Figure 3 - uploaded by Eman Al-Sharif
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Case 3. (a)–(c) preoperative clinical and radiological appearance – note the shoulder dislocation; (d) and (e) postoperative clinical and radiological appearance at age 4 years. Note the poorly defined volar creases.
Source publication
Thumb reconstruction in mirror hands is usually done by pollicization. However, objective pinch strength and power grip data in mirror hands following pollicization are lacking. Alternative thumb reconstruction techniques include doing nothing, rotation osteotomy or syndactylization of the radial digits. In this article, we report a series of four...
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To evaluate the outcome of 20 claw hands corrected with the Zancolli lasso procedure.
20 patients aged 7 to 48 (mean, 28) years with claw hand deformity for a mean of 33.3 months secondary to leprosy (n=17), traumatic ulnar nerve injury (n=2), or tardy ulnar nerve palsy (n=1) underwent the Zancolli lasso procedure, in which the flexor digitorum sup...
Citations
... The surgery for correction of this anomaly includes excision of supernumerary digits, after preserving 1 digit for thumb reconstruction [7] . Various surgical procedures have been employed to repair the thumb, ranging from doing nothing to formal pollicization [10] , which may necessitate numerous complicated and repetitive surgeries. Because mirror hand defects are dynamic in nature, functional and anatomical alterations may occur even after a complete restoration [3] . ...
Introduction:
Mirror hand is an extremely rare congenital abnormality characterized by polydactyly and duplication of the ulna, with the absence of the radius and thumb. Atypical presentations of mirror hand were described, including the presence of the radius in a few cases; here the authors report one of the atypical cases of mirror hand that underwent successful management.
Case presentation:
A 2-year-old and 7-month-old female child presented with 7 well-developed digits, with an absent thumb; the X-ray imaging of the forearm showed a well-formed ulna and radius with proximal fusion. The patient has good shoulder movement, minor limitations in supination and pronation, and elbow flexion restriction. The patient underwent multiple surgical interventions for pollicization. Follow-up revealed a significant improvement of hand function and appearance.
Clinical discussion:
In the literature review, mirror hand is a rare congenital malformation and has many varieties. The management of this deformity is a challenge and differs from case to case; here the authors described a novel variant of this deformity and its successful management.
Conclusion:
Mirror hand is a rare congenital abnormality and has a wide spectrum of variants. The management challenge, but with early pollicization with appropriate functional considerations, the outcome is promising.
... A true pollicization was performed in most cases, with a pseudo-pollicization as the alternative technique (Barton et al., 1986b;Gropper, 1983). Other techniques, such as shortening metacarpal osteotomies, have also been done (Al-Qattan et al., 2013;King and Hoyes, 1982). Postoperative splinting can improve functional results (Pintilie et al., 1964;Winge and Bye, 2013). ...
Ulnar dimelia is a very rare unilateral congenital upper limb anomaly (CULA) affecting the whole extremity. Treatment remains difficult because of the complexity and multi-level involvement. Twenty-four cases with duplicated ulna, absent radius and polydactyly from seven European centres were reviewed according to a structured list of parameters. At first consultation, median age 8 months (1–178), the shoulder movement was good in 17 patients or poor in six, and the median passive elbow range of motion was 20° (0°–90°). The resting wrist position was flexed in 22/24 patients. Following stretching and splinting, elbow surgery included resection of the lateral proximal ulna in 11 patients and muscle transfers in six to improve passive movement and increase active elbow motion, respectively. Tendon transfers were performed in eight wrists and a pollicization or pseudo-pollicization in 23 patients. Overall, patients demonstrate acceptable function postoperatively. Guidelines for treatment of this severe CULA are presented.
Level of evidence: IV
... Mirror hand deformity is classified into two main types according to the bones of the forearm: The classic type (also known as ulnar dimelia) in which the forearm contains two ulnae and the non-classic type in which the forearm contains a radius and one or two ulnae [1]. ...
Congenital hand and forearm anomalies pose a unique challenge in plastic and pediatric surgery. We present a case report of an 8-months-old girl with a congenital left sided hand and forearm anomaly, provisionally diagnosed as atypical left mirror hand anomaly. Classically there is absence of radius and duplication of ulna; however, our case had normal radius and ulna and a hand with seven digits arranged in two groups. We did a surgery which involved a ray amputation of the finger ulnar to the most radial digit, aiming to preserve an adequate first web space to reconstruct the thumb. The result of the surgical treatment in both functional and cosmetic aspects was, in authors’ opinion, good.
... In some other cases, there is scapular hypoplasia, recurrent glenohumeral dislocations, hypoplasia of the capitellum in the forearm articulating with 2 bones of ulnar morphology described in corpses as rotated between 70 °and 180 °from each other. These changes generate biomechanical alterations in the elbow joint, which result in limitation of the normal range of motion [7] . ...
Ulnar dimelia is an infrequent congenital anomaly of the upper limb characterized by the duplication of the ulna, absence of the radial ray, and polydactyly. We report on 2 cases of young girls with upper limb abnormalities who were brought to our Advanced Imaging Department for specialized imaging evaluation. The first case is a 15-month-old girl with a typical manifestation of ulnar dimelia. X-ray images of her right forearm and hand showed 2 ulnar-like bones, absence of the radial ray, and post-axial polydactyly. Our second case is a 7-year-old girl with evident limited pronosupination and shortening of the left forearm. Findings from computed tomography evaluation included ulnar duplication, one of which had a bifid aspect, with characteristics that are similar to ulnar proximal and distal epiphysis. The latter case which, to the best of our knowledge, has not been reported in the literature, is a rare variant of ulnar dimelia, that we have called ulnar trimelia . In this contribution, we emphasize the importance of an adequate diagnostic imaging approach for accurate recognition of upper limb anomalies, so that the treatment selection is appropriate to achieve better health outcomes and improve patients' quality of life. Our descriptions and images presented in this contribution form the basis for further research.
... Several procedures have been described to improve elbow flexion. Subperiosteal resection of the proximal portion of the lateral ulna may permit the medial forearm to flex and extend at the residual-medial ulnotrochlear joint [68,72] (Figs. 21.5 and 21.6). ...
... Surgery of the wrist is designed to address the flexed and deviated posture of the hand and establish active wrist extension. Procedures to release the flexion deformity and to augment wrist extension include palmar skin z-plasty, fractional lengthening of the flexor carpi ulnaris tendons, volar capsulotomy, dorsal capsule plication, extensor tendon shortening, and proximal row carpectomy [68,72]. Tendon transfer can confer active extension of the wrist. ...
... The transfer may be inserted into the extensor carpi radialis, if present, or directly into the second metacarpal. Donor motors include the flexor carpi ulnaris or residual flexor or extensor tendons from amputated fingers during digital reduction surgery [51,54,59,68,72]. Though wrist arthrodesis can help position the wrist and improve grip strength, it should be reserved for extreme cases not amenable to contracture release and tendon transfers [48]. ...
Ulnar polydactyly is the most frequent form of hand polydactyly, while ulnar dimelia represents one of the most unusual forms of congenital duplication. Ulnar polydactyly is common in affected families and in individuals of African ancestry. Ulnar dimelia is sporadic. Both of these conditions demonstrate variable pathologic anatomy due to failure of formation or differentiation of the anterior-posterior axis of the upper limb. Ulnar polydactyly may occur as an element of several congenital syndromes. Ulnar polydactyly and ulnar dimelia may be diagnosed prenatally. Evaluation of affected children should consider the possibility of associated abnormalities. Surgical reconstruction of the affected extremity aims to normalize appearance and maximize functional capabilities.
... Ulnar dimelia, or known as mirror hand syndrome, is a rare congenital deformity of the upper limb with only three cases reported in Saudi Arabia and around 70 worldwide. [1][2][3][4][5][6][7] It is characterized by a duplication of the ulna, absence of the radius, and thumb with polydactyly (usually seven or eight digits). Apart from the skeletal deformity, there might be malformation of the arteries and nerves of the forearm, secondary to the skeletal deformities such as duplication of the ulnar nerve, duplication of the ulnar artery, or absence of the radial artery. ...
... The management, in our case, was similar to a case that has been successfully managed by Al-Qattan et al. by excision of the olecranon of the preaxial (the lateral) ulna. [1] Another case that has also been managed by Al-Qattan et al. did not require any surgical procedure of the patient's elbow and achieved great improvement with physiotherapy only. [1] Furthermore, a third case managed by Rabah et al. by excision of part of the upper end of one of the ulnae resulted in an excellent outcome. ...
... [1] Another case that has also been managed by Al-Qattan et al. did not require any surgical procedure of the patient's elbow and achieved great improvement with physiotherapy only. [1] Furthermore, a third case managed by Rabah et al. by excision of part of the upper end of one of the ulnae resulted in an excellent outcome. [2] However, shoulder, wrist, and elbow stiffness are the major problems in this disorder as they are difficult to manage and improve. ...
... Classical congenital mirror hand, also known as ulnar dimelia, 1 consists of an absent radius, ulna duplication and polydactyly with 7-8 digits symmetric around the midline. 2,3 It is an extremely rare deformity with only a few dozen case reports described in the worldwide literature. ...
... 2,3 It is an extremely rare deformity with only a few dozen case reports described in the worldwide literature. [1][2][3][4][5] Reconstruction consists of creating unusually oriented skin flaps, osteotomies, ray transpositions, and amputations. Such procedures present the surgeon with significant challenges: the geometry of these flaps may be unfamiliar, the vascular supply may be aberrant, and the bony anatomy may be highly unusual. ...
Mirror hand is an extremely rare congenital anomaly. We modeled and simulated the reconstruction of mirror hand in a 2-year-old boy utilizing a preoperative 3-dimensional model to aid surgical planning. A soft-tissue hand model was created using preoperative imaging, 3-dimensional printing, and silicone casting, and the model was used to perform trial surgery. Given the complexity of the deformity, the model greatly facilitated the final operative plan for reconstruction.
... As illustrated in table 1, among the research reports which were published in peer-reviewed journals, nine different journals belonging to different medical disciplines were published (10)(11)(12)(13)(14)(15)(16)(17)(18). Among the published reports, one was a case series and one was a mixed methodology report, and the remaining were cross-sectional studies. ...
Medical students at King Saud University (KSU) conduct research projects during their undergraduate study. However, only a minority of research projects reports are disseminated. This study aims to evaluate KSU medical students research projects conducted during 2011 and 2012, and to assess the association between research reports' quality and publication propensity. Methods: This is a case-control study where cases were defined as published reports and controls were defined as unpublished reports. 25% of the research reports submitted in 2011 and 2012 were targeted for this study. The selected studies were critically and independently appraised by two reviewers. Results: Twenty-six research reports were included in this study, where nine reports were published in peer-reviewed journals. All of the included studies were observational, whereas most of the conducted studies were cross-sectional. Quality scores given by each reviewer for each evaluated study were highly correlated (Pearson correlation coefficient = 0.897, p-value < 0.01). The average quality score for published report is 39.3, which is similar to the average quality score of non-published reports (38.5). There is no statistically significant difference in the means of average quality scores of included studies, neither according to the publication status nor the study year. Conclusions: These findings suggest that the propensity of a student's research report being accepted for publication does not necessarily rely on the quality of the project report, and that other factors are likely to impact the dissemination rate.
BACKGROUND: The most rare and extremely rare malformation of the upper limb is polymelia, i.e., doubling (triple) limb. In the literature, no more than 20 cases of an increase in the number of lower limbs and only three cases of doubling (triple) of the upper limb (in humans) have been described. CLINICAL CASE: A 2-year-old child diagnosed with polymelia of the right upper limb was examined and treated at our clinic. Clinical examination revealed two shoulder blades and one clavicle, with a normal anatomical location and development. Behind, an upper limb ended with the stump of the forearm, represented by an underdeveloped ulna. Moreover, the elbow joint was in a state of flexion contracture at an angle of 170, and the ulna was turned posteriorly and 1520 medially. The anterior humerus in the proximal section was underdeveloped, and in the distal section, it articulated in a normally formed elbow joint with the radius and ulna. The hand had five fingers with moderate underdevelopment and deformity of the 5th finger. After examining the bone, muscle, and vascular anatomy of the doubled limb, surgery was performed; as a result, a nearly complete anatomical and functional limb was reconstructed from the two segments of one upper limb. DISCUSSION: Polymelia is an extremely rare variant of a congenital anomaly of the limbs; thus, only a few cases are reported in the literature, which differs significantly from each other in clinical manifestations. Many questions related to the etiology and pathogenesis of these malformations are unexplored. The doubling of the limbs in animals is caused by both teratogenic environmental factors and genetic mutations. In any case, reconstructive surgery makes it possible to effectively treat children with such anomalies in the development of the limbs and obtain good results. CONCLUSIONS: The restoration of the upper limb of children with polymelia is quite difficult, but doable. Adequate analysis of all examination data and rational planning of surgical intervention in such cases creates conditions that enable achieving the maximum anatomical and functional results of upper limb reconstruction in children with this developmental anomaly.
