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Clinical Neuroscience

JANUARY 20, 2017

Combination of severe facial and cervical vascular malformation with obstructive sleep apnea syndrome: diagnostic and therapeutic approaches

FALUDI Béla, IMRE Marianna, BÜKI András, KOMOLY Sámuel, LUJBER László

The combination of obstructive sleep apnea syndrome and vascular malformation within the head and neck region is a rare condition, and interestingly, only a few cases have recently been published. Propagation of the vascular mass to the larynx and pharynx can cause breathing and swallowing difficulties. Due to these sypmtoms, examination and initiation of appropriate therapy for such patients are indeed challenging. We reviewed the literature available and present our case of a 64 year old woman emphasizing the complaints of sleep apnea syndrome and vascular malformation of the face and neck region. Polygraphic examination detected severe obstructive sleep apnea syndrome. The MR examination of the neck revealed extensive vascular mass narrowing the pharyngo-laryngeal region, thereby causing temporal bone destruction on the right side with intracranial propagation. ENT examination demonstrated significant narrowing of the pharyngeal lumen and the laryngeal aditus caused by multiple hemangiomas. CPAP titration showed the minimalization of the apnea-hypopnea index on the effective pressure level. Regular CPAP usage resulted in diminishing a majority of the patient’s complaints. Our examination clearly demonstrates, obstructive sleep apnea syndrome coupled with significantly obstructing vascular malformation in the head and neck region can be effectively treated safely with a CPAP device, if surgical therapy is not possible. We summarized our findings and the data available in the literature to set up recommendations for the appropriate examination and therapy (including mask fit, etc.) of vascular malformations and hemangiomas causing pharyngo-laryngeal obstruction.

Clinical Neuroscience

JULY 30, 2016

[Angioneuritic edema in ischaemic stroke patients treated with rt-PA]

LOVÁSZ Rita, SAS Attila, KOLLÁR Tibor, PETERCSÁK Edina, FEKETE István, BILINSZKI Erika, VALIKOVICS Attila

[Data of our 254 patients who were treated with rt-PA between 1st of Jan, 2011 and 31st of Dec, 2014 were processed. We focused on angioneurotic oedema as allergic complication of thrombolysis which caused life threatening respiratory obstruction in two cases. We describe these two patients’ history. Out of 254 patients six (2.3%) suffered angioneurotic edema caused respiratory obstruction in two (0.90%) cases. This occurrence is approximately 1.3-5.1% in literature. Five, out of six patients who suffered from angioneurotic oedema, had been treated with ACE inhibitors or ARB before. The role of ACE inhibitors is known in metabolism of bradykinin cascade. Plasmin which present during thrombolysis, precipitates biochemical mechanisms of this potential life threatening complication. Therefore rt-PA alone can be the cause of angioedema, but it can be more frequent together with ACE inhibitors therapy.]

Clinical Neuroscience

JULY 30, 2013

[Shunt insufficiency due to knot formation in the peritoneal catheter]

FEKETE Gábor, NAGY Andrea, PATAKI István, BOGNÁR László, NOVÁK László

[The authors report a rare case of the peripheral obstruction of a ventriculoperitoneal shunt. Premature baby was operated on hydrocephalus due to germinal matrix bleeding. After two months of implantation of venticuloperitoneal shunt peripheral insufficiency of the system was emerged. During the shunt revision extensive knot formation became visible. We simply cut the catheter above the knot and the working shunt was replaced into the abdominal cavity. The postoperative course was uneventful and the baby was free of complaints for more than one year. The pathomechanism of knot formation is not clear thus the discovery of the problem during the operation is an unexpected event. In our opinion tight knot cannot be spontaneously formed intraabdominally. Loose knots can be developed and can reduce the capacity of liquor flow. We think that the knot tightens during pulling out. Longer peritoneal catheters can precipitate multiple looping and/or axial torquations and increase the peripheral resistance of the shunt. In such cases when the pulling out is challenged conversion to laparotomy is suggested.]

Lege Artis Medicinae

JULY 20, 2012

[Idiopathic retroperitoneal fibrosis (Morbus Ormond) associated with severe cardiomyopathy in a young man: a case report]

KOVÁCS Erzsébet, ZEHER Margit, SZÁNTÓ János, PÁSZTOR Éva, TÓTH László, PFLIEGLER György

[INTRODUCTION - The cause of idiopathic retroperitoneal fibrosis is unknown, but probably involves a chronic, nonspecific, autoimmune inflammation. This rare disease mostly affects middle-aged or elderly people, it hardly ever occurs in young people. It causes obstruction of the urinary tract, postrenal insufficiency as well as functional disorder of vital organs in the retroperitoneum and its surrounding area. The diagnosis is based on diagnostic imaging and histological examinations. Surgical treatment of the urinary obstruction is only efficient in the long-term if combined with immunomodulatory treatment. CASE REPORT - A young, 34-year-old man who presented with oedema in his legs was diagnosed with retroperitoneal fibrosis. Autoimmune serology was negative. He needed temporary nephrostomy on the right side. Treatment with steroid and colchicine was initially successful. His severe cardiomyopathy improved gradually. No regression occurred with tamoxifen, so we started to administer azathioprine with methylprednisolone. The control abdominal CT showed stagnation of the process. CONCLUSIONS - Because of the rarity of this disease, no randomised controlled trials are available regarding its treatment. The aim of treatment is to eradicate the symptoms, solve the urinary obstruction, maintain renal function and prevent progression of the disease. Improvement may be achieved by corticosteroids, cyclophosphamide, azathioprine, leflunomide, mycophenolate mofetil and tamoxifen. To our knowledge, this case is unique as no similar case with severe cardiomyopathy and idiopathic retroperitoneal fibrosis has been reported in the literature previously.]

Clinical Neuroscience

MAY 30, 2012

[Effect of two month positive airway pressure therapy on the structure of sleep, cognitive function and anxiety]

CSÁBI Eszter, VÁRSZEGI Mária, SEFCSIK Tamás, NÉMETH Dezsõ

[Obstructive sleep apnea is a common disorder, characterized by repeated episodes of upper airway obstruction during sleep, resulting intermittent hypoxia and disruption of the normal sleep pattern, which caused cognitive dysfunction in these patients. Nasal continuous positive airway pressure is the treatment of choice for this disorder. The aim of the study is to evaluate the effect of short-term positive airway pressure on sleep pattern (polisomnographic measures), cognitive function and anxiety. Twenty four newly diagnosed and previously untreated patients with obstructive sleep apnea were evaluated a battery of neuropsychological tests before and after 2 and a half months of the treatment. We focused on working memory, short and long-term episodic memory, executive functions, anxiety and subjective sleepiness. Our results showed that the two and half month of treatment improved the respiration during sleep, sleep pattern and the subjective sleepiness. We found improvement in short- and long-term verbal memory, and complex working memory. Despite of treatment we did not find improvement in visuospatial learning. These results reveal that 2 and a half months of positive airway pressure treatment restored not only the normal respiration during sleep and normal sleep pattern, but also the cognitive functions. Our study suggests that cognitive dysfunction is at least partial reversible in obstructive sleep apnea patients after positive airway pressure treatment.]

Hungarian Radiology

JUNE 20, 2007

[Investigation of colon transit with radiopaque m]

WENINGER Csaba, KIRÁLY Ágnes

[The constipation is a frequent disorder, which often requires gastroenterological examination. Colon transit study and defecography are indicated in case of making difference between slow colon transit time and outlet obstruction. The method, the mode of administration of the radiopaque markers and the evaluation of the radiological study are discussed. Radiopaque markers ingested on the first day are followed by abdominal X-ray taken at regular times. The normal value of the colon transit time is less than 70 hours. The etiology of constipation has to be clarified by functional examinations, since the treatment of outlet obstruction is different from slow transit constipation. To determine the large bowel transit time is necessary also in the differential diagnosis of slow colon transit. The method is a simple, reliable and easily reproducible.]

Hungarian Radiology

JUNE 20, 2002

[Ogilvie’s syndrome associated with excretory urography]

SZÁNTÓ Dezső, SZŰCS Gabriella

[INTRODUCTION - The Ogilvie's syndrome is a disturbance of colonic innervation with parasympathic overreaction was assumed to be cause of large bowel segment spasm and poststenotic accumulation of gas. There is no distention in small intestine. CASE REPORT - In case of a 15 years old male during excretory urography six minutes after the administration of contrast material infusion Ogilvie's syndrome had occured. On 6, 12 and 18 min. abdominal plain film were demonstrated the acute spasm and poststenotic large bowel distention by air lumenogram phenomenon in the kidneys ambilateral renal tuberculosis by the whitening-like contrast opacity arising from centre calyx (pyelotubular reflux) in right side and by clubbing of calyces in left side (daisy flower sign). Not involved the small intestine. The colonic spasm and accumulation of gas lasted approximately 6 hours and ceased without medical aid. CONCLUSION - The Ogilvie's syndrome accompanying excretory urography is a toxic effect attributing to transient injury of peripheric neures and neurovisceral synapses.]

Lege Artis Medicinae

DECEMBER 20, 2004

[BOUVERET’S SYNDROME]

KESZTHELYI László, FÓGEL Kristóf, VADINSZKY Péter, SÁVOLT Ákos, PŐCZE Balázs, BALOGH István

[INTRODUCTION - Gallstone ileus is a rare disorder among elderly population. The gallstone coming through a bilioenteral fistula into the gastrointestinal tract is stuck into the duodenal lumen causing ileus in approximately 0,5% of all cases. The syndrome is named after Bouveret, the first describer. CASE REPORT - Author present the case of 72 year old female patient with known cholelithiasis. Investigations proved existing cholelithiasis causing the patient abdominal pain one year before admission but she gave no consent to surgical intervention. An emergency admission to our department occurred after four days of abdominal pain and vomiting. The clinical status of the patient and abdominal X-ray examination suggested stomach evacuation disturbance. Double contrast barium swallow test and gastroscopy proved bowel obstruction caused by a gallstone. During surgery gastrotomy and the removal of gallstone was carried out. There were no postoperative complications and the patient was discharged home. CONCLUSION - Cholelithiasis should be resolved at any chosen time well before complications may occur. The simultaneous appearance of stomach evacuation problems and cholelithiasis should draw attention to this rare disease.]

Hungarian Radiology

MARCH 20, 2007

[Diagnostic and therapeutical possibilities in constipation]

ILLÉS Anita, KIRÁLY Ágnes

[Constipation is a common gastrointestinal problem. The prevalence of symptoms related with constipation fluctuates from 3 to 20 per cent. Constipation occurs more frequently in the elderly people and in females and more frequent in case of inactivity and less fiber intake. Assesment of patients with severe constipation includes specialized investigations. Exclusion of primary organic causes has to be the first step, then metabolic, neurological and iatrogenic causes (such as medicament side effects, etc) have to be excluded. After these considerations special functional gastroenterological investigations are needed which contribute to the diagnosis and differential diagnosis of the cause of the constipation. Anorectal manometry, ballon expulsion test, defecography and colon transit studies allow us to distinguish between slow colon transit, colon inertia, different subtypes of outlet obstruction, and the constipation predominant irritable bowel syndrome. The evaluation of these specific studies leads to the exact diagnosis and appropriate treatement for their problem can be given to the patients, which always has to be individually planed in all cases.]

Hungarian Radiology

APRIL 07, 2009

[Self-expanding metallic stents in intrahepatic biliary strictures after liver transplantation]

DOROS Attila, NÉMETH Andrea, HARTMANN Erika, DEÁK Pál Ákos, JUHAROSI Gyöngyi, LÉNÁRD Zsuzsa, KOZMA Veronika, GÖRÖG Dénes, GERLEI Zsuzsa, FEHÉRVÁRI Imre, NEMES Balázs, KÓBORI László

[INTRODUCTION - Bile duct complications remain a key problem of liver transplantation. Two main types are recognized: anastomotic and intrahepatic. In cases of anastomotic strictures good results can be achieved with surgery or minimally invasive therapy. Intrahepatic stenosis usually requires retransplantation. In this report the results of intrahepatic metallic stent placements are analyzed. PATIENTS AND METHODS - Since 1995, 20 patients with intrahepatic bile strictures were referred for percutaneous treatment. Of 34 percutaneous transhepatic cholangiography, 33 successful drainages were performed and 58 balloon dilatations were employed to overcome. In 13 patients, 20 metallic stents were implanted. One bleeding complication was successfully treated with selective embolization. RESULTS - The average follow up time was 35 months. 14 patients have no symptoms, 12 of them after metallic stent placements and 4 of them after retransplantation (2 patients had metallic stents at retransplantation). One patient has metallic stent and an external drain waiting for retransplantation. Three patients died after 7 retransplantations. Two patients died on the waiting list, one with and one without external drain. There were no deaths after successful metallic stent placement. CONCLUSION - After meticulous preparations metallic stent placement is safe and effective in intrahepatic biliary stenosis after liver transplantation. The patients can be stabilized till the retransplantation, or it can even be avoided.]