Unfolding the outcomes of surgical treatment of lumbar spinal stenosis-a prospective 5- and 10-year follow-up study.

PURPOSE: In this prospective study, we aim to determine surgical outcomes in patients with lumbar spinal stenosis (LSS) 10 years after surgery. METHODS: The study population consisted of 96 LSS patients who underwent decompressive surgery, 72 of whom participated in the 10-year follow-up. The patients completed a questionnaire preoperatively and 3 months, 5 years, and 10 years postoperatively. Outcome measures were satisfaction with the surgical outcomes, the Oswestry Disability Index (ODI), the visual analog scale (VAS), the numeric rating scale (NRS-11), and walking ability quantified in meters. Postoperative improvements at 5 and 10 years were analyzed using linear mixed models. Furthermore, comparisons between postoperative time points were made for clinical courses of pain, disability, and walking ability. RESULTS: At the 10-year follow-up, 68% of the patients were satisfied with the surgical outcomes. All the measured outcomes showed statistically significant improvement from baseline to the 5- and 10-year follow-up. The mean VAS score was 9.8 mm higher at the 5-year follow-up and 7.8 mm at the 10-year follow-up compared to the 3-month follow-up point. Similarly, the mean ODI was 4.8% higher at the 10-year follow-up compared to the 3-month follow-up point. CONCLUSION: This study reports the clinical course of pain, disability, and walking distance after LSS surgery with the 10-year follow-up. Based on our study results, patients with LSS could expect to have positive effects of their back surgery up to 10 years. However, minor worsening in pain and disability may occur and one-fourth of the patients may need a reoperation during the 10-year follow-up period.

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%).

Contemporary management of abdominal surgical emergencies in infants and children.

BACKGROUND: Acute abdominal complaints in children are common presentations in the emergency department. The aetiology, presentation, diagnosis and management often differ from those in adults. METHODS: This review was based on expert paediatric surgical experience confirmed by evidence from the literature obtained by searching PubMed and the Cochrane Library. Keywords used were the combinations of 'abdominal emergencies', 'acute abdomen' and the disorders 'acute appendicitis', 'intussusception', 'volvulus', 'Meckel's diverticulum', 'incarcerated inguinal hernia', 'testicular torsion' and 'ovarian torsion' with 'children'. Information was included from reviews, randomized clinical trials, meta-analyses, and prospective and retrospective cohort studies. RESULTS: Presentation and symptoms of abdominal emergencies, especially in young children, vary widely, which renders recognition of the underlying disorder and treatment challenging. Critically targeted imaging techniques are becoming increasingly important in obtaining the correct diagnosis without unnecessary delay. Minimally invasive techniques have become the method of choice for the diagnosis and treatment of many abdominal emergencies in children. CONCLUSION: Knowledge of abdominal disorders in childhood, their specific presentation, diagnosis and treatment facilitates management of children with acute abdomen in emergency departments. Imaging and minimally invasive techniques are becoming increasingly important in the diagnosis of acute abdomen in children. Urgent operation remains the cornerstone of therapy for most acute abdominal disorders.

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.

Vocal Cord Paralysis after Repair of Esophageal Atresia.

OBJECTIVE: Etiology of vocal cord paralysis (VCP) and laryngeal dysfunction may be congenital or surgical trauma of recurrent and superior laryngeal nerves. We assessed the incidence, risk factors, and morbidity of VCP after repair of esophageal atresia (EA). METHODS: Medical records of 201 EA patients from 2000 to 2022 were reviewed for this retrospective study. Postrepair vocal cord examination (VCE) included awake nasolaryngeal fiberoscopy by otolaryngologist or laryngoscopy under spontaneous breathing anesthesia. Before 2017, postoperative VCE was performed in symptomatic patients only and routinely after 2017. MAIN RESULTS: Overall, VCE was performed on 79 (38%) patients (52 asymptomatic), whereas 122 asymptomatic patients underwent no VCE. VCP was diagnosed in 32 of 79 patients (right 12, left 10, and bilateral 10; symptomatic 25 and asymptomatic unilateral 7) corresponding with extrapolated overall VCP incidence of 16 to 24% among 201 patients including asymptomatic ones. Ten patients (bilateral VCP 8 and left VCP 2) required tracheostomy. Of 10 patients with bilateral VCP, three underwent laryngotracheal expansion surgery (left VC lateralization in one and laryngoplasty in two with acquired subglottic stenosis), three remained tracheostomy dependent, three were off tracheostomy, and one died of complications after redo esophageal reconstruction. All patients with unilateral VCP managed without tracheostomy. Cervical dissection or ostomy formation was a major risk factor of VCP. CONCLUSION: Repair of EA is associated with a considerable risk of VCP and associated morbidity. Cervical EA surgery significantly increased the risk of VCP. Bilateral VCP may eventually require laryngotracheal expansion surgery.

General health, health-related quality of life and sexual health after pediatric liver transplantation: a nationwide study.

The long-term impact of pediatric liver transplantation (LT) and its complications on general health, health-related quality of life (HRQoL) and sexual health were assessed. We conducted a national cross-sectional study of all pediatric recipients who underwent LT between 1987 and 2007. Of 66 survivors, 57 participants (86%) were compared to randomly chosen healthy controls (n = 141) at 10.7 ± 6.6 years posttransplant. PedsQL4.0, SF-36, DISF-SR and AUDIT questionnaires for appropriate age groups were used. Patients and controls <7 years had similar HRQoL and 54% of patients aged over 7 scored within the controls' normal range on all HRQoL domains. In adult survivors, physical functioning and general health were decreased (p < 0.05). Biliary complications, reoperations and obesity were independently associated with reduced HRQoL (p < 0.05 for all). Still 64% of adult survivors considered their health excellent. Sexual health was similar to controls but LT recipients may experience problems with their orgasm strength (p = 0.050) and condom-based contraception was more common after LT than among controls (58% and 12%, p < 0.001). In conclusion, normal HRQoL and sexual health are achievable post-LT.

Cholesterol metabolism altered and FGF21 levels high after pediatric liver transplantation despite normal serum lipids.

Liver transplantation (LT) predisposes to metabolic derangements and increases the risk for cardiovascular disease. We conducted a national cross-sectional study of all pediatric recipients who underwent LT between 1987 and 2007. We measured serum levels of noncholesterol sterols (surrogate markers of cholesterol synthesis and intestinal absorption) and fibroblast growth factor 21 (FGF21) in 49 patients (74% of survivors) at a median of 10 years posttransplant and in 93 controls matched for age and gender. Although serum cholesterol levels were similar in patients and controls, patients displayed increased whole-body synthesis and decreased intestinal absorption of cholesterol compared with controls (lathosterol to cholesterol ratio 129 ± 55 vs. 96 ± 41, respectively, p < 0.001; campesterol to cholesterol ratio 233 ± 91 vs. 316 ± 107, respectively; p < 0.001). Azathioprine (r =-0.383, p = 0.007) and low-dose methylpredisolone (r =-0.492, p < 0.001) were negatively associated with lathosterol/sitosterol ratio reflecting a favorable effect on cholesterol metabolism. FGF21 levels were higher in patients than in controls (248 pg/mL vs. 77 pg/mL, p < 0.001). In healthy controls, FGF21 was associated with cholesterol metabolism, an association missing in LT recipients. Normal serum lipids are achievable in long-term survivors of pediatric LT, but changes in cholesterol metabolism and increased FGF21 levels may explicate later cardiovascular risk.

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.

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.

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.

Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood.

BACKGROUND: Surgery for total colonic aganglionosis (TCA) is designed to preserve continence and achieve satisfactory quality of life. This study evaluated a comprehensive group of clinical and social outcomes. METHODS: An international multicentre study from eight Nordic hospitals involving examination of case records and a patient-reported questionnaire survey of all patients born with TCA between 1987 and 2006 was undertaken. RESULTS: Of a total of 116 patients, five (4·3 per cent) had died and 102 were traced. Over a median follow-up of 12 (range 0·3-33) years, bowel continuity was established in 75 (73·5 per cent) at a median age of 11 (0·5-156) months. Mucosectomy with a short muscular cuff and straight ileoanal anastomosis (SIAA) (29 patients) or with a J pouch (JIAA) (26) were the most common reconstructions (55 of 72, 76 per cent). Major early postoperative complications requiring surgical intervention were observed in four (6 per cent) of the 72 patients. In 57 children aged over 4 years, long-term functional bowel symptoms after reconstruction included difficulties in holding back defaecation in 22 (39 per cent), more than one faecal accident per week in nine (16 per cent), increased frequency of defaecation in 51 (89 per cent), and social restrictions due to bowel symptoms in 35 (61 per cent). Enterocolitis occurred in 35 (47 per cent) of 72 patients. Supplementary enteral and/or parenteral nutrition was required by 51 (55 per cent) of 93 patients at any time during follow-up. Of 56 responders aged 2-20 years, true low BMI for age was found in 20 (36 per cent) and 13 (23 per cent) were short for age. CONCLUSION: Reconstruction for TCA was associated with persistent bowel symptoms, and enterocolitis remained common. Multidisciplinary follow-up, including continuity of care in adulthood, might improve care standards in patients with TCA.

ANTECEDENTES: La cirugía de la aganglionosis colónica total (total colonic aganglionosis, TCA) está diseñada para preservar la continencia y lograr una calidad de vida satisfactoria. Este estudio evaluó un gran número de resultados clínicos y sociales. MÉTODOS: Se realizó un estudio internacional multicéntrico en ocho hospitales nórdicos en el que se incluyeron las historias clínicas de todos los pacientes nacidos con TCA entre 1987 y 2006. Se invitó a los pacientes y a sus cuidadores a responder una encuesta sobre la función intestinal, el crecimiento y las necesidades nutricionales, así como la repercusión social de la enfermedad a largo plazo. RESULTADOS: De un total de 116 pacientes, 5 (4,3%) habían fallecido y 102 respondieron la encuesta. Con una mediana de seguimiento de 12 años (rango 0,5-33), se había restablecido la continuidad intestinal en 75/102 (74%) a una mediana de edad de 11 meses (0,5-156). La mucosectomía con un manguito muscular corto y anastomosis ileoanal directa (short muscular cuff and straight ileo-anal anastomosis, SIAA) n = 29 o con reservorio en J (JIAA) n = 26 (55/72, 76%) fueron las reconstrucciones más habituales. Las complicaciones postoperatorias precoces que precisaron una intervención quirúrgica fueron muy poco frecuentes, pero se presentaron en 4/72 (5%) pacientes. Los síntomas a largo plazo relacionados con la función intestinal tras la reconstrucción, valorados en 57 niños mayores de cuatro años, fueron la dificultad para retener la defecación en 14 (25%), la encopresis en 21 (37%), las pérdidas fecales > 1/semana en 9 (16%) y el aumento de la frecuencia de defecación en 51 (89%). A largo plazo, se desarrolló una enterocolitis en 35/72 (47%) pacientes. Se precisó de nutrición enteral y/o parenteral suplementaria en algún momento del período de seguimiento en 51/93 (55%) pacientes. En los pacientes que respondieron a la encuesta entre 2 y 20 años (n = 56) de edad, se detectó un índice de masa corporal menor en 20 (35%) y una altura baja en 13 (23%) para su edad. En 35/57 (61%) pacientes mayores de 4 años con restauración del tránsito intestinal había restricciones sociales debido a los síntomas intestinales, de los que en 10 (17%) casos fueron moderadas o graves. CONCLUSIÓN: La reconstrucción de una TCA se asocia con síntomas intestinales persistentes y la enterocolitis sigue siendo frecuente. Un seguimiento multidisciplinario, incluso en la edad adulta, podría mejorar los resultados en la cirugía de la TCA.

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.

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.

Increased prevalence of pancreaticobiliary maljunction in biliary malignancies.

AIM: The incidence of pancreaticobiliary maljunction is thought to approximate 1:100,000 within Western populations. We aimed to study the significance of pancreaticobiliary maljunction in biliary tract malignancies. METHODS: Medical records and magnetic resonance cholangiopancreatography images of 252 consecutive patients treated for biliary malignancies during 2005-2016 were reviewed. Patients with other known risk factors for biliary cancers (n = 27) were excluded. A common pancreaticobiliary channel measuring ⩾10 mm outside the duodenal wall was defined as pancreaticobiliary maljunction. MAIN RESULTS: Of the 225 patients, a reliably interpretable preoperative magnetic resonance cholangiopancreatography was available for 73 (32%). Sex (47% vs 57% females) and age at diagnosis (67 vs 66 years) were similar among patients with or without an magnetic resonance cholangiopancreatography (p = ns for both). In magnetic resonance cholangiopancreatography, a pancreaticobiliary maljunction with a median length of 20 mm (range 10-23 mm) was identified in four patients (5.5%, 95% confidence interval 1.6-14), while none had evident accompanying biliary tree dilatation. Pancreaticobiliary maljunction patients were significantly more often females (100% vs 43%, p = 0.043), less likely to have intrahepatic bile duct cancer (0% vs 65%, p = 0.019) while more likely to have gallbladder cancer (75% vs 22%, p = 0.044) compared to the others. Age at diagnosis (66 vs 67 years, p = 0.898), extrahepatic bile duct cancer incidence (25% vs 13%, p = 0.453), and survival status at last follow-up (50% vs 42% alive, p = 1.000) were comparable between the subgroups. CONCLUSION: The prevalence of pancreaticobiliary maljunction is substantially higher in adults with biliary malignancies than one would expect based on its incidence, reinforcing the etiologic role of pancreaticobiliary maljunction especially in females with gallbladder cancer.

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.

European biliary atresia registries: summary of a symposium.

Biliary atresia (BA) is a rare but potentially devastating disease. The European Biliary Atresia Registry (EBAR) was set up to improve data collection and to develop a pan-national and interdisciplinary strategy to improve clinical outcomes. From 2001 to 2005, 100 centers from 22 countries registered with EBAR via its website (www.biliary-atresia.com). In June 2006, the first meeting was held to evaluate results and launch further initiatives. During a 5-year period, 60 centers from 19 European countries and Israel sent completed registration forms for a total of 514 BA patients. Assuming the estimated incidence of BA in Europe is 1:18,000 live births, 35% of the expected 1488 patients from all EBAR participating countries were captured, suggesting that reporting arrangements need improvement. At the meeting, the cumulative evaluation of 928 BA patients including patients from other registries with variable follow-up revealed an overall survival of 78% (range from 41% to 92%), of whom 342 patients (37%) have had liver transplants. Survival with native liver ranged from 14% to 75%. There was a marked variance in reported management and outcome by country (e.g., referral patterns, timing of surgery, centralization of surgery). In conclusion, EBAR represents the first attempt at an overall evaluation of the outcome of BA from a pan-European perspective. The natural history and outcome of biliary atresia is of considerable relevance to a European population. It is essential that there is further support for a pan-European registry with coordination of clinical standards, further participation of parent support groups, and implementation of online data entry and multidisciplinary clinical and basic research projects.

Outcome of Surgery for Pediatric Gastroesophageal Reflux: Clinical and Endoscopic Follow-up after 300 Fundoplications in 279 Consecutive Patients.

PURPOSE: Clinical and endoscopic assessment of the outcome after fundoplication for pediatric gastroesophageal reflux. BASIC PROCEDURES: Hospital records of 279 consecutive patients who underwent fundoplication for gastroesophageal reflux from 1991 to 2014 were reviewed. Underlying disorders, clinical and endoscopic findings, imaging studies, pH monitoring, and surgical technique were assessed. Main outcome measures were patency of fundoplication, control of symptoms and esophagitis, complications, redo operations, and predictive factors of failures. MAIN RESULTS: A total of 279 patients underwent 300 fundoplications (277 primaries and 23 redos). Underlying disorders in 217 (72%) patients included neurological impairment (28%) and esophageal atresia (22%). Indications for fundoplication included recalcitrant gastroesophageal reflux symptoms (44%), failure to thrive (22%), respiratory symptoms (15%), esophageal anastomotic stricture (4%), apneic spells (2%), and regurgitation (2%). Preoperative endoscopy was performed in 92% and pH monitoring in 49% of patients. Median age at primary fundoplication was 2.2 ((IQR = 0.5-7.5)) years. Fundoplication was open in 205 (74%; Nissen n = 63, Boix-Ochoa n = 97, Toupet n = 39, and other n = 6), laparoscopic in 72 (24%; Nissen n = 67 and Toupet n = 5), and included hiatoplasty in 73%. Clinical follow-up was a median of 3.9 (IQR = 1.2-9.9) years. Mortality related to surgery was 0.3%. Symptom control was achieved in 87% of patients, and esophagitis rate decreased from 65% to 29% (p < 0.001). Fundoplication failed in 41 (15%) patients. Failure was predicted by esophageal atresia risk ratio = 3.9 (95% confidence interval = 1.3-11, p = 0.01), any underlying disorder risk ratio = 3.1 (95% confidence interval = 1.1-9.1, p = 0.04), and hiatoplasty risk ratio = 2.6 (95% confidence interval = 1.1-6.6, p = 0.03). Of the 23 redo-fundoplications, 32% failed. CONCLUSION: The majority of patients who underwent fundoplication had an underlying disorder. Primary fundoplication provided control of symptoms in almost 90% of patients and also reduced the rate of esophagitis. Failure of primary fundoplication occurred in 15% of patients, and an underlying disorder, esophageal atresia, and hiatoplasty increased the risk of failure.

Gastrointestinal Complications After Surgical Correction of Neuromuscular Scoliosis: A Retrospective Cohort Study.

STUDY DESIGN: A retrospective cohort study of consecutively operated neuromuscular scoliosis patients. BACKGROUND AND AIM: Surgical correction of neuromuscular scoliosis can be complicated by early gastrointestinal complications, but data on the extent and severity of them is scarce. The aim of the study was to determine the incidence, course, and risk factors of gastrointestinal complications after neuromuscular scoliosis correction. MATERIAL AND METHODS: Ninety-one patients (<21 years of age) were consecutively operated on for neuropathic neuromuscular scoliosis during 2000-2011. Patients who developed marked postoperative gastrointestinal complications were identified and clinical, operative, and radiographic records, death certificates, and post-mortem reports were examined. RESULTS: The average age at surgery was 14.5 (SD 2.9) and follow-up time was 4.9 (SD 2.3) years. Gastrointestinal complications occurred in 12 (13%) patients and included prolonged paralytic ileus (7%, 6/91), dysphagia (7%, 6/91), and gastroparesis (1%, 1/91). Hospital stay was 22 (SD 11) days in patients with gastrointestinal complications and 16 (SD 20) days in non-complicated patients (p = 0.005). Dysphagia required permanent feeding gastrostomy in one patient whereas other complications were transient and none caused death. The risk factors for postoperative gastrointestinal complications were preoperative main curve correction <30% in traction/bending radiographs (Relative Risk (RR) = 28 (95% Confidence Interval (CI) 4.4-180); p < 0.001), preoperative main curve >90° (RR = 5.5 (95% CI 1.3-23); p = 0.020), disturbance in intraoperative spinal cord monitoring (RR = 6.0 (95% CI 1.1-34); p = 0.043), and intravenous opioid medication over 5 days postoperatively (RR = 7.9 (95% CI 1.8-35), p = 0.006). CONCLUSION: Gastrointestinal complications occurred in 13% of patients after neuromuscular scoliosis correction. Marked gastrointestinal complications extended postoperative hospitalization period, but they were transient in majority (92%) of cases and none caused death. Rigid scoliosis was the most significant risk factor for gastrointestinal complications. Gastrointestinal complications appear to be less frequent after posterior only spinal fusion with total pedicle screw instrumentation and Ponte osteotomies.