Flexion Deformity Of Fingers Research Articles

Airway hitches and management in Maroteaux-Lamy syndrome.

Dear Editor, Maroteaux-Lamy Syndrome (mucopolysaccharidosis type VI [MPS VI]) is caused by deficiency of N-acetylgalactosamine-4-sulfatase resulting in accumulation of dermatan sulfate which evokes the varied spectra of clinical symptoms.[1] Dysmorphic facial features with multi-systemic involvement pose a formidable challenge to anesthetists. We present a case of an 8-year-old female child with progressive bilateral flexion deformity of fingers, mouth breathing and snoring. She was posted for cervical (C1-C2) fixation surgery. Child had coarse facial features, macrocephaly (head circumference: 60 cm), macroglossia, short neck (thyromental distance: 4.5 cm, sternomental distance: 7 cm) and restricted neck mobility [Figure 1a]. Our plan A was C-MAC videolaryngoscopic (C-MAC®VL)-guided intubation with backup plans of fiber-optic bronchoscope (FOB)-guided intubation, retrograde intubation, and front of neck access as plans B, C, and D respectively. Consent of the mother was obtained. ASA standard monitors were attached. Continuous high flow nasal cannula (HFNC) oxygen therapy was started [Figure 1b]. Fentanyl 2mcg/kg (IV) was given and inhalational induction with sevoflurane was started. There was difficulty in mask ventilation even after oral airway insertion. So, immediate insertion of supreme laryngeal mask airway (LMA) size-2 enabled adequate ventilation [Figure 2a]. Injection rocuronium 1 mg/kg (IV) was administered. LMA was removed and endotracheal intubation was performed using C-MAC®VL at second attempt using bougie. Apneic oxygenation with HFNC and manual in-line stabilization was ensured until endotracheal intubation was achieved. When the patient was turned from prone to supine position after surgery, we noticed edema of the lips and tongue [Figure 2b]. Videolaryngoscopy revealed edema of posterior commissure which resolved with intravenous steroids and then extubated 24 hours later in ICU.Figure 1: (a) shows facial and airway profile in this child with MPS. (b) shows continuous HFNC therapyFigure 2: (a) Supreme LMA inserted to facilitate ventilation before endotracheal intubation. (b) Edematous tongue and lips noted when patient turned supine after surgeryMaroteaux–Lamy syndrome is a type of mucopolysaccharidosis characterized by learning difficulties, mental retardation, coarse facial features, frontal bossing, depressed nasal bridge, macroglossia, adeno-tonsillar hypertrophy, narrowed airway, short, stiff neck and temporomandibular joints leading to difficult airway.[2,3] Multi-systemic involvement includes obstructive or, restrictive lung disease, valvular heart disease and cardiomyopathy. Instability of the atlantoaxial joint requires special care during intubation to prevent further compression of the cervical spinal cord.[4] Difficulty in maintaining adequate ventilation may be due to obstructive and restrictive lung disease. Intraoperative hemodynamic instability may occur due to underlying cardiac disease. Prone position can further aggravate existing airway narrowing as it can lead to airway edema which may pose difficulty during extubation. In our case, we chose inhalational induction over intravenous induction as it was anticipated difficult airway. We could overcome the difficulty of mask ventilation by expeditious insertion of LMA. C-MAC®VL-guided intubation was chosen over the fiber-optic technique as it provides fixed wide view of the glottis that aids in quick recognition of airway landmarks.[5] FOB-guided intubation would be difficult as the patient had poor comprehension and, airway collapse leads to difficulty in maneuvering and advancement. HFNC oxygen therapy prevented desaturation. Thus, from this case, we infer that in a child with mucopolysaccharidosis, inhalational induction, ventilation with LMA followed by intubation with C-Mac videolaryngoscope would be a logical method of airway management. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Repair of severe flexion contracture deformity of adjacent fingers with trifoliated flap from toe web in six patients

From May 2014 to January 2018, 6 patients with severe flexion contracture deformity caused by palmar cicatricial hyperplasia of adjacent 2 or 3 fingers were admitted to the Yidu Central Hospital of Weifang. There were 4 males (8 fingers) and 2 females (5 fingers), aged 13-51 years.The degrees of flexion of proximal interphalangeal joint of affected fingers and metacarpophalangeal joint were respectively 60-90°and 60-80°, and the total degree of passive extension of metacarpophalangeal joint, proximal interphalangeal joint, and distal interphalangeal joint was from -180 to -120°. After scar resection, tendon release, and extension of affected fingers, bone and/or tendon were exposed, and the wound area was 4.0 cm×2.0 cm-8.5 cm×4.0 cm.The wound was repaired with trifoliated flap composed of fibular flap of great toe, abdominal flap of the second toe, and plantar metatarsal flap, with flap area of 4.5 cm×2.5 cm-9.0 cm×4.5 cm.The plantar metatarsal wound of the donor site was sutured directly, and the remaining wound was repaired with full-thickness skin graft from the outside of the thigh. All the wounds of patients were healed. All flaps survived and had good blood circulation without vascular crisis after operation.During follow-up of 6 months to 2 years, the flaps were with good appearance, no swelling, similar in color to the surrounding skin, soft in texture, good in sensations of touch and pain, and the distance of discrimination of 6-8 mm. The active and passive flexion and extension of the affected fingers were good, and there was no dysfunction in the donor site of foot, only with slight scar. The article showed that trifoliated flap from toe web is one of the best methods to repair the severe contracture flexion deformity of fingers caused by severe cicatricial hyperplasia of palmar side, especially the soft-tissue defect of the skin after the cicatricial hyperplasia of adjacent 2 or 3 fingers is removed.

Fixed Flexion Deformity of the Middle and Ring Fingers in Adult Caused by Intramuscular Hemangioma of the Forearm: A Case Report

Intramuscular hemangioma (IMH) of the upper extremity is extremely rare and mostly found in children. The authors presented a 39-year-old female with an unusual case of fixed flexion deformity of the middle finger and ring finger with deep forearm pain for three years. The patient was diagnosed with IMH of the flexor digitorum superficialis muscle. Excision of the IMH was performed and intra-operatively passive extension of PIP joints was achieved. After 12 months follow-up, the patient was satisfied with the outcomes with no recurrence of flexion contracture. IMH of the upper extremity is an uncommon disease, especially in adults. It should be considered in patient who presented with deep forearm pain and finger flexion deformity. Early investigation should be performed and surgical removal remains the treatment of choice. Furthermore, tendon transfer should be reserved in case of functional deficits. Keywords: Intramuscular hemangioma, Flexion contracture, Forearm, Soft tissue tumor

Atypical musculoskeletal manifestations of gout in hyperuricemia patients.

Deposition of tophus is a common feature in chronic gout; however, signs and symptoms are not always well-pronounced in cases of uncommon sites. We report a case series of three patients, one with an acute locked knee suspected to be meniscus tear or intra-articular loose body, a second case with a lump on the dorsal hand suspected to be a benign lesion, and a third case with a finger flexion deformity with carpal tunnel syndrome; all three cases were later on found out to be tophaceous gout. White chalky monosodium urate crystal deposition was found to be the culprit of the symptoms of the patients.

Identification of Critical Region Responsible for Split Hand/Foot Malformation Type 3 (SHFM3) Phenotype through Systematic Review of Literature and Mapping of Breakpoints Using Microarray Data.

Split hand/foot malformation (SHFM) is a limb malformation with underdeveloped or absent central digital rays, clefts of hands and feet, and variable syndactyly of the remaining digits. There are six types of SHFM. Here, we report a boy with SHFM type 3 having normal 4th and 5th digits, absent 2nd and 3rd digits, and a 4th finger flexion deformity, as well as absent 2nd, 3rd and 4th toes bilaterally. His father, two paternal uncles, and two paternal first cousins have similar phenotype. Chromosome analysis showed a normal male karyotype. A 514 kb gain at 10q24.31–q24.32 (chr10:102,962,134–103,476,346, hg19) was identified using 6.0 Single nucleotide polymorphism (SNP) microarray, resulting in the duplication of nine genes, including BTRC and FBXW4. A detailed systematic review of literature and mapping of breakpoints using microarray data from all reported cases in PubMed and DECIPHER were conducted, and exon 1 of BTRC gene was identified as the critical region responsible for the SHFM3 phenotype. The potential mechanism and future studies of this critical region causing the SHFM3 phenotype are discussed.

Dupuytren’s contracture of hand and its medical and social review

IntroductionDupuytren's contracture is a disease of connective tissue with the affection of palmar aponeurosis, frequently with chronic recurrent course, the flexion deformities of fingers and dysfunction of the hand. It composed 11,8 % among of all types of the hand pathology. The mostly it develops in men of middle-aged and elderly. At young age it occurs more rarely, but with fast progressing current, which in 67 % of cases leads to impaired ability to perform professional hand-work and in 3 % patients to physical inability. Predisposing factors for this fibromatosis are numerous, but one of the popular opinion among of authors is a hereditary character of disease. In clinical practice, the most frequently used fourth degree’s classification of Dupuytren's contracture includes severity of deformation and function disorders of the hand. It is correspond to the normative standards of medical and social expertiseThe aim of research: to specify the most optimal methods of surgical treatment, the criteria of medical and social examination and rehabilitation, clinical and labor prognosis, indications to establishment of disability after operations concerning of Dupuytren’s contracture.In our clinic was performed three methods of operation: aponeurotomia “open hand”, partial aponeuroectomia, total aponeuroectomia. Errors in the treatment of palmar fibromatosis we shared on: preoperated, intraoperated and associated with violations of the mode of rehabilitation. The next clinical observation of 68 patients (up to 3 years after surgery) with Dupuytren’s contracture of hand showed the recurrence rate amounted to 31,7%, and physical inability level is 5,9% of cases. In our opinion the most optimal operative methodic is total aponeuroectomia with rational surgical approaches.Clinical and labour prognosis depends on the manifestations of disease which can develop regardless of the stage, methods of treatment and patient's age. Disadvantageous prognostic factors include: the formation of keloid scars, persistent development of contractures and arthritis of the joints of fingers. For the expert diagnosis, the most important are functional disorders such as the capture and retention of items. Other types of disorders are less important. The restriction of mobility little joints with vicious position of fingers of the hand limit the ability to work and ability to self-service. The case of the bilateral affected leads to disability group III. At carrying out of medical and social examination and its conclusion, it is necessary to take into account the age, concomitant pathology, compensatory abilities of the patient, as well as the dynamics of recovery function of the hand after correction.Conclusions:Dupuytren’s contracture is the complex orthopedic pathology that can be interpreted as dysplasia of connective tissue. It makes 11,8% of all diseases of hand and affects mainly men of the middle age.In clinical practice is most often applied four-degree’s classification considering severity of deformation and function disorders of hand, which needs clarification of criteria for medical and social examination.The optimal method of Dupuytren’s contracture treatment is operative – the total aponeuroectomia, however the quantity of unsatisfactory outcomes reaches 13 % of cases, and cases on physical inability up to 3 %. According to our clinical observation of the 68 patients (up to 3 years after surgery) the recurrence rate amounted to 31,7%, and physical inability level is 5,9% of cases.In our opinion, aponeuroectomia should be regarded as complex microsurgical operation, by using rational approaches and obligatory plastic substitution of the defects of the skin.The basic criteria of the clinical and labor prognosis and medical and social examination are characters of morphological changes and a degree of the basic functions disorders of hand. They reduce work capacity, quality of life, and at bilateral affection results to ІІІ group of physical inability and to 1-st degree of disability. At conclusions of medical and social examination it is necessary to consider compensatory possibilities of the person and concomitant pathology, as well as its profession and age.

Mean Platelet Volume Seems To Be a Valuable Marker in Patients with Systemic Sclerosis

The predictors for the development of cardiovascular diseases and peripheral arterial diseases in patients with systemic sclerosis (SSc) were not clearly established, and there is no specific study conducted to investigate the mean platelet volume (MPV) levels in SSc patients. Therefore, this study evaluates the MPV levels in SSc and possible relationship between SSc, its clinical features and activity/severity scores, and MPV. In total, 76 SSc patients (67 women and 9 men, mean age 50.44 ± 13.21 years) diagnosed according to the classification criteria of the American College of Rheumatology and 45 healthy volunteers were enrolled into study. Data relating to anamnesis, physical examination, MPV, erythrocyte sedimentation rate, C-reactive protein levels, electrocardiography, echocardiography, high-resolution computerized tomography findings, complaints, and treatment processes were recorded into the database. Of the total cases, 17 had (22.3 %) cardiac involvement, 45 had gastrointestinal involvement (59.2 %), 47 had (61.8 %) lung involvement, 31 (32 %) had finger flexion deformity, and 27 (35.5 %) had digital ulcers at the fingertips. The mean MPV levels of SSc patients were significantly higher than those of the control group (p = 0.008). The mean MPV levels of SSc patients with cardiac involvement, digital ulcers, and gangrene presence were significantly high, and lower in Ilomedin-receiving patients than in the Ilomedin naives (p < 0.05). A negative relationship was discovered between the mean MPV levels, Valentini score, and Disease Severity Index of the patients with systemic sclerosis (p = 0.006, r = -0.310; p = 0.047, r = -0.229). MPV levels were significantly elevated in SSc patients and they were negatively correlated with disease activity scores. Increased MPV levels would be a predictive marker in the diagnosis of macrovascular and microvascular disease involvement in SSc patients.

Brachialis muscle transfer to the forearm for the treatment of deformities in spastic cerebral palsy

The use of the brachialis muscle for tendon transfers in cerebral palsy has not been described previously. In this study, the brachialis muscle was used for transfer in 11 patients with spastic cerebral palsy for the restoration of forearm supination, wrist extension, or finger extension. Four patients underwent brachialis rerouting supinatorplasty. Active supination increased in two (60° and 50°), minimally increased in one (5°), and did not change in one patient. Five patients had a brachialis to extensor carpi radialis brevis transfer. The mean gain in postoperative active wrist extension was 65°. Two patients with finger flexion deformity and no active metacarpophalangeal joint movement underwent a brachialis to extensor digitorum communis transfer, and they attained an improved posture of finger extension although their postoperative metacarpophalangeal flexion-extension movement arc was 5° and 25°. None of the patients developed any loss of active flexion at the elbow. Our preliminary experience suggests that the brachialis muscle may serve as an alternative tendon transfer in cerebral palsy.

Association Between Thr21Met and Ser89Asn Polymorphisms of the Urotensin II Gene and Systemic Sclerosis

Systemic sclerosis (SSc) is an autoimmune chronic fibrotic disorder. Urotensin II (U-II) is predominantly a vasoactive peptide with fibrotic and prothrombotic features. Like endothelin-1 (ET-1), U-II could play an important role in SSc pathogenesis. We evaluated the possible role of the U-II gene polymorphisms (Thr21Met and Ser89Asn) in the genetic susceptibility to SSc in a Turkish population. A total of 189 patients with SSc and 205 healthy controls were enrolled in our study. We analyzed the genotype and allele frequencies of the U-II (UTS2) gene polymorphisms Thr21Met and Ser89Asn in patients with SSc and in controls. We found that the Thr21Met polymorphism of the UTS2 gene was markedly associated with the risk of developing SSc (p < 0.0001), but there was no relationship between the Ser89Asn polymorphism and SSc (p > 0.05). Two haplotypes (MS and TS) were markedly associated with SSc (p < 0.05). There were significant associations between the genotype and allele frequencies of UTS2 gene Thr21Met polymorphism and cases with diffuse or limited SSc, systemic or lung involvement, finger flexion deformity, pitting scars at the fingertips, positive anticentromere, or positive antitopoisomerase 1 antibody groups. Our study shows the association between Thr21Met, but not Ser89Asn, in the UTS2 gene and SSc. The results strongly suggest that this single-nucleotide polymorphism may be an important risk factor in the development of SSc, and a powerful indicator of severe skin and lung involvement in patients with SSc.

Simultaneous reconstruction of a palmar skin defect and the digital artery with an arterialized venous flap after correction of camptodactyly with severe flexion deformity

When severe flexion deformities of fingers are corrected surgically, vascular circulation of the fingers is sometimes impaired. The poor perfusion of the digits is caused by inadvertent injuries, thrombosis formation, or vasospasm of the bilateral digital arteries of the fingers. If the vascular impairment is caused by inadvertent injury to the digital arteries, this injury should be repaired immediately using an operating microscope. Thrombosis formation and vasospasm are usually treated successfully by relaxation of the positional soft tissue tension on the affected fingers, irrigation of the wound or fingers by warm saline solution, bathing the digital arteries with local anesthesia, or application of vasodilators. Camptodactyly is a congenital anomaly of the fingers that demonstrates flexion deformities of the proximal interphalangeal (PIP) joints. Most of these deformities can be treated successfully by manipulation and splinting [1, 6, 15, 18]. However, some of them develop into severe flexion contracture of the PIP joints and demonstrate so-called pseudo-boutonniere deformities [flexion contracture of the PIP joint with hyperextension of the distal interphalangeal (DIP) joint] [8]. Surgery was performed on a 9-year-old girl to correct severe flexion contracture of the PIP joint of her right small finger resulting from camptodactyly. After extension of the finger to the neutral position, the finger failed to pink-up following release of the pneumotourniquet. This poor perfusion of the finger was improved when the PIP joint was flexed more than 40°. A skin flap including a subcutaneous vein was harvested from the distal forearm and the vein of the skin flap was interposed between the transected stumps of the palmar digital artery of the finger. The skin defect created after extension of the PIP joint was covered by the skin flap. The PIP joint was fixed temporarily at the neutral position for 5 weeks by a K-wire. The arterialized venous skin flap successfully survived without any adverse events. The finger having showed complete numbness just after surgery retuned to have normal sensation within 2 months. Nine years after surgery, the active arc of the PIP joint of the operated finger was −5° to 60°.

Dupuytren Contracture in a Patient with Congenital Camptodactyly and Incidental Carpal Tunnel Syndrome

Dupuytren contracture is commonly seen in northern European populations but not in Asians. Even more rare is a presentation of flexion deformity of fingers involving two different pathologies with incidental carpal tunnel syndrome in the same patient. We report herein a case of Dupuytren contracture with congenital camptodactyly and unilateral carpal tunnel syndrome.

Sarcoidosis Manifested by Gradual Flexion Deformity of Fingers of Both Hands

A 59 years old woman was first seen by us in December 1953 with chief complaint of gradual flexion deformity of fingers of both hands.The flexion position of the digits was diminished by palmar flexion of the wrist. The volar aspect of the right forearm was explored. The flexor digitorum profundus muscle was quite dark in color and tightened. Tendon transfers were done.The microscopic examination of the muscle revealed tubercle formation consisting of epitheloid cells without necrosis. The case was diagnosed as muscular sarcoidosis. Since then, the patient has been treated with cortisone.The flexion deformity of the left hand cannot be improved. On the other hand, all digits of the right hand can be fairly well extended.