An unusual cause of electrical storm in a young woman.
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ABSTRACT:
SUBMITTER: Resende CX
PROVIDER: S-EPMC8717761 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Introductionand importance: Zinner syndrome is a rare congenital malformation of the seminal vesicles and the homolateral upper urinary tract. While the majority of patients remain asymptomatic and are discovered incidentally, others present symptoms such as micturition or ejaculatory difficulties, or pain. We report a case of Zinner syndrome in a 32-year-old patient with painful ejaculation and discuss the diagnosis and treatment difficulties.Case presentationA 32-year-old married patient was consulted for pelvic pain associated with painful ejaculation that had been evolving for six months. The clinical examination was normal. Routine laboratory studies of blood and urine were normal. The patient was explored by ultrasound which showed the absence of the right kidney and the presence of a 7 cm right lateral prostatic cystic mass. On MRI, the right kidney was not visualized. Multiple cysts were seen in the right seminal vesicle. Surgical excision of the cyst by laparotomy was performed. The patient had an uneventful recovery and was discharged on the third postoperative day.Clinical discussionCongenital malformations of the seminal vesicles are often associated with those of the ipsilateral upper urinary tract, as the ureteral and seminal vesicle buds originate from the mesonephric duct. The syndrome often occurs in the second and third decades of life, especially after the onset of sexual activity. The most common symptoms were dysuria, perineal pain, epididymitis, and painful ejaculation. Diagnostic modalities include ultrasound, MRI, and cystoscopy. In patients with symptoms, the therapeutic management of the cyst includes ultrasound-guided aspiration and laparoscopic or open surgical excision.ConclusionSeminal vesicle cysts associated with homolateral renal agenesis or hypoplasia are a rare urologic anomaly. The treatment depends on the patient's symptoms. surgical excision of seminal vesicle cysts may be needed for large cysts causing obstructive symptoms.
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Project description:BackgroundWhile transient loss of consciousness is a frequent presenting symptom, differential diagnosis between syncope and epilepsy can be challenging. Misdiagnosis of epilepsy leads to important psychosocial consequences and eliminates the opportunity to treat patient's true condition.Case summaryA 39-year-old woman presenting with recurrent seizures since her childhood was referred to neurological consultation. Electroencephalograms (EEGs) and magnetic resonance imaging previously performed were normal. A sleep-deprived video-EEG was performed and highlighted after 12 h of sleep deprivation a progressive dropping of the heart rate followed by a complete heart block without ventricular escape rhythm and asystole for about 30 s. Her EEG recording later showed diffuse slow waves traducing a global cerebral dysfunction and suffering. The diagnosis of vaso-vagal syncope with predominant cardioinhibitory response was made and a dual-chamber pacemaker with rate-drop response algorithm was implanted. After a 2 years of follow-up, the patient remained free of syncope.DiscussionPatients presenting with loss of consciousness and convulsion are often diagnosed with epilepsy despite normal EEGs. In patients presenting with recurrent seizures with unclear diagnosis of epilepsy or in a situation of drug-resistant epilepsy, syncope diagnosis should always be considered and a risk stratification is necessary. The benefit of pacemaker implantation in patients with recurrent vaso-vagal syncope is still very controversial. Only patients presenting with spontaneous asystole should be considered for pacemaker implantation in case of recurrent vaso-vagal syncope.
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Project description:BackgroundThe study sought to assess the prognostic impact of chronic kidney disease (CKD) in patients with electrical storm (ES). ES represents a life-threatening heart rhythm disorder. In particular, CKD patients are at risk of suffering from ES. However, data regarding the prognostic impact of CKD on long-term mortality in ES patients is limited.MethodsAll consecutive ES patients with an implantable cardioverter-defibrillator (ICD) were included retrospectively from 2002 to 2016. Patients with CKD (MDRD-GFR < 60 ml/min/1.73 m2) were compared to patients without CKD. The primary endpoint was all-cause mortality at 3 years. Secondary endpoints were in-hospital mortality, cardiac rehospitalization, recurrences of electrical storm (ES-R), and major adverse cardiac events (MACE) at 3 years.ResultsA total of 70 consecutive ES patients were included. CKD was present in 43% of ES patients with a median glomerular filtration rate (GFR) of 43.3 ml/min/1.73 m2. CKD was associated with increased all-cause mortality at 3 years (63% vs. 20%; p = 0.001; HR = 4.293; 95% CI 1.874-9.836; p = 0.001) and MACE (57% vs. 30%; p = 0.025; HR = 3.597; 95% CI 1.679-7.708; p = 0.001). In contrast, first cardiac rehospitalization (43% vs. 45%; log-rank p = 0.889) and ES-R (30% vs. 20%; log-rank p = 0.334) were not affected by CKD. Even after multivariable adjustment, CKD was still associated with increased long-term mortality (HR = 2.397; 95% CI 1.012-5.697; p = 0.047), as well as with the secondary endpoint MACE (HR = 2.520; 95% CI 1.109-5.727; p = 0.027).ConclusionsIn patients with ES, the presence of CKD was associated with increased long-term mortality and MACE.
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