Alveolar capillary dysplasia with left heart obstruction - rare but lethal.

Alveolar capillary dysplasia (ACD) is a rare neonatal lung disease characterized anatomically by a defective and hypoplastic development of pulmonary alveoli leading to persistent pulmonary hypertension (PPHN) and finally lethal respiratory failure. It is often associated with congenital left heart obstruction. Given the fatal prognosis an early diagnosis is important. However, due to the fast onset of PPHN in neonates and lack of pathognomonic signs for its cause, safe and fast detection of ACD is challenging. Therefore, following the exclusion of cardiac and common pulmonary causes, lung biopsy becomes essential for diagnosis.We hereby report a case of ACD with atrial septal defect type one and hypoplastic aortic arch with an ante-mortem diagnosis and discuss the current state of medicine in relation to ACD.

Acute Fatty Liver of Pregnancy and its Differentiation from Other Liver Diseases in Pregnancy.

Background: There are a number of threatening liver diseases that occur during pregnancy. Acute fatty liver of pregnancy is a rare disease associated with high maternal and foetal mortality. Case Report: We report on a young gravida 1 woman who presented to our level 1 perinatal centre in the 36 + 5 week of pregnancy with an isolated elevation of transaminases together with diffuse upper abdominal complaints. After comprehensive diagnostic work-up we performed an emergency delivery by Caesarean section. This was followed by interdisciplinary management. Discussion: The differentiation from other liver diseases seems not to be obvious in all cases. Here we consider the following differential diagnoses: hyperemesis gravidarum, intrahepatic gestational cholestasis, preeclampsia, HELLP syndrome. Conclusion: Rapid diagnosis and delivery as well as interdisciplinary aftercare are necessary in order to reduce maternal and foetal mortality.

[Abdominal ultrasonography in patients with diabetes mellitus. Part 1: Liver]. / Abdominelle Sonografie bei Patienten mit Diabetes mellitus. Teil 1: Leber.

In patients with diabetes mellitus, abdominal ultrasonography is the appropriate diagnostic technique to detect and to follow-up secondary and accompanying diseases of the liver, the kidneys, the pancreas, the gastrointestinal tract and of abdominal vessels. Moreover, pancreatic and hepatic diseases may be realized which are of etiological importance for diabetes mellitus. Based on a systematic survey of the published literature, this review in 3 parts will describe the value of abdominal ultrasonography in patients with diabetes mellitus. Part 1 deals with the diagnostic relevance and particular findings of ultrasonographic methods in hepatic manifestations and complications of diabetes mellitus.

Fetomaternal hemorrhage (FMH), an update: review of literature and an illustrative case.

BACKGROUND: Blood trafficking from fetus to mother and vice versa is a well-known physiological event that occurs at any stage in pregnancy. If the fetus looses high blood quantities to the maternal blood stream it becomes symptomatic. These symptoms can vary from cardiovascular distress to fetal death. MATERIALS AND METHODS: We give a review of current literature on Fetomaternal hemorrhage (FMH). CONCLUSION: This article highlights the importance of physician's awareness on detecting this rare but life threatening entity with both severe consequences for mother and neonate. The traditional measurement of FMH and the co-usage of alpha-fetoprotein are debated. To conclude we describe and discuss an illustrative case of FMH. This article gives an applicatory overview of symptoms, diagnostics and treatment of FMH to facilitate physicians to detect this disease precociously.

[How safe is sedation in gastrointestinal endoscopy? A multicentre analysis of 388,404 endoscopies and analysis of data from prospective registries of complications managed by members of the Working Group of Leading Hospital Gastroenterologists (ALGK)]. / Wie sicher ist die Sedierung in der gastrointestinalen Endoskopie? Eine multizentrische Auswertung von 388 404 Endoskopien und Auswertung der Daten aus prospektiv geführten Komplikationsregistern von Mitgliedern der Arbeitsgemeinschaft leitender Gastroenterologen im Krankenhaus (ALGK).

BACKGROUND: Gastrointestinal endoscopies are increasingly being carried out with sedation. All of the drugs used for sedation are associated with a certain risk of complications. Data currently available on sedation-associated morbidity and mortality rates are limited and in most cases have substantial methodological limitations. The aim of this study was to record severe sedation-associated complications in a large number of gastrointestinal endoscopies. METHODS: Data on severe sedation-associated complications were collected on a multicentre basis from prospectively recorded registries of complications in the participating hospitals (median documentation period 27 months, range 9 - 129 months). RESULTS: Data for 388,404 endoscopies from 15 departments were included in the study. Severe sedation-associated complications occurred in 57 patients (0.01 %). Forty-one percent of the complications and 50 % of all complications with a fatal outcome (10/20 patients) occurred during emergency endoscopies. In addition, it was found that 95 % of the complications and 100 % of all fatal complications affected patients in ASA class ≥ 3. CONCLUSIONS: Including nearly 400,000 endoscopies, this study represents the largest prospective, multicenter record of the complications of sedation worldwide. The analysis shows that sedation is carried out safely in gastrointestinal endoscopy. The morbidity and mortality rates are much lower than previously reported in the literature in similar groups of patients. Risk factors for the occurrence of serious complications include emergency examinations and patients in ASA class ≥ 3.

[Enteroscopy and imaging in sclerosing mesenteritis]. / Enteroskopie und Bildgebung bei sklerosierender Mesenteritis.

Sclerosing mesenteritis is a rare, benign, and chronic fibrosing inflammatory disease of the mesenteric fatty tissue. Its aetiology is unknown. In the present report we describe a 56-year-old women who presented with postprandial abdominal pain, and weight loss. Ultrasound, computed tomography, and magnetic resonance imaging revealed a mesenteric mass of 15 cm. The findings were typical for this disease. Additionally the patient underwent a single ballon enteroscopy in which the mucosa showed a considerable hyperergic reaction. The histological examination of the ileum was appropriate to support the suspicion. The patient's symptoms responded to a therapy with tamoxifen.

Aortic arch repair: let it beat!

Objective aortic arch repair (AAR) on the beating heart may reduce cross-clamping times and offer improved postoperative cardiac function.Methods A single-center review of all patients (n = 24) who underwent surgical AAR during biventricular repair between 01/2006 and 01/2008 was done. All patients were operated on under cardiopulmonary bypass (CPB) with antegrade cerebral perfusion (ACP). During AAR, 13 patients (group 1) received cardioplegic arrest, and were compared to 11 patients (group 2) who underwent a beating-heart modification with selective myocardial perfusion. Seventeen patients had additional intracardiac lesions and underwent simultaneous correction during the procedure.Results Durations of CPB, AAR and ACP did not differ statistically between groups. Cardioplegic arrest time was significantly lower in group 1 (34 ± 13 vs. 76 ± 11 min, p = 0.02) and resulted in a subsequent reduction of myocardial ischemic damage as borne out by lower postoperative levels of troponin T and CK-MB (2.5 ± 0.7 vs. 7.1 ± 1.4 ng/mL, p = 0.02; 68.7 ± 11.5 vs. 149.1 ± 27.2 U/l, p = 0.03). We observed an enhanced patient recovery with shorter inotropic and ventilatory support times (p < 0.05).Conclusion Pediatric aortic arch correction on a CPB beating heart with selective myocardial perfusion is technically feasible and safe. The reduction of the myocardial ischemic time is effective and results in less myocardial damage.

[Symptomatic choledocholithiasis during pregnancy - the role of ultrasound, ERCP and EUS]. / Symptomatische Choledocholithiasis in der Schwangerschaft - Einsatz der Sonografie, ERCP und EUS.

We present an overview of the management of acute bilary pancreatitis in pregnancy with particular reference to endoscopy. Ultrasound is the gold standard for diagnosis. If clinical symptoms, laboratory parameters and transabdominal ultrasound do not allow appropriate decision making, endoscopic ultrasound (EUS) is the method of choice. EUS is preferably performed prior to ERCP during the same clinical session, this is of particular relevance for pregnant women with regards to the safety of mother and fetus. However, because the procedure is performed under fluoroscopic guidance, there are potential risks related to the radiation exposure. ERCP, especially in pregnant patients demands high expertise from the endoscopist, and with due diligence and attention to detail, the procedure can be safely performed.

[Ultrasound-guided interventions and description of the equipment]. / Sonografisch gestützte Interventionen[nl](auch eine kleine Materialkunde).

For many years ultrasound-guided biopsy and drainage has represented a routine part of clinical practice. Percutaneous biopsy and drainage are minimally invasive procedures for sampling tissue or the removal of fluid collections within the body. Differing image guidance techniques for the positioning of the needles/catheters are in use. This is due to the advancement of effectiveness and safety of modern guidance methods. For the management of abscesses, percutaneous drainage is more effective (depending on the size) than percutaneous needle aspiration and is being increasingly used as the first-line treatment. This shift in practice is driven by the preference for low-risk, minimally invasive procedures and thus using surgical interventions only when percutaneous drainage has been unsuccessful. However, the variety of biopsy needles and drainage systems available to the clinician can appear overwhelming. We present a literature review regarding the devices and materials currently available to achieve the most reliable results with the lowest level of complications. With regard to drainage systems, particular considerations include the consistency of fluid, path of access and guidance technique. Although we present comparisons between published studies, it is important to consider that the personal experience of the interventionalist will also have an impact on the device chosen.

[Duodenal diverticulum--a therapeutic challenge]. / Blutendes Duodenaldivertikel - eine therapeutische Herausforderung.

We report the case of an 81-year-old women with a duodenal diverticular bleeding in the second portion of the duodenum. Various therapies are used to halt haemorrhaging in this tissue, including surgical or endoscopic intervention and transarterial embolisation. The lesion was identified and treated endoscopically with hypertonic saline solution and epinephrine. Because of a fistula and a continual bleeding we use a hemoclip two days later. The bleeding was completely controlled. However, a small mucosal defect was noted at the site where the hemoclip was applied, and an operation was necessary. Finally we discuss the therapy optoins based on a review of the literature.

[Splenic injury after colonoscopy: a retrospective study and review of the literature]. / Milzverletzung nach Koloskopie. Eine retrospektive Studie und Analyse der Literatur.

BACKGROUND AND OBJECTIVE: The increasing number of preventive screening investigations in the course of obtaining early diagnosis of colon cancer, has been leading to an increase in the number of colonoscopies in Germany. Splenic injury is one of the complications to be aware of, if postinterventional abdominal symptoms occur. PATIENTS AND METHODS: This retrospective study was based on the answers to questionnaires sent to 220 members of the Working Party of Leading Hospital-Based Gastroenterologists. The response rate was 40%. RESULTS: Thirteen of the gastroenterologists reported a total of 16 splenic injuries, a rate of about one injury per 87,000 (about 0.001 %) this compares to a worldwide published splenic injury rate of 0.004 %. But the actual numbers are probably higher in Germany as well. CONCLUSIONS: In order to obtain a more exact complication rate in the future, splenic injuries should be reported centrally. This is possible by sending a report of the complication by e-mail to splenicinjury_colonoscopy@web.de.

[Ultrasound guided interventions, part 1, diagnostic procedures]. / Sonografisch gestützte Interventionen (auch eine kleine Materialkunde).

Ultrasound-guided punctures have been part of clinical routine for many years. Meanwhile the variety of biopsy needles has become huge, so that we can offer a special gadget for each indication and each organ. The results of investigations of the materials may help one to gain a general idea of the available possibilities and help to in the choice of the right one. In the special chapters about organs and tumour types one can find out which biopsy needles can achieve best results and guarantee the lowest possible level of complications. The path of access and possible injuries to important structures play an important role when choosing the size of the biopsy needle and the technique of puncture. Besides the results of comparative studies, the personal experience of the interventionalist is an important factor for choosing the right material because the individual's manual skills have an important impact on the results.

The Norwood procedure - does the type of shunt determine outcome?

BACKGROUND: Stage I palliation of hypoplastic left heart syndrome (HLHS) and its variants is usually performed by a Norwood operation. The management of pulmonary blood flow during this procedure remains controversial. The RV-to-PA conduit (RVPAC) has been proposed as the better alternative compared to a systemic-to-pulmonary shunt (SPS). METHODS: A retrospective single center chart review of consecutive patients who underwent a Norwood I procedure between 01/1997 and 09/2006 was performed. All patients were operated in deep hypothermia, with or without circulatory arrest, using different shunt modifications according to surgeon's preference. Patients were divided into two groups depending on surgical management for pulmonary blood flow (modified BT shunt [BT] and non-valved RVPAC [Sano]). RESULTS: Fifty-four patients were included in the study (BT: 31 patients vs. Sano: 23 patients). Diastolic blood pressure during the first 24 hours postoperatively was significantly lower in the BT group (BT: 38.6 +/- 6.9 mmHg vs. Sano: 42.4 +/- 7.2 mmHg; P < 0.01) with a trend towards a higher systolic blood pressure (BT: 74.1 +/- 13.5 mmHg vs. Sano: 69.8 +/- 12.1 mmHg; P = 0.08). Mean circulatory arrest time in the BT group was significantly longer compared to the Sano patients (BT: 41 +/- 21 min vs. Sano: 25 +/- 23 min; P < 0.01). The mean hospital stay was 18.5 days for BT patients and 20 days for Sano patients ( P = 0.45). Early mortality for the total cohort was 14.8 % (n = 8) (BT 19.4 % [n = 6] vs. Sano 8.7 % [n = 2]; P = 0.12). There was no significant difference in inter-stage mortality between the two groups (BT: 18.2 % vs. Sano: 21.1 %; P = 0.47). CONCLUSION: The results for both established surgical methods (BT and Sano) for the palliation of HLHS and its variants have improved over time and are reaching acceptable early mortality rates. There was a trend towards a favorable early outcome for Sano patients, which did not reach statistical significance in this study due to the low patient numbers.

Endoscopy in Barrett's oesophagus: adherence to standards and neoplasia detection in the community practice versus hospital setting.

OBJECTIVE: Potential process differences between hospital and community-based endoscopy for Barrett's oesophagus have not been examined. We aimed at comparing adherence to guidelines and neoplasia detection rates in medical centres (MC) and community practices (CP). DESIGN: Retrospective analysis. SETTING: All histologically confirmed Barrett cases seen over a 3-year period in six MC and 19 CP covering a third of all upper gastrointestinal endoscopies (n = 126,000) performed annually in Berlin, Germany. MAIN OUTCOME MEASURE: Rate of relevant neoplasia (high-grade intraepithelial neoplasia or more) in both settings in relation to adherence to standards. RESULTS: Of 1317 Barrett cases, 66% were seen in CP. CP patients had a shorter mean Barrett length (2.6 cm vs. 3.8 cm; P < 0.001) with fewer biopsies taken during an examination (2.5 vs. 4.1 for Barrett length

[Accidental cannulation of the hepatic artery following needle-knife sphincterotomy]. / Akzidentelle Sondierung der A. hepatica im Rahmen der Nadelmessersphincterotomie.

The use of needle-knife sphincterotomy has become an established technique for precut sphincterotomy to achieve am otherwise inaccessible bile duct. The present case report describes an accidental cannulation of the proper hepatic artery following needle-knife sphincterotomy. The endoscopic retrograde cholangiopancreaticography (ERCP) was performed for diagnosis and treatment of a pancreatitis presumably caused by cholelithiasis. After guide-wire placement and angiography of the artery through the papilla, there were no further complications. The distance between the catheter and the hepatic hilus was seen under X-ray control.

[Pseudoaneurysm of the left hepatic artery as a complication of ERCP with sphincterotomy]. / Pseudoaneurysma der Arteria hepatica sinistra als Komplikation einer ERCP mit Sphinkterotomie.

A pseudoaneurysm of the hepatic artery is a rare complication of interventional endoscopy or transhepatic puncture. The present case report describes a symptomatic pseudoaneurysm of the left hepatic artery following endoscopic retrograde cholangio-pancreatography (ERCP). The indication was a biliary pancreatitis, and it was treated by guide-wire-sphincterotomy and extraction of sludge with a Dormia basket. A superselective angiographic embolization was not necessary because of a spontaneous thrombotic obstruction of the pseudoaneurysm.

Bacterial biofilm within diseased pancreatic and biliary tracts.

BACKGROUND: Bacterial community structures in human pancreatic and biliary tracts were evaluated. METHODS: Gall bladder stones from 153 patients, 20 gall bladder walls, six common duct stones, 52 biliary stents, 21 duodenal biopsies, nine pancreatic duct biopsies, and five bile ducts were investigated using fluorescence in situ hybridisation (FISH) with ribosomal RNA targeted Cy3/Cy5 (carbocyanine) labelled oligonucleotide probes. RESULT: Duodenal, gall bladder, and bile duct walls were free of bacteria. A dense multispecies bacterial biofilm was present within the pancreatic duct of patients with calcific pancreatitis and within biliary stents, irrespective of diagnosis. The concentration, density, and amenability of the biofilm to FISH and DNA staining declined progressively with the grade of stent occlusion. The lowest detectable bacterial concentrations were found by FISH in completely occluded stents and brown/mixed gall stones. Bacteria were not detectable with FISH in cholesterol gall stones. CONCLUSIONS: A wide range of different branches and groups of bacteria participate in the development of biofilms on the surfaces of foreign bodies, such as biliary stents, mixed gall stones, or calcific pancreatic ducts, but not on the surface of pure cholesterol gall stones. Occlusion of stents leads to progressive extinction of the biofilm and mummification of its components. Deposition of cholesterol or other substances within the biofilm matrix may be a novel mechanism of host defence against bacteria present in these biofilms.

[Cyst of the common bile duct with acute abdomen]. / Choledochuszyste mit akutem Abdomen.

Congenital cysts of the bile duct are well-documented anomalies of the biliary tree. Choledochal cysts frequently cause malignant changes in the epithelial lining. In such patients, the prognosis is very poor mainly because of the lack of typical symptoms in the early stages. The incidence of carcinoma in patients with bile duct cysts is estimated at 2.5 % to 15 %, compared to an incidence of 0.012 % to 0.48 % in patients without bile duct cysts. We report the case of a 32-year-old Vietnamese woman with a history of acute epigastric pain. Exploratory surgery was performed, and the segment containing the cyst and the ectopic pancreas was resected. Sonography pointed the way, and resection would have been necessary even without the abdominal symptoms.

[Intrahepatic calcification--a differential diagnostic problem]. / Intrahepatische Verkalkungen--ein differential-diagnostisches Problem.

HISTORY AND CLINICAL FINDINGS: A 57-year-old woman was admitted for investigation of intrahepatic calcifications. Intrahepatic cholangiolithiasis was suspected. In 1993 she underwent cholecystectomy because of lithiasis. The examination with endoscopic retrograde cholangiography (ERC) was planned. INVESTIGATIONS: The laboratory findings were normal except for a mild elevation of alkaline phosphatase and gammaglutamyl transpeptidase. The sonographic examination of the abdomen showed multiple hyperechoic calcifications along non-dilated bile ducts. In the spleen of normal size there were found a lot of intraparenchymatous calcifications. The abdominal roentgenography revealed calcifications also in the pancreas. In ERC, the intra- and extrahepatic bile ducts were normal with simultaneous proof of parenchymatous calcifications. TREATMENT AND COURSE: Because of the medical history of the patient and the radiologic findings the multiple parenchymatous calcifications could be referred to a miliary tuberculosis during childhood. In miliary tuberculosis, the liver almost always is involved by acute granulomatous inflammation. The therapy of hepatic tuberculosis follows the guidelines of systemic tuberculostatic therapy according to other presentations of this disease. Under sufficient therapy, tuberculotic granulomas normally heal without cicatrization. Sometimes tissue reactions in form of local fibrosis and calcification lead to a mild reduction in hepatic function as seen in this case. CONCLUSION: In the European population, intrahepatic cholangiolithiasis is a rare cause of focal hyperechoic liver lesions. In differential diagnosis, numerous diseases of possible systemic course have to be considered, which may induce calcifications of the liver or other organs. Among systemic diseases characterized by granulomatous inflammation and possible calcification tuberculosis and sarcoidosis have to be mentioned first.