Diagnostic value of chest computed tomography images in adult Poland syndrome: a report of two cases

Poland syndrome is a rare congenital developmental deformity characterized by unilateral agenesis or hypoplasia of thoracic wall soft tissue. We report two adult cases of Poland syndrome detected by computed tomography (CT) images. CT images of the two cases depicted an asymmetric chest wall with the absence of a breast and agenesis of the pectoralis muscles. A physical examination of case 1 showed a thin right chest wall with depression of the right nipple region. Hand deformities were also observed, including brachydactyly and syndactyly. In case 2, hand deformities were not found in a physical examination. Using multi-planar reconstruction, the size, position, origin, and termination of bilateral pectoral muscles could be compared symmetrically. For patients with Poland syndrome, a timely diagnosis and treatment are important. The use of chest CT in clinical practice could play an important role in the early diagnosis and treatment of Poland syndrome.

Correction of Hand Deformities after Burn

Hand burns can lead to deformities even after successful primary healing. They are the most common cause of skin contractures involving the hand. This review article discusses ways to correct claw deformity, flexion contracture in palm and finger, and web space contracture, which are post-burn hand deformities commonly encountered in clinical practice. Loss of skin is the end result in many causes of hand deformities after burn. Therefore, reinforcing the lost skin is the principle of corrective surgery. Even if the skin is thicker than the full-thickness skin, it will engraft if damage to the tissue and blood vessels of the recipient is minimized. The thicker the skin, the less is the re-contraction and growth. The foot is an ideal donor site for skin graft on the hand. In particular, the instep or the area below the malleolar is a very good donor site. First web space of the hand is very important for hand function, and it must be reconstructed with Z-plasty, skin graft, and free flap step by step according to the degree of contraction.

Considerations When Performing Arthrodesis in the Scleroderma Hand

Background Hand deformities secondary to scleroderma can limit activities of daily living and be associated with substantial disability. This study aimed to evaluate the outcomes following arthrodesis performed to treat digital contractures secondary to scleroderma. Methods We performed a retrospective review of all patients with scleroderma who underwent arthrodesis by a single surgeon from 2015 to 2020. We collected demographic information, operative variables, and outcomes variables. Our primary outcome was occurrence of any postoperative complication, which we defined to include wound dehiscence, digital ischemia, malunion, nonunion, cellulitis, and osteomyelitis. We calculated descriptive statistics and performed all analyses at the joint level. Results We identified 9 patients who underwent arthrodesis of 19 joints. All patients were women with a mean age of 55.3 years. At the time of surgery, most patients were taking disease-modifying antirheumatic drugs (DMARDs). Kirschner wires (K-wires) were used in most cases (n = 18), 15 of which were removed uneventfully at an average of 4.8 months after surgery. With a mean follow-up time of 15.4 months, the overall complication rate was 5.3% (n = 1). This patient developed digital ischemia in 1 of 4 operative digits, which became gangrenous and required amputation. Conclusions Our study suggests that arthrodesis can be performed safely in the scleroderma hand, even when patients are taking DMARDs. Given the uneventful K-wire removal in all joints and the high risk of exposure of buried hardware in this population, we recommend nonpermanent placement of K-wires. Hand surgeons may consider arthrodesis in the scleroderma hand before proceeding to revision amputation.

Postburn Hand Deformities: Principles of Management and Approach

The hand is one of the top 3 locations for burn scar contracture deformity. The functionality of the hands is one of the primary determinants of burns survivors’ quality of life. Although most burn abnormalities are preventable, they do arise when adequate treatment is not delivered in an emergency or when they are part of a major burn. Reconstructive surgeries can significantly improve hand function. For a burn’s survivor, the right methods and timing of surgery, followed by supervised physiotherapy, can be a game changer.

Poland’s syndrome mimicking traumatic chest injury

Poland’s syndrome (PS) is a rare developmental anomaly that can manifest mild (pectoralis muscles involvement) to severe deformities (rib hypoplasia and hand deformities). We report a case of 69-year-old man who presented to the emergency department with a traumatic chest injury after a fall. It was initially thought to have a significant chest injury as the trauma survey revealed a palpable defect and tenderness in the right anterior chest wall. There was also a symbrachydactyly deformity in the right hand. CT of the chest showed lack of right pectoralis muscles, which were consistent with PS. This case highlights the importance of gathering detail history in adult trauma patients such as congenital disorder especially in the presence of bony deformity. With possibilities of several traumatic conditions in trauma patients eliminated, one can expand the non-traumatic differential, keeping in mind the possibility of a congenital disorder that can mimic traumatic chest injury.

Comprehensive assessment of alterations in hand deformities over 11 years in patients with rheumatoid arthritis using cluster analysis and analysis of covariance

Background Although drug therapy for rheumatoid arthritis (RA) has recently improved, treating patients with established disease, whose hands have three major deformities (thumb deformity, finger deformities, and ulnar drift), remains a challenge. The underlying complex pathophysiology makes understanding these deformities difficult, and comprehensive assessment methods require accumulated skill with long learning curves. We aimed to establish a simpler composite method to understand the pathophysiology of and alterations in the hand deformities of patients with RA. Methods We established a rheumatoid hand cohort in 2004 and clinically evaluated 134 hands (67 patients). We repeated the evaluations in 2009 (100 hands of 52 patients) and 2015 (63 hands of 37 patients) after case exclusion. Thumb deformities, finger deformities (swan-neck and boutonnière deformity), and ulnar drift were semi-quantitated and entered as parameters into a two-step cross-sectional cluster analysis for the data in 2004. The parameters in each cluster were plotted at each evaluation point. Two-way analysis of covariance was used to examine whether differences existed between evaluation points and clusters of deformity parameters. Results Five clusters most appropriately described hand deformity: (i) cluster 1, minimal deformity; (ii) cluster 2, type 1 thumb deformity; (iii) cluster 3, thumb deformity and severe boutonnière deformity; (iv) cluster 4, type 2 or 3 thumb deformity and severe ulnar drift; and (v) cluster 5, thumb deformity and severe swan-neck deformity. Clusters 1 and 2 had higher function than cluster 5, and cluster 3 had moderate function. Clusters 1–4 had similar disease duration but showed different paths of deformity progression from disease onset. Clusters 1 and 2 represented conservative deformity parameters and clusters 3, 4, and 5 represented progressive deformity parameters. Over time, thumb deformity evolved into other types of deformities and swan-neck deformity worsened significantly. Conclusions Our comprehensive analysis identified five deformity patterns and a progressive course in the rheumatoid hand. Knowledge of the characteristics of progressive deformity parameters may allow rheumatologists to more easily implement practical interventions and determine functional prognosis.

Comprehensive assessment of alterations in hand deformities over 11 years in patients with rheumatoid arthritis using cluster analysis and analysis of covariance

BackgroundAlthough drug therapy for rheumatoid arthritis (RA) has recently improved, treating patients with established disease, whose hands have three major deformities (thumb deformity, finger deformities, and ulnar drift), remains a challenge. The underlying complex pathophysiology makes understanding these deformities difficult, and comprehensive assessment methods require accumulated skill with long learning curves. We aimed to establish a simpler composite method to understand the pathophysiology of and alterations in the hand deformities of patients with RA.MethodsWe established a rheumatoid hand cohort in 2004 and clinically evaluated 134 hands (67 patients). We repeated the evaluations in 2009 (100 hands of 52 patients) and 2015 (63 hands of 37 patients) after case exclusion. Thumb deformities, finger deformities (swan-neck and boutonnière deformity), and ulnar drift were semi-quantitated and entered as parameters into a two-step cross-sectional cluster analysis for the data in 2004. The parameters in each cluster were plotted at each evaluation point. Two-way analysis of covariance was used to examine whether differences existed between evaluation points and clusters of deformity parameters.ResultsFive clusters most appropriately described hand deformity: (i) cluster 1, minimal deformity; (ii) cluster 2, type 1 thumb deformity; (iii) cluster 3, thumb deformity and severe boutonnière deformity; (iv) cluster 4, type 2 or 3 thumb deformity and severe ulnar drift; and (v) cluster 5, thumb deformity and severe swan-neck deformity. Clusters 1 and 2 had higher function than cluster 5, and cluster 3 had moderate function. Clusters 1–4 had similar disease duration but showed different paths of deformity progression from disease onset. Clusters 1 and 2 represented conservative deformity parameters and clusters 3, 4, and 5 represented progressive deformity parameters. Over time, thumb deformity evolved into other types of deformities and swan-neck deformity worsened significantly.ConclusionsOur comprehensive analysis identified five deformity patterns and a progressive course in the rheumatoid hand. Knowledge of the characteristics of progressive deformity parameters may allow rheumatologists to more easily implement practical interventions and determine functional prognosis.

Possibilities of using microsurgical autotransplantation of tissue complexes in children

Background. Applications of traditional treatment methods in children with congenital and acquired pathology of the musculoskeletal system are often limited, as wound defects of significant area and depth with a soft tissue and bone deficit may occur during the reconstruction of the segment. Microsurgical techniques, including autotransplantation of blood-supplied tissue complexes, make it possible to realize the aims of musculoskeletal segment reconstruction and reduce surgical treatment time. Aim. This study aimed to perform a retrospective (statistical) analysis of using microsurgical autotransplantation of tissue complexes in children. Materials and methods. Treatment outcomes of 871 patients with congenital and development deformities of the musculoskeletal system who underwent 1048 microsurgical autotransplantations of various tissue complexes in 19842018 were analyzed. Complications associated with impaired blood supply to transplanted autografts, requiring revision microsurgical interventions, were also statistically processed. Results. The mean patient age was 5.8 years (range, 10 months to 17 years). In children with congenital pathology (n = 597), transplantation of blood-supplied tissue complexes in 85.9% of the cases was performed in cases with hand deformities. In 285 cases of acquired deformities, post-traumatic finger stumps accounted for 45.5%, scar changes of soft tissues for 39.6%, and other pathological conditions for 14.9%. Most of the microsurgical operations were toe-to-hand transfers, which accounted for 81.8% of the total number of surgeries. In 79.4% of the cases, the second toe was used for the toe-to-hand transfer. Accordingly, the remaining toes were transferred in 20.6% of the cases. When replacing soft tissue defects, a thoracodorsal flap was used in 84 cases, which was 5.6% (of the total number of autotransplantations), and a groin flap was used in 22 patients. To replace bone defects, a blood-supplied fibula graft was used in 47 patients and a metatarsal bone graft in 41 children. Circulatory disorders in the postoperative period were noted in 5.9% of the total operations, which in 3.1% of cases resulted in the necrosis of the transplanted autograft. Conclusion. The treatment outcomes of using microsurgical autotransplantation of blood-supplied tissue complexes in the reconstruction of tissues and segments of the musculoskeletal system confirm their high efficacy.

“Striatal Hand Signs” and early diagnosis of Parkinson’s Disease: The “Monkey-Wrench Sign”

Background: Striatal hand signs, first described by Charcot, are reported in 10% of advanced Parkinson’s Disease patients as flexion of the metacarpophalangeal joints, extension of proximal interphalangeal joints, flexion of distal interphalangeal joints and ulnar deviation. Another deformation is the metacarpophalangeal joints flexion and thumb medialization (U shaped - “Monkey-Wrench sign”). We believe that mild hand deformities are present in earlier phases of the disease and may help the diagnosis of PD. Objectives: To identify hand deformities in PD patient. Methods: We evaluated 36 PD patients, according to UKPDS Brain Bank Diagnostic Criteria, side of onset, disease duration, severity by UPDRS part III - left/ right side, hand deformities and the “monkey-wrench sign”. Results: We had 14 patients 10 years. 2/3 had left side onset. 33 patients (91.67%) presented the “Monkey- Wrench sign”, unilaterally or asymmetrically, and it was present in all PD patients > 4 years of disease onset, but it was present only in 78.6% less than 4 years. The “MW” sign was easily detected in the more affected side, with a mean UPDRS score of 31.1 on the side of striatal hand signs compared to the mean score of 24.7 on the less affected side. Conclusions: Striatal hand deformities and the “Monkey-Wrench” sign may be useful to diagnosis PD, if unilateral or asymmetrical signs, as well as, it could be used to differentiate between tremors of PD and other etiologies, in a more visual way.