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.

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.

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.

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.

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.

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.

Is routine dilatation after repair of esophageal atresia with distal fistula better than dilatation when symptoms arise? Comparison of results of two European pediatric surgical centers.

BACKGROUND/PURPOSE: The aim of this study was to determine whether routine dilatation of the anastomosis after repair of an esophageal atresia with distal fistula (EADF) is superior to a wait-and-see policy with dilatation only when symptoms arise. METHODS: The records of 100 consecutive patients operated on for EADF in 2 European pediatric surgical centers (A [n = 63], B [n = 37]) were reviewed. In center A, dilatation of the anastomosis was carried out in symptomatic cases only, whereas in center B dilatation was begun 3 weeks postoperatively and repeated every 1-3 weeks until a stable diameter of 10 mm was reached. Particular attention was paid to the number of dilatations per patient, dilatation-related complications, and differences in results after 2 years. RESULTS: The patient materials of both centers did not differ with respect to the incidence of prematurity, tracheomalacia, gastroesophageal reflux (GER), and major postoperative complications. The incidence of associated anomalies was higher in center B (P < .05). In center A, 26 of 63 patients underwent dilatation; in center B, all 37 patients were dilated (P < .05). Median number of dilatations per patient was 4 in center A and 7 in center B (P < .05). In center A, 23 of 26 and in center B, 20 of 37 of the patients received medical treatment for GER at the time of the dilatations. Dilatation-related complications developed in 7 of 26 patients of center A and in 3 of 37 patients in the center B (P value, not significant). The median primary hospital stay was 24 days in center A and 33 days in center B (P < .05), median secondary hospital stay for dilatation was 6 days in center A and 13 days in center B (P < .05). After 2 years of follow-up, the incidence of dysphagia, respiratory problems, or bolus obstruction did not differ significantly between the 2 centers. CONCLUSIONS: A wait-and-see policy and dilatations based on clinical indications for patients with repaired EADF is superior to routine dilatations. It appears that more than half of the patients do not require dilatations at all.

Does postoperative pH monitoring predict complicated gastroesophageal reflux in patients with esophageal atresia?

Gastroesophageal reflux (GER) is common after repair of esophageal atresia with a distal tracheoesophageal fistula (EATOF). In a retrospective study we assessed whether early 18-h pH monitoring can predict the development of EATOF-associated gastroesophageal reflux. During 1980-1997, 90 consecutive patients had primary repair for EATOF. Development of GER was classified as favorable if the patient developed no esophagitis or mild esophagitis and needed no antireflux medication, and as unfavorable if the patient developed moderate or secondary esophagitis or required an antireflux procedure. Patients who developed unfavorable GER outcome before pH monitoring or needed secondary reconstruction or those whose endoscopic follow-up data were insufficient were excluded. Eighteen-hour pH monitoring was considered pathologic if esophageal pH was <4 more than 10% of the recorded time or 5% of the recorded time minus 2 h after each meal, or if there were more than three preprandial reflux periods lasting longer than 5 min. A total of fifty patients were included into the study. pH monitoring was performed at the median age of 9.2 (range 2.5-95.0) months and classified as pathologic in 10 and normal in 40 patients. After a median follow-up of 59 (0.3-217.6) months, nine of 10 (90%) patients with pathologic pH monitoring and five of 40 (12.5%) patients with normal pH monitoring developed unfavorable outcomes ( p<.05). We conclude that early pH monitoring predicts the development of significant GER, but because 12.5% of patients with normal early pH monitoring also developed significant GER, early pH monitoring alone does not rule out the development of significant GER.

Comparison of functional outcomes of Duhamel and transanal endorectal coloanal anastomosis for Hirschsprung's disease.

PURPOSE: The aim of this study was to determine the morbidity and medium-term functional outcome of the Duhamel operation and laparotomy and transanal endorectal coloanal anastomosis (TECA) for Hirschsprung's disease (HSCR). METHODS: The study populations were 34 consecutive children who underwent the Duhamel operation (or Lester Martin modification) and 37 who had the TECA. Demographic details were obtained by case note review, and functional outcome was determined by a combination of outpatient interview, questionnaire, and telephone enquiry. RESULTS: There was no difference between the groups with respect to age, gender, and length of aganglionic segment. Seventy percent presented as neonates (Duhamel, 24 of 34; TECA, 26 of 37). A single-stage primary pull-through was performed in 17 of 37 children in the TECA group, and in 1 of 34 from the Duhamel group. There was a single perioperative death in the Duhamel group and an unrelated, late death in the TECA group. Postoperative enterocolitis was seen in 13 of 37 TECA children and in a single child from the Duhamel group. A stricture of the pull-through segment was seen in 7 of 37 children after TECA and required temporary diversion in 2 of 9. Late division of a rectal spur was required in 6 of 33 Duhamel children. Requirement for late myectomy was the same in both groups (Duhamel 3 of 33, TECA 4 of 37). Complications requiring stoma formation occurred in 5 of 37 after TECA and 2 of 33 after the Duhamel operation. Two children from the TECA group and 1 from the Duhamel group remain diverted. One child from each group required a re-pull-through procedure. Two patients were lost to follow-up in the TECA group, leaving 34 children in this group and 33 in the Duhamel group in whom functional outcome could be assessed. Functional outcome was similar in the 2 groups. CONCLUSIONS: TECA and Duhamel procedures have similar medium-term functional outcomes. TECA has a high incidence of postoperative enterocolitis and transient stricture formation but is suitable for single-stage neonatal treatment of HSCR.

Early outcome of transanal endorectal pull-through with a short muscle cuff during the neonatal period.

BACKGROUND/PURPOSE: Transanal pull-through has been advocated recently for classic Hirschsprung's disease. The procedure leaves no scars, is associated with less postoperative pain and discomfort, and shortens hospital stay. Long-term functional outcome of patients having transanal pull-through is unclear, but short-term function is reported to be very similar to that after open or laparoscopic procedures. One-stage neonatal repair of Hirschsprung's disease has been suggested to be associated with less cost and demand of resources without jeopardizing functional outcome. The aim of this study was to ascertain the feasibility and safety of transanal pull-through in the neonatal period. METHODS: Case notes of 40 consecutive patients who had undergone transanal endorectal pull-through for Hirschsprung's disease between January 2000 and February 2003 were reviewed. The patients were divided in to 3 groups: patients with neonatal primary pull-through (group I, 15 patients), patients operated on beyond the neonatal period (group II, 11 patients), and patients with a previous colostomy (group III, 14 patients). All colostomies except one were taken down and pulled through concomitantly with the transanal procedure. The case notes were evaluated for hospital stay, time to full oral feedings, operative and postoperative complications, need for postoperative dilatations, and occurrence of postoperative enterocolitis. RESULTS: There was no difference in median hospital stay (group I, 5 days; group II, 4; group III, 5) and median time to full oral feedings (group I, 4 days; group II, 2; group III, 3) between groups. Two patients (group III) had immediate postoperative prolapse of the pulled-through colon that was reduced without further sequels; 1 (group III) had infection of the stoma closure wound. Perianal skin rash that usually resolved within 6 weeks occurred more often in neonatal patients (group I, 10 of 15; group II: 4 of 11; group III: 8 of 14). Anastomotic dilatation regimen was required more often in neonatal cases (group I, 6 of 15; group II, 1 of 11; group III, 2 of 14). Enterocolitis requiring hospital care occurred in 2 patients (group I), 5 further patients (group II, 1; group III, 4) were treated as outpatients for symptoms suggesting mild enterocolitis or bacterial overgrowth. The median follow-up was 6 months. CONCLUSIONS: Transanal endorectal pull-through in neonatal patients is as feasible and safe as in older children or in those with a levelling colostomy. However, temporary postoperative skin rash occurs more frequently in neonatal patients, and postoperative dilatations are required more often than in older children.

[Transanal endorectal pull-through with short muscular cuff in the treatment of Hirschsprung disease. Preliminary study with 37 patients]. / Descenso endorrectal transanal con manguito muscular corto en el tratamiento de la enfermedad de Hirschsprung. Estudio preliminar de 37 pacientes.

INTRODUCTION: Totally transanal operation for classic Hirschsprung's disease has gained increasing popularity during the last few years. The procedure leaves no scars, is associated with less postoperative pain and discomfort and shortens hospital stay. The most commonly used technique for transanal pull-through is long endorectal dissection leaving a long muscular cuff, which is usually split posteriorly. We present our preliminary results following transanal endorectal operation with a short unsplit muscular cuff. MATERIALS AND METHODS: Thirty-seven patients underwent short-cuff transanal endorectal operation for Hirchsprung's disease between years 2000 and 2002. Patients' hospital records were analysed retrospectively. The collected data included age at the operation, associated conditions, hospital stay and time to full enteral feeds, occurrence of pre- and postoperative enterocolitis and preoperative stoma, operative complications, need for postoperative anal dilatations, postoperative perianal skin problems and preliminary data on bowel function. RESULTS: The median age at operation was 5 months (range 0-60 months), 13 patients were operated in the neonatal period. Six patients had Down's syndrome, 1 had cartilage-hair hypoplasia and one Ondine's syndrome. Six patients had preoperative enterocolitis. Thirteen patients had undergone levelling stoma formation for unremitting obstruction or enterocolitis, two of these had a long segment aganglionosis. The proximal ganglionic stoma was concomitantly pulled-through and anastomosed to the anus in all 13 patients with a stoma. Complications related to surgery occurred in 3 patients, 2 had prolapse of the pulled-through bowel (reduced immediately without later sequels) and 1 infection in the stoma closure wound. Median postoperative hospital stay was 4 days (range 2-21 days) and median time to full enteral feeds 3 (range 1-14) days. The median follow-up time was 6 months (range 1-22 months). Postoperative enterocolitis occurred in 1 patient, further 3 patients had symptoms suggesting mild enterocolitis. Nine patients required anal dilatations, two of those for a period of 3 weeks. Twenty-two patients had perianal skin rash, which resolved usually within 6 weeks. Early postoperative bowel function was characterised by frequent bowel movements in most patients. These usually resolved within few months; of the 17 patients with a follow-up for longer than 6 months only 2 have more than 3 bowel movements per day. At last follow-up frank soiling occurred in 2 patients, one patient needs oral laxatives for constipation. CONCLUSIONS: Transanal endorectal pull-through with a short cuff is a safe operation with low incidence of operative and postoperative complications. Hospital stay and time to full enteral feeds is significantly shorter than following conventional procedures; this associates with lower hospital costs. Long-term functional outcome of patients having totally transanal pull-through is unclear but short tern function is very similar than after procedures where transanal mucosectomy is combined with rectosigmoid dissection through laparotomy.

Descenso endorrectal transanal con manguito muscular corto en el tratamiento de la enfermedad de Hirschsprung. Estudio preliminar de 37 pacientes / Transanal endorectal pull-through with short muscular cuff in the treatment of hirschsprung disease. Preliminary study of 37 patients

Introducción. El los últimos años está ganando creciente popularidad la operación totalmente transanal para la Enfermedad de Hirschsprung clásica. Esta operación no deja cicatrices, produce menos dolor postoperatorio y acorta la estancia hospitalaria. La técnica más común para realizar este descenso transanal es la disección endorrectal prolongada dejando un manguito muscular largo que generalmente se abre en su cara posterior. Presentamos nuestros resultados preliminares con una técnica similar pero con un manguito muscular más corto e intacto. Material y métodos. Entre 2000 y 2002 operamos 37 pacientes con Enfermedad de Hirschsprung mediante esta técnica. Las historias se analizaron retrospectivamente con atención a la edad, malformaciones asociadas, estancia hospitalaria, tiempo hasta alimentación oral completa, enterocolitis pre y postoperatoria, ostomía preoperatoria, complicaciones operatorias, necesidad de dilataciones, problemas cutáneos perineales postoperatorios y datos preliminares sobre la función intestinal. Resultados. La edad en el momento de la operación fue de 5 meses (mediana, rango de 0-60), Trece niños fueron operados en el periodo neonatal. Seis tenían Síndrome de Down, 1 una hipoplasia cartílago-pelo y otro un Síndrome de Ondine. Seis pacientes tuvieron enterocolitis preoperatoria. Trece habían tenido un estoma previo por obstrucción intratable o enterocolitis y 2 de ellos tenían un aganglionosis de segmento largo. En todos los pacientes se pudo bajar al ano el cabo proximal gangliónico de la ostomía. Hubo complicaciones relacionadas con la operación en 3 pacientes, 2 tuvieron prolapso del intestino descendido (reducido inmediatamente sin secuelas) y 1 infección de la herida de cierre de la ostomía. La estancia postoperatoria fue de 4 días (mediana, rango 2-21) y el tiempo medio hasta la alimentación oral total de 3 (mediana, rango 1-14).El tiempo de seguimiento fue 6 meses (mediana, rango 1-22). Ocurrió enterocolitis en un paciente y tres más tuvieron síntomas que sugerían enterocolitis leve. Nueve niños requirieron dilataciones anales de los que 2 durante un periodo de tres semanas. Veintidós pacientes tuvieron irritación de la piel perineal que generalmente desapareció en las primeras 6 semanas. La función intestinal postoperatoria temprana se caracterizó por deposiciones frecuentes que fueron disminuyendo a lo largo de los siguientes meses en la mayoría de los pacientes. De los 17 niños con un seguimiento de más de 6 meses sólo 2 tienen más de 3 deposiciones al día. En el último control 2 pacientes se manchan y uno necesita laxantes por estreñimiento. Conclusiones. El descenso endorrectal transanal con manguito corto es una operación segura con pocas complicaciones operatorias y postoperatorias. La estancia hospitalaria y el tiempo hasta la alimentación oral completa son significativamente más cortos que tras los procedimientos convencionales lo que genera menores costes hospitalarios. El resultado funcional a largo plazo es aún desconocido pero la función a corto plazo es muy similar a la obtenida tras otros procedimientos en los que la mucosectomía transanal se combina con abordaje intraabdominal por laparotomía (AU)

Transanal coloanal pull-through with a short muscular cuff for classic Hirschsprung's disease.

INTRODUCTION: A totally transanal operation for classic Hirschsprung's disease has become increasingly popular during the last few years. The procedure leaves no scars, is associated with less postoperative pain and discomfort and shortens hospital stay. The most commonly used technique for transanal pull-through is long endorectal dissection leaving a long muscular cuff, which is usually split posteriorly. We present our preliminary results following transanal endorectal operations with a short unsplit muscular cuff. MATERIALS AND METHODS: Twenty-six patients underwent short-cuff transanal endorectal operation for Hirschsprung's disease between years 2000 and 2002. Patients' hospital records were analysed retrospectively. The collected data included age at operation, associated conditions, hospital stay and time to full enteral feeding, occurrence of pre- and postoperative enterocolitis and preoperative stoma, operative complications, need for postoperative anal dilatations, postoperative perianal skin problems and preliminary data on bowel function. RESULTS: The median age at operation was 1 month (range 0 - 60 months), 13 patients were operated on in the neonatal period. Four patients had Down's syndrome, 1 had cartilage-hair hypoplasia and one Ondine's syndrome. Five patients had preoperative enterocolitis. Four patients had undergone levelling stoma formation for unremitting constipation or enterocolitis. The proximal ganglionic stoma was concomitantly pulled-through and anastomosed to the anus in all 4 patients with a stoma. One patient with aganglionosis extending to the proximal sigmoid required additional laparoscopic colonic mobilisation. Complications related to surgery did not occur in the present series. Median postoperative hospital stay was 3 days (range 2 - 21 days) and median time to full enteral feeding was 3 days (range 1 - 14). Six patients required anal dilatations, two of those for a period of 3 weeks. The median follow-up time was 6 months (range 1 - 22 months). Fourteen patients had perianal skin rash, which usually resolved within 6 weeks. Postoperative enterocolitis requiring hospitalisation occurred in 1 patient, another patient had mild symptoms suggesting enterocolitis; these responded to oral antibiotic treatment. Early postoperative bowel function was characterised by frequent bowel movements in most patients. This usually resolved within a few months; of the 15 patients with a follow-up of longer than 6 months only 2 have more than 3 bowel movements per day. At the last follow-up frank soiling occurred in 1 patient with Down's syndrome, one patient requires oral laxatives for constipation. CONCLUSIONS: Transanal endorectal pull-through with a short cuff is a safe operation with a low incidence of operative and postoperative complications. Hospital stay and time to full enteral feeds is significantly shorter than after conventional procedures; this is associated with lower hospital costs. Long-term functional outcome is unclear but short-term function is very similar to that after procedures where transanal mucosectomy is combined with open rectosigmoid dissection.

Morbidity and quality of life in adult patients with a congenital abdominal wall defect: a questionnaire survey.

BACKGROUND: In children with congenital abdominal wall defects (CAWD), surgical treatment of the abdominal defect and the associated anomalies cause considerable morbidity in the first years of life. Afterward, most of the CAWD patients with correctable anomalies develop as other children. The morbidity and quality of life (QoL) of CAWD patients who have reached their adulthood is less well known and the subject of this study. METHODS: A 3-part questionnaire was sent to 75 former patients with CAWD, aged 17 years or more. The first part included questions about health, symptoms, and education; the second part consisted of 3 tests of psychosocial functioning; and the third part was a SF-36 questionnaire measuring the QoL. RESULTS: Of the 75 patients, 57 (76%) answered: (25 males, 32 females); omphalocele (n = 16) gastroschisis (n = 11); median age, 27 (range, 17 to 48) years. With the exception of rheumatoid arthritis (in 7% of patients), the prevalence of acquired diseases in CAWD patients was comparable with that of the general population; 50 of 57 (88%) considered their health good. The most frequent causes of morbidity were disorders in the abdominal scar in 21 (37%) patients, and functional gastrointestinal disorders in 29 (51%) of patients. Low self-esteem was found in 12% of patients, but the QoL and educational level of CAWD patients were not different from that of the general population. CONCLUSIONS: In CAWD patients the morbidity from acquired disorders is similar to morbidity in the general population. Disorders with the abdominal scar and various functional gastrointestinal disorders are common, but they rarely cause serious problems. The majority of CAWD patients have a quality of life not different from the general population.