Muscle Anomalies Research Articles

Dark papillary muscles sign is a new prognostic indicator in patients with dilated cardiomyopathy: A multi-center study

ObjectivesThe prognostic value of left ventricular (LV) papillary muscle anomalies in dilated cardiomyopathy (DCM) patients is unclear. The objective of this study was to evaluate the prognostic significance of LV papillary muscle anomalies in DCM patients using cardiac magnetic resonance (CMR). Methods369 DCM patients who underwent CMR at two Chinese medical facilities from January 2019 to June 2023 were retrospectively and consecutively included in total. The various features of the LV papillary muscles were taken into consideration: thickness, attachment, supernumerary papillary muscles, angles, and signal intensity. The end-systolic signal hypointensity of both papillary muscles in early post-contrast cine CMR images was identified as Dark-Paps. Major adverse cardiac events (MACEs) were assessed, and all patients were followed up. Results119 patients (32.2 %) had Dark-Paps and 141 patients (38.2 %) experienced MACE during a median follow-up of 22 months. According to Kaplan-Meier curve analysis, patients who had Dark-Paps had a lower survival rate free from MACE (log-rank, p < 0.001). Dark-Paps maintained an independent predictor of MACE in a multivariate model that included left ventricular ejection fraction (LVEF) and late gadolinium enhancement (LGE) extent (HR: 3.49; p < 0.001). Furthermore, adding Dark-Paps to the multivariate model greatly enhanced the prognostic role of endpoint events (C-statistic improvement: 0.652–0.777, Delong test: p < 0.001). ConclusionDark-Paps is a potent independent indicator of major adverse cardiac events in dilated cardiomyopathy patients. In addition, Dark-Paps can provide additional prognostic value over the multivariable baseline clinical model.

Unusual anatomical variants of infrahyoid muscles - case report.

Anatomical anomalies of neck muscles are rarely observed and usually comprise variations of digastric and omohyoid muscles. Neck muscles' abnormalities might be correlated with embryological development and are observed in individuals with aneuploidies such as Edward's syndrome (18-trisomy) or Down syndrome (21-trisomy). Some infrahyoid muscles are important landmarks during surgery, therefore their anatomical variations of these muscles are related to higher risk of surgical complications. Herein, we present a rare case of infrahyoid muscles anomalies found during routine dissection of male cadaver. Redundant muscle bellies of sternohyoid muscle (sternohyoid azygos muscle), presence of levator glandulae thyroideae and also one hypoplastic superior belly of the omohyoid muscle were observed. Presence of muscle fibers within found structures was confirmed using Masson's trichrome staining method.

A RARE CASE OF INTERVENTRICULAR SEPTUM DUPLICATION

Abstract Case Report A 62–year–old man, history of hypertension. In 2015, there was suspicion of non–compaction cardiomyopathy, and he underwent cardiac MRI at another facility (report not available). The patient reported palpitations and dyspnea with exertion, leading to a follow–up visit. EKG showed sinus rhythm 90/min, nonspecific intraventricular conduction delay and isolated monomorphic ventricular ectopic beats. Echo revealed a mildly dilated left ventricle (end–diastolic volume 88 ml/m), systolic function at the lower limits of normal (EF 53%), and prominent muscular structures near the posterior interventricular septum (SIV), likely related to anomalies of the papillary muscles (prominent and possibly duplicated posterior–medial papillary muscle). There was apical trabecular protrusion, which did not meet the criteria for non–compaction (NC/C ratio in systole approximately 1), with no other significant findings. The cardiac MRI, performed to assess dilated cardiomyopathy, revealed a conspicuous structural abnormality in the septo–papillary region, located medio–apically and paraseptal–endocardial in the left ventricle. This anomaly lacked evident chordae tendineae with the mitral valve leaflets and exhibited synchronous systo–diastolic motion with the left ventricular wall, suggesting septal duplication. The septal thickness was slightly reduced, particularly in the medio–apical region. Additionally, there was hypertrabeculation of the posterior and lateral walls of the left ventricle, exceeding the threshold for non–compaction (NC/C ratio in end–diastole: 2.6 – normal range: &amp;lt;2.3). Left ventricular mass was normal (mass: 173.52 g; mass/body surface area (BSA): 86.58 g/m). Basal septum, medio–apical septum and medio–apical lateral wallshowed hypokinetic contractility. The left ventricle was dilated (end–diastolic diameter: 55.0 mm; end–systolic diameter: 45.3 mm – end–diastolic volume: 230 ml; stroke volume/BSA: 57.6 ml/m² – EF: 51%). Areas of late gadolinium enhancement (LGE) were observed in the anterior medio–basal septum and at the site of the described structural muscular anomaly, both displaying an intramural non–ischemic pattern. Conclusions The described findings suggest a picture of dilated cardiomyopathy and an associated structural septo–papillary anomaly of uncertain diagnostic interpretation (septum duplication with associated non–compacted myocardium? duplicated posterior–medial papillary muscle with associated non–compacted myocardium?).

A rare variation of omohyoid and its embryological correlations- A case report

The omohyoid muscle is important in radical neck dissection, as it is a landmark for this operation. Because it divides the anterior and posterior cervical triangles into smaller triangles and its particular relationship to the large cervical vessels, the presence of an anatomical variation of the omohyoid muscle is important. Either belly may be absent or double; and the inferior belly may be attached directly to the clavicle and the superior is sometimes fused with sternohyoid. Variations in the omohyoid muscle might be linked to differences in embryological development, which could influence the muscle’s final form and function. Anderson (Anderson, 1881) theorized that the superior belly of the omohyoid muscle is a true infrahyoid muscle, whereas the inferior belly most likely shares a common embryology with the subclavius muscle. As omohyoid mucle is used to achieve the reconstruction of the laryngeal muscles and bowed vocal folds, the knowledge of the possible anomalies of the omohyoid muscle is important.In the present study, we have found a case with two inferior bellies &amp; aberrant course of superior belly of omohyoid on right side of 40 years female cadaver embalmed with formalin during routine dissection at Department of Anatomy AIIMS Gorakhpur.Omohyoid muscle is closely related to large vessels of neck &amp; brachial plexus, so variations in this muscle are clinically important.

Anomaly originated flexor digitorum superficialis muscle of the small finger: A case report.

We present a clinical case of flexor digitorum superficialis (FDS) muscle belly of the small finger originating from the palm of a patient undergoing carpal tunnel surgery with a literature review. A 28-year-old right-handed woman visited our hospital with a chief complaint of a continuous tingling sensation and weakness in the right hand, which began after the volar side of her wrist was crushed by a machine during work 2 weeks prior. The patient complained of a continuous tingling sensation in the thumb, index, and middle fingers. The patient had a positive result on Tinel test of the median nerve of the wrist. As electromyography and nerve conduction velocities showed signs of severe injury in the right median nerve, exploration and carpal tunnel release were planned. Carpal tunnel release was performed under regional anesthesia using the classical open approach. The median nerve in the distal forearm and distal portion of the flexor retinaculum appeared to be narrowed and compressed. An anomalous muscle originating from the flexor retinaculum is also observed. The FDS muscle of the small finger was excised at the flexor retinaculum and musculotendinous junction and sutured to the flexor digitorum profundus tendon. At the 37-month follow-up, the patient did not experience any tingling sensation or weakness. She showed excellent range of motion of the right small finger. The grip strength was 20 kg on both the right and left sides. Quick disabilities of the arm, shoulder, and hand score was 2.3. Asymptomatic small finger FDS muscle anomalies can occur, as demonstrated in this case study. Thus, physicians should familiarize themselves with small finger FDS muscle anomalies during interactions with patients to facilitate future treatments of patient complaints related to the hand, as well as wrist laceration or trauma requiring hand exploration.

Magnetic Resonance Imaging of the Velopharynx: Clinical Findings in Patients with Velopharyngeal Insufficiency.

Magnetic resonance imaging (MRI) is the only imaging modality capable of directly visualizing the levator veli palatini (LVP) muscles: the primary muscles responsible for velopharyngeal closure during speech. MRI has been used to describe normal anatomy and physiology of the velopharynx in research studies, but there is limited experience with use of MRI in the clinical evaluation of patients with velopharyngeal insufficiency (VPI). MRI was used to evaluate the velopharyngeal mechanism in patients presenting for VPI management. The MRI followed a fully awake, nonsedated protocol with phonation sequences. Quantitative and qualitative measures of the velopharynx were obtained and compared with age- and sex-matched individuals with normal speech resonance. MRI was completed successfully in 113 of 118 patients (96%). Compared with controls, patients with VPI after cleft palate repair had a shorter velum (P < 0.001), higher incidence of LVP discontinuity (P < 0.001), and shorter effective velar length (P < 0.001). Among patients with persistent VPI after pharyngeal flap placement, findings included a pharyngeal flap base located inferior to the palatal plane [11 of 15 (73%)], shorter velum (P < 0.001), and higher incidence of LVP discontinuity (P = 0.014). Patients presenting with noncleft VPI had a shorter (P = 0.004) and thinner velum (P < 0.001) and higher incidence of LVP discontinuity (P = 0.014). MRI provides direct evidence of LVP muscle anomalies and quantitative evaluation of both velar length and velopharyngeal gap. This information is unavailable with traditional VPI imaging tools, suggesting that MRI may be a useful tool for selecting surgical procedures to address patient-specific anatomic differences.

The crucial role of titin in fetal development: recurrent miscarriages and bone, heart and muscle anomalies characterise the severe end of titinopathies spectrum

BackgroundTitin truncating variants (TTNtvs) have been associated with several forms of myopathies and/or cardiomyopathies. In homozygosity or in compound heterozygosity, they cause a wide spectrum of recessive phenotypes with a...

A phenotypic rescue approach identifies lineage regionalization defects in a mouse model of DiGeorge syndrome.

ABSTRACTTBX1 is a key regulator of pharyngeal apparatus (PhAp) development. Vitamin B12 (vB12) treatment partially rescues aortic arch patterning defects of Tbx1+/− embryos. Here, we show that it also improves cardiac outflow tract septation and branchiomeric muscle anomalies of Tbx1 hypomorphic mutants. At the molecular level, in vivo vB12 treatment enabled us to identify genes that were dysregulated by Tbx1 haploinsufficiency and rescued by treatment. We found that SNAI2, also known as SLUG, encoded by the rescued gene Snai2, identified a population of mesodermal cells that was partially overlapping with, but distinct from, ISL1+ and TBX1+ populations. In addition, SNAI2+ cells were mislocalized and had a greater tendency to aggregate in Tbx1+/− and Tbx1−/− embryos, and vB12 treatment restored cellular distribution. Adjacent neural crest-derived mesenchymal cells, which do not express TBX1, were also affected, showing enhanced segregation from cardiopharyngeal mesodermal cells. We propose that TBX1 regulates cell distribution in the core mesoderm and the arrangement of multiple lineages within the PhAp.

EP218: A novel FGFR2 variant in a family with a spectrum of anterior segment anomalies

Pathogenic variants in fibroblast growth factor receptor 2 (FGFR2) have been associated with a wide spectrum of craniofacial anomalies, ranging from severe multiple craniosynostoses to unilateral coronal craniosynostosis. The degree of craniosynostosis and the presence or absence of additional clinical features involving the hands and feet may establish diagnoses of either Crouzon, Pfieffer, or Apert syndromes. However, phenotypic overlap is common. Ocular anomalies in FGRF2-related craniosynostosis including exophthalmos, strabismus due to extraocular muscle anomalies or orbit malformation, and anisometropia have been well described.

Management of resistant congenital clubfeet by the new operative procedure (Hussain's procedure): An experience of a fellowship program

Background and Objectives: Pakistan Society for Rehabilitation of the Disabled Orthopedic Hospital is renowned for the correction of orthopedic deformities including foot and ankle deformities. Consultant orthopedic surgeon Afzal Hussain, pioneers the treatment of orthopedic deformities and has developed a new operative technique for congenital clubfoot. This research was planned to report the success of Hussain's Procedure in detecting and managing the anomalous structures.&#x0D; Material and Methods: This research was mixed retrospective and prospective research carried out during fellowship of first author in which, operative procedure of the resistant congenital clubfoot associated with anomalous structures viz. anomalous muscles and tarsal coalitions, were performed with the new operative procedure by Dr. Hussain, were assisted by the first author and a minimum of 5 years of follow up of the cases was done by the senior author (Afzal Hussain). Cumming’s modification of Laavag and Ponseti score was applied for the calculation of the results at the follow-ups.&#x0D; Results: During the fellowship, 10 cases of clubfeet with muscle anomalies were managed. Similarly 7 clubfeet with tarsal coalitions were managed. There was full correction of the clubfoot deformity. The cases were followed up by the senior author (Afzal Hussain) for at least 5 years. During the minimum of 5 years followup post completion of treatment, Cumming’s modification of Laavag and Ponseti scores of the operated clubfeet were found to be excellent.&#x0D; Conclusion: The resistance to management of congenital clubfoot by casting may be because of anomalous structures. New operative procedure by Consultant Afzal Hussain names as Hussain's Procedure was helpful in detecting and managing the anomalous structures.

Pathophysiological Effects of Overactive STIM1 on Murine Muscle Function and Structure.

Store-operated Ca2+ entry (SOCE) is a ubiquitous mechanism regulating extracellular Ca2+ entry to control a multitude of Ca2+-dependent signaling pathways and cellular processes. SOCE relies on the concerted activity of the reticular Ca2+ sensor STIM1 and the plasma membrane Ca2+ channel ORAI1, and dysfunctions of these key factors result in human pathologies. STIM1 and ORAI1 gain-of-function (GoF) mutations induce excessive Ca2+ influx through SOCE over-activation, and cause tubular aggregate myopathy (TAM) and Stormorken syndrome (STRMK), two overlapping disorders characterized by muscle weakness and additional multi-systemic signs affecting growth, platelets, spleen, skin, and intellectual abilities. In order to investigate the pathophysiological effect of overactive SOCE on muscle function and structure, we combined transcriptomics with morphological and functional studies on a TAM/STRMK mouse model. Muscles from Stim1R304W/+ mice displayed aberrant expression profiles of genes implicated in Ca2+ handling and excitation-contraction coupling (ECC), and in vivo investigations evidenced delayed muscle contraction and relaxation kinetics. We also identified signs of reticular stress and abnormal mitochondrial activity, and histological and respirometric analyses on muscle samples revealed enhanced myofiber degeneration associated with reduced mitochondrial respiration. Taken together, we uncovered a molecular disease signature and deciphered the pathomechanism underlying the functional and structural muscle anomalies characterizing TAM/STRMK.

ЗНАЧИМОСТЬ ЭЛЕКТРОНЕЙРОМИОГРАФИИ И СПИРАЛЬНОЙ КОМПЬЮТЕРНОЙ ТОМОГРАФИИ В ДИАГНОСТИКЕ И ХИРУРГИЧЕСКОМ ЛЕЧЕНИИ СИНДРОМА ШЕЙНОГО РЕБРА

The study aims to assess the significance of the results of electroneuromyography and spiral computed tomography in the diagnostics and surgical treatment of cervical rib syndrome. Material and methods. The analysis of examination and surgical treatment of 72 patients, aged 16 to 46 years, operated for cervical rib syndrome for the period 2013–2019 is carried out. Results. In patients of group I (n = 48), the following postoperative complications were found: brachioplexitis in 16.7% (n = 8) of cases, phrenic nerve injury in 4.1% (n = 2) of cases, hemothorax and pneumothorax in two (4.1%) patients. Patients of group II (n = 24) additionally underwent electroneuromyography and spiral computed tomography for selection of the optimal surgical approach, which made it possible to reduce the incidence of early postoperative complications. It was possible to eliminate anomalies of the scalene muscles in 58% (n = 14) of cases; fibrous cords compressing the neurovascular bundle in 42% (n = 10) of cases. Brachioplexitis was observed in 4.2% of cases, which is less than in group I. There were no other complications in group II, as well as deaths in both groups.

Development and evolution of a robotic surgical technique for the treatment of thoracic outlet syndrome

ObjectiveWe describe the development and evolution of a surgical technique that uses the robotic da Vinci Surgical System (Intuitive Surgical, Inc, Sunnyvale, Calif) for the transaxillary approach to repair the disabling thoracic outlet syndrome (TOS). We report our patient outcomes associated with the use of this robotic technique. MethodsWe present a retrospective review and analysis of data collected from a 16-year experience of a single surgeon using a robotic surgical system and technique for TOS surgery. From the initial design of an endoscope attached to a microvideo camera in 1982 to the adoption of the monorobotic arm with integrated voice in 1998, the main objective of the transaxillary approach has always been to improve visualization of congenital cervical anomalies of the scalene muscles. From February 2003 to December 2018, we performed 412 transaxillary decompression procedures using the robotic da Vinci Surgical System. The surgical procedure has been described in further detail and includes the following steps: (1) positioning of the patient into a lateral decubitus position and using a monoarm retractor; (2) creation of a mini-incision in the axillary area and creation and maintenance of the subpectoral anatomic working space; (3) placement of endoscopic ports and engagement of the robotic instrumentation; (4) dissection of extrapleural and intrapleural soft tissue; (5) creation of the “floater” first rib; (6) excision of the cervical bands and first rib; and (7) placement of thoracostomy tubes for drainage and closure of the incisions. ResultsNone of the patients died, and no patient experienced permanent neurovascular damage of the extremity. Of the 306 patients, 22 (5% of 441 operations) experienced complications. One patient developed postoperative scarring that required a redo operation with a robotic-assisted transaxillary approach. ConclusionsWith its three-dimensional visual magnification of the anatomic area, the endoscopic robotic-assisted transaxillary approach offers safe and effective management of disabling TOS symptoms. The endoscope facilitates observation of the cervical bands and the mechanism (pathogenesis) of the neurovascular compression that causes TOS, thereby allowing complete excision of the first rib, cervical bands, and scalene muscle. We sought to develop and perfect this robotic approach. The present study was not intended to be a comparative study to nonrobotic TOS surgery.

Magnitude, Trends, and Determinants of Iodized Salt Availability among Households in Ethiopia: Analysis of Ethiopian Demographic and Health Surveys (2000-2016).

Background:Iodine deficiency causes various health problems such as mental defects, goiter, reproductive damage, hypo and hyperthyroidism, stillbirth, abortion, congenital abnormalities, cretinism, mental retardation, muscle anomalies, and reduced work output. Although the adverse effects on health and socio-economic development are well known, they persisted as a public health problem worldwide. Salt iodization is recommended as a simple cost-effective method to prevent iodine deficiency disorders. This study aimed to determine the magnitude, trends, and determinants of iodized salt availability in the household in Ethiopia.Methods:The current study used the Ethiopian Demographic and Health Surveys conducted from 2000 to 2016 with a total of 57 939 households. Descriptive statistics were performed on selected background characteristics to provide an overall picture of the sample after considering sample weights. To ensure the representativeness of the sample we applied a complex sample design considering household weights, primary sampling units, and the strata associated with it. The Cochran–Armitage test was performed to assess the trend of iodized salt availability in the household. Multivariate logistic regression was used to determine the association between the dependent variable and independent variables. A significance level of .05 was chosen for all analyses.Results:The magnitude of iodized salt availability in the household was 28.45% in 2000, 54.34% in 2005, 15.42% in 2011, and 89.28% in 2016. Iodized salt availability increased from 28.45% [95% CI: 27.69-29.21] in 2000 to 89.28% [95% CI: 88.79-89.75] in 2016. Despite the decline from 2005 to 2011 in the percentage of households with iodized salt, overall, there was a significant increment from 2000 to 2016 in Ethiopia (P-value <.001). There were differences in the status of salt iodization in the administrative region, wealth, family size, and ownership of radio or television.Conclusion:Remarkable progress has been made in Ethiopia regarding iodized salt availability in recent years. Besides the current efforts to achieve universal salt iodization, future interventions should prioritize specific groups like those with lower socioeconomic status and geographic areas with lower availability of iodized salt in the household.

STIM1/ORAI1 Loss-of-Function and Gain-of-Function Mutations Inversely Impact on SOCE and Calcium Homeostasis and Cause Multi-Systemic Mirror Diseases.

Store-operated Ca2+ entry (SOCE) is a ubiquitous and essential mechanism regulating Ca2+ homeostasis in all tissues, and controls a wide range of cellular functions including keratinocyte differentiation, osteoblastogenesis and osteoclastogenesis, T cell proliferation, platelet activation, and muscle contraction. The main SOCE actors are STIM1 and ORAI1. Depletion of the reticular Ca2+ stores induces oligomerization of the luminal Ca2+ sensor STIM1, and the oligomers activate the plasma membrane Ca2+ channel ORAI1 to trigger extracellular Ca2+ entry. Mutations in STIM1 and ORAI1 result in abnormal SOCE and lead to multi-systemic disorders. Recessive loss-of-function mutations are associated with CRAC (Ca2+ release-activated Ca2+) channelopathy, involving immunodeficiency and autoimmunity, muscular hypotonia, ectodermal dysplasia, and mydriasis. In contrast, dominant STIM1 and ORAI1 gain-of-function mutations give rise to tubular aggregate myopathy and Stormorken syndrome (TAM/STRMK), forming a clinical spectrum encompassing muscle weakness, thrombocytopenia, ichthyosis, hyposplenism, short stature, and miosis. Functional studies on patient-derived cells revealed that CRAC channelopathy mutations impair SOCE and extracellular Ca2+ influx, while TAM/STRMK mutations induce excessive Ca2+ entry through SOCE over-activation. In accordance with the opposite pathomechanisms underlying both disorders, CRAC channelopathy and TAM/STRMK patients show mirror phenotypes at the clinical and molecular levels, and the respective animal models recapitulate the skin, bones, immune system, platelet, and muscle anomalies. Here we review and compare the clinical presentations of CRAC channelopathy and TAM/STRMK patients and the histological and molecular findings obtained on human samples and murine models to highlight the mirror phenotypes in different tissues, and to point out potentially undiagnosed anomalies in patients, which may be relevant for disease management and prospective therapeutic approaches.

Normal anatomy and abnormalities of the rectus extraocular muscles in humans

The development of modern surgical techniques makes a deep knowledge of anatomy, in the case of ophthalmic surgery, also functional problems of the extraocular muscles. Thus, the purpose of our review was to summarize the latest findings on normal anatomy and anomalies of rectus extraocular muscles (ERMs). Particular attention was paid to the presentation of detailed and structured data on the general anatomy of the ERMs, including their attachment, topography, vascularization and innervation. The question of ERMs innervation is presented in detail, taking into account recent studies on human material using methods.

Evaluation of peroxide index and oxidative rancidity of broiler diets and the occurrence of breast muscle anomalies

The incidence of breast muscle anomalies, such as white striping (WS) and wooden breast (WB), has increased over the last two decades. These myopathies have different implications on the quality of poultry meat, which depending on the degree of the anomaly, may be rejected due to undesirable appearance or lower technological and nutritional characteristics. Despite the high economic impact, the causes of these myopathies have not yet been defined. The increased susceptibility of poultry to the oxidation process may influence the occurrence of hemorrhagic disorders and muscular dystrophies, necrosis, and fibrosis associated with myopathy with unknown etiology development. Coagulants such as ferric chloride (FC) used in the treatment of effluents can be involved in the feed peroxidation process. Our aim was to evaluate whether the peroxides generated by the presence of FC in poultries’ diets are related to myopathies. Broilers were treated with feed supplemented with 4, 8, or 12 mL FC/Kg of feed. The presence of WS and WB, feed peroxide indices (PI), mean weights, and feed conversion ratios were evaluated. The highest FC concentration indicated higher peroxide production. Addition of 4, 8 and 12 mL of FC yielded 60.0%, 70.0%, and 70.7% of myopathies, respectively, which indicated a possible relationship between food peroxidation and myopathies. Lipoperoxidation resulted in the lowest mean final weight and worsening of the feed conversion in the birds treated with 12 mL of FC. Feed with a high peroxide index is a factor that can increase the incidence of WB and WS.

Normal Anatomy and Anomalies of the Rectus Extraocular Muscles in Human: A Review of the Recent Data and Findings.

Development of modern surgical techniques is associated with the need for a thorough knowledge of surgical anatomy and, in the case of ophthalmologic surgery, also functional aspects of extraocular muscles. Thus, the leading idea of this review was to summarize the most recent findings regarding the normal anatomy and anomalies of the extraocular rectus muscles (ERMs). Particular attention was paid to the presentation of detailed and structured data on the gross anatomy of the ERMs, including their attachments, anatomical relationships, vascularization, and innervation. This issue of ERMs innervation was presented in detail, considering the research that has recently been carried out on human material using advanced anatomical techniques such as Sihler's technique of the nerves staining. The text was supplemented with a carefully selected graphic material (including anatomical specimens prepared specially for the purpose of this review) and discussion of the clinical cases and practical significance of the presented issues.

Anomalous Muscles of the Wrist Encountered During Endoscopic Carpal Tunnel Surgery.

ObjectiveAnomalous muscles of the wrist are infrequently encountered during carpal tunnel surgery. Anatomic variants of the palmaris longus (PL), flexor digitorum superficialis, lumbricalis and abductor digiti minimi (ADM) have been reported but are usually clinically insignificant. Anomalies of the wrist muscles, encountered during endoscopic carpal tunnel surgery have rarely been described. I conducted this study to evaluate muscular anomalies of the volar aspect of the wrist, encountered during endoscopic carpal tunnel surgery. MethodsI studied a consecutive series of 1235 hands in 809 patients with carpal tunnel syndrome who underwent single-portal endoscopic carpal tunnel release (ECTR) from 2002 to 2014. Nine hundred seventy-three hands in 644 patients who had minimal 6-month postoperative follow-up were included in the study. The postoperative surgical outcome was assessed at least 6 months after surgery. ResultsIn eight patients, anomalous muscles were found under the antebrachial fascia at the proximal wrist crease and superficial to the ulnar bursa, passing superficial to the transverse carpal ligament . Those anomalous muscles were presumed to be variants of the PL or accessory ADM muscle, necessitating splitting and retraction to enter the carpal tunnel during the ECTR procedure. Other muscle anomalies were not seen within the carpal tunnel on the endoscopic view. The surgical outcome for all eight wrists was successful at the 6-month postoperative follow-up. ConclusionCarpal tunnel surgeons, especially those using an endoscope should be familiar with unusual findings of anomalous muscles of the wrist because early recognition of those muscles can contribute to avoiding unnecessary surgical exploration and unsuccessful surgical outcomes.

Clinical Spectrum and Management Implications of Left Ventricular Outflow Obstruction With Mild Ventricular Septal Thickness in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HC) has historically been characterized as a disease of substantial left ventricular hypertrophy, often associated with dynamic left ventricular outflow tract obstruction. However, we have recently encountered patients with subaortic obstruction and only minimal basal septal thickness, raising important management implications. Thereby, we sought to characterize the natural history and treatment strategies for this under-recognized subgroup of HC patients with dynamic obstruction. Of 1,591 consecutive patients with a HC diagnosis, 113 (7%) were identified with outflow obstruction due to elongated mitral valve leaflets producing systolic anterior motion and septal contact typical of HC (gradient, 84 ± 29 mm Hg at rest or with exercise), with maximal ventricular septal thickness ≤15 mm, including 14 patients with only 10 to 12 mm. In addition to the mechanism of outflow obstruction, other evidence supporting a HC diagnosis prominently included: positive HC family history and/or pathogenic sarcomere mutation, arrhythmic sudden death event, typical histopathology of septal muscle, and characteristic mitral valve and papillary muscle anomalies. Over 3.8 ± 3.5 years, 41 patients (36%) developed severe heart failure including 36 who have undergone myectomy associated with reconstruction of the outflow tract and mitral valve apparatus, resulting in relief of gradient without iatrogenic ventricular septal defect or mitral valve replacement. Postoperatively, all 36 patients have survived with symptom relief to New York Heart Association classes I/II. In conclusion, these observations expand the HC clinical profile and phenotype to include an under-appreciated subgroup in which disease expression includes outflow obstruction due primarily to an elongated mitral valve, associated with only minimal (or normal) ventricular septal thickness. Such HC patients can develop marked functional limitation amenable to an operative strategy that effectively relieved symptoms due to outflow obstruction, but without mitral valve replacement.