Assessment of the carpal tunnel and associated neural structures with superb microvascular imaging: a scoping review.

INTRODUCTION: Superb microvascular imaging (SMI) is an advanced ultrasound technique that portrays microcirculation. Its clinical applications have been studied in various diseases, including carpal tunnel syndrome (CTS) i.e. the most common entrapment neuropathy. This scoping review explores the role of SMI in diagnosing CTS or the assessment of relevant neural structures. METHODS: We conducted a comprehensive search of electronic databases (PubMed, Embase and Web of Science) up to 26 September 2023. Two independent authors conducted the literature search, quality assessment, and data extraction. RESULTS: This review includes seven studies comprising 385 wrists. SMI consistently revealed increased intraneural vascularity in the median nerves of patients with CTS compared to healthy individuals. While SMI demonstrated higher sensitivity than traditional Doppler methods for detecting CTS, its specificity was somewhat lower. Combining SMI with B-mode ultrasound appears to enhance the diagnostic accuracy for CTS. However, the relationship between SMI findings and CTS severity remains unclear. CONCLUSIONS: This review highlighted the ability of SMI to provide detailed vascular structures in both healthy wrists and those with CTS. Additional research is crucial to determine the typical SMI findings of the carpal tunnel and within that context, tailor more precise diagnostic/therapeutic applications for the CTS population.

Utility of shear wave elastography and high-definition color for diagnosing carpal tunnel syndrome.

OBJECTIVE: The diagnostic values of measuring median nerve (MN) stiffness and vascularity with shear wave elastography (SWE) and high-definition (HD) color were investigated in carpal tunnel syndrome (CTS). METHODS: Seventy patients (123 wrists) with CTS and thirty-five healthy volunteers (70 wrists) were enrolled. Based on nerve conduction studies (NCS), the patients were subdivided into NCS-negative, mild-to-moderate, and severe CTS groups. MN and abductor pollicis brevis (APB) SWE and MN HD color were performed on a longitudinal plane. RESULTS: The mild-to-moderate and severe CTS groups showed increased MN stiffness at the wrist and MN stiffness ratio (wrist-to forearm) compared with the control (p < 0.001). The NCS-negative CTS group showed increased MN stiffness at the wrist (p = 0.022) and MN stiffness ratio (p = 0.032) compared with the control. The severe CTS group showed increased MN stiffness at the wrist compared with the mild-to-moderate CTS group (p = 0.034). The cutoff-values in diagnosing NCS-confirmed CTS were 50.12 kPa for MN stiffness at the wrist, 1.91 for MN stiffness ratio, and grade 1 for HD color. CONCLUSIONS: SWE and HD color are good supportive tools in diagnosing and assessing severity in CTS. SIGNIFICANCE: SWE and HD color demonstrated that MN in CTS was associated with increased stiffness and hypervascularity.

Influence of temperature on sonographic images of the median nerve for the diagnosis of carpal tunnel syndrome: a case control study.

BACKGROUND: In addition to nerve conduction studies (NCSs), ultrasonography has been widely used as an alternative tool for diagnosing carpal tunnel syndrome (CTS). Although the results of NCSs are influenced by local skin temperature, few studies have explored the effects of skin temperature on ultrasonography of the median nerve. Since swelling and intraneural blood flow of the median nerve might be influenced by local temperature changes, the aim of this study was to evaluate the cross-sectional area (CSA) and intraneural blood flow of the median nerve under three skin temperatures (30 °C, 32 °C, 34 °C). METHODS: Fifty patients with CTS and 50 healthy volunteers were consecutively recruited from a community hospital. Each participant received physical examinations and NCSs and underwent ultrasonography, including power Doppler, to evaluate intraneural vascularity. RESULTS: The CSA of the median nerve in the CTS patients was significantly larger than that in the healthy controls at all three temperatures. However, significant differences in the power Doppler signals of the median nerve between the two studied groups were observed only at 30 and 32 °C, not at 34 °C. CONCLUSION: The significant difference in the intraneural vascularity of the median nerve between the patients with CTS and the healthy subjects was lost at higher temperatures (34 °C). Therefore, the results of power Doppler ultrasonography in diagnosing CTS should be cautiously interpreted in patients with a high skin temperature or those who reside in warm environments.

Median Nerve's Microcirculation in Carpal Tunnel Syndrome: Superb Microvascular Imaging.

BACKGROUND: The objectivity of physical and electrodiagnostic tests is limited in detecting carpal tunnel syndrome and its recurrence. Predicting the median nerve blood supply using superb microvascular imaging will allow exact diagnosis and a good follow-up system. The aims of this study include using superb microvascular imaging to correlate with electromyographic diagnosis, and to determine the impact of surgery on improvement in superb microvascular imaging. METHODS: Between July of 2019 and January of 2020, 32 wrists of 21 patients were evaluated prospectively. After preoperative electrodiagnostic studies and vascular index measurement with superb microvascular imaging, open carpal tunnel release was performed by a single surgeon, and 3 months later standardized superb microvascular imaging was performed. Preoperative vascular indexes were compared with the mild, moderate, and severe electrodiagnostic study results. Preoperative and postoperative vascular index results were compared. RESULTS: The average of the preoperative and postoperative imaging groups was 2.77 and 1.48, respectively, and there was a statistically significant difference between the two groups (p < 0.05). Although no significant difference was found between preoperative and postoperative vascular index values in patients presenting with mild carpal tunnel syndrome (p > 0.05), there was a significant decrease in vascular index values in patients presenting with moderate and severe carpal tunnel syndrome after surgical decompression. CONCLUSIONS: Superb microvascular imaging is emerging as a groundbreaking, new, and reliable technique. Evaluation of the median nerve blood supply is a reliable method that would be helpful for early diagnosis, planning treatment, determining the severity of carpal tunnel syndrome, and postoperative follow-up. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, IV.

Superb microvascular imaging for detecting carpal tunnel syndrome compared with power Doppler ultrasonography: A protocol for systematic review and meta-analysis.

BACKGROUND: Sonography enhances diagnostic accuracy by the detection of the epineural or intraneuronal blood flow.[1-4] Power Doppler ultrasonography (PDUS) in carpal tunnel syndrome (CTS) has been previously studied and shown to be valid and reliable for grading the intraneural flow.[3,4] However, superb microvascular imaging (SMI) represents a new era in diagnostic sonography, and this new technology enables accurate visualization of vascular structures with intensive clutter suppression to provide flow signals for large to small vessels, and it presents these data at high frame rates.[5-7] Studies suggested that SMI is more sensitive in demonstrating blood flow in the diagnosis of CTS compared with PDUS.[7] In order to gain clarity, a meta-analysis to systematically review and synthesize relevant data on the evaluating intraneural blood flow of the median nerve (MN) using SMI and PDUS was undertaken. METHODS: We will search PubMed, Web of Science, Cochrane Library, and Chinese biomedical databases from their inceptions to the December 31, 2020, without language restrictions. Two authors will independently carry out searching literature records, scanning titles and abstracts, full texts, collecting data, and assessing risk of bias. Review Manager 5.2 and Stata 14.0 software will be used for data analysis. RESULTS: This systematic review will investigate whether superb microvascular imaging is more sensitive to display the blood flow in the MN with CTS than PDUS. CONCLUSION: Its findings will provide strong evidence for the feasibility of superb microvascular imaging on the detection of the neovascularization of the MN with CTS. SYSTEMATIC REVIEW REGISTRATION: INPLASY202110018.

Ultrasound examination predicts 6-month progression in carpal tunnel syndrome patients.

Carpal tunnel syndrome (CTS) is a peripheral neuropathy resulting from chronic median nerve compression. Chronic compression leads to neurological changes that are quantified through nerve conduction studies (NCS). Although NCS represents the gold standard in CTS assessment, they provide limited prognostic value. Several studies have identified ultrasound as a tool in diagnosing and potentially predicting the progression of CTS in patients. The purpose of this study was to evaluate the predictive value of ultrasound examination in CTS patients. Twenty patients recruited at their first visit with the neurologist completed two NCS and ultrasound examinations approximately 6 months apart. Ultrasound examination consisted of B-mode, pulse-wave Doppler and colour Doppler ultrasound videos and images to quantify median nerve cross-sectional area, intraneural blood flow velocity in three wrist postures (15° flexion, neutral, and 30° extension), and displacement of the flexor digitorum superficialis (FDS) tendon and the adjacent subsynovial connective tissue (SSCT) of the middle finger during repetitive finger flexion-extension cycles. A questionnaire was administered to assess the work-relatedness of CTS. Linear regression analyses revealed that intraneural blood flow velocity (R2 = 0.36, p = .03), assessed in wrist flexion, and relative FDS-SSCT displacement (R2 = 0.27, p = .04) and shear strain index (R2 = 0.28, p = .04) were significant predictors of nerve sensory and motor changes at 6 months. Results suggest the possibility of using a battery of ultrasound measures as viable markers to predict median nerve functional changes within 6 months.

Sonographic assessment of nerve blood flow in diabetic neuropathy.

AIMS: To undertake sonographic assessment of nerve blood flow in people with Type 2 diabetes and correlate the findings with neuropathy severity scores and electrophysiological measurements. METHODS: Median and tibial nerve ultrasound scans were undertaken in 75 people with diabetes and 30 aged-matched controls without diabetes, using a high-resolution linear probe at non-entrapment sites. Nerve blood flow was quantified using power Doppler techniques to obtain the vessel score and the maximum perfusion intensity. Neuropathy severity was assessed using a total neuropathy score. RESULTS: Diabetic nerves had higher rates of nerve blood flow detection (28%) compared to the control group (P < 0.0001). Significant correlations were found between nerve blood flow measurements and nerve size (P <0.001), reported sensory symptoms (P < 0.05) and neuropathy severity scores (P < 0.001). The cohort with diabetes had significantly larger median (8.5 ± 0.3 mm2 vs 7.2 ± 0.1 mm2 ; P < 0.05) and tibial nerves (18.0 ± 0.9 mm2 vs 12.8 ± 0.5 mm2 ; P < 0.05) compared with controls. CONCLUSION: Peripheral nerve hypervascularity is detectable by ultrasonography in moderate to severe diabetic neuropathy with prominent sensory dysfunction.

Carpal tunnel ultrasound: is the "safe zone" on the ulnar side of the median nerve really avascular?

INTRODUCTION: Numerous publications have studied the regional anatomy of the carpal tunnel to define a "safe zone" to reduce the risk of perioperative neurovascular complications. This zone, located between the ulnar neurovascular bundle and the median nerve, is considered to be safe mainly because of the absence of vascular structures. This study aims to assess the presence of arterioles within this area using superb microvascular imaging (SMI). MATERIALS AND METHODS: The images from patients who underwent a bilateral routine wrist ultrasound with SMI, between January 28 and February 28, 2019, were retrospectively reviewed by two radiologists to evaluate the presence and location of arterioles in the safe zone. In addition, cadaveric wrists injected with intra-arterial red latex underwent dissection of the carpal tunnel. RESULTS: The images from 27 patients (54 wrists) were reviewed. In the safe zone, arterioles were seen superficial to the retinaculum in 36 wrists (36/54; 66.7%) and deep to the retinaculum in 21 wrists (21/54; 38.9%). The arterioles located deep to the retinaculum were more frequently found close to the median nerve (21/54; 38.9%) than to the ulnar artery (9/54; 16.7%). In five cadaveric wrists, arterioles were detected superficial to the retinaculum in 3 wrists (3/5; 60%) and deep to the retinaculum in 2 wrists (2/5; 40%). CONCLUSION: Arterioles can be seen in the safe zone both superficial and deep to the flexor retinaculum. Deep to the retinaculum, they are mainly observed in the proximal aspect of the carpal tunnel and more frequently close to the median nerve. KEY POINTS: • Superb microvascular imaging (SMI) enables the visualization of arterioles within the "safe zone" of the carpal tunnel (visible both superficial and deep to the flexor retinaculum). • Arterioles were more frequently observed in the proximal aspect of the carpal tunnel. • Deep to the retinaculum, arterioles were more frequently seen in proximity to the median nerve.

[Acute carpal tunnel syndrome caused by a thrombosis of a persistent median artery: 2 case reports]. / Canal carpien aigu : penser à la thrombose de l'artère persistante du nerf médian !

Carpal tunnel syndrome is a common peripheral neuropathy, usually idiopathic or post-traumatic due to the compression of the median nerve. Numbness and paresthesias in the median nerve distribution are the most common symptoms associated with this condition. Persistent median artery is a rare anatomic variation, thrombosis of this additional artery can be responsible for an acute carpal tunnel syndrome, and patients frequently complain about coldness and acute hand swelling. These unusual features must lead clinicians to think of a vascular cause. The diagnosis can be easily confirmed by using ultrasound doppler, but CT-scan and MRI are sometimes helpful. We describe 2 cases of acute carpal tunnel syndrome due to thrombosed persistent median artery, including a case of thromboangiitis obliterans. These thrombosis might also be due to traumatic causes. No guidelines are currently available to help physicians for the management of carpal tunnel syndrome from thrombosed persistent median artery. Antiplatelet therapy, statin, anticoagulant might be helpful, and surgery has sometimes be reported as effective.

Persistent Median Artery as an Unusual Finding in the Carpal Tunnel: Its Contribution to the Blood Supply of the Hand and Clinical Significance.

BACKGROUND Knowledge of the variable relation of the persistent median artery (PMA) to the median nerve and its contribution to the formation of the superficial palmar arch is of great clinical significance. This study presents a proposal of specific variables which might be introduced to characterize the PMA in the wrist region. MATERIAL AND METHODS One hundred and twenty-five randomly selected, isolated upper limbs fixed in 10% formalin solution were subjected to anatomical dissection. RESULTS Of the 125 upper limbs, PMA was found in 5 specimens (4% of the total number of limbs). In the carpal tunnel, the artery occupied the anterolateral position (2 cases), the anterior position (2 cases) or the anteromedial position (1 case) in relation to the median nerve. Two types of superficial palmar arches with significant contributions from the PMA were observed in the studied material: complete medio-ulnar arch and an incomplete arch without a connection between the territories of the ulnar and median arteries. The mean ratio of the diameter of the PMA to the diameter of ulnar artery at the level of the wrist was 0.59 (min.=0.38, max=0.83, SD=0.19). CONCLUSIONS Orthopedic and hand surgeons should be aware of the probability of occurrence of the PMA in both planning and conducting surgeries within the wrist region and within the carpal tunnel, as this anomalous vessel might present significant contributions to the arterial blood supply of the hand and might potentially play an important role in the presence of notable clinical symptoms and presentations.

Neural Perforasomes of the Upper Extremity.

BACKGROUND: In the setting of the rapid advancement of integumentary vascular knowledge, the authors hypothesized that the extrinsic blood supply to the major peripheral nerves of the upper extremity could be categorized into discrete neural "perforasomes." METHODS: Total limb perfusion of the arterial system was performed with gelatin-red lead oxide in cadaveric upper limbs. The perforating vessels to the radial, median, and ulnar nerves were identified, confirmed with fluoroscopy, and dissected. Distances to major anatomical landmarks of the upper extremity were measured. Additional cadaveric limbs' nerves were dissected and source arteries were selectively cannulated and injected to assess specific contribution to extrinsic nerve perfusion. The perfusion of each nerve was then calculated among all specimens. RESULTS: The radial, median, and ulnar nerve perforators were mapped. The corresponding neural perforasomes were mapped. The distal portions of the superficial radial nerve and the posterior interosseous nerve demonstrated a lack of staining. Similarly, at the carpal tunnel and at the proximal 25 percent of the median nerve (corresponding to the pronator teres), the nerve lacked vascular staining. At the Guyon canal and the flexor carpi ulnaris, the ulnar nerve demonstrated a lack of vascular staining. CONCLUSIONS: Peripheral nerves can be divided into neural perforasomes with limited overlap. The extrinsic perfusion of peripheral nerves is highly segmental. Absent stains within the nerves correspond to common sites of compression: carpal tunnel and pronator teres for the median nerve, supinator for the posterior interosseous nerve, and the Guyon canal and the flexor carpi ulnaris for the ulnar nerve.

Superb Microvascular Imaging of the Median Nerve in Carpal Tunnel Syndrome: An Electrodiagnostic and Ultrasonographic Study.

OBJECTIVE: To evaluate intraneural blood flow of the median nerve using superb microvascular imaging (SMI) and power Doppler ultrasonography (PDUS), and to examine their correlation with electroneuromyography in patients with carpal tunnel syndrome (CTS). METHODS: A cross-sectional survey was used, and the study was conducted in the research unit of a training and research hospital. Patients diagnosed with CTS according to electroneuromyography studies were included in the study. Ultrasound measurements were taken using an Aplio-500 (Toshiba Medical System Corporation, Tokyo, Japan) device and a linear multifrequency 14-MHz probe. The cross-sectional area of the median nerve at the carpal tunnel level was measured by the direct tracing method using electronic calipers. The power Doppler ultrasonography and superb microvascular imaging scores were recorded by grading the vascularity between 0 and 3. RESULTS: Evaluation was made of a total of 113 hands of 80 patients (18 men, 62 women) with a mean age of 34.67 ± 12.82 years. The mean duration of symptoms was 12.34 ± 6.66 months. When the patients were grouped as mild, moderate, and severe CTS, there was a statistically significant difference between the SMI and PDUS grades (P < .05). As the severity of CTS increased, an increase in SMI and PDUS scores was observed. There was a strong correlation between SMI scores and motor distal latency (r = .71/P = .026), amplitude of sensory action potential (r = -.77/P = .029), and sensory neurotransmission rate (r = .77/P = .029). CONCLUSION: SMI seems to be more sensitive than PDUS for evaluating the vascularity of the median nerve in patients with CTS, and SMI grading is correlated with the ENMG results.

Blood Flow Changes in Subsynovial Connective Tissue on Contrast-Enhanced Ultrasonography in Patients With Carpal Tunnel Syndrome Before and After Surgical Decompression.

OBJECTIVES: Although qualitative alteration of the subsynovial connective tissue in the carpal tunnel is considered to be one of the most important factors in the pathophysiologic mechanisms of carpal tunnel syndrome (CTS), little information is available about the microcirculation in the subsynovial connective tissue in patients with CTS. The aims of this study were to use contrast-enhanced ultrasonography (US) to evaluate blood flow in the subsynovial connective tissue proximal to the carpal tunnel in patients with CTS before and after carpal tunnel release. METHODS: The study included 15 volunteers and 12 patients with CTS. The blood flow in the subsynovial connective tissue and the median nerve was evaluated preoperatively and at 1, 2, and 3 months postoperatively using contrast-enhanced US. RESULTS: The blood flow in the subsynovial connective tissue was higher in the patients with CTS than in the volunteers. In the patients with CTS, there was a significant correlation between the blood flow in the subsynovial connective tissue and the median nerve (P = .01). The blood flow in both the subsynovial connective tissue and the median nerve increased markedly after carpal tunnel release. CONCLUSIONS: Our results suggest that increased blood flow in the subsynovial connective tissue may play a role in the alteration of the microcirculation within the median nerve related to the pathophysiologic mechanisms of CTS. The increase in the blood flow in the subsynovial connective tissue during the early postoperative period may contribute to the changes in intraneural circulation, and these changes may lead to neural recovery.

Value of superb microvascular imaging ultrasonography in the diagnosis of carpal tunnel syndrome: Compared with color Doppler and power Doppler.

The aim of this study was to compare the value of superb microvascular imaging (SMI) in carpal tunnel syndrome (CTS) with that of color Doppler ultrasonography (CDUS) and power Doppler ultrasonography (PDUS).Fifty patients with symptomatic CTS and 25 healthy volunteers were enrolled. The cross-sectional area (CSA), CDUS score, PDUS score, and SMI score of the median nerve (MN) at the carpal tunnel were recorded. The value of different ultrasonography (US) diagnostic strategies was calculated.The blood flow display ratio in the MN of the healthy volunteers had no statistical difference between CDUS, PDUS, and SMI (20%, 32%, and 48%, respectively, P >.05). The blood flow display ratio for SMI in patients was significantly higher than that of CDUS and PDUS (90%, 52%, and 60%, respectively, P <.005). The accuracy of SMI score ≥2 (79%) was much higher than that of CDUS and PDUS (61% and 63%, respectively, P <.05). Comprehensive consideration of SMI and CSA, CSA≥10.5 mm, and/or SMI score ≥2 has the highest accuracy (83%), significantly higher than that of CSA combination with CDUS or PDUS (68% and 69%, respectively, P <.05).SMI is more sensitive to display the blood flow in the MN with CTS than CDUS and PDUS. It might significantly improve the diagnosis value for CTS.

Effects of Wrist Posture and Fingertip Force on Median Nerve Blood Flow Velocity.

Purpose. The purpose of this study was to assess nerve hypervascularization using high resolution ultrasonography to determine the effects of wrist posture and fingertip force on median nerve blood flow at the wrist in healthy participants and those experiencing carpal tunnel syndrome (CTS) symptoms. Methods. The median nerves of nine healthy participants and nine participants experiencing symptoms of CTS were evaluated using optimized ultrasonography in five wrist postures with and without a middle digit fingertip press (0, 6 N). Results. Both wrist posture and fingertip force had significant main effects on mean peak blood flow velocity. Blood flow velocity with a neutral wrist (2.87 cm/s) was significantly lower than flexed 30° (3.37 cm/s), flexed 15° (3.27 cm/s), and extended 30° (3.29 cm/s). Similarly, median nerve blood flow velocity was lower without force (2.81 cm/s) than with force (3.56 cm/s). A significant difference was not found between groups. Discussion. Vascular changes associated with CTS may be acutely induced by nonneutral wrist postures and fingertip force. This study represents an early evaluation of intraneural blood flow as a measure of nerve hypervascularization in response to occupational risk factors and advances our understanding of the vascular phenomena associated with peripheral nerve compression.

Median artery of the forearm in human fetuses in northeastern Brazil: anatomical study and review of the literature.

A persistent median artery is a rare anomaly. It accompanies the median nerve along its course in the forearm and is of variable origin. It is associated with other local anatomical variations and may contribute significantly towards formation of the superficial palmar arch. In embryos, it is responsible mainly for the blood supply to the hand. The objective of this study was to research the frequency, type (forearm or palmar) and origin of the median artery in fetuses, correlating its presence with sex and body side. Red-colored latex was injected into 32 brachial arteries of human fetuses until its arrival in the hand could be seen. Twenty-four hours after the injection, the median arteries were dissected without the aid of optical instruments. Among the 32 forearms dissected, the median artery was present in 81.25 % (26) of the cases, and it was found more frequently in females and on the left side. Regarding origin, most of the median arteries originated in the common interosseous artery (38.5 %) and anterior interosseous artery (34.6 %). The mean length of the median arteries was 21.1 mm for the palmar type and 19.8 mm for the forearm type. The median artery has a high rate of persistence. It is important to be aware of this anatomical variation, since its presence may give rise to difficulties during routine surgical procedures on the wrist. Its presence may cause serious functional complications in the carpal tunnel, anterior interosseous nerve, round pronator syndromes, and ischemia of the hand.

Detection of Intraneural Median Nerve Microvascularity Using Contrast-Enhanced Sonography: A Pilot Study.

OBJECTIVES: Demonstrating vascularity within the human median nerve may be difficult using power Doppler sonography. To this end, a pilot study documenting contrast-enhanced vascularity of the median nerve was conducted. METHODS: Patients undergoing contrast-enhanced transthoracic echocardiography were recruited for this study (n = 24). During echocardiography, a simultaneous contrast-enhanced sonographic examination of the median nerve was conducted. The study and study protocol were built from preclinical evidence. Image analysis was based on the power Doppler pixel intensity within a defined region of interest to obtain quantitative data representing the average pixel intensity, maximum pixel intensity, and power Doppler pixel dot count. Semiquantitative data representing the power Doppler dot count grading were also obtained. RESULTS: Spearman correlations between analytical methods showed strong positive, statistically significant (P< .05) correlations between the average pixel intensity and maximum pixel intensity and between the power Doppler dot count and dot count grading. Statistically significant increases in the average pixel intensity and power Doppler dot count were seen at all but 1 time point throughout the contrast-enhanced sonographic examination when compared to precontrast administration. Statistically significant increases in the maximum pixel intensity were seen at all but 4 time points. CONCLUSIONS: These pilot results represent early evidence that contrast-enhanced sonography can be used to image median nerve vascularity. In this convenience sample, median nerve contrast-enhanced sonographic data collection was feasible, safe, and consistent.

Spontaneous Compartment Syndrome of the Hand in Systemic Sclerosis.

Compartment syndrome refers to a condition of compromised circulation within a limited space due to increased pressure within that space. The reduced tissue perfusion results in reduced venous drainage, leading to increased interstitial tissue pressure and subsequent compromised arterial flow. Although not as common as compartment syndrome of the leg and forearm, compartment syndrome of the hand is not rare and can lead to devastating sequelae as a result of tissue necrosis. Compartment syndrome of the hand has several etiologies, including trauma, arterial injury, thermal injury, and constrictive bandaging. The cardinal clinical sign is pain that is aggravated by passive stretching of the muscles within the involved compartments. Extremity function is usually restored with expeditious fasciotomy of the involved myofascial compartments, and complications, such as intrinsic muscular dysfunction and Volkmann's ischemic contracture, can usually be prevented. There are no reported cases of compartment syndrome of the hand in patients with systemic sclerosis or Raynaud's phenomenon. Systemic sclerosis is a form of scleroderma that affects the skin and internal organs. The limited cutaneous subset affects the skin of the extremities but is associated with a set of characteristic features that includes calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia. This report describes an unusual case of a patient who had spontaneous compartment syndrome of the hand. The patient's concomitant limited cutaneous systemic sclerosis may have played a role in this unusual occurrence. The diagnosis was based on the clinical picture, and the symptoms resolved after surgical decompression.