Continuous Arrhythmia Monitoring in Pediatric and Adult Patients With Left Ventricular Noncompaction.

Patients with left ventricular noncompaction (LVNC) are at risk of clinically significant arrhythmias and sudden death. We evaluated whether implantable loop recorders could detect significant arrhythmias that might be missed in these patients during annual Holter monitoring. Selected pediatric and adult patients with LVNC who consented to implantable loop recorder placement were monitored for 3 years (study duration, 10 April 2014-9 December 2019). Fourteen subjects were included (age range, 6.5-36.4 yr; 8 males). Of 13 patients who remained after one device extrusion, one underwent implantable cardioverter-defibrillator placement. Four patients (31%) had significant arrhythmias: atrial tachycardia (n=2), nonsustained ventricular tachycardia (n=1), and atrial fibrillation (n=1). All 4 events were clinically asymptomatic and not associated with left ventricular ejection fraction. In addition, a high frequency of benign arrhythmic patterns was detected. Implantable loop recorders enable continuous, long-term detection of important subclinical arrhythmias in selected patients who have LVNC. These devices may prove to be most valuable in patients who have LVNC and moderate or greater ventricular dysfunction.

Glomangiopericytoma of the inferior nasal turbinate: A case report.

INTRODUCTION: Glomangiopericytoma is defined as a sinonasal tumor with perivascular myoid phenotype, which was first described in 1942 by Stout and Murray as a soft tissue tumor with characteristic proliferation including branching vessels and small vessel perivascular hyalinization. The tumor accounts for less than 0.5 % of all sinonasal neoplasms. The World Health Organization (WHO) classified this tumor as glomangiopericytoma in 2005. CASE REPORT: A 47-year-women presented with two years history of permanent left nasal obstruction and frequent epistaxis. Rhinoscopy revealed a friable grayish pink polypoidal mass, fully occupying the left anterior naris. Computed tomography showed a lesion involving the left nasal cavity, with a soft tissue density (70 UH) measuring 50 × 16 mm, widely infiltrative. Endoscopic surgery was performed to remove the mass, considering the size, limited expansion and the accessible location of the tumor. The immunohistochemistry examination showed positive staining b-catenin tumor cells which confirmed the diagnostic of glomangiopericytoma. After a 2 years follow-up, the patient showed no signs of recurrence. CONCLUSION: Glomangiopericytomas generally arises in the nasal cavity and may extend into the paranasal sinuses. It is categorized as a borderline low malignancy tumor by the WHO classification. Complete transnasal endoscopic excision is the optimal treatment.

Atypical first branchial cleft fistula: A case report.

INTRODUCTION: First branchial cleft anomalies (FBCA) are rare. They have an estimated incidence of 1 in 100,000. Type I are those that embryologically duplicate the membrane (cutaneous) external auditory canal. The aim of this case is to describe an unusual path of a type II first branchial cleft fistula tract in a 3 years old child and its surgical management in the academic hospital of Casablanca. CASE PRESENTATION: This case is about a 3 year old girl who presented to the Ear Nose Throat (ENT) consultation for recurrent right lateral cervical infection. Clinical examination found an unsightly scar attached to an orifice giving pus located near the right mandibular angle suggesting type II first branchial cleft anomaly. Surgical excision was performed under general anesthesia by the superficial parotidectomy approach, the facial nerve was identified and preserved. The fistula cord was dissected and followed, it went under the facial nerve and the parorid gland to end under the digastric muscle where we tied it up. The postoperative check-up did not show any complications. The follow-up period was 12 months; the clinical examination did not find any sign of recurrence. CONCLUSION: First branchial cleft fistula are rare and can be in form of cyst or fistula. Its management is surgical excision keeping the tract cyst of the fistula intact with facial nerve preservation.

Quality of life assessment in patients treated with nasal corticotherapy for allergic rhinitis: A cohort study.

INTRODUCTION: Allergic rhinitis (AR) is a chronic nasal pathology induced by an inflammation of the mucous membranes due to a dependent IgE reaction following allergenic exposure. The main symptoms are rhinorrhea, nasal itching, nasal obstruction and sneezing bursts. It highly affects the patient quality of life (QoL) in many levels making it a public health issue.The aim of this study is to assess the QoL of patients with AR as well as the level of its improvement after nasal corticotherapy. MATERIALS AND METHODS: From June 2019 to February 2020, a prospective study was carried out based on the use of the validated Arabic version of the RQLQ (Rhinoconjunctivitis Quality of Life Questionnaire) on a cohort of AR patients to measure their QoL before and after three months of treatment by "Budesonide". RESULTS: A total of 70 patients participated in the study with an average age of 39.54 years with a sex ratio of 0.60. Their total RQLQ score was up to 4.28, improved to 2.35 after treatment. Also, a statistically significant improvement in sub-scores was also observed (p < 0.001): activities (from 4.43 to 2.29), nasal symptoms (from 5.00 to 2.80), eye symptoms (from 3.38 to 1.80), practical problems (from 4.29 to 2.18), general problems (from 4.63 to 2.78) and emotional state (from 4.28 to 2.43). CONCLUSION: The RQLQ is a reliable tool to evaluate the QoL in AR patients, stating the negative influence of AR on patients daily life and the effectiveness of nasal corticosteroid treatment.

Non-invasive Risk Stratification in Pediatric Ventricular Pre-excitation.

Children with ventricular pre-excitation are at risk for sudden death. This retrospective pediatric study identified patients > 8 years of age who had undergone electrophysiology study (EPS). Our primary objective was to determine the performance characteristics of non-invasive risk stratification. Subjects were separated into two groups. Group 1 was asymptomatic or had non-specific symptoms (palpitations, chest pain, and light headedness) without documented supraventricular tachycardia (SVT). Group 2 had syncope, documented SVT, or a life-threatening event. As a secondary aim, we tested whether patients with severe symptoms had a shorter time from the date of diagnosis to the date of invasive risk stratification. Among 93 patients with an average age of 14.2 years, 25 patients had documented SVT, 6 had syncope, and 1 had a life-threatening event. The sensitivity of non-invasive risk stratification was 7%. The specificity was 91%. The positive predictive valve was 14% and the negative predictive value was 84%. Even patients with severe symptoms commonly underwent non-invasive risk stratification prior to EPS, albeit at a lower rate (Group 1, 98%; Group 2 84%, p = 0.02). The median time to EPS was 4.2 months (Group 1) and 4.5 months (Group 2, p = 0.63). Non-invasive risk stratification was a poor predictor of invasive risk stratification. Cardiologists should counsel families about the limitations of non-invasive risk stratification and consider starting with invasive risk stratification and possible ablation. Counterintuitively, severe symptoms were not associated with a shorter time to electrophysiology study.

Extensive epithelioid hemangioendothelioma of the maxillary sinus: A case report.

BACKGROUND: Epitheliod hemandioendothelioma (EHE) is a rare vascular tumor which was first reported by Weiss and Enzinger in 1982. It can be seen in many locations whose paranasal sinus is extremly rare. Its main treatment is surgery. To our best knowledge, no report of EHE treated by radiation therapy and chemotherapy has been described. CASE SUMMARY: A 18 years old man presented to our hospital with a 3 months history of right intermittent epistaxis, permnanent nasal obstruction and right hearing loss. The physical exam found a right exophtalmia, swelling deformatted right hemifacia and a bulky whitish tumor filling the right nasal cavity. The magnetic resonance imaging (MRI) showed an extensive hyperascularized nasopharyngeal process filling the right nasal cavity whose histopathological exam revealed an epitheloid hemangioendothelioma. Due to intracranial extension, surgery was contraindicated. The patient received 65 Gy of radiation therapy and chemotheray (cisplatin). After 18 months of follow up, the exopthalmous regressed and the MRI showed a 50% regression of the tumour size. CONCLUSION: Extensive EHE can be treated by radiation therapy and chemotherapy when surgery can't be perfomed.

Risk factors for complications in the implantation of epicardial pacemakers in neonates and infants.

BACKGROUND: Complications related to epicardial pacemakers in infants have been reported, though limited data are available on their incidence and associated risk factors. OBJECTIVE: The hypothesis of the study is that younger, smaller patients and larger devices would be associated with complications in neonates and infants. METHODS: This is a retrospective study of all patients at a single center receiving an epicardial pacemaker at ≤12 months of age (1996-2015). Patient and device characteristics were obtained. Characteristics of patients with and without complications were compared. RESULTS: There were 86 patients with a median age of 73 days (interquartile range 13-166 days), of whom 12 (14%) had a complication. Eight (9%) needed surgical intervention, of whom 5 (6%) required explantation. Younger age (9 days vs 89 days; P = .01) and lower weight (2.91 kg vs 4.44 kg; P = .004) at implantation were associated with complications. Device characteristics were not statistically different. Patients ≤3 kg in weight and/or <5 days of age had an odds ratio of 18.1 (3.6-91.2; P < .001) for developing a complication with a negative predictive value (NPV) of 97%. Regardless of weight, patients aged >21 days were found to be at lower risk with an NPV of 96%; and regardless of age, patients weighing >4 kg had an NPV of 98%. CONCLUSION: Young age and low weight at the time of implantation are risk factors for complications, while device characteristics appear to play a minor role. Reserving pacemaker implantation for patients >3 kg in weight and 5 days of age may predict patients at low risk of developing complications.