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Results for “Hand Deformities”

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At least 937 records · Page 52Linked to original sources
PubMed2002

Congenital bilateral perisylvian syndrome associated with congenital constriction band syndrome.

We report a 7-year-old boy with congenital bilateral perisylvian syndrome and congenital constriction band syndrome. The former is a congenital neurologic syndrome characterized by pseudobulbar palsy, mental retardation, epilepsy, and bilateral perisylvian polymicrogyria The latter is a malformative disorder with digital ring constrictions and amputations, probably caused by early amnion rupture resulting in entanglement of fetal parts by amniotic strands. We believe that the combination of these two malfornative disorders was not coincidental; instead, fetal circulatory disturbance related to chronic abruptio placentae could account for this combination.

Amniotic Band Syndrome↗
PubMed2004

Correlation between upper limb functional ability and structural hand impairment in an early rheumatoid population.

OBJECTIVE: To explore the relationship in individuals with early rheumatoid arthritis (RA) between self-report upper limb function, therapist-assessed upper limb function and therapist-assessed measures of structural impairment (handgrip, active hand motion and metacarpophalangeal (MCP) joint ulnar deviation). DESIGN: Thirty-six patients with early RA were recruited across seven outpatient occupational therapy departments. OUTCOME MEASURES: Upper limb functional activity and ability was measured using the Disability of the Arm, Shoulder and Hand (DASH) questionnaire and the Grip Ability Test (GAT). Upper limb impairment was assessed by bilateral power handgrip using the MIE Digital Grip Analyser, goniometry measures of bilateral metacarpophalangeal (MCP) joint ulnar deviation and bilateral active motion of the wrist. RESULTS: Strong correlations (> 0.7) were seen between the self-report DASH questionnaire and the therapist-rated GAT assessment. Bilateral power handgrips were also strongly correlated with both functional assessments. Dominant ulnar deviation at the MCP joints demonstrated a weak correlation (0.3-0.4) with both self-report and therapist-rated functional ability and a weak to moderate. (0.1-0.5) correlation on the nondominant side. CONCLUSION: In this early RA population handgrip strength is an accurate indicator of upper limb ability. Ulnar deviation at the MCP joints shows only a weak to moderate association with upper limb functional activity and ability. Although the DASH and the GAT were strongly correlated, the DASH was a more discriminating measure than the GAT in assessing upper limb ability in this sample population.

Activities of Daily Living↗
PubMed1996

Magnetic resonance imaging in macromelia and macrodactyly.

We report five cases presenting with soft tissue and bone overgrowth that demonstrate the ability of MRI to establish a diagnosis in the absence of specific clinical features. Disorders included macrodystrophia lipomatosa, angiolipomatosis, Klippel-Trenaunay-Weber syndrome, blue rubber bleb naevus syndrome and one case of segmental limited hypertrophy. The MRI appearances, and other radiological features of these conditions are discussed. MRI is recommended in all cases of macrodystrophy when the clinical features and plain film findings are indeterminate.

Adult↗
PubMed2001

Limb deficiencies in newborn infants.

OBJECTIVE: The prevalence rate of all types of limb reduction defects in general and those that potentially are caused by vascular disruption in particular is needed to provide a baseline for the evaluation of infants who are exposed in utero to teratogens that cause vascular disruption. The objective of this study was to determine this prevalence rate. METHODS: All infants with any limb deficiency among 161 252 liveborn and stillborn infants and elective terminations were identified in a hospital-based Active Malformations Surveillance Program in Boston in the years 1972 to 1974 and 1979 to 1994. An extensive search was made to identify infants who were missed by the Surveillance Program; an additional 8 infants (7.3% of total) were identified. The limb reduction defects were classified in 3 ways: 1) by the anatomic location of the defect, that is longitudinal, terminal, intercalary, etc; 2) for infants with absence/hypoplasia of fingers or toes, a tabulation of which digit or digits were affected; and 3) by apparent cause. RESULTS: The prevalence rate for all types of limb deficiency was 0.69/1000. The apparent causes included single mutant genes, familial occurrence, and known syndromes (24%); chromosome abnormalities (6%); teratogens (4%); vascular disruption (35%); and unknown cause (32%). CONCLUSIONS: A hospital-based surveillance program can be used to establish the prevalence of limb reduction defects, if ascertainment is extended to include elective terminations for fetal abnormalities. An apparent cause can be established for most limb defects when the clinical findings are used rather than reliance only on the International Classification of Diseases, Ninth Revision, codes of the discharge diagnoses. The prevalence rate of limb reduction defects as a result of presumed vascular disruption was 0.22/1000.

Amniotic Band Syndrome↗
PubMed2004

Mandibular widening by distraction osteogenesis in the treatment of a constricted mandible and telescopic bite.

OBJECTIVE: Documentation of the application of mandibular widening by distraction osteogenesis and orthodontics. PATIENTS: Three patients with telescopic bite resulting from an extremely constricted mandible related to hypoglossia-hypodactyly syndrome. INTERVENTION: Mandibular widening by distraction osteogenesis using an extraoral device and subsequent orthodontic treatment. RESULTS: The extremely constricted mandible and telescopic bite were dramatically improved by mandibular widening using distraction osteogenesis and subsequent orthodontics. Two of the three patients had transient complications; one reported temporomandibular joint pain and the other showed evidence of periodontal damage. CONCLUSION: Mandibular widening by distraction osteogenesis is an effective technique for the treatment of telescopic bite resulting from an extremely constricted mandible.

Child↗
PubMed1995

Craniosynostosis with joint contractures, ear deformity, cleft palate, scoliosis, and other features.

A case of craniosynostosis with joint contractures, ear deformity, cleft palate, scoliosis, and many other features is presented. The patient's skull was round with craniosynostosis of the coronal suture. The fingers were slender and long; the finger joints were contracted. Trismus was also present. Contracture of the temporomandibular joint was suspected. The helices were flat, antihelices were minimal, and the ears protruded. Furthermore, the facial and cranial features of this patient included mild hypertelorism, ocular proptosis, short pons nasi, flat radix nasi, mild retrognathia, and small oral fissure. In the oral cavity, a relatively wide cleft extended from the soft palate to the uvula. A frontal chest radiograph revealed a mild scoliosis. Differential diagnoses are discussed in reported syndromes with craniosynostosis.

Abnormalities, Multiple↗
PubMed2002

Bone-lengthening for symbrachydactyly of the hand with the technique of callus distraction.

BACKGROUND: Bone-lengthening in the hand and foot is a relatively new application for distraction osteogenesis. We present the operative treatment and postoperative outcome for four patients with M ller type-D symbrachydactyly of the hand who underwent metacarpal lengthening with use of a distraction device to establish pinch function. METHODS: Four patients who underwent distraction osteogenesis for the treatment of congenital symbrachydactyly of the hand were evaluated over a thirteen-year period. The nondominant right hand was treated in two patients, and the nondominant left hand was treated in the other two. The patients included three boys and one girl; all patients had the operation between the ages of five and eleven years. Distraction osteogenesis was performed on the fifth metacarpal in one patient and on the fourth and fifth metacarpals in the remaining three, in whom both bones were lengthened simultaneously with use of a single device. Postoperative bone elongation was analyzed with radiographs made at the time of removal of the distractor. The sensory function of the treated fingers and any growth disturbance of the distracted bones were evaluated. RESULTS: The mean duration of distraction was 37.3 days (range, thirty-two to forty-nine days), and the distractor was removed at a mean of eighty-four days after surgery. The bones were lengthened by a mean of 22.3 mm (81.6% of their original length) at a rate of 0.6 mm/day. Pinch function was improved in all patients. CONCLUSIONS: On the basis of our limited experience, we found that distraction osteogenesis of the metacarpals was an effective technique for the establishment of pinch function. We also found that an intramedullary Kirschner wire could maintain the alignment of the osteotomized bone. Although distraction requires a longer treatment period, it is apparently more effective than bone-grafting in terms of achieving adequate bone length. Simultaneous lengthening of two metacarpals also was found to be an effective technique.

Child↗
PubMed2004

Advanced Hirayama disease with successful improvement of activities of daily living by operative reconstruction.

Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is a benign and non-progressive motor neuron disease. Application of a cervical collar is believed to prevent progression of symptoms in the early stages, but there is no effective therapy for the advanced disease. We found that tendon transfer improved the activities of daily living (ADL) of a patient with advanced Hirayama disease. An operative reconstruction can be valuable, even in patients with Hirayama disease who have developed impaired ADL due to extensive intrinsic hand muscle atrophy.

Activities of Daily Living↗