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Palmar fibromatosis (Dupuytren disease/contracture) is the most common type of fibromatosis, defined as a benign proliferation of fibroblasts and myofibroblasts. The disease process is most common in white, middle-aged and older men occurring at the distal palmar crease leading to nodules and contracture, which in many cases recur after surgical treatment. In a similar process, plantar fibromatosis (Ledderhose disease) is a proliferation of fibroblasts and myofibroblasts on the plantar aponeurosis of mostly middle-aged patients that may lead to painful nodules but usually does not lead to contracture. Both processes are histologically similar, composed of a bland cellular proliferation of spindle cells with a bluish appearance and with a variable amount of background collagen, depending on the age of the lesion. The etiology of both lesions is still uncertain, while treatment ranges from observation to surgery, with some pharmacologic agents being investigated with mixed success. In this paper we provide an overview of both processes with regards to clinical and radiologic findings, pathophysiology, diagnosis, treatment, and prognosis.

Purpose: There is little information regarding the starting points for intramedullary K-wires and their effect on the soft tissues surrounding the metacarpophalangeal (MCP) joint. This study attempted to identify starting points for intramedullary K-wire fixation and determine the effect of these K-wires on soft tissues surrounding the MCP joint. Methods: We placed intramedullary K-wires in the index, middle, and little finger metacarpals and proximal phalanges in 6 cadaveric specimens. We measured the starting points of the K-wires and performed dissection to evaluate which structures were penetrated. A Fisher exact test determined statistical significance between K-wire penetration of soft tissue structures and the starting point for K-wires. Results: Metacarpal K-wires started 8.5 ± 1.5 mm volar to the dorsal aspect of the metacarpal head, 19.1 ± 4.6 mm proximal to the digital palmar crease, and 8.6 ± 1.2 mm from the metacarpal head midline. All MCP K-wires crossed at least one soft tissue structure about the MCP joint; the most commonly the sagittal band. Proximal phalanx K-wires started 8.9 ± 2.9 mm proximal to the distal aspect of the metacarpal head, 25.5 ± 5.9 mm proximal to the digital palmar crease, and 9.9 ± 1.5 mm from metacarpal head midline. All proximal phalanx K-wires crossed at least one soft tissue structure about the MCP, most commonly the joint capsule. No relation was established between K-wire start point and penetration of soft tissue structures. Conclusions: Use of these measurements may aid the physician in placing intramedullary K-wires into the proximal phalanges and metacarpals. Starting points that decrease the rate of penetration of soft tissue structures around the MCP joint have not been described, and all pins crossed at least one soft tissue structure adjacent to the MCP joint. Type of study/level of evidence: Therapeutic IV Key words: complication, fracture, K-wire, metacarpal, phalangeal

Background Musculocontractural Ehlers–Danlos Syndrome (mcEDS) is a rare connective tissue disorder caused by biallelic loss‐of‐function variants in CHST14 (mcEDS‐CHST14) or DSE (mcEDS‐DSE), both of which result in defective dermatan sulfate biosynthesis. Forty‐one patients with mcEDS‐CHST14 and three patients with mcEDS‐DSE have been described in the literature. Methods Clinical, molecular, and glycobiological findings in three additional patients with mcEDS‐DSE were investigated. Results Three patients from two families shared craniofacial characteristics (hypertelorism, blue sclera, midfacial hypoplasia), skeletal features (pectus and spinal deformities, characteristic finger shapes, progressive talipes deformities), skin features (fine or acrogeria‐like palmar creases), and ocular refractive errors. Homozygous pathogenic variants in DSE were found: c.960T>A/p.Tyr320* in patient 1 and c.996dupT/p.Val333Cysfs*4 in patients 2 and 3. No dermatan sulfate was detected in the urine sample from patient 1, suggesting a complete depletion of DS. Conclusion McEDS‐DSE is a congenital multisystem disorder with progressive symptoms involving craniofacial, skeletal, cutaneous, and cardiovascular systems, similar to the symptoms of mcEDS‐CHST14. However, the burden of symptoms seems lower in patients with mcEDS‐DSE., Clinical, molecular, and glycobiological findings in three additional patients from two families with musculocontractural Ehlers–Danlos syndrome (mcEDS‐DSE) are described. McEDS‐DSE is a congenital multisystem disorder with progressive symptoms involving craniofacial, skeletal, cutaneous, and cardiovascular systems, similar to the symptoms of mcEDS‐CHST14. However, the burden of symptoms seems lower in patients with mcEDS‐DSE.

Systemic sclerosis (SSc) is a collagen-vascular disorder characterized by fibrosis and vasculopathy. Delta finger to palm distance (delta FTP) is an index measuring the distance between the tip of the third finger to the distal palmar crease in the flexed and extended position. The present study aimed to evaluate the clinical value of delta FTP and to assess the correlation of delta FTP with modified Rodnan skin score (mRSS) and forced vital capacity (FVC) over the 12-month follow-up. This prospective longitudinal study began with 50 participants who were followed for twelve months. Lowess smoothing and linear regression were applied to detect and assess the relationship between delta FTP and mRSS. p-values were adjusted by the Benjamini-Hochberg method (BHM) as a control for false discovery rate. Delta FTP was lower among patients with higher disease duration (p-valueadj: 0.008), diffuse cutaneous SSc (p-valueadj: 0.006), digital ulcers (p-valueadj: 0.003), telangiectasia (p-valueadj: 0.006) and dysphagia (p-valueadj: 0.036). The mRSS has a significant negative linear effect on the delta FTP at the baseline and the end of the follow-up (r: -0.31 and -0.40, respectively). Moreover, changes of mRSS and delta FTP showed a negative linear association over time (r: -0.22). These linear effects remained significant after regrouping the patients based on their SSc subtype. Delta FTP and FVC were not correlated either at the baseline or at the end. It seems that the delta FTP can be a valuable clinical index, supported by its correlated changes with mRSS and other SSc clinical manifestations over the one-year follow-up.

Background: Electrophysiologic studies have been considered the “gold standard” in diagnosing carpal tunnel syndrome (CTS); however, reports of false-negative results, as well as discomfort for the patient during the procedure has paved the use of ultrasound, being a painless and cost-efficient tool, as an alternative means for its diagnosis. Various ultrasound parameters assessing the median nerve and wrist dimensions have been described, but description of landmarks to assess these in a reliable manner has been lacking. Methodology: A systematic search of different databases yielded data regarding ultrasound parameters for CTS diagnosis, the landmarks used, and presence of reliability testing. Based on this, three sonologists discussed the external and sonographic landmarks that will be used in measuring the median nerve measurements, bowing of the flexor retinaculum and the carpal tunnel dimensions. A pilot test with two consecutive healthy participants using the discussed ultrasound parameters was carried out, and results were subjected to inter- and intra-rater reliability testing. Modifications were accordingly made on the acquisition of ultrasound image using external landmarks. The reliability testing proper was done with ten consecutive healthy participants. Results: Based on the systematic review and the pilot study, external landmarks were used to locate the median nerve in the forearm, carpal tunnel inlet and outlet. For the forearm measurement, it was taken 10 cm proximal from the distal palmar crease. The distal palmar crease was the external landmark used for the carpal tunnel inlet, while for the carpal tunnel outlet; it was measured 1 cm distal to the distal palmar crease. Instead of using the inner edge of the hook of hamate and trapezium, the apices of these bones were used as the landmarks in measuring the carpal tunnel outlet dimensions. There was excellent intra-rater reliability (mid-forearm, carpal tunnel inlet and outlet) except for the following: cross-sectional area (CSA) of the median nerve at the carpal tunnel inlet and outlet; and bowing of the flexor retinaculum. All the parameters had an excellent inter-rater reliability measured at the three levels (intraclass correlation [ICC]: Of 0.77–0.99) except for CSA of the median nerve at the levels of the forearm (fair-to-good with ICC of 0.71) and the carpal tunnel inlet (fair-to-good reliability of ICC: 0.43). Conclusion: There was an improved inter- and intra-rater reliability when external landmarks were used instead of sonographic landmarks.

Introduction: A lipoma is a benign tumor of the adipose tissue and the most common tumor of the subcutaneous tissue which is most commonly found on the trunk. It appears as a round or oval subcutaneous mass of soft consistency, which is not attached to the skin or to the deeper tissues. Its growth can cause compressive symptoms, the most common being pain and paresthesia. Case report: Considering the fact that lipomas very rarely occur in the hand and foot, in this paper we present a case of a large lipoma in the hand of a 70-year-old female patient, with a subcutaneous tumor, in the area of the ulnar side of the left palm, and the pain and tingling in the fourth and fifth finger. Longitudinal incision on the medial side of the palm and transversal incision up to the proximal palmar crease were performed under general endotracheal anesthesia. The extirpated tumor was, due to its dimensions (5.6 x 3.4 x 2.5 cm), categorized as a giant hand lipoma. A histopathological analysis confirmed the diagnosis of lipoma. Conclusion: A hand lipoma requires surgical treatment exclusively, involving a qualified hand surgeon, which is important because of the high functional and aesthetic importance of the hand.

Background More than 1.5 billion people are infected with soil-transmitted helminths (Ascaris lumbricoides, hookworm, Strongyloides stercoralis, and Trichuris trichiura), causing an estimated global burden in excess of 3 million disability-adjusted life years. However, the relationship between soil-transmitted helminth infection, adverse health consequences, and beneficial effects of deworming are not well understood. Methodology We pursued a detailed longitudinal clinical evaluation of school-age children to evaluate morbidity associated with soil-transmitted helminth infection and responses to treatment. This exploratory study was embedded into a randomized controlled trial. Overall, 434 children, aged 7–14 years, underwent a detailed medical history, physical examination, stool microscopy for soil-transmitted helminths, and hemoglobin (Hb) measurement at baseline. Medical history and stool examination were repeated at 3 and 18 weeks posttreatment. Additionally, Hb measurement was performed at the 18-week treatment follow-up. Logistic regression was employed to assess clinical factors associated with soil-transmitted helminth infection at baseline, and longitudinal data analysis to examine change in health outcomes following treatment over time. Principal findings All enrolled children were infected with T. trichiura, and randomized into four different treatment interventions. None of the medical history, physical examination, and laboratory (i.e., Hb) findings were associated with A. lumbricoides, hookworm, or S. stercoralis infection at baseline. A composite of physical exam findings for anemia, including pallor of the conjunctiva, nail beds, and palmar creases predicted lower Hb values (-3.8 g/dl, 95% confidence interval (CI): -6.9, -0.6 g/dl). When examining longitudinal trends, we did not find improvements to Hb or face Wong-Baker Likert scale among children with soil-transmitted helminth infection compared to those without infection, although there was a slight trend toward improving Hb values after treating hookworm infection. Conclusions/significance Our study demonstrates the challenges of measuring morbidity in the context of soil-transmitted helminth infection and treatment, thus confirming the mainly subtle morbidity effects of infection., Author summary Soil-transmitted helminth infections frequently affect children and impoverished individuals in low-resource settings and are associated with negative health effects. We attempted to measure morbidity in school-age children from Pemba Island, Tanzania who are infected with soil-transmitted helminths but with relatively low risk for other infections known to cause chronic morbidity, such as malaria and schistosomiasis. We used a composite of clinical history, physical examination, and hemoglobin measurements. Our study was embedded into a randomized controlled trial, and by design, all individuals were infected with whipworms (Trichuris trichiura). A detailed clinical evaluation for morbidity in school-age children was not associated with an infection of roundworms (Ascaris lumbricoides), hookworm, or threadworms (Strongyloides stercoralis) but demonstrated the utility of the clinical examination to detect anemia. Indeed, anemia slightly trended toward resolution after deworming. While many metrics for soil-transmitted helminth infection-related morbidity may have been masked by ongoing deworming campaigns, simple physical exam findings for anemia can be useful to highlight individuals that require further evaluation.

Hyperlipidemic myeloma is a rare and poorly understood variant of multiple myeloma. We report the case of a 53-year-old woman with hyperlipidemic myeloma, skin xanthomas and hyperviscosity syndrome who underwent allogeneic bone marrow transplantation. A comprehensive literature search identified 52 additional cases with plasma cell disease and hyperlipidemia. A detailed analysis revealed several characteristics of these patients as compared to multiple myeloma with normal lipid status: (1) IgA paraprotein was present in the majority (53% vs. 21% in classical multiple myeloma). (2) Skin xanthomas, especially in the palmar creases, elbows, and knees were common (70%). (3) Hyperviscosity syndrome occurred more often (26% vs. 2–6%). While conventional lipid-lowering therapy had only marginal effects, successful anti-myeloma therapy also reduced hyperlipidemia. Analyses of the mechanisms leading to hyperlipidemia documented complexes of paraprotein and lipoprotein in 75% of the 32 cases tested, suggesting an inhibitory role of the paraprotein on lipid degradation. In conclusion, the clinical characteristics, the therapeutic options, and the pathophysiologic mechanisms of hyperlipidemic myeloma are comprehensively reported using the available data from all 53 published cases in the literature., Annals of hematology, 89 (6), ISSN:0939-5555, ISSN:1432-0584

Dear editor, After carefully reading the recent paper by Gu et al ,1 a few questions and concerns arose. In the Background it is mentioned that “LI4 is supplied by the median nerve”.1 No reference is given to support this statement. Also, it is stated that determination of electroacupuncture (EA) intensity “was generally accompanied by mild muscle contraction in the index finger”.1 According to Anatomy for Acupuncture by Dorsher and Cummings (http://www.anatomy.tv/atv2/mainwindow.aspx?ref=0&titleid=26&svrid=0&App=acupuncture), sensation from the skin of the first dorsal web space is conveyed by the terminal radial nerve, and the muscles most often needled at this point are the first dorsal interosseous and adductor pollicis brevis, both of which are innervated by the ulnar nerve (myotome C8-T1). Deep needling closer to the first metacarpal bone may penetrate flexor pollicis brevis, which is innervated by the ulnar and median nerves (myotome C8-T1), but this is not usually achieved with needling to 1.25 cm depth at LI4. If the needle were to have been directed ventrally to the second metacarpal bone (in the direction of SI3, on the ulnar border of the hand at the level of the palmar crease), the needle might have reached the first lumbrical, which is also innervated by the median nerve.2 In this case, flexion of the metacarpophalangeal joint with extension of the interphalangeal joints of the second finger would have been elicited with EA. When describing …

Little is known about the prevalence and phenotype of fetal alcohol syndrome (FAS) or spectrum disorders (FASD) in Korea. This study was performed to describe the distribution of alcohol-related physical features in a genetically homogeneous sample of children and adolescents in institutional settings in Korea. Children and adolescents receiving services in one of seven institutions in Seoul, Korea were screened for growth deficiency. Those who screened positive were assessed using a structured protocol for the key cardinal features of FAS, and for 11 additional alcohol-related dysmorphologic features. Based on these findings, children and adolescents were categorized as FAS, Deferred (some characteristic features of FAS), and No FAS. Groups were compared on the prevalence of specific additional features and number of additional features, stratified by gender and age. Of 307 children and adolescents screened, 87 received the dysmorphology evaluation. Thirteen were classified as FAS, 44 Deferred, and 30 No FAS. The frequency of 10 of the 11 additional alcohol-related features did not differ significantly by FAS category. Palmar crease abnormalities were more common in FAS (53.8%) than in the Deferred category (25.0%) or the No FAS category (6.7%) (P = 0.003). A high prevalence across all groups was found for midfacial hypoplasia and epicanthal folds, whereas only one child exhibited ptosis. This study suggests that an FASD phenotype variant related to ethnic differences in the range of defects specific to prenatal alcohol exposure may be present in the Korean population.

Dear Sir, We describe two modifications to simplify cannulation and passage of fine feeding catheter through flexor sheath for closed irrigation of infective flexor tenosynovitis. Cannulating the flexor sheath and controlled passage of fine feeding catheter through the retinacular system can be difficult due to its pliability, often requiring multiple attempts and causing additional trauma to flexor tendons. Conventionally, irrigation solution is pumped out through the tip of the catheter and intrathecal lavage is dependent on the pressure jet and continuous flow [2]. The potential drawbacks of the latter are incomplete evacuation of infected material and tedious dressings. Other authors have described single and multiple fenestrations along catheter in human and veterinary patients [1, 4]. The advantage of side fenestration is the delivery of irrigation solution to sanctuaries such as the sub-chiasms and intertendinous interfaces. Based on the above techniques, we have modified the closed irrigation technique in two ways to ease cannulation and intrathecal lavage. In all cases, the flexor sheath is visualized and accessed through hemi-Brunner incision centered over A4/5 or A3/4, and a palmar crease incision centered on the A1. The first modification introduces an intraluminal 24-gauge wire to stiffen the tube for cannulation at either A3/4 or A4/5 and retrograde passage to the A1 window (Fig. 1). The second modification involves fenestrating the catheter along its middle portion to increase turbulence of intrathecal flow. During irrigation, the catheter is drawn back and forth, while an assistant flushes the tube after clamping its tip. The end point is the evacuation of clear fluid from the A1 window. Fig. 1 Middle segment of a feeding catheter is marked (black arrows) and fenestrated with a no. 11 blade. Intraluminal 24-gauge wire to increase stiffness of catheter to ease cannulation and manipulation of catheter We believe that the combination of mechanical lavage and fluid flow through the fenestrations increases direct clearance of intrathecal material as opposed to indirect methods that rely on continuous flow (Fig. 2). The catheter is removed after irrigation and postoperative irrigation is not practiced due to the lack of perceived benefits [3]. Fig. 2 Left to right. Direct irrigation—tip of catheter clamped to facilitate intrathecal flow of irrigation fluid (blue arrows), and oscillating motion (thick black arrows) to promote mechanical lavage. Indirect irrigation—flexor sheath is ...

Summary Objectives We conducted preliminary research to validate the insertion depth ranges of the five phase points by using ultrasonography. Design and setting The insertion depth ranges of the five phase points were investigated by reviewing acupuncture and moxibustion literature. The following lengths were measured in centimetres in order to substitute B-cun values with centimetre values: the distance from the cubital crease to the palmar crease, from the inferior border of the medial tibia condyle to the medial malleolus, and from the popliteal crease to the prominence of the lateral malleolus. The five phase points were represented by pen marks on the skin, per the World Health Organization acupuncture point specifications. The anatomical structures of the five phase points within the insertion depth ranges were observed using colour Doppler ultrasound. Results The five phase points for which the insertion depth ranges needed to be modified were LU11, HT9, PC9, LI1, LI2, SI1, SI2, TE1, ST45, BL66, GB44, SP1, SP2, LR1, and BL67. The five phase points that required careful insertion of acupuncture needles were LU8, LU9, LU10, LI5, ST36, ST41, SP3, HT4, HT7, HT8, SI3, SI8, BL40, KI2, KI3, KI10, KI27, GB34, GB38, LR3, and LR4. Conclusion This preliminary study provided evidence on the insertion depths in acupuncture treatment. To generate more concrete evidence regarding insertion depths, further research should be conducted in a participant group with more diverse body mass index (BMI) values by using acupoint-specific ultrasonography transducers that can measure the acupoints properly.

Purpose The aim of this study was the examination of the superficial anatomy of palmar creases and their relation to deeper neuro-vascular structures. Methods Four creases: distal wrist flexion crease, thenar crease, proximal palmar crease and distal palmar crease were evaluated with reference to the following structures: palmar cutaneous branch of median nerve, palmar cutaneous branch of ulnar nerve, the nerve of Henle, transverse palmar branches from ulnar nerve, recurrent motor branch of median nerve, radial proper palmar digital nerve to the index and the ulnar proper palmar digital nerve to the thumb, Berrettini’s communicating branch, ulnar nerve and artery, superficial palmar arch. We performed dissections of 20 cadaveric upper limbs derived from a homogenous Caucasian group. In our study we measured the location of surgically important structures with reference to palmar skin creases. Results Among the other observations we noticed that the palmar cutaneous branches of the median and ulnar nerves were located at least 0.5 cm away from the thenar crease. The superficial palmar arch was found between the thenar and proximal palmar crease and never crossed the proximal or distal palmar creases. Conclusions These anatomical dissections will provide reference material for further ultrasound studies on the arrangements of neuro-vascular structures in reference to superficial palmar creases.