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.

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.

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.

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.

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.

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.

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.

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.

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.

In-hospital treated pediatric injuries are increasing in Finland--a population based study between 1997 and 2006.

BACKGROUND AND AIMS: Injuries are an important public health problem as well as the leading cause of death and disability among children. Our aim was to longitudinally explore the incidence of in-hospital treated traumas, their operative treatment and related mortality among pediatric patients in Finland. METHODS: The National Hospital Discharge Register and the Official Cause-of-Death Statistics data of in-hospital treated pediatric trauma patients between 1997 and 2006 in Finland were evaluated for hospitalizations, treatment modality and mortality. RESULTS: Fractures (69%) and head injuries (28%) were the most common in-hospital treated traumas (477/100,000 persons/year). These were followed by injuries of intra-abdominal (1.4%), thoracic (1.2%) and urological organs (0.6%). Head traumas constituted 67% of injury-related deaths. During the ten-year follow-up period, the annual incidence (per 100,000 persons) of head injuries decreased by 13.6% (152 in 1997 vs. 131 in 2006, p < 0.0001) mainly contributing to a 30% decrease in overall injury-related mortality incidence (from 5.7 in 1997 to 4.0 in 2006, p = 0.0519). The overall trauma incidence, and incidence of fractures and abdominal injuries significantly increased by 5.0% (p < 0.0001), 13.5% (p < 0.0001) and 37% (p < 0.05), respectively, while the incidence of thoracic and urological injuries remained unchanged. Up to 15% of spleen injuries lead to splenectomy. CONCLUSIONS: Although overall and head trauma-related mortality is decreasing, the increasing incidence of fractures and abdominal injuries has amplified the overall incidence of severe injuries among children in Finland. A significant number of unnecessary splenectomies are still performed among children.

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.

Cholesterol, non-cholesterol sterols and bile acids in paediatric gallstones.

BACKGROUND: Depending on underlying aetiopathogenetic factors human gallstones contain various amounts of cholesterol, non-cholesterol sterols and bile acids, which have remained unexplored in paediatric gallstone patients. AIMS: To evaluate sterol and bile acids compositions of paediatric gallstones. PATIENTS AND METHODS: Study group included 21 consecutively cholecystectomised children. Gas-liquid chromatography was used to quantitate gallstone sterols and bile acids. Results were compared to adult gallstones (n=194). RESULTS: Cholesterol stones (n=9) had higher proportions of cholesterol and lathosterol, but lower those of lanosterol and phytosterols than pigment stones (n=12) (p<0.05 for each). Patients with gallstone cholesterol content over 70% were female. Gallstone cholesterol positively reflected body mass index and, in cholesterol stones-group, age (r=approximately +0.700, p<0.05). Three patients on parenteral nutrition had brown pigment stones consisting of high amounts of campesterol and sitosterol ranging 483-9303 microg/100 mg of stone. Pigment stones had 13-fold higher amount of bile acids than cholesterol stones (p<0.05). Black pigment stones contained approximately 3-fold higher phytosterol proportions, and pigment stones and cholesterol stones had approximately 43% lower proportions of deoxycholic acid than adults (p<0.05). CONCLUSION: Gallstones in patients on parenteral nutrition are rich in phytosterols. With respect to gallstone sterols, gallstone disease of adolescent girls resembles that of adults. Composition of bile acids in paediatric gallstones is different from adults.

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.

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.