Karl-Ludwig Waag

Congenital diaphragmatic hernia: a modern day approach.

Centralization of all complicated congenital diaphragmatic hernias (CDH) was organized in Germany from 1998, collecting 325 consecutive patients with striking increasing survival rates. This series report 244 patients from 2002 to 2007. Today, large defects are detected early in pregnancy by ultrasound and magnetic resonance imaging (MRI). In extracorporeal membrane oxygenation (ECMO) patients, prenatal lung head ratio (LHR) was 1.2 (median) at the 34th week of gestation or less than 25 ml lung tissue in MRI. This means that all patients below LHR of 1.4 should be transferred prenatally in a tertiary center. High risk group for survival was defined as LHR below 0.9, ie, 10 ml in MRI planimetry. Inborn patients show better results than outborns. In algorithm therapy, gentle ventilation plays an important role in preventing damage to the lung tissue and avoiding long term ventilation. When PaCO(2) was more than 75 mmHg, ventilation was changed to high frequency oscillatory ventilation (HFOV). Indication for ECMO was seen in preductal PaO(2) less than 50 mmHg over 2-4 h or less than 40 mmHg over 2 h. ECMO related risks included intracerebral bleeding (9%), intrapulmonary bleeding (14%), and convulsions (16%). Surgically, a longitudinal midline incision for exposure of the defect, the duodenal kinking, and probably for abdominal patching was perfect. A cone formed goretex patch provided more abdominal space and reduced abundant intrathoracical cavity. No drain was used. Postoperative complications were described. Overall survival in 244 consecutive patients was 86.5% for all patients born alive. All those who needed ECMO survived in 71%, underlining ECMO as a treatment of last choice. Follow-up for quality of life after CDH is described.

Hemarthrosis after trauma to the pediatric knee joint: what is the value of magnetic resonance imaging in the diagnostic algorithm?

In children, compulsory arthroscopy for hemarthrosis after knee trauma is not justified because ligamentous and meniscal damage is rare. In a prospective study, we analyzed the diagnostic value of radiography, magnetic resonance imaging (MRI), and arthroscopy in 51 patients up to 14 years of age with acute knee trauma. Plain radiography revealed 16 osseous lesions (5 metaphyseal, 3 patellar, 4 physeal fractures, 3 avulsions of the tibial spine, and 1 osseous ligamentous tear). In 29 patients, the cause of hemarthrosis remained unclear. All patients were evaluated by MRI. A diagnosis could be assigned to all 29 patients. MRI demonstrated lesions in 38 patients. In addition, the following lesions were discovered: 8 patellar dislocations, 13 bruises, 1 rupture of the anterior cruciate ligament, 1 osteochondritis dissecans, and 13 joint effusions. In 13 patients, MRI was followed by arthroscopy to confirm the diagnosis. Both, MRI and arthroscopy missed two osteochondral fractures. In addition, three chondral lesions were not picked up by MRI. MRI is a reliable tool for assessing the extent of knee lesions in children.

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

INTRODUCTION Short 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. PATIENTS AND METHODS A 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. RESULTS Liver 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. CONCLUSION After 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.

Long-term total parenteral nutrition-induced hepatobiliary dysfunction in a rabbit model

BACKGROUND/PURPOSE Currently, the reason for hepatobiliary dysfunction associated with long-term total parenteral nutrition (TPN) is much debated and still unclear. No agreement can be achieved about whether bacteriotoxins and sepsis, enteral starvation, consequences of abdominal operations, or the TPN solution itself is the real cause for the disease. Animal models were criticized for their short period of TPN and their failure to demonstrate cholestasis and bile duct proliferation. The aim of this study was to establish an animal model for long-term TPN in which the same alterations of the hepatobiliary system as observed in humans could be produced. METHODS In this model, rabbits could be kept for the first time under continuous TPN for 4 weeks. Three serial liver biopsy sections were taken operatively from each animal and biochemical analyses were performed four times. A control group of enterally fed rabbits underwent exactly the same procedure in respect to operations and handling, so that differences in macroscopical, biochemical, and histological changes between both groups could be attributed exclusively to TPN. RESULTS Only in the TPN group gallbladder distension developed in all animals after 1 week. After 3 and 4 weeks, viscous dark bile, sludge and stones, a slight rise in direct bilirubin, and a decline in plasma albumin and alkaline phosphatase was noted. In both groups liver biopsy results showed a similar degree of mild portal inflammation and single-cell necrosis at equivalent time points. These changes could be caused by antiseptics, antibiotics, anesthesia, and operations. Although mild to moderate proliferative changes and no hydropic degeneration developed in the control group during the same time, the TPN group generated marked proliferative and degenerative changes. We noted as early as 1 week after starting TPN a severe hydropic degeneration in 90% of the animals. Fibrosis and bile duct proliferation increased from a slight degree after 1 week up to a moderate to severe degree after 3 and 4 weeks, respectively. CONCLUSIONS The hepatobiliary alterations associated with TPN in children, which cannot be separated clinically from consequences of multiple other factors, can almost identically be reproduced in our rabbit model as a clear consequence of TPN. Furthermore, the hydropic degeneration of the liver cells begins in zone 3 and is an early predominant feature of hepatobiliary dysfunction in rabbits and infants. It must be rated as a response to a direct cytotoxic effect on the liver cell.

Parenteral nutrition-induced hepatobiliary dysfunction in infants and prepubertal rabbits

Abstract We analyzed clinical, biochemical, and histo- logic parameters of ten infants with parenteral nutrition-induced hepatobiliary dysfunction. The data were compared with the results of a rabbit model. All infants were born prematurely with low birth weight. Their clinical diagnoses were necrotizing enterocolitis (6), gastroschisis (1), intrauterine volvulus (1), and lung hypoplasia (2). All required total (TPN) or partial parenteral nutrition for at least 8 weeks. All had repeated episodes of infections or sepsis. A rise in bilirubin and aminotransferase levels occurred after a minimum of 5 weeks; peak bilirubin levels ranged from 4 to 14 mg% and aminotransferases from 40 to 140 IU/l. One child later developed gallstones. Liver biopsies after 1 to 24 months showed fibrosis, bile-duct proliferation, cholestasis, and hydropic degeneration. All of the above-mentioned clinical factors have been accused of causing the observed biochemical and histologic changes. In our rabbit model we were able to produce almost identical symptoms by TPN alone: gallbladder distension, sludge, and stones developed after 1–4 weeks of TPN as well as uncharacteristic changes in aminotransferases and bilirubin after 4 weeks. Liver histology revealed severe hydropic degeneration of zone 3 as early as 1 week after beginning TPN. A rise of fibrosis and bile-duct proliferation after 1 to 4 weeks of infusion was statistically significant. Cholestasis, as was observed in the infants, could not be detected. In our model, all alterations observed could be attributed exclusively to TPN. We therefore assume that TPN was the true cause of the dysfunction. In a second experimental series infusions were reduced to 80% PN and free access to lab chow. These animals produced normal feces, indicating physiologic enteral stimulation. They developed the same degenerative and proliferative histologic changes, whereas gallbladder distension, sludge, and stones were not noted. We conclude that: (1) The TPN solution itself is responsible for the histologic changes in the liver, which is supported by the fact that hydropic degeneration of zone 3 is typical of a direct toxic effect; and (2) Complete enteral starvation with an absence of enteral stimulation causes disease of the lower biliary tract.

Transanal Pull-Through Procedure for Hirschsprung's Disease: A 5-Year Experience

BACKGROUND/PURPOSE Transanal endorectal pull-through (TEPT) has become a widely used approach for the treatment of Hirschsprungs Disease. The technique is safe and, according to previous reports, it has a good clinical outcome. In this study our experience with TEPT in the early postoperative period is evaluated. METHODS The clinical course of 34 children (28 boys and 6 girls) who underwent one-stage pull-through operation according to De la Torre for Hirschsprungs disease from January 2003 to December 2007 was reviewed. Their ages ranged from 2 months to 4 years. Complications occurring within the first four weeks after operation were analyzed. RESULTS Eight of 34 children (24 %) had early complications in the form of dehiscences of the anastomosis. Two children (6 %) had symptomatic anastomotic dehiscences. One child had an almost full retraction of the colon that had to be pulled down and resutured. One child developed a retrorectal abscess three weeks postoperatively due to anastomotic leakage. The dehiscences of 6 children (18 %) were asymptomatic. These dehiscences were detected only with standardized routine examination. The dehiscences healed uneventfully after resuturing. Two other patients (6 %) developed an anastomotic stricture that could be treated with rectal dilatations. Four children (12 %) showed a single episode of postoperative enterocolitis. CONCLUSION The rate of early clinical and particularly subclinical complications such as anastomotic dehiscences after TEPT is higher than previously estimated. Patients should be monitored carefully during the early postoperative period. Severe complications can only be avoided with a thorough examination. Early resuturing of dehiscences might be helpful to prevent hazardous sequelae.

Diagnostische und therapeutische Konsequenzen nach Nierenverletzungen bei kindlichem stumpfen Bauchtrauma

ZusammenfassungDie Nierenverletzungen gehören zu den häufigsten Folgen des stumpfen Bauchtraumas. In einer retrospektiven Studie untersuchten wir, wie häufig Nierenverletzungen im Rahmen des stumpfen Bauchtraumas vorkamen, und welche diagnostischen und therapeutischen Schritte indiziert waren. Zwischen 1976–1996 wurden 308 Patienten stationär wegen eines stumpfen Bauchtraumas behandelt. Diagnostisch kamen die Peritoneallavage (bis 1983), die Sonographie und der Combur-Test, ferner gezielt i.v.-Urograpie, CT und Angiographie zur Anwendung. Die Nierenverletzungen wurden entsprechend dem “organ injury scaling” (OIS) nach 5 Schweregraden klassifiziert.Es fanden sich 69 relevante intraabdominelle Verletzungen, darunter 36 Nierenverletzungen ab Grad 2; 20 Kinder waren polytraumatisiert. Nach dem OIS teilten wir alle 67 Nierenverletzungen auf in 28 Grad-1-(Kontusion), 22 Grad-2-(Hämatom bzw. Lazeration < 1 cm), 8 Grad-3-(Lazeration ohne Urinaustritt), 5 Grad-4-(Lazerationmit Kelchbeteiligung bzw. vaskuläre Läsion), 1 Grad-5-Verletzung (Nierenzertrümmerung bzw. Devaskularisation) und 3 Läsionen der ableitenden Harnwege. Im Screening und in der Verlaufskontrolle erwiesen sich Sonographie und Harnanalyse als die optimalen diagnostischen Mittel. Ab Grad 2 war das CT in der Diagnostik am zuverlässigsten, so dass die i.v.-Urographie in den Hintergrund trat. Bei fehlender Kontrastmittelausscheidung im CT ist auf jeden Fall eine Angiographie indiziert. Nur in 10 Fällen war die operative Therapie notwendig.Im Verlauf der Jahre entwickelte sich ein Konzept zugunsten eines abwartenden Verhaltens mit sekundären, z. T. minimal-invasiven Eingriffen zum elektiven Zeitpunkt. Ab Grad 4 ist die operative Versorgung der Verletzungen, sei es als minimal-invasive Maßnahme, zwingend.AbstractRenal lesions are frequently encountered in blunt pediatric abdominal trauma. In this retrospective study, we analyzed the incidence of renal trauma in these trauma patients to determine which diagnostic and therapeutic approaches were most predictive. From 1976 to 1996, 308 children sustaining blunt abdominal trauma were admitted to our department. Patients were evaluated using abdominal paracentesis, ultrasonography, and urinary analysis. In specific cases, IV urography, CT scan, and/or angiography were applied. We used the Organ Injury Scale (OIS) for classification of renal trauma into five grades.We encountered 69 serious abdominal traumas. Thirty-six patients sustained renal lesions grade 2 (G2) or higher; 20 children were polytraumatized. There were 67 renal lesions with 28 G1, 22 G2, 8 G3, 5 G4, 1G5, and 3 lesions of the urinary tract. Ultrasonography and urinary analysis were found to be optimal diagnostic means for screening and observing the course of renal lesions. For lesions G2 or higher, CT scan was the most reliable in classifying and diagnosing renal lesions. This superceded IV urography. If no contrast medium was excreted in the CT scan, angiography was indicated. Only ten patients proceeded to operative therapy.During the period reviewed, a shift from operative to conservative treatment was notable with a tendency toward minimally invasive therapy. If lesions were G4 or G5, operative treatment was always indicated.

Peutz-Jeghers-Syndrom Beobachtungen am klinikum mannheim über 25 jahre

Observations in our clinic and others reviewed in the literature result in a new picture of the Peutz-Jeghers syndrome. It turns out to be a hereditary polypose syndrome which is hard to define. The tendency to malignant degeneration of polyps and development of associated neoplasms is almost impossible to forecast. In addition operations frequently involve complications and often need to be repeated more than once. Radical removal of all polyps must be the aim of treatment; the patients must then be closely followed up by clinical examination, endoscopy and radiology to avoid complications of regrowth and to make sure degeneration and associated neoplasms are detected at an early stage.ZusammenfassungAn Hand eigener Beobachtungen sowie einer Literaturübersicht wird ein neues Bild des Peutz-Jeghers-Syndroms entworfen. Es stellt sich als ein schwer abgrenzbares, hereditäres Polyposesyndrom mit einer kaum einschätzbaren Neigung zu Entartung und Entwicklung assoziierter Neoplasmen einerseits und wiederholten komplikationsträchtigen Operationen andererseits dar. Deshalb ist therapeutisch eine möglichst vollst↭dige Entfernung der Polypen zu fordern. Die Patienten müssen engmaschig klinisch und endoskopisch bzw. radiologisch kontrolliert werden, um polybedingten Komplikationen vorzubeugen und Entartungen und assoziierte Neoplasmen rechtzeitig zu erkennen.

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.

Totale und Obere Sternumspalte — Eine einfache Methode der chirurgischen Korrektur / Total and Superior Clefts: A Simple Technique for Surgical Correction

Angeborene Sternumspalten sind selten und in der Literatur nur als einzelne Falle beschrieben. Sie werden eingeteilt in eine totale, obere und untere Sternumspalte und in solche im Rahmen einer totalen ventralen Spaltbildung. Seit 1964 behandelten wir insgesamt 10 Patienten. 7 mit einer oberen und 3 mit einer totalen Sternumspalte. Unsere Methode beruht darauf, die Knochenbrucke zu separieren und somit die partielle in eine totale Spalte umzuwandeln. Nach Auffrischen der Sternalrander ist die Adaptation und der primare Verschlus ohne autologes Material moglich. So behandelten wir 8/10 Patienten. Alle waren postoperativ komplikationsfrei. 1 Patient mit oberer Spalte verstarb 3 Monate postoperativ an einem VSD. 1 Patient mit totaler Spalte verstarb gleich nach Geburt an seinen Fehlbildungen. Unserer Meinung nach ist die operative Versorgung schon in den ersten Lebenstagen indiziert, bei einfacher Technik, geringen Komplikationen und gutem kosmetischem Ergebnis.