Konrad Reinshagen

The Impact of Longitudinal Intestinal Lengthening and Tailoring on Liver Function in Short Bowel Syndrome

INTRODUCTIONnShort bowel syndrome is a functional or anatomic loss of major parts of the small bowel leading to severe malnutrition. The limiting factor for the survival of these patients remains parenteral nutrition-related liver damage leading to end-stage liver failure. Longitudinal intestinal lengthening and tailoring (LILT) has been proven to enhance peristalsis, to decrease bacterial overgrowth and to extend the mucosal contact time for the absorption of nutrients. The aim of this study was to show the impact of LILT on the development of parenteral nutrition-related liver damage.nnnPATIENTS AND METHODSnA cohort of 55 patients with short bowel syndrome managed with LILT in our institution between 1987 and 2007 was retrospectively reviewed. LILT was performed at a mean age of 24 months (range 4 - 150 months). Mean follow-up time was 83.76 months (range 5 - 240 months). We obtained reliable data from 31 patients with regard to liver enzymes and function parameters in blood samples before LILT and at the present time. Liver biopsy was performed in 14 patients prior to LILT.nnnRESULTSnLiver enzymes ALAT (mean 121 U/l), ASAT (mean 166 U/l) and bilirubin (mean 2.49 mg/dl) were elevated preoperatively in 27/31 children. After the lengthening procedure, ALAT (mean 50 U/l), ASAT (mean 63 U/l) and bilirubin (mean 1.059 mg/dl) normalized except in 5 of 8 patients who could not be weaned from parenteral nutrition after LILT. Liver function parameters such as the international normal ratio (INR) were slightly elevated in 5/31 patients. Albumin was generally low, probably due to parenteral nutrition. Liver biopsy was performed in 14 patients preoperatively, showing 4 patients with low-grade, 6 patients with intermediate and 4 patients with high-grade fibrosis. End-stage liver disease with cirrhosis was an exclusion criterion for LILT. All patients with liver fibrosis showed a normalization of liver enzymes when they were weaned from parenteral nutrition. But patients with higher grade liver fibrosis tend to develop more complications perioperatively.nnnCONCLUSIONnAfter LILT, all patients with liver fibrosis who could be weaned from parenteral nutrition showed a normalization of liver enzymes. Preoperative liver biopsy is mandatory in order to differentiate reversible liver fibrosis from end-stage liver disease. A higher grade of liver fibrosis and elevated INR has been shown to be a sensitive parameter for peri- and postoperative complications.

Right- versus left-sided congenital diaphragmatic hernia: postnatal outcome at a specialized tertiary care center.

Objective: To systematically investigate the impact of the location of the defect in congenital diaphragmatic hernia on neonatal mortality and morbidity with a special focus on survival at discharge, extracorporeal membrane oxygenation requirement, and the development of chronic lung disease. Design: Retrospective tertiary care center study with a matched-pair analysis of all fetuses that were treated for congenital diaphragmatic hernia between 2004 and 2009. Setting: A specialized tertiary care center for fetuses with congenital diaphragmatic hernia. Patients: Complete sets of data were available for 106 patients with congenital diaphragmatic hernia. For 17 of 18 infants with right-sided congenital diaphragmatic hernia we were able to allocate infants with left-sided congenital diaphragmatic hernia with no relevant difference in previously described prognostic factors, such as pulmonary hypoplasia and liver herniation. Interventions: None. Measurements and Main Results: There was a strong trend toward better survival in infants with right-sided congenital diaphragmatic hernia than with left-sided congenital diaphragmatic hernia (94% vs. 70%; p = .07). More neonates with left-sided congenital diaphragmatic hernia died of severe pulmonary hypertension despite extracorporeal membrane oxygenation. Fewer neonates with right-sided congenital diaphragmatic hernia died, yet higher degrees of pulmonary hypoplasia and oxygen requirement were observed despite extracorporeal membrane oxygenation. Conclusions: In congenital diaphragmatic hernia, the location of the defect has a substantial impact on postnatal survival and the development of chronic lung disease. In left-sided congenital diaphragmatic hernia, pulmonary hypertension resistant to therapeutic management, including extracorporeal membrane oxygenation, is more common and is associated with a higher rate of neonatal demise. Right-sided congenital diaphragmatic hernia infants have an increased benefit from extracorporeal membrane oxygenation but the better survival entails a higher rate of chronic lung disease.

Preventive antireflux surgery in neonates with congenital diaphragmatic hernia: a single-blinded prospective study

OBJECTIVEnCongenital diaphragmatic hernia (CDH) is known to be a predisposing factor in gastroesophageal reflux (GER) leading to pulmonary and nutritional problems. The aim of this prospective, randomized, patient-blinded study was to evaluate the benefit of antireflux surgery at the time of CDH repair.nnnMETHODSnFrom 2003 to 2009, 79 neonates with left-sided CDH were included. Forty-three had regular hernia closure. Thirty-six patients additionally had fundoplication at hernia repair. Follow-up was at 6, 12, and 24 months after birth with a standardized questionnaire and a thorax radiograph. Patients with clinical signs for GER were evaluated with upper gastrointestinal series and 24-hour pH-metry.nnnRESULTSnSeventy-nine of 263 patients participated in this prospective trial. Survival rate was 88.61%. The GER symptoms were almost significantly more frequent in the group without concomitant fundoplication at the age of 6 months. At 24 months, the difference between both groups was not significant anymore. Development of body weight in the first 2 years of life was similar in both groups. No complications related to initial antireflux surgery were noted.nnnCONCLUSIONnPatients profit from fundoplication at CDH repair only within the first year of life. At the present point of this study, simultaneous fundoplication at the time of primary CDH repair cannot be recommended as a standard procedure in all patients with left-sided CDH.

Candidiasis caused by Candida kefyr in a neonate: Case report

BackgroundSystemic Candidia infections are of major concern in neonates, especially in those with risk factors such as longer use of broad spectrum antibiotics. Recent studies showed that also term babies with underlying gastrointestinal or urinary tract abnormalities are much more prone to systemic Candida infection. We report a very rare case of candidiasis caused by Candida kefyr in a term neonate.Case PresentationRenal agenesis on the left side was diagnosed antenatally and anal atresia postnatally. Moreover, a vesico-ureteral-reflux (VUR) grade V was detected by cystography. The first surgical procedure, creating a protective colostoma, was uneventful. Afterwards our patient developed urosepsis caused by Enterococcus faecalis and was treated with piperacillin. The child improved initially, but deteriorated again. A further urine analysis revealed Candida kefyr in a significant number. As antibiotic resistance data about this non-albicans Candida species are limited, we started liposomal amphotericin B (AMB), but later changed to fluconazole after receiving the antibiogram. Candiduria persisted and abdominal imaging showed a Candida pyelonephritis. Since high grade reflux was prevalent we instilled AMB into the childs bladder as a therapeutic approach. While undergoing surgery (creating a neo-rectum) a recto-vesical fistula could be shown and subsequently was resected. The child recovered completely under systemic fluconazole therapy over 3 months.ConclusionsCandidiasis is still of major concern in neonates with accompanying risk factors. As clinicians are confronted with an increasing number of non-albicans Candida species, knowledge about these pathogens and their sensitivities is of major importance.

A case of severe Pierre Robin sequence with failure to thrive and tachycardia resolved after redo-fundoplication and hiatoplasty

We report an infant suffering from Pierre Robin sequence complicated by gastro-oesophageal reflux and failure to thrive, which were resistant to conservative therapy and a hemifundoplication. Gastro-oesophageal reflux was accompanied by supraventricular tachycardia, treated with propafenone. Tachycardia may be present in Pierre Robin sequence as a consequence of cardiac parasympathetic imbalance. The patient recovered completely from the gastro-oesophageal reflux and tachycardia after redo-fundoplication (Nissen) and a hiatoplasty were performed. Conclusion:This case shows that a thorough search for gastro-oesophageal reflux is indicated in each case of Pierre Robin sequence with failure to thrive.

Ultra late onset group B streptococcal sepsis with acute renal failure in a child with urethral obstruction: a case report.

IntroductionGroup B streptococci are a well-known cause of early and late onset sepsis. In neonates and older children gram-negative bacteria are mostly found in urinary tract infections and urosepsis. In adults predisposing factors for group B streptococci urinary tract infection may include diabetes mellitus and chronic renal failure.Case presentationWe present a rare case of a five-month-old Caucasian boy with ultra late onset urosepsis and acute renal failure caused by group B streptococci serotype V. Excretion urography showed a subvesical obstruction that consequently was surgically corrected after antibiotic treatment of the acute infection.ConclusionsGroup B streptococci serotype V, urogenitary tract malformations, previous hospitalization and medical interventions may be important risk factors for the development of ultra late onset Group B streptococci sepsis in non-neonates.

Langzeitergebnisse nach Carotisrekonstruktion, strukturelle Hirnveränderungen und neurologische Entwicklung nach ECMO-Therapie bei Neugeborenen mit kongenitaler Zwerchfellhernie

Hintergrund: Die extracorporale Membranoxygenierung (ECMO) beim respiratorischen Versagen des Neugeborenen ist eine invasive Therapie mit schwerwiegenden Komplikationen wie intracerebraler Blutung oder Ischamie nach Kanulierung der Arteria carotis communis. Fragestellung: Im Rahmen dieser propspektiven Studie wurde die Uberlebensqualitat von Kindern mit kongenitaler Zwerchfellhernie nach ECMO-Therapie evaluiert. Material und Methoden: Im Rahmen unseres standardisierten Nachsorgeprogrammes erfolgt eine regelmasige Verlaufskontrolle. Im Alter von 2 Jahren wird auch ein Schadel-MRT zur Detektion cerebraler Veranderungen und zur Evaluation des Gefasstatus nach Carotisrekonstruktion durchgefuhrt. Initial erfolgte ein MRT bei allen Kindern, mittlerweile nur noch bei Patienten nach ECMO-Therapie. Dies erlaubt eine Auswertung von Kindern mit derselben Grunderkrankung im Hinblick auf den Einflussfaktor ECMO-Therapie. Ergebnisse: Von den 64 Patienten mit MRT-Kontrolle im Alter von 2 Jahren waren 50 ECMO-Patienten, die Kontrollgruppe ohne ECMO-Therapie umfasste 14 Kinder. Die mediane ECMO-Dauer betrug 8 Tage [4d; 15d]. Bei 41 Neugeborenen wurde die Arteria carotis zum Zeitpunkt der Decanulierung rekonstruiert. Nach zwei Jahren war eine sekundare Okklusionsrate von 53,7% nachweisbar. Eine langere ECMO-Dauer war mit einer hoheren Komplikationsrate nach Carotisrekonstruktion assoziiert. Hirnveranderungen (erweiterte Liquorraume, Blutungs- und Infarktresiduen) fanden sich ausschlieslich in der ECMO-Gruppe (64%). Klinisch zeigten 15/64 Patienten eine leichte Entwicklungsverzogerung; hierunter waren auch 2 Kinder aus der Kontrollgruppe. Eine schwere psychomotorische Retardierung wurde ausschlieslich in der ECMO-Gruppe nach stattgehabter intracranieller Blutung oder Infarzierung beobachtet (9/50 Patienten). Strukturelle Hirnveranderungen wurden bei 2/3 der Patienten auf der Gegenseite der arteriellen Kanulierung festgestellt. Insgesamt zeigten 80% der Kinder eine normale oder leicht retardierte psychomotorische Entwicklung nach neonataler ECMO-Therapie, obwohl in 64% strukturelle Hirnveranderungen MR-morphologisch nachgewiesen werden konnten. Die Dauer der ECMO-Therapie (p<0,0005) und das mannliche Geschlecht (p=0,0051) waren statistisch signifikante Risikofaktoren fur Hirnveranderungen. Fur Gestationsalter, Geburtsgewicht und Seite der kongenitalen Zwerchfellhernie konnte kein Einfluss nachgewiesen werden. Das relative Risiko fur Hirnveranderungen war fur Jungen im Vergleich zu Madchen doppelt so hoch (RR=2,0 [1,14; 3,52]). Schlussfolgerung: Insgesamt konnten wir bei Patienten mit kongenitaler Zwerchfellhernie und der Notwendigkeit zur ECMO-Therapie eine Langzeitprognose mit Lebensqualitat beobachten. Eine schwerwiegende psychomotorische Retardierung findet sich nach cerebralen Einblutungen oder Infarkten (22%). Auserdem zeigte sich, dass das Risiko fur Hirnveranderungen und Komplikationen nach Carotisrekonstruktion mit der Dauer der ECMO-Therapie steigt sowie bei Jungen signifikant erhoht ist. Trotz der hohen Re-Okklusionsrate zeigte sich fur die Rekonstruktion der Arteria carotis im Vergleich zu Patienten mit primarer Ligatur ein positiver Effekt auf nachweisbare Hirnveranderungen und die neurologische Entwicklung.

Der Kurzdarm-Patient - Indikation zur Operation und operative Ergebnisse

Es wird uber 53 Patienten mit einem Kurzdarm und einem mittleren Alter von 25 Monaten berichtet, die nach der Methode von Bianchi einer longitudinalen Halbierung des Darmes zur Darmverlangerung aufgrund eines Kurzdarmsyndroms unterzogen wurden. Die Operationsindikation ergab sich aus der Notwendigkeit persistierender parenteraler Ernahrung, aus der aggravierenden Cholestase, aus unkontrollierbarer Stuhlfrequenz und Stuhlqualitat und aus der Ausschopfung der letzten adaquaten zentral-venosen Zugange. Das Follow-up betrug 79,5 Monate. 77,36% aller Patienten haben bis heute uberlebt. 80% der Patienten werden langfristig komplett enteral ernahrt. Darmverlangerungsoperationen sind bei Patienten mit einem Kurzdarmsyndrom, die eine sekundare Dunndarmdilatation und noch keine irreversible Leberfunktionsstorung zeigen, die Therapie der ersten Wahl. Der Transplantation sollten nur solche Patienten zugefuhrt werden, die nach der Dunndarmverlangerung nicht von der parenteralen Ernahrung entwohnt werden konnen und eine zunehmende Leberfunktionsstorung entwickeln.