomy-induced rib fusions (OR 3.4, 95% CI 1.3-8.7; p = 0.01) and oesophageal epithelial metaplasia (OR 3.0, 95% CI 1.0-8.9; p = 0.05). After repair of OA, respiratory-related morbidity, restrictive ventilatory defect and BHR extended into adulthood. Nearly half the patients had BHR and over half had a restrictive ventilatory defect. Thoracotomy-induced rib fusions and gastro-oesophageal reflux-associated oesophageal epithelial metaplasia were the strongest risk factors for restrictive ventilatory defect.

Surgical management of constipation.

This review addresses the range of operations suggested to be of contemporary value in the treatment of constipation with critical evaluation of efficacy data, complications, patient selection, controversies and areas for future research.

Botox injection treatment for anal outlet obstruction in patients with internal anal sphincter achalasia and Hirschsprung's disease.

BACKGROUND AND AIM: Botox injection treatment (BIT) is a potentially effective but yet unproven treatment of functional anal outlet obstruction that is caused by non-relaxing internal anal sphincter. We present a single institution experience of BIT from 2005 to 2008. PATIENTS AND METHODS: Sixteen patients (11 males), eight with Hirschsprung's disease (HD) (one with total colon aganglionosis, TCA) and eight with internal sphincter achalasia (ISA) were included. Median ages were 3.8 years (0.4-9.3) for HD and 8.1 years (range 1.5-11.4) for ISA. ISA was defined as the absence of rectoanal inhibitory reflex with normal rectal biopsies. Seven HD patients had previous coloanal pull-through (CAPT), and one (TCA) colectomy and ileoanal J-Pouch anastomosis. Two of the ISA patients had undergone internal sphincter myectomy and two had Malone procedure [antegrade colonic enema (ACE)]. Indication for BIT in 16 patients was anal outlet obstruction (n = 11) with soiling and recurring HD-associated enterocolitis (n = 5) and in one patient (HD, TCA) soiling with enterocolitis (n = 1). Before BIT, all patients underwent anorectal manometry, rectal biopsies and barium enema. The effect of BIT was evaluated after 2 months and BIT was repeated if necessary. Effect of BIT was scored as follows: 0 no, 1 little, 2 significant effect and 3 symptoms disappeared. RESULTS: Median follow-up was 19 months (range 3-43). The median number of injections was two per patient (range 1-4) and the median Botox dose was 80 U (range 40-100). Scores of BIT effect were 3 or 2 in five (31%) and 0 or 1 in 11 (69%). After adjunctive treatment modalities (myectomy n = 1, CAPT n = 1, adjusted ACE/laxative treatment), the end result was good or satisfactory in 11 (69%) but remained poor in 5 (31%) patients. Patient age, diagnosis, anorectal resting pressure or findings in barium enema were not correlated with BIT efficiency score (R range -0.06 to 0.39, P = 0.12-0.91). CONCLUSION: Although successful in some patients, the role of BIT remains undetermined. It is difficult to predict which patients will profit from BIT. Continuing other treatment modalities after BIT may improve the results.

Outcome of esophageal atresia beyond childhood.

Survivors of esophageal atresia are reaching their adulthood in large numbers for the first time enabling assessment of true long-term outcome among this group of patients. This review summarizes the current knowledge on the subject focusing on late symptoms and complications, esophageal pathology and pulmonary function. Relationships between esophageal dysmotility, gastroesophageal reflux, esophagitis and epithelial metaplastic changes including esophageal cancer are outlined. In addition to pertinent literature, institutional experience, and follow-up of patients with esophageal atresia for more than 60 years is included.

Antegrade continence enema in the treatment of congenital fecal incontinence beyond childhood.

PURPOSE: The role of antegrade continence enema for the treatment of congenital fecal incontinence in adult patients remains unclear. MATERIALS: Twenty-seven patients, median age 19 (range, 17-43) years, with congenital fecal incontinence underwent surgery for antegrade continence enema and were prospectively followed up for functional outcome after a median of 25 (range, 3-117) months. RESULTS: The diagnoses included myelodysplasia (n = 14), anorectal malformations (n = 6), and others (n = 7). Antegrade continence enema conduits included appendicostomy (n = 22) and cecal (n = 2), ileal (n = 2), and sigmoid (n = 1) tubes. Thirteen (48 percent) patients had complications. Eighteen (66 percent) patients became fully continent, six (23 percent) had minor, and three (11 percent) major soiling. Antegrade continence enema became unnecessary in three patients (11 percent). Treatment with antegrade continence enema failed in three cases. Of the 21 patients who continued with antegrade continence enema, 16 (76 percent) are fully continent, and bowel function and quality of life was improved in 15 (71 percent) and 13 (62 percent) patients, respectively. The scores of convenience (1 = easy, 5 = difficult) and overall satisfaction (1 = poor, 10 = excellent) were median 2 (range, 1-4) and 8 (range, 3-10). CONCLUSIONS: Despite numerous complications and occasional treatment failures, 90 percent of adult patients with congenital fecal incontinence benefited from antegrade continence enema.

Respiratory distress secondary to nasopharyngeal glial heterotopia.

Nasal glial heterotopia (nasal glioma) is a rare congenital malformation of neural origin. We present a newborn baby with life-threatening respiratory distress secondary to nasopharyngeal glial heterotopia that obstructed the nasopharyngeal or nasal airway. A high degree of suspicion, early diagnosis and surgical management are essential to cure this rare and potentially life-threatening disorder.

Preoperative distal loop contrast radiograph before closure of an enterostomy in paediatric surgical patients. How much does it affect the procedure or predict early postoperative complications?

Before the closure of an enterostomy, a distal loop contrast radiograph (DLCR) is widely used to disclose pathology which may affect the performance of the procedure. We studied whether DLCR of paediatric patients caused actual alterations in the surgical plan and whether it predicted postoperative complications. Between 1991 and 2006, 105 patients (small bowel enterostomy, SBE; n = 51), (colostomy, CO; n = 54) underwent closure of an enterostomy. All 105 patients had preoperative DLCR. The indications for enterostomy included anorectal malformation (n = 38), neonatal intestinal perforation (n = 25), J-Pouch ileoanal anastomosis (n = 20), anorectal trauma (n = 5), and miscellaneous (n = 17). We recorded sensitivity, specificity, and positive and negative predictive value (PPV and NPV) of DLCR for complications within 6 postoperative weeks. DLCR was considered complete and interpreted as normal in 94 (90%) and abnormal (incomplete n = 3 or pathological n = 8) in 11 (10%) patients. None of the 11 abnormal findings caused cancellation of surgery, but in three (27%) patients it was possible to surgically correct a stricture seen in DLCR. The frequency of surgical complications was 17/105 (16%), SBE (15/51,29%) and CO (2/54, 4%), (P < 0.05). Most common complications (9/17, 53%) were those associated with the intestinal anastomosis. For postoperative complications DLCR had sensitivity, specificity, and PPV and NPV of 47, 97, 73 and 90% (SBE and 47, 97, 88 and 81%), (CO 50, 96, 33 and 98%). The pathology seen in DLCR, however, seldom directly hinted the complications which actually occurred. Abnormal DLCR changed the surgical plan in less than one-fifth of the cases. For surgical complications DLCR had poor sensitivity, good specificity and NPV, and moderate PPV. The pathology suggested by DLCR, however, correlated poorly with the actual complications. Poor sensitivity reflects the high frequency of anastomotic complications, which are practically unpredictable by preoperative radiographs.

Laparoscopic herniorrhaphy after manual reduction of incarcerated inguinal hernia.

BACKGROUND: After a manual reduction (MR) of an incarcerated inguinal hernia (IIH), it is recommended that an open herniotomy (OH) be performed after a one-day delay because of the postincarceration tissue edema. We assumed that performing laparoscopic herniorrhaphy (LH) shortly after MR reduces the hospital stay for IIH. We compared LH with OH retrospectively. We expected equal results but a shorter hospital stay with LH. METHODS: From May 2002 to April 2006, 40 successive patients with IIH were admitted. OH was scheduled two days after MR, whereas no delay for performing LH was required. Patients in whom MR failed and who required immediate surgery (n = 4) and patients whose medical condition prevented surgery within the schedule (n = 3) were excluded from the study. Follow-up consisted of an outpatient visit and telephone survey. RESULTS: Thirty-three patients (31 male, 15 OH, 18 LH) were included. For the LH patients, the median age was 15 (0.7-81) months and that for OH patients was 8.6 (0.6-61) months. For LH patients, weight = 11.5 (3.6-22) kg and for OH patients, weight = 9.8 (3.5-17) kg (p = NS). Median delay from MR to OH was 2 (2-4) days, and from MR to LH median delay was 1 (0-3) day (p < 0.05). Length of the operation was 29 (10-80) min in OH and 39 (20-60) min in LH (p = NS). Total theatre time was 44 (17-111) min in OH and 66 (44-86) min in LH (p < 0.05), and hospital time was 3 (3-6) days in OH and 2 (1-4) days in LH (p < 0.05). Median cost (surgery + hospitalization) of OH was euro 2315 (1910-3530) and that of LH was euro 3215 (2605-3650) (p < 0.05). Median follow-up was 26 (4-49) months, one patient (LH) had re-LH for recurrent hernia. CONCLUSION: After MR, LH can be performed with minimal delay and similar results as OH. Despite increased theatre time and total hospital costs, LH shortened hospital stay.

Treatment of a leaking ACE conduit with Deflux injections.

Faecal leakage from the catheter conduit can be troublesome and reduces the convenience of use of antegrade continence enema (ACE). We report the results of Deflux injection treatment for leaking ACE conduits in nine patients. From 1994 to the end of 2005, 81 patients underwent a procedure for ACE [appendicostomy with wrap (AW) n = 29, appendicostomy straight (AS) n = 44, Monti-Yang ileal tube (MY) n = 5, lateral caecal flap (LCF) n = 1, caecal tube (CT) n = 1, sigmoid tube (ST) n = 1]. Nine (11%) patients (AW n = 3, AS n = 1, MY n = 3, LCF n = 1, ST n = 1) had persistent stomal leak and underwent Deflux injections of the ACE conduit. With the patients in general anaesthesia, the conduit was visualised with CH10.5 cystoscope. At the junction of the conduit and colon, 0.5-1.0 ml of Deflux was injected submucosally at three sites to create intermingling mounds in order to narrow the conduit. After the injections, a balloon catheter was left in the conduit for 3-7 days. When necessary, the injections were repeated. The result was graded as no improvement, moderate improvement, significant improvement. Nine patients underwent a median of 2 (range 1-4) injections. No major complications occurred. The results after a median follow-up of 22 (range 3-53) months from the first injection were as follows: no improvement (n = 1), moderate improvement (n = 5), significant improvement (n = 3). Repeated injection of the ACE conduit with Deflux is a low invasive method and provides moderate to good results in the majority of the patients who suffer from faecal leakage from conduit.

Laparoscopic suture rectopexy in the treatment of persisting rectal prolapse in children: a preliminary report.

BACKGROUND: The repair of choice for persistent rectal prolapse (PRP) in children is disputed. Laparoscopic suture rectopexy (LSRP) is effective in adults, but its usefulness in pediatric PRP is unknown. We compared LSRP with posterosagittal rectopexy (PSRP). METHODS: Sixteen children, with a median age of 6.5 years (range, 0.8-16.8) and duration of symptoms of 2.8 years (range, 0.5-10.2), underwent surgery for PRP. Eight (1991-2000) had PSRP, and eight (2002-2005) had LSRP. Three patients with LSRP were healthy; the others had mental retardation and epilepsy (n = 1), cerebral palsy (n = 1), Aspeger's syndrome (n = 1), meningomyelocele (n = 1), and bladder extrophy (n = 1). Preoperative cologram (n = 6), sigmoideoscopy (n = 3), and anorectal manometry (n = 2) were normal in patients with LSRP. In LSRP, the rectum was mobilized and sutured to the sacral periosteum. RESULTS: Median operation time for LSRP was 80 min (range, 62-90) and for PSRP 40 min (range, 25-70) (p < 0.05); median hospital time was 6 days (range, 3-8) for LSRP and 6 days (range, 3-9) for PSRP (not significant). Six patients with LSRP had a median follow-up of 13 months (range, 4-24). None have had recurrences, and two patients (33%) require laxatives. Of the patients with PSRP, two (25%) had recurrence and underwent abdominal rectopexy with sigmoid resection. CONCLUSION: Medium-term results indicate that LSPR is effective in pediatric PRP. Constipation is the only postoperative problem in a significant proportion of patients.

Revision of prolapsed feeding gastrostomy with a modified Janeway 'gastric tube'.

After creation of open or percutaneous endoscopic gastrostomy, gastric prolapse and leak of gastric contents may cause serious skin rash and infection which are often difficult to treat. We present four patients in whom these problems were solved with gastrostomy revision by a modified Janeway 'gastric tube' technique. The patients were aged 7 months and 7, 10 and 16 years at the time of the revision. The underlying conditions were hypoxic encephalopathy with epilepsy, infantile spasm and epilepsy with arthrogryposis, dystonic tetraplegy, and total colon aganglionosis. All patients had gastrostomy prolapse with peristomal skin rash and cellulitis. Prior to modified Janeway revision, the four patients had undergone a total of 16 failed attempts to cure the prolapse. At the operation, the previous gastrostomy was detached and closed. A longitudinal gastric tube of 6 cm was created along the greater curvature with a GIA stapler and brought through the abdominal wall leaving 3-5 cm of free intra-abdominal gastric tube. A balloon catheter was left for 6 weeks, and replaced with a long Mickey tube according to patient's or caretaker's preference. There were no surgical complications. Hospitalisation after revision was median 6 (range 4-11) days. Six weeks after the revision, prolapse, leak and peristomal infections were cured in all patients, and feeding through the gastrostomy presented no problems. One patient underwent minor excision of excess stomal mucosa. Two patients opted for Mickey tube, two for a feeding catheter. A median of 9 (6-16) months after the revision, all patients have a functioning gastrostomy without prolapse or leak. Modified Janeway 'gastric tube' revision is feasible and, within short to medium term follow-up, controls efficiently gastrostomy prolapse and leak.

Long-term outcome after internal sphincter myectomy for internal sphincter achalasia.

Anal internal sphincter achalasia is a rare cause of refractory constipation. It is usually treated by internal sphincter myectomy (ISM). The aim of this study was to evaluate the long-term outcome of patients who had undergone ISM for internal sphincter achalasia. Bowel habits, fecal continence, and quality of life were evaluated using a questionnaire. Fecal continence was quantitatively assessed by a score described by Holschneider. A questionnaire was sent to 13 patients (11 male, two female) operated on by ISM for intractable constipation caused by internal sphincter achalasia between 1983 and 1993. Ten patients responded and were included in the study. At the time of the study, three of the 10 patients required oral medication for constipation and had one to three bowel movements per week. One of the 10 patients had three to five bowel movements per day, and the others had one to two bowel movements per day. Four patients had normal and six patients had good continence scores. Four of the patients reported mild social problems, and one had problems associated with sports. Refractory constipation in the majority of patients with internal sphincter achalasia can be treated by ISM. However, in the long term, a significant number of patients suffer from soiling-related social problems.