Δευτέρα 14 Φεβρουαρίου 2022

Aggressive paediatric camptodactyly: The evolution of a proposed treatment algorithm

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J Plast Reconstr Aesthet Surg. 2022 Jan 22:S1748-6815(22)00034-1. doi: 10.1016/j.bjps.2022.01.020. Online ahead of print.

ABSTRACT

INTRODUCTION: It is a long-established teaching to avoid operating on camptodactyly unless there is a failure of non-operative treatment, such as serial splinting and hand therapy, and there is an established proximal interphalangeal joint (PIPJ) contracture of 60°; a recent systematic review reflects this continuing approach, with some papers advocating intervention with a lesser degree of contracture.

AIM: To evaluate whether early flexor digitorum superficialis (FDS) release, followed by gentle passive manipulation (GPM), will correct severe 'congenital' camptodactyly, if undertaken at an earlier age than usual, thus avoiding the more aggressive surgical approach required in the established adolescent cases.

METHOD: The surgical technique and treatment algorithm are described. A multi-centre case series is presented; data analysis included patient demographics, syndromic association, side/digit affected, ages at onset, progression, referral and at surgery, operation details, pre- and post-operative contracture and range of motion.

RESULTS: There were 12 patients (3 males, 9 females) who underwent 15 operations for 24 involved digits. Patients had surgery by 3 months (median) post-referral, and there was a significant improvement in median (range) PIPJ contracture (90°(30°-90°) vs. 0°(0°-45°); p<0.001) and range of motion (0°(0°-60°) vs. 90°(50°-95°); p<0.001), at a median post-operative follow-up of 2.5 years. According to the Siegert grade, 87.5% of digits had excellent/good post-operative outcomes and 12.5% had fair outcomes.

CONCLUSION: This paper specifically addresses the problem of aggressive and progressive camptodactyly in the young child. By this, we mean patients who have failed non-operative treatment and have PIPJ contractures ≥60°, and those whose contractures have increased by 30° within 1 year. All cases responded to early FDS release and GPM, hence correcting the PIPJ contracture. However, cases with multiple digital involvement, whether syndromic or not, and failed previous surgery or the older child, required additional procedures to restore a dynamic dorsal apparatus and active extension.

PMID:35153164 | DOI:10.1016/j.bjps.2022.01.020

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Treatment of Graves' Disease Associated With Severe Neutropenia

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Cureus. 2022 Jan 7;14(1):e21014. doi: 10.7759/cureus.21014. eCollection 2022 Jan.

ABSTRACT

Severe neutropenia in newly diagnosed hyperthyroidism is a diagnostic and therapeutic dilemma since antithyroid drugs (ATDs) cannot be started if the absolute neutrophil count (ANC) is <1 x 109/L. We report the case of a patient followed for hyperthyroidism associated with severe neutropenia treated with dexamethasone and ATD. The patient was 51 years old and was hospitalized fo r hyperthyroidism with a thyroid stimulating hormone (TSH) level <0.005 (0.4-4) mUI/L, T4 at 415 (9.3-17.1) ng/L and T3 at 148 (2-4.4) pg/mL on Graves' disease (GD) confirmed by the TSH receptor antibodies at 38 IU/mL and scintigraphy, associated with neutropenia, with ANC at 0.4 x 109/L. He was put on prednisolone 60 mg/day and propranolol 60 mg/day for three weeks without improvement. Faced with the association of hyperthyroidism and severe neutropenia, we could not start the ATD for fear of agranulocytosis; we put the patient on propranolol 60 mg and dexamethasone 6 mg with progressive degression resulting in a spectacular increase of ANC from 0.4 x 109/L to 7.1 x 109/L, which allowed us to start the ATD (carbimazole) at a dose of 30 mg, and then 50 mg, with monitoring of ANC and transaminases every 48 hours. Euthyroidism was achieved after 15 days. A curative treatment with radioactive iodine ablation was administered. Our patient did not resp ond to prednisolone but responded dramatically to dexamethasone; this leads us to consider using dexamethasone for the rapid preparation for radical treatment of patients with GD.

PMID:35154986 | PMC:PMC8818315 | DOI:10.7759/cureus.21014

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Prevalence of abnormal vestibular responses in children with sensorineural hearing loss

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Eur Arch Otorhinolaryngol. 2022 Feb 14. doi: 10.1007/s00405-021-07241-2. Online ahead of print.

ABSTRACT

PURPOSE: Dysfunction of the vestibular organs has a great deal of influence on children's balance. Children with sensorineural hearing loss (SNHL) may often have accompanying abnormal vestibular responses. Video head impulse test (vHIT) combined with a test battery of cervical and ocular vestibular evoked myogenic potentials (cVEMPs, oVEMPs) make it possible to determine the prevalence of abnormal vestibular response in any of the five paired vestibular organs amongst children with SNHL.

METHOD: Prospective cross-sectional study including children aged 3-17 years with uni- or bilateral SNHL (bone-conduction (BC) pure tone average (PTA4) above 20 dB). Assessments included vHIT, cVEMP, and oVEMP. Descriptive data were analyzed. T-tests were performed to detect any correlation between the degree of SNHL and abnormal ve stibular responses.

RESULTS: 42 children (27 males), a total of 63 ears with SNHL, mean BC PTA4 SNHL of 42.3 dB (32.0; 58.4 IR) were included. 28.6% had at least one ear with abnormal vestibular response. 97.6% (41/42) completed one and 75.0% (27/36) completed all vestibular examinations. Ears with two pathological examinations had significantly more severe SNHL than ears with one pathological or normal vestibular examinations (p = 0.008, p = 0.005).

CONCLUSION: All test methods used with this study were easy, fast, and comfortable for children (as young as 3 years of age) to undergo. Even a moderate SNHL might be associated with abnormal vestibular response. Structural damage to the organs is the most likely cause of vestibular deficit. Consensus on criteria defining pathological examinations with both vHIT and VEMPs are required for more accurate comparison with previous studies.

PMID:35156132 | DOI:10.1007/s00405-021-07241-2

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Commentary on can sex improve nasal function?-An exploration of the link between sex and nasal function

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Ear Nose Throat J. 2022 Feb 14:1455613221077601. doi: 10.1177/01455613221077601. Online ahead of print.

NO ABSTRACT

PMID:35152782 | DOI:10.1177/01455613221077601

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Bow Hunter's syndrome with a dominant aberrantly coursing right vertebral artery (VA), presenting with persistent dizziness and syncope

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Bow hunter syndrome (BHS) is an uncommon cause of vertebrobasilar insufficiency that results from occlusion or injury to the vertebral artery (VA) during neck rotation. The cause is often a bony abnormality that may compress the VA compromising distal flow or lead to vessel wall injury resulting in thromboembolism.

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Ann Otol Rhinol Laryngol. 2022 Feb 12:34894221077477. doi: 10.1177/00034894221077477. Online ahead of print.

ABSTRACT

OBJECTIVES AND METHODS: We report a unique case of Bow Hunter's syndrome with a dominant aberrantly coursing right vertebral artery (VA), presenting with persistent dizziness and syncope despite previous decompressive surgery at vertebral levels C5-C6.

RESULTS: Re-evaluation with computed tomography-scan during provocation of dizziness by neck rotation revealed compression of the right VA at level C6 from against the ipsilateral posterior border and superior cornu of the thyroid cartilage. Laryngoplasty resulted in complete resolution of symptoms.

CONCLUSION: This extremely rare cause of Bow's Hunter's syndrome should be considered, especially in refractory cases after neurosurgical decompression, and surgical management is straightforward and successful.

PMID:35152775 | DOI:10.1177/00034894221077477

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Sclerotherapy for Hereditary Hemorrhagic Telangiectasia-Related Epistaxis: A Systematic Review

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Ann Otol Rhinol Laryngol. 2022 Feb 12:34894221078075. doi: 10.1177/00034894221078075. Online ahead of print.

ABSTRACT

OBJECTIVES: Hereditary hemorrhagic telangiectasia (HHT) is a common inherited condition characterized by mucosal telangiectasias, recurrent epistaxis, and arteriovenous malformations. HHT results in detriment to quality of life. Morbidity and mortality result from severe anemia. Conventional interventions for HHT-related epistaxis include nasal packing, diath ermy, lasers, coblation, microdebridement, bevacizumab (topical and systemic), as well as septodermoplasty and nasal closure. Sclerotherapy has been recently described in the literature as a novel approach to HHT-related epistaxis. We hypothesize that sclerotherapy is an effective treatment for HHT-related epistaxis and improves upon the current standard of care for this disease.

METHODS: A systematic review was conducted to study sclerotherapy for treating HHT-related epistaxis. Ovid MEDLINE, Ovid EMBASE, Scopus, and Web of Science were searched. Articles were evaluated and excluded according to PRISMA guidelines and reviewed by 2 authors. Reported variables included number of injections, months of follow up, changes in Epistaxis Severity Score, previous treatments used to control epistaxis, and post-injection side effects.

RESULTS: Seven studies with a total of 196 patients met inclusion criteria. Three studies reported significant improvement as measured by the Epista xis Severity Score scale. One reported improvement through subjective patient surveys and others used the Bergler-Sadick scale to measure frequency and intensity of epistaxis. All studies reported improvement in HHT-related epistaxis. The lack of uniform reporting measures however precluded formal meta-analysis.

CONCLUSIONS: Based on limited data, sclerotherapy appears to be effective for treating HHT-related epistaxis and offers promise for treating this recalcitrant condition. However, larger, prospective, multi-centered studies using universally validated instruments for epistaxis are needed to definitively evaluate outcomes from sclerotherapy.

PMID:35152768 | DOI:10.1177/00034894221078075

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Thyroid Cartilage Compression Causing Bow Hunter's Syndrome

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Ann Otol Rhinol Laryngol. 2022 Feb 12:34894221077477. doi: 10.1177/00034894221077477. Online ahead of print.

ABSTRACT

OBJECTIVES AND METHODS: We report a unique case of Bow Hunter's syndrome with a dominant aberrantly coursing right vertebral artery (VA), presenting with persistent dizziness and syncope despite previous decompressive surgery at vertebral levels C5-C6.

RESULTS: Re-evaluation with computed tomography-scan during provocation of dizziness by neck rotation revealed compression of the right VA at level C6 from against the ipsilateral posterior border and superior cornu of the thyroid cartilage. Laryngoplasty resulted in complete resolution of symptoms.

CONCLUSION: This extremely rare cause of Bow's Hunter's syndrome should be considered, especially in refractory cases after neurosurgical decompression, and surgical management is straightforward and successful.

PMID:35152775 | DOI:10.1177/00034894221077477

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Low MICA Gene Expression Confers an Increased Risk of Graves' Disease: A Mendelian Randomization Study

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Thyroid, Volume 32, Issue 2, Page 188-195, February 2022.
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Serratus Anterior-Rib Composite Flap as a Novel Approach for Tracheal Reconstruction

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Ann Otol Rhinol Laryngol. 2022 Feb 13:34894211067608. doi: 10.1177/00034894211067608. Online ahead of print.

ABSTRACT

OBJECTIVE: To report a novel case of tracheal reconstruction using a serratus anterior-rib composite flap.

METHODS: Case report and literature review.

RESULTS: A 46-year-old male with a 4 cm anterior tracheal wall defect underwent reconstruction with a serratus anterior-rib composite flap. The patient experienced excellent results regarding phonati on, swallowing, and cosmesis.

CONCLUSION: The serratus anterior-rib composite flap appears to be a suitable candidate for tracheal reconstruction and merits further analysis in this context. The flap's intrinsic incorporation of a perfused rib segment allows for reliable reconstruction of the neotrachea and maintenance of proximal dynamic airway support.

PMID:35156399 | DOI:10.1177/00034894211067608

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