[Middle ear salivary gland choristoma related to branchio-oto-renal syndrome diagnosed by array-CGH]. / Speicheldrüsenchoristom im Mittelohr bei mittels Array-CGH diagnostiziertem branchiootorenalem Syndrom.

Branchio-oto-renal (BOR) syndrome is characterized by ear malformations associated with sensorineural or mixed hearing loss. In addition, preauricular tags, preauricular pits, branchial cleft fistulas and cysts, as well as renal dysplasia are seen. A genetic mutation on chromosome 8, either autosomal dominantly inherited or occuring as a spontaneous mutation, is the cause in the majority of cases. Using array-based comparative genomic hybridization (CGH), it is possible to detect even the smallest genetic changes. Salivary gland choristoma in the middle ear is very rare. Surgical removal and histological clarification are required.

[A new spruelike disease as a cause of severe diarrhea]. / Eine neue Sprue-ähnliche Erkrankung als Ursache schwerer Diarrhoe.

UNLABELLED: History and presentation at admission: A 77-years old woman presented with an 8-week history of pronounced diarrhea and weight loss of 10 kg. INVESTIGATIONS: Infectious enteritis and chronic inflammatory bowel disease were excluded. Duodenal biopsies showed villous atrophy and 50 intraepithelial lymphocytes per 100 enterocytes, but antibody testing for celiac disease was negative. TREATMENT AND COURSE: The diarrhea resolved within 2 weeks after withdrawing olmesartan, which she had received for hypertension for 5 years, without a gluten-free diet. 6 months later the histological abnormalities in the duodenum regressed markedly. A diagnosis of sprue-like enteropathy associated with olmesartan was made. CONCLUSION: The syndrome is probably rare and was first described in 2012. It should be suspected in patients on olmesartan treatment who have clinical and histological findings, but not serological markers of celiac disease.

Spinal cord ischemia after endovascular repair of the descending thoracic aorta in a sheep model.

OBJECTIVES: Spinal cord ischemia remains a devastating complication after thoracic aortic surgery. The aim of this study was to investigate the pathophysiology of spinal cord ischemia after thoracic aortic endografting and the role of intercostal artery blood supply for the spinal cord in a standardized animal model. METHODS: Female merino sheep were randomized to either I, open thoracotomy with cross-clamping of the descending aorta for 50 min (n=7), II, endograft implantation (TAG, WL Gore & Ass.), (n=6) or III open thoracotomy with clipping of all intercostal arteries (n=5) . CT-angiography was used to assess completion of surgical protocol and assess the fate of intercostal arteries. Tarloy score was used for daily neurological examination for up to 7 days post-operatively. Histological cross sections of the lumbar, thoracic and cervical spinal cords were scored for ischemic damage after stained with Hematoxylin-Eosin, Klüver-Barrrera and antibodies. Exact Kruskall-Wallis-Test was used for statistical assessment (p<0.05). RESULTS: Incidence of paraplegia was 100% in group I and 0% in group II (p=0.0004). When compared to the endovascular group, there was a higher rate of histological changes associated with spinal cord ischemia in the animals of the control group (p=0.0096). Group III animals showed no permanent neurological deficit and only 20% infarction rate (p=0.0318 compared to group I). CONCLUSIONS: In sheep, incidence of histological and clinical ischemic injury of the spinal cord following endografting was very low. Complete thoracic aortic stent-grafting was feasible without permanent neurologic deficit. Following endovascular coverage or clipping of their origins, there is retrograde filling of the intercostal arteries which remain patent.

Acute haemorrhage into a microcystic meningioma leading to cerebral herniation.

Low-grade (WHO level I) meningiomas are slow-growing, benign tumours typically presenting with unspecific symptoms (e.g. headache), seizures, cranial nerve compression and neuropsychological symptoms determined by location and size of the lesion. Haemorrhagic onset and sequelae are rare, and have been described infrequently. This is a case of a 50-year-old male presenting with signs of tentorial herniation secondary to hyperacute intratumoural haemorrhage (ITH) into a previously undiagnosed meningioma. Emergency surgical decompression and exstirpation of the lesion helped to achieve a favourable outcome. ITH has been described in all including benign intracranial neoplasms. Factors associated with a higher risk for haemorrhage in meningiomas are discussed. Though haemorrhages associated with meningiomas have been reported, ITH into low-grade meningiomas leading to herniation remains a rarity. Bearers of known lesions and their treating physicians who opt for conservative or delayed treatment should be aware of this remote complication.