[Chronic anaemia in a patient with hereditary haemorrhagic telangiectasia and juvenile gastric polyposis]. / Chronische Anämie bei hereditärer hämorrhagischer Teleangiektasie und juveniler Polyposis des Magens.

HISTORY: A 52-year-old woman with chronic anaemia presented with recurrent epistaxis, telangiectasias and haemangiomas, suggesting the presence of hereditary haemorrhagic telangiectasia (Osler-Rendu-Weber disease). Moreover, previous investigations had also shown multiple polyps of the stomach. INVESTIGATIONS: A severe microcytic iron deficiency anaemia in combination with hepatic haemangiomas and duodenal angio-dysplasia were detected. Gastrointestinal endoscopy revealed multiple juvenile polyps in the gastric fundus and body, indicating the diagnosis of hereditary haemorrhagic telangiectasia in combination with juvenile polyposis. THERAPY AND COURSE: A gastrectomy was performed because of recurrent gastrointestinal bleeding and the malignant potential of juvenile gastric polyposis. Histopathology confirmed the diagnosis and did not reveal any malignancy. CONCLUSION: The association of hereditary haemorrhagic telangiectasia and juvenile polyposis, as seen in this patient, has been reported repeatedly. A diagnosis of one of the described entities should initiate the screening for evidence of the other one to prevent (life-threatening) complications.

[Lipoma of the small bowel]. / Dünndarmlipom - Seltene Ursache von Abdominalschmerzen und chronischen, blutigen Diarrhöen.

CLINICAL FEATURES: A 40-year-old female presented with chronic diarrhoea, intermittent abdominal pain, and melena. She reported of a 7 kg loss of weight within the preceding 6 weeks and showed signs of mild abdominal pain upon palpation of the right lower quadrant. Our clinical diagnosis was Crohn's disease or infectious gastro-enteritis as a CT scan showed thickening of the colonic wall in the right lower quadrant. However a previously performed ileocolonoscopy was normal. TECHNICAL EXAMINATIONS: Magnetic resonance imaging showed a thickened terminal ileum with extensive narrowing of the bowel lumen, in addition a polyp obstructing the lumen of the terminal ileum at 30 cm was detected during colonoscopy. THERAPY: The involved portion of ileum was resected. Intraoperatively an intussusception with the polyp forming the leading edge was found. Histological analysis led to the diagnosis "lipoma". Postoperatively the patient is now feeling well without any abdominal pain. CONCLUSION: Tumours of the small bowel are rare and are therefore often forgotten in the list of differential diagnoses of abdominal pain. If symptomatic, these tumours present mainly with uncharacteristic and unspecific signs, often leading to a delay in correct diagnosis as in the presented case. We therefore suggest that small bowel tumours should be considered at earlier stages in the differential diagnosis of unexplained abdominal pain in the middle-aged patient with signs of intestinal obstruction.

[Fatal sinus vein thrombosis in a patient with mixed connective tissue disease and secondary antiphospholipid antibody syndrome]. / Letale Sinusvenenthrombose bei einer Patientin mit Mischkollagenose und sekundärem Antiphospholipid-Antikörper-Syndrom.

BACKGROUND: The antiphospholipid (Huges) syndrome is a complication of connective tissue diseases characterized by thromboembolic occlusions of arterial and venous blood vessels. CASE REPORT: At the age of 13, the patient developed connective tissue disease with arthritis and myositis. The course of her disease was characterized by frequent relapses despite immunosuppressive treatment. She developed deep venous thrombosis of her right leg as a manifestation of secondary antiphospholipid antibody syndrome at the age of 15 and was subsequently started on oral anticoagulation therapy. Approximately 10 months later, however, she decided to try alternative medicine and stopped both anticoagulation and immunosuppressive therapy. Only after 4 weeks she developed seizures followed by respiratory arrest with the need for cardiopulmonary resuscitation. Despite intensive care she died 2 days later with the signs of severe cerebral edema causing herniation of the brainstem. Autopsy confirmed the diagnosis of severe edema of the brain as a result of extensive thrombosis of all sinus veins. CONCLUSION: A complete sinus vein thrombosis is a rare manifestation of antiphospholipid antibody syndrome. The lethal thrombosis in this case occurred during a period of reactive hypercoagulability after termination of immunosuppressive and/or anticoagulation therapy. This case report underlines the need for long-term anticoagulation in patients with the antiphospholipid syndrome.

Asymmetrical coronal synostosis, cutaneous syndactyly of the fingers and toes, and jejunal atresia in a male infant.

We report on a male infant with an unusual type of acrocephalosyndactyly presenting with unilateral coronal craniosynostosis, cutaneous syndactyly of toes 2 and 3, loss of distal triradii, and transverse alignment of dermal ridges of the palm suggesting syndactyly, atresia of the proximal jejunum, and anal stenosis.

[Three-dimensional analysis of the tunica media of umbilical arteries. Scanning electron microscopy study]. / Die dreidimensionale Analyse der Media der Umbilikalarterien. Eine rasterelektronenmikroskopische Untersuchung.

The three-dimensional media structure of the umbilical arteries in their various functional states has up to now been object of numerous investigations, which are predominantly based on light-microscopic findings and have come to extremely differing results. For that reason the electron microscope method with an optically higher resolution was chosen for this thesis. In total, 20 umbilical cords were recovered, and in each case one of the two umbilical cord arteries was kept in a condition of non-contraction by means of perfusion fixation immediately after the omphalotomy. After the necessary preparation longitudinal, diagonal and cross-sections of both non-contracted and fully contracted umbilical arteries as well as of the folds of Hoboken, which occur in the arteries postnatally, were investigated by the scanning electron microscope. During this examination the following findings were made: The media in the wide umbilical cord artery consists of two layers. The outer layer accounts for some three quarters of the wall and is composed of lamellae of parallel muscle fibres which surround the vessel alternatively in gently rising and falling thread-like turns. The inner layer accounts for the remaining quarter and also consists of lamellae the thread-like turns of which, however, run at a much higher pitch. Exactly subendothelial, a very thin layer of irregularly arranged fibres with longitudinal main direction can be clearly identified. The folds of Hoboken are caused by local media contraction in the course of which the outer media restructures itself, thus being the main carrier of the fold.(ABSTRACT TRUNCATED AT 250 WORDS)

Karyotype and placental structure of first-trimester spontaneous abortions: a morphometrical study.

Size of placental villi, thickness and condition of trophoblast, stromal density of villi as well as perivillous fibrinoid deposits were used as criteria for patho-morphological classification of karyotyped first-trimester spontaneous abortions. Significant differences in all the criteria were noted. Diagnostic values were verified using discriminant analysis. Using morphological values, 67% of the cases could be grouped correctly. Including further criteria (week of gestation, age of mother) it was possible to improve this to 72%. Normal karotype and trisomy could be accurately diagnosed using morphology; however, it is not suitable for finding monosomy X and tetraploidy.

[Experimental studies of wound healing in the paranasal sinuses. I. A model of respiratory wounds in the rabbit maxillary sinus]. / Experimentelle Untersuchungen zur Wundheilung in den Nasennebenhöhlen. I. Ein Modell respiratorischer Wunden in der Kaninchenkieferhöhle.

We present an animal model in rabbits for the investigation of normal wound healing in the paranasal sinuses. The blood and lymphatic stream and the mucociliary pathways are demonstrated on the medial maxillary sinus wall to study the influence on wound geometry. Standardized and circular wounds are created in the same area exposing the underlying bone of the maxillary sinus. Using a computer aided system for 3-D-reconstruction, it can be shown that respiratory wound closure is independent of the stream of blood, lymph or mucus. The circular wound diminishes in size concentrically. The standardized wound model may be suitable for comparison of different therapeutic methods of wound care.

[Primary chondrosarcoma of the ureter]. / Primäres Chondrosarkom des Ureters.

A 62-year-old patient had complained of recurring colicky pain in the right renal bed, seven days before he was admitted as an inpatient. Macrohaematuria was an accompanying sign during one of these attacks. Sonography revealed right urinary obstruction of medium severity; the right kidney could not be demonstrated by urography. Since CT did not yield any clue, retrograde ureteropyelography was performed. An obstructive tumour of the right ureter was identified. This tumour, which had a length of 3.5 cm and a width of 1.2 cm, was completely resected by ureteronephrectomy. Examination revealed an extraskeletal mesenchymal chondrosarcoma of predominantly intraluminal growth, and a tumour-free external ureteral wall. A chondrosarcoma at this site has not been reported as yet. Pathogenesis may be ascribed either to degeneration of an existing chorista or to the development of a pluripotent tumour parent cell.

Coarctation of the aorta in the syndrome of absent pulmonary valve with ventricular septal defect.

The case of a male child is reported in which the syndrome of absent pulmonary valve and ventricular septal defect was associated with anomalous drainage of the right superior pulmonary vein into the superior vena cava, atrial septal defect, a right aortic arch, and an aortic isthmic coarctation. The combination of aortic coarctation with a high degree of right-ventricular outflow obstruction has not been reported so far in the literature. This case shows that there is no proof of aplasia of the ductus arteriosus in the syndrome of absent pulmonary valve and ventricular septal defect. Most likely, there is an early involution of the ductus arteriosus; persistence of the ductus arteriosus would not be compatible with fetal survival.

Multiple middle cerebral artery aneurysms in an infant. Case report.

An 11-month-old boy was admitted for evaluation of drowsiness, vomiting, and convulsions. Computerized tomography showed subarachnoid blood in the left sylvian fissure and a small intracerebral hematoma in the temporal lobe. Angiography revealed several aneurysms of the left middle cerebral artery (MCA). During surgery, 13 aneurysms were found arising from one main branch of the left MCA, and this segment of the MCA was trapped. Somatosensory evoked potentials did not show any change during surgery. The diseased arterial segment was examined histologically and the pathogenetic aspects of the case are discussed. Control angiography 6 months later excluded systemic disease or other aneurysms. The rarity of such lesions in childhood and their successful surgical treatment are discussed briefly.

Surgical removal of pontomesencephalic cavernous hemangiomas.

Cavernous hemangiomas of the brain stem are usually discovered accidentally during evacuation of a hematoma, and successful surgical treatment of these lesions is seldom achieved. With the increasing use of magnetic resonance imaging, the presence of a cavernous hemangioma can be detected before surgery, allowing an elective surgical approach. We successfully removed pontomesencephalic cavernous hemangiomas from 2 patients and pontomedullary hemangiomas from 2 others. Elective surgery was performed with perioperative bimodal monitoring of somatosensory and auditory evoked potentials. Performing surgery soon after the hemorrhage minimizes the risk of additional postoperative neurological deficit, since surgical excision is facilitated when the hematoma is not completely organized. Pontine hemangiomas are approached via the 4th ventricle. Mesencephalic hemangiomas are removed by a midline supracerebellar approach when they are lateralized by using a subtemporal approach. The lesion can be removed through a small incision in the brain stem at the site of the lesion. The favorable results, which include marked improvement of preoperative neurological deficits and documentation of complete removal of the lesion by magnetic resonance imaging, support a more aggressive approach to the treatment of symptomatic cavernous hemangiomas of the brain stem. Further investigation of the natural history of these lesions is mandatory.

Physiological and pathological morphology of the umbilical and placental circulation.

Closure mechanisms of fetal vessels are essential after birth. They are physiological. The speedy and characteristic constriction is made possible by the special construction of the fetal vessels. The so-called "folds of Hoboken" in the umbilical artery initially form tapered constrictions which quickly extend to longer sections of the vessel. The contractions continue to include the chorion plate and the villi. Due to a lack of lamina elastica interna, a protrusion of pear shaped, expanded mediamyocytes and myofibroblasts is possible which reduces the cross-sectional area of these vessels. Similar, but lasting reductions in the cross-section of the villous arteries due to a fibrous-muscular media occur in the second half of pregnancy (IVth sign of maturity). Closure of the fetal vessels during intrauterine life leads to placental insufficiency of fetal death in utero. The endarteritis obliterans closes villous vessels by means of a connective tissue plug which starts on one side of the vessel and continues across the whole width of the lumen. The periphery dependent of these villi remains avascular, as the placenta is limited in its resorptive capabilities. In the case of intrauterine asphyxia, intravasal fibrinthrombi form as a sign of subacute insufficiency in the utero-placental circulation. A decompression collapse occurs in the fetal circulation, when fetal death occurs with continuing maternal circulation.

Surface and branching of placental villi in early abortion: relationship to karyotype. Scanning electron microscopic study.

The placental villi of 61 early abortions with known karyotype and 7 legally induced abortions were investigated by scanning electron microscopy and documented in standardised enlargements. Five groups were established from the findings: uniformly branched villi with a velvety surface (group A) were found in 4 of the 7 induced abortions, abundant syncytial sprouts (group B) in 4 of the 6 cases with monosomy X; all 5 cases of triploidy were classified in the group bulbous or spherical villi (group C); 13 out of 25 cases of trisomy were found to have little branching and a surface densely covered with microvilli (group D), while 14 out of the 25 cases of euploidy belonged in the group with slender villi and surface with focal areas of denudation (group E). Forty of the 68 cases were properly assignable to the correct groups (58.8%). The non-uniformity of the villous morphology in the case of induced abortions shows that there is no uniform development of the (early) placenta. The variable morphology seen in abortions with euploidy reflects the various mechanisms of abortion applicable to this group.

Lethal blockage of a Bjørk-Shiley artificial heart valve caused by strut fracture--the metallurgical aspect.

The fracture of the outlet strut of a Bjørk-Shiley artificial heart valve implanted in a 21 year old man lead--6 years after the operation--to the valve disc jamming closed with a resulting lethal heart failure and circulation standstill. The probable cause of the valve strut fracture could be determined by metallurgical/metallographical investigation of the material and the production methods.

Neuro-rhinosurgical treatment of aesthesioneuroblastoma.

The results of a combined neuro-rhinosurgical procedure in eight cases of aesthesioneuroblastoma are presented. All patients were suffering from tumours in the advanced stage (stage C according to Kadish). Diagnosis was established by the clinical history, neuro-radiological imaging and by endoscopic endonasal biopsy. Contrary to most reports in the literature the authors performed a one step operative removal of the whole tumour mass by a combined transcranial-transbasal approach alone. A second transfacial operation was unnecessary in all our cases. To the best of our knowledge only Loew (see Jakumeit 1971) already in the 1960ties used a comparable one step transcranial approach for tumour removal. The long-term survival rate in our patients is 50%, a result comparable to reported series in the literature. Mortality is due to early recurrences and metastases within a few months after the initial treatment including post-operative irradiation. Four patients are living without evidence of tumour recurrence 1.5 to 5 years after treatment. The authors surgical technique is presented in detail and compared with the results of other treatment modalities.

Risk factors of hepatocellular carcinoma in Germany: hepatitis B or liver cirrhosis?

In a retrospective study, 42 out of 44 cases of hepatocellular carcinoma were investigated immunohistochemically for chronic hepatitis B infection. Surface antigen was found in the liver tissue of only 4 of these cases. In 41 of the patients, mildly to moderately active cirrhosis of the liver was found to be underlying the carcinoma. The age distribution and case histories showed that hepatocellular carcinoma often developed from low-complication cirrhosis of long standing and of various etiologies, and must thus be considered a late complication of cirrhosis.

[Cholaemic placentosis--a contribution to metabolic disorders of the placenta]. / Cholämische Plazentose--Ein Beitrag zur Stoffwechselstörung der Plazenta.

The deposition of bile substances produces intracellular metabolic disorders in the organs so affected. Since severe cholaemia is rare in pregnancy, similar disorders of the placental cells have been not discussed up to now. We report on morphological observations in a placenta of a stillbirth in the middle of pregnancy. Bile substances are deposited in the syncytiotrophoblast and Hofbauer cells, pointing to the probable function of the Hofbauer cells as macrophages.