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The aim of this study was to assess whether adolescents following anorectal malformation repair have a decreased cardiorespiratory performance capacity and impaired motor skills. All eligible children treated for ARMs between 2000 and 2014 were invited to participate in a prospective study consisting of a clinical examination, evaluation of Bowel function and Quality of Life, spirometry, spiroergometry and assessment of the motor activity. The results were compared to a healthy age- and sex-matched control group. There was no statistically significant difference in height, weight, BMI, muscle mass or body fat percentage between the study and the control group. Nine out of 18 patients (50%) had an excellent functional outcome with a normal Bowel Function Score. Spirometry revealed no significant differences between ARM patients and controls, four patients showed a ventilation disorder. Spiroergometry revealed a significantly lower relative performance capacity and the overall rating of the motor activity test showed significantly decreased grades in ARM patients. ARM patients were affected by an impaired cardiopulmonary function and decreased motor abilities. Long-term examinations consisting of routine locomotor function evaluation and spiroergometry are advisable to detect impaired cardiopulmonary function and to prevent a progression of associated complications and related impaired quality of life.

Highlights • Laparoscopic repair of intermediate anorectal malformation (ARM) lead to full-thickness rectal prolapse (RP) in a boy. • As conservative treatment modalities failed, the parents opted for surgical treatment at the patient’s age of 4 years. • Intractable RP of 2 cm with ulcerations was successfully treated by Well’s laparoscopic posterior mesh-rectopexy. • The mid-term outcome was favorable with no prolapse recurrence. • Well’s procedure represents a feasible treatment option for RP secondary to ARM repair in children., Introduction Intractable full-thickness rectal prolapse (IRP) unresponsive to conservative treatment remains a major problem after anorectoplasty for high or intermediate anorectal malformation (ARM). Surgical management must aim for a permanent fixation of the rectum to the presacral fascia. While in children with IRP following ARM repair the optimal procedure has not been established yet, laparoscopic posterior mesh-rectopexy (Well’s procedure) has demonstrated efficacy in adults. Presentation of case A male infant with intermediate ARM received laparoscopic-assisted anorectal pull-through at the age of 4 months. Three months later he developed mucosal prolapse and received multimodal conservative treatment. Because of progression into a full-thickness RP with ulcerations, the parents opted for surgical management. Well’s procedure was performed at the age of 4 years. Using four ports, the rectum was circumferentially mobilized down to the pelvic floor and pulled inside. A 5 × 5 cm prolene mesh was tacked to the sacrum, enveloped posteriorly 270° around the rectum, fixed with interrupted prolene sutures on both edges and carefully covered with peritoneum. Any redundant external mucosa was excised from a perineal approach. There were no intra- and postoperative complications. Within 1.5 years of follow-up the boy had voluntary bowel movements and was toilet trained. No prolapse recurrence could be observed nor provoked. Discussion We present the first pediatric case of IRP secondary to laparoscopic ARM repair which has been successfully treated by combined Well’s procedure and perineal mucosal resection. Conclusion Well’s procedure is a successful technique and should be further explored in children with ARM and IRP.

Padiatrische Palliativpatienten sind Kinder, Jugendliche und junge Erwachsene mit meist chronischen, lebenslimitierenden Erkrankungen, die eine hohe Symptomdichte aufweisen. Im Rahmen eines ganzheitlichen Betreuungskonzepts, das die Lebensqualitat der Kinder und ihrer Familien in den Mittelpunkt stellt, spielt das Thema der optimalen Ernahrung bei fast allen Krankheitsverlaufen zumindest zeitweise eine wichtige Rolle. Wahrend es fur einige Diagnosegruppen sehr klare Richtlinien zur Ernahrung gibt, ist fur den Grosteil dieser Patienten weder der Begriff Fehlernahrung klar definiert, noch existieren – abgesehen von dem Gebiet der Ernahrung und der Flussigkeitszufuhr in der Lebensendphase – klare Empfehlungen. Ziele dieses fachgruppenubergreifenden, interdisziplinaren Projekts waren zum einen, fur die in dem Bereich der padiatrischen Palliativmedizin Tatigen praktisch anwendbare Kriterien zur Erkennung und zur Beurteilung einer Fehlernahrung zu definieren, und zum anderen, Leitlinien fur die optimale Ernahrung dieser Patientengruppen zu identifizieren. Diese Arbeit bezieht sich auf die „Leitlinien zur Ernahrung in der padiatrischen Palliativmedizin“, die in der Zeitschrift Padiatrie & Padologie (1. Supplement) im Marz 2015 publiziert wurden. Diese enthalten einen ausfuhrlichen Anhang mit praktischen Hinweisen, Fallbeispielen und einer Auflistung der derzeit erhaltlichen Sondennahrungen sowie eine umfassende Literaturliste und sollen eine praktische Hilfestellung fur alle im Bereich der padiatrischen Palliativmedizin Tatigen darstellen.

Background Surgical correction of congenital anorectal malformations could be complicated by fecal incontinence. Some authors believe that preservation of the fistula is associated with improved outcome. Rectal manometry is a well-established method to evaluate postoperative functional outcome in these patients and can demonstrate successful transplantation of the fistula. Objective Herein, we report the results of our series of patients with anorectal malformations and an externally accessible fistula, who underwent pre- and postoperative rectal manometry studies. Design This is a prospective cohort study. Settings This study was conducted at a tertiary neonatal and pediatric surgical center. Patients Patients with anorectal malformations, who underwent preoperative rectal manometry of the fistula and postoperative rectal manometry of the neoanus between January 2002 and December 2011 were included. Main outcome measures Pre- and postoperative rectal manometry results were compared by using paired t test or contingency tables (p values Results Twelve female patients with rectoperineal (n = 7, 58%) or rectovestibular (n = 5, 42%) fistula were treated by anterior sagittal anorectoplasty or minimal posterior sagittal anorectoplasty. Complete transposition of the fistula was achieved in all patients. Normal presence of rectoanal inhibitory reflex was demonstrated in all pre- and postoperative rectal manometry studies. There were no differences between pre- and postoperative rectal manometry in the length of the high-pressure zone (2.3 ± 0.6 cm vs 2.5 ± 0.8 cm (p = 0.5)) and resting pressure (59.4 ± 18.2 mm Hg vs 62.1 ± 19.2 mm Hg (p = 0.62)). At a median follow-up of 665 days (range, 290-1165 days), all patients have voluntary bowel movements, with no incontinence or soiling. Limitations This study is limited by its small sample size and by single-institution bias. Conclusion Preoperative rectal manometry of rectoperineal or rectovestibular fistula showed the presence of functional anal structures within the fistula in all patients. We speculate that fistula-preserving surgery in patients with anorectal malformations is associated with improved bowel function outcome.

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function. One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9.6 years (range, 3–27 years). Comparative statistics were used to evaluate temporal changes between an early (1975–1989) and late (1990–2011) study period. Gross types of EA were A (n = 6), B (n = 5), C (n = 89), D (n = 7), and E (n = 2). Seventy (64.2%) patients had coexisting anomalies, 13 (11.9%) of whom died before EA correction was completed. In the remaining 96 infants, surgical repair was primary (n = 66) or delayed (n = 25) anastomosis, closure of TEF in EA type E (n = 2), and esophageal replacement with colon interposition (n=2) or gastric transposition (n=1). Long-gap EA was diagnosed in 23 (24.0%) cases. Postoperative mortality was 4/96 (4.2%). Overall survival increased significantly between the two study periods (42/55 vs. 50/54; P = 0.03). Sixty-nine (71.9%) patients presented postoperatively with anastomotic strictures requiring a median of 3 (range, 1–15) dilatations. Revisional surgery was required for anastomotic leakage (n = 5), recurrent TEF with (n = 1) or without (n=9) anastomotic stricture, undetected proximal TEF (n = 4), and refractory anastomotic strictures with (n = 1) or without (n = 2) fistula. Normal dietary intake was achieved in 89 (96.7%) patients, while 3 (3.3%) remained dependent on gastrostomy feedings. Manometry showed esophageal dysmotility in 78 (84.8%) infants at 1 year of age, increasing to 100% at 10-year follow-up. Fifty-six (60.9%) patients suffered from dysphagia with need for endoscopic foreign body removal in 12 (13.0%) cases. Anti-reflux medication was required in 43 (46.7%) children and 30 (32.6%) underwent fundoplication. The rate of gastroesophageal reflux increased significantly between the two study periods (29/42 vs. 44/50; P = 0.04). Twenty-two (23.9%) cases of endoscopic esophagitis and one Barrett’s esophagus were identified. Postoperative complications after EA/TEF repair are common and should be expertly managed to reduce the risk of long-term morbidity. Regular multidisciplinary surveillance with transitional care into adulthood is recommended in all patients with EA/TEF.

Hintergrund Kindesmisshandlung war lange Zeit eine der Haupttodesursachen im Kindesalter. Sie ist in ihrer Ursache, ihrem Erscheinungsbild, ihrer Konsequenz und Voraussage komplex. Der Umgang mit den Patienten ist emotional stark belastend.

This study aimed to evaluate a possible link between gastroesophageal reflux (GER) and behavior pattern indicating pain experience in a group of children with severe neurologic deficits.We prospectively evaluated 19 patients with severe neurologic impairment (10 males and 9 females; mean age, 12.7 years) suspected for GER before and after initial treatment. Repeated 24-hour pH monitorings were performed in 19 children, whereas additional repeated endoscopic investigations were carried out in 18. In these children a histologic examination of mucosal biopsies was performed. Behavior pattern was described by parents or caregivers. The level of activity was classified as no/occasional agitation, daily agitation, and autoaggressive behavior.The behavioral pattern of the children was most frequently (n = 24) described as "agitated" followed by "autoaggressive" behavior (n = 10). Neither autoaggression nor agitation was less frequent (n = 4). Children with autoaggressive behavior had a significantly higher reflux index compared with the neurologically impaired patients with agitation only (P.01). The reflux index was significantly higher in children with behavioral abnormalities than in children without abnormalities (P.0004). In children without agitation or autoaggression, the pH measurements were all normal. Biopsies of esophageal mucosa revealed inflammation in 27 cases (first degree in 9; second degree in 12, and third degree in 6). Patients with autoaggressive behavior and those with agitation only had a significantly higher degree of inflammation compared with children without behavioral abnormalities (1.77 and 1.35 vs 0.25, respectively; P.05). The degree of inflammation did not differ significantly among children with behavioral abnormalities.The present study suggests that in children with severe neurologic impairment, autoaggressive behavior or considerable agitation may be a marker for reoccurring or first-time-diagnosed pathologic GER. The severity of reflux correlates significantly to the behavior pattern resulting from experiencing pain. Pathologic GER should be excluded in all neurologically impaired children with behavioral abnormalities to prevent reflux-related complications and improve health-related quality of life.

In 1989, Sauer introduced a technique to treat patients with total colonic aganglionosis (TCA) where he preserved the ileo-cecal valve and the right hemicolon to retain water and electrolyte absorption. This report examines the long-term outcome of patients who underwent this technique. All TCA patients treated between 1981 and 2005 according to Sauer were invited in 2013 to participate in a questionnaire survey to assess their long-term outcome focusing on bowel function and quality of life (QoL), using standardized scores. Of eight TCA patients (2 females, 6 males), seven participated in the follow-up survey (median follow-up time 13.5 years (range 8–31.6). Early postoperative complications involving bouts of enterocolitis in 3 and anal strictures in 5 patients ceased with age. Bowel movements ranged from median 3–4 times a day (range 1–8). Bowel-function score (BFS) was reduced in 6 patients (median 16 points, range 8–19, max 20), who also reported soiling incidences with the need for night-time protective aids in two. QoL assessment, however, documented a good outcome with a median score of 10 points (range 7–13, max 13). This long-term investigation of TCA patients treated according to Sauer’s technique documented a good QoL despite a reduced BFS.

In neurologically impaired patients (NIP), surgical management of gastroesophageal reflux disease (GERD) has become a widespread standard. Several follow-up studies have shown a high incidence of complications and recurrent reflux. As one of the possible causes epilepsy is mentioned repeatedly in the literature. The aim of this study was to determine the effective impact of epilepsy on the postoperative outcome by comparing results of our patients suffering from epilepsy to those without this disorder. From 1984 to 1999, a total of 45 NIP underwent antireflux surgery, including 20 patients suffering from epilepsy (EP) and 25 patients without this disorder (nEP). The existence of epileptic disorders, incidence of epileptic seizures and administration of anticonvulsatory drugs as well as results of diagnostic procedures, postoperative complications and incidence and time of recurrence of pathologic GER were recorded and analysed retrospectively. Preoperatively all patients had pathologic results in 24 h pH-monitoring. Median RI was 15.75 (EP, range 5.2-28.6) and 17.55 (nEP; range 7.2-26.5). 12-months-postoperative 24 h pH-monitoring showed a median RI of 3.8 (EP; range 1.3-25.6) versus a median RI of 3.3 (nEP; range 0.7-26.3). During the long-term follow-up evaluation of 3.9 years, 17 EP suffered from persistent epileptic seizures despite medical treatment. 19 patients (42%) developed recurrent reflux. This included seven EP (35%) and 12 nEP (48%). The necessity of reoperation was higher in nEP (n = 5, 20%) than in EP (n = 1, 5%). This analysis did not show a significant correlation between recurrence of pathologic GER after fundoplication and the incidence of epileptic seizures. We consider our findings to strongly question the widespread opinion considering this correlation. We conclude, that cerebral seizures alone do not alter the operative outcome of antireflux surgery in NIP.

To evaluate the influence of proton pump inhibitors (PPI) in predominantly milk-fed infants with symptoms of GERD by 24-h pH-multichannel intraluminal impedance (24-h pH-MII). Ten infants (8 males and 2 females) with a mean gestational age of 39 weeks (28–40) were included. 24-h pH-MII was performed before prescription and during intake of PPI. Total acid exposure time, bolus exposure time (acidic/non-acidic/total) and the number of refluxes (acidic/non-acidic/total) were determined. Clinical symptoms were recorded and used to calculate the Reflux Symptom Index (RSI) and the Symptom Severity Index (SSI). There was a significant decrease in the number of acidic refluxes, total acid exposure and acidic bolus exposure time. However, this went along with a significant increase in non-acidic bolus exposure time. The total number of refluxes and the total bolus exposure time remained unchanged. Under PPI, a decrease of SSI and RSI for pain-related symptoms could be observed. For respiratory symptoms and vomiting however no significant changes could be demonstrated. Under PPI, an improvement of pain-related symptoms could be shown. The decrease of acid exposure went along with an increase of non-acidic refluxes resulting in almost constant total reflux numbers. This finding is interpreted as main reason for some persisting symptoms despite adequate PPI dosage. Concluding from our data PPI therapy should only be indicated in case of pain, but has no effect in case of vomiting or recurrent respiratory symptoms.

Goessler A, Huber-Zeyringer A, Hoellwarth M.E. “Recurrent gastroesophageal reflux in neurologically impaired patients after fundoplication.” Acta Paediatr 00, Stockholm. ISSN 0803-5253. Aim: To evaluate the outcome and analyse the main causes of complications and failures of antireflux surgery for gastroesophageal reflux disease (GERD) in neurologically impaired patients (NIP). Methods: From 1985 to 1999 44 NIP (mean age 12 years) underwent surgery for GERD. Type of surgery, complications and recurrent reflux were analysed. Results: Twenty-seven patients (61%) showed preoperatively severe failure to thrive. All patients showed pathologic results in 24-h pH monitoring. Surgical treatment consisted of ventral (n = 25, Thal) or dorsal (n = 4, Toupet) semifundoplication or a Nissen fundoplication (n = 15). Postoperatively, all patients showed an impressive growth, regress of symptoms and improvement of results of diagnostic investigations. Late complications and recurrence of reflux were significantly related to preoperative extreme dystrophy (p < 0.0025). In six patients (15%) severity of symptoms related to recurrent reflux required a reoperation 17.8 month postoperatively (range 8–35 month). Recurrent reflux was found in 40% after ventral semifundoplication and in 46% after Nissen fundoplication (8 and 47 months postoperatively, respectively). Conclusion: Symptoms improved impressively after fundoplication in mentally retarded children. The incidence of recurrent reflux is not related to the type of surgery, however, it occurs significantly earlier with ventral semifundoplication when compared with Nissen fundoplication. Both late complications as well as recurrent reflux are related significantly to preoperative dystrophy.