Characteristics of patients with congenital clasped thumb: a prospective study of 40 patients with the results of treatment

Purpose  Congenital clasped thumb is a deformity that is associated with heterogeneous congenital anomalies and it has been addressed in many congenital syndromes. The aim of this study was to diagnose and evaluate cases of clasped thumb as regards the associated congenital anomalies and syndromes, and evaluation of the results of treatment of such cases. Methods  A prospective study on 40 patients with 73 clasped thumbs was done. All the patients’ data regarding their personal, family, pregnancy and developmental histories were recorded. All the patients were exposed to thorough clinical and radiological examination and genetic assessment. The cases were classified using the Tsuyuguchi et al. (J Hand Surg [Am] 10:613–618, 1985) classification into three types. Conservative treatment was adopted in ten hands, and surgical treatment was performed for 28 hands in 17 patients, with an average follow-up of 26 months. Results  Positive consanguinity was recorded in 57.5% of cases. Associated anomalies were recorded in 77.5% of cases. Type I was the most common one, followed by type III and then type II. Conservative treatment is effective in type I cases when presented early, and all patients were satisfied with the results of surgical treatment. Conclusions  We reported associated anomalies which are to our knowledge have not mentioned before in the literature which include; congenital blindness, radial deviation of the index finger and ventricular septal defect. We found that 68% of the patients had associated syndromes, and this has not been mentioned before. In this study, we found that there were no difference between type II and type III clasped thumb as regards the pathological findings, severity, the operative procedures, the treatment protocol and the operative results. Properly planned treatment gives satisfactory results.     

Surgical management of the Blauth 1 to 3A thumb hypoplasia

Thumb hypoplasia is a sequence of congenital abnormalities, from minor hypoplastic thumb with a preserved function to complete thumb aplasia. We only discuss in this chapter on the management of the thumb hypoplasia type 1 to 3A according to the modified Blauth's classification. Their common feature is the conservative treatment, in opposition with severe hypoplastic thumbs usually treated by index pollicisation. Whatever is the degree of hypoplasia, patients should be carefully examined for bilaterality, other anomalies and syndrome. Surgical treatment, when indicated, requires systematically widening of the first web space, stabilization of the metacarpophalangeal joint and restoration of opposition and extension. Reconstruction of the infant thumb should be started in the first year of life. Functional result depends on the degree of hypoplasia and differs dramatically whether if the thumb hypoplasia is isolated or associated with a radial club hand.     

Conduite à tenir devant une hypoplasie du pouce de type 1 à 3A

Thumb hypoplasia is a sequence of congenital abnormalities, from minor hypoplastic thumb with a preserved function to complete thumb aplasia. We only discuss in this chapter on the management of the thumb hypoplasia type 1 to 3A according to the modified Blauth’s classification. Their common feature is the conservative treatment, in opposition with severe hypoplastic thumbs usually treated by index pollicisation. Whatever is the degree of hypoplasia, patients should be carefully examined for bilaterality, other anomalies and syndrome. Surgical treatment, when indicated, requires systematically widening of the first web space, stabilization of the metacarpophalangeal joint and restoration of opposition and extension. Reconstruction of the infant thumb should be started in the first year of life. Functional result depends on the degree of hypoplasia and differs dramatically whether if the thumb hypoplasia is isolated or associated with a radial club hand.     

Surgical reconstruction of congenital thumb hypoplasia

This review article introduces a new classification of congenital hand anomalies. It then considers the classification of congenital thumb hypoplasia and describes the the authors indications for surgery. Specifically, attention is directed towards surgical techniques and problems encountered in the reconstruction of grade 2 hypoplastic thumbs and in pollicisation of the index finger for grades 3, 4 and 5 thumb hypoplasia.KEY WORDS: Congenital thumb hypoplasia, Surgical reconstruction     

Classification of congenital thumb hypoplasia and deformity

BackgroundCongenital thumb deformities account for one-third or more of all cases of congenital hand deformity. However, the current classification schemes of congenital thumb hypoplasia are no longer adequate due to their lack of adaptability to increasing knowledge in the field. Hence, a modified system with the potential to adapt to ongoing advances in knowledge and understanding is desperately needed.MethodsBased on the photographs collected from thousands of cases of congenital deformities of the hand and upper limb over multiple decades in our department, we subdivided thumb hypoplasia according to the variables of morphological characteristics, anatomical structures, functional status, the relationship between thumb deformity and hand deformity, the relationship between congenital hand deformity syndrome and thumb hypoplasia, and the selection of treatment methods.ResultsA total of 10 types were presented, which were elucidated with nomenclatures as well as pathological feature and symptoms.ConclusionThis modified system may shed additional light on the classification of congenital thumb anomalies, which will assist in a more effective selection of treatment modalities and offers significant benefits to both patients and practice.     

Thumb duplication and contralateral thumb hypoplasia in infant of mother with diabetes

Uncontrolled maternal diabetes frequently results in congenital anomalies. This report describes an infant of a mother with diabetes born with thumb hypoplasia and contralateral thumb duplication without other associated anomalies. Maternal diabetes should be part of the etiologic differential diagnosis of thumb anomalies. The concomitant occurrence of thumb duplication and hypoplasia, apparently as a result of the same systemic insult, suggests a close relationship between hypoplasia and duplication.     

Carpal coalition: a rare coincidence with hand deficiencies

Three cases in which congenital carpal fusion was associated with formation deficiencies of the hand are presented. In two patients with a lunate-triquetral and a capitate-hamate-trapezoid coalition, respectively, an ulnar deficient hand was also diagnosed. A subdivision of the first type of ulnar club hand deformities is proposed. Other skeletal anomalies included duplication of a phalanx and pseudoepiphysis of a metacarpal, respectively. In the latter case hypoplasia of the flexor tendons to the fingers, excluding the thumb, was also noted. In the third case, a proximal capitate-hamate coalition coexisted with aplasia of the flexor tendons to the middle finger.     

The spectrum of radial longitudinal deficiency: a modified classification

The records of 119 patients with 196 extremities with radial longitudinal deficiency seen between 1923 and 1996 were reviewed. We propose a global classification system that includes the spectrum of pathology affecting the radial side of the extremity, including deficiency of the radius, carpal abnormalities, and hypoplastic thumbs. Radial deficiency could be classified for 181 extremities of 104 patients using this classification system. Type N has a normal length radius and a normal carpus with thumb hypoplasia, type O has a normal length radius and radial side carpal abnormalities, type 1 has more than 2 mm shortening of the radius, type 2 has a hypoplastic radius, type 3 has a partial radius with absence of the distal physis, and type 4 has complete absence of the radius. All patients had thumb hypoplasia. Eighty-two percent of extremities with thumb hypoplasia but no deficiency of the radius that were available for carpal bone classification had carpal anomalies, including absence, hypoplasia, and coalitions. All the extremities with type 1 radial deficiency had carpal anomalies. Carpal abnormalities could not be determined for types 2, 3, and 4 deficiency because most had a prior centralization. Proximal radioulnar synostosis or congenital dislocation of the radial head was seen in 44% of extremities with type 1 radial deficiency. This classification includes carpal anomalies and thereby links isolated thumb hypoplasia and deficiency of the radius into one system.     

Autosomal dominant transmission of bilateral "opposable" triphalangeal thumb

Triphalangeal thumb occurs in two functional types: opposable and non-opposable. The opposable type presents a rudimentary middle phalanx and the thumb is frequently angulated. The non-opposable type presents a finger-like thumb (five-fingered hand). Triphalangeal thumb often occurs in syndromes where other anomalies are present, so its pattern of inheritance has often been described jointly with the syndrome itself, then conflicting patterns of inheritance have been reported in literature. The present study reports upon the character of "bilateral opposable triphalangeal thumb" occurring isolated, without association of other anomalies. Authors studied the phenotype distribution in a family over five generations. They found that this character is transmitted with an autosomal dominant pattern of inheritance, and affected individuals are heterozygotes.     

Innovative techniques in bony reconstruction to facilitate hand salvage

Mutilating injuries of the hand and congenital hand anomalies can present challenging reconstructive scenarios for salvage and restoration of function. During a 5-year period from 1993 to 1997, the plastic and reconstructive surgical unit of East Carolina University Medical Center was presented with a series of unique reconstructive challenges as a result of complex hand injuries that resulted in unexpected opportunities for the salvage of distal components. These traumatic injuries were unique in that, although devastating to the hand, they left the opportunity for salvage of distal vascularized and sensate components of the hand. Other unique challenges arose as a result of patients who did not want to pursue alternative reconstructive options such as toe-to-hand transfers or pollicization. These cases are presented to emphasize alternative algorithms to standard hand reconstruction in complex scenarios. Three patients presented with distal viable (vascularized and sensate) phalangeal components with proximal complex bony defects, 1 patient presented with a complex thumb defect and declined standard therapy, and 1 patient presented with a congenital thumb anomaly and declined standard therapy. All flaps survived and all hands were saved. These patients illustrate the clinical feasibility of osteocutaneous and free osseous grafting to provide strut stabilization in metacarpal defects and to preserve an opposable post after thumb amputation or thumb anomaly.     

示指拇化治疗重度拇指发育不全

目的 介绍示指拇化治疗先天性重度拇指发育不全(ⅢB～V型)的手术方法.方法 对Ⅳ型(漂浮拇)2例、ⅢB型(腕掌关节缺如)2例采用示指拇化,将示指自掌骨部位转位重建拇指.结果 4例移位指全部存活.术后随访2～3年,虎口开大70°～90°,接近健侧.拇指掌指关节屈曲后可与所有手指对指,近指间关节活动度从0°至100～120°,远指间关节活动度从0°至90°.指端两点分辨觉同健侧,外观和功能满意.结论 采用示指转位治疗重度发育不全的拇指,外形和功能满意,克服了以往采用皮瓣和骨瓣移植的缺点,是一种实用而可取的方法,值得推广.     

Triphalangies du pouce

Many teratogenic mechanisms converge to create a three-phalangeal thumb: duplication, fusion and deletion. Fine observation of the position of growth centres and of associated hand malformations suggests that the intermediate delta phalanx of the three-phalangeal thumb results of the fusion of a three-phalanx ray with a two-phalanx thumb. The ulnar or radial side of the deviation is determined by the radial or ulnar position of this three-phalangeal ray fusing with the thumb. An opposable three-phalangeal thumb ulnarly deviated with a radial intermediate delta phalanx is the result of the fusion of a duplicated Wassel VII type thumb composed by a radial three-phalangeal ray and an ulnar two-phalangeal ray. In split hands, the three-phalangeal thumb is most often radially deviated because it presumably results from the fusion of a radial two phalanx thumb with the index, deleted in this fusion. The aligned opposable three-phalangeal thumb is quite uncommon and is probably the result of a fusion of two Wassel VII-type duplicated three-phalangeal rays. Conversely, the five-fingered hand or non-opposable three-phalangeal thumb, usually has no deviation and could result of a thumb deletion associated to an index duplication. This understanding of three-phalangeal thumbs makes usual classifications obsolete, but explains the various pathologic associations found in literature and in our own series. Treatment varies with age and takes in account all fundamental aspects of this congenital hand anomaly: the extraphalanx, the clinodactyly due to a delta phalanx, the thumb duplication or the absence of opposable thumb.     

Three-phalangeal thumbs

Many teratogenic mechanisms converge to create a three-phalangeal thumb: duplication, fusion and deletion. Fine observation of the position of growth centres and of associated hand malformations suggests that the intermediate delta phalanx of the three-phalangeal thumb results of the fusion of a three-phalanx ray with a two-phalanx thumb. The ulnar or radial side of the deviation is determined by the radial or ulnar position of this three-phalangeal ray fusing with the thumb. An opposable three-phalangeal thumb ulnarly deviated with a radial intermediate delta phalanx is the result of the fusion of a duplicated Wassel VII type thumb composed by a radial three-phalangeal ray and an ulnar two-phalangeal ray. In split hands, the three-phalangeal thumb is most often radially deviated because it presumably results from the fusion of a radial two phalanx thumb with the index, deleted in this fusion. The aligned opposable three-phalangeal thumb is quite uncommon and is probably the result of a fusion of two Wassel VII-type duplicated three-phalangeal rays. Conversely, the five-fingered hand or non-opposable three-phalangeal thumb, usually has no deviation and could result of a thumb deletion associated to an index duplication. This understanding of three-phalangeal thumbs makes usual classifications obsolete, but explains the various pathologic associations found in literature and in our own series. Treatment varies with age and takes in account all fundamental aspects of this congenital hand anomaly: the extraphalanx, the clinodactyly due to a delta phalanx, the thumb duplication or the absence of opposable thumb.     

Surgical management of the hand in Freeman-Sheldon syndrome

Freeman-Sheldon syndrome is a rare form of distal arthrogryposis characterized by craniofacial anomalies, a rheumatoid-appearing hand, and pedal deformities. The hand deformities include ulnar deviation of the fingers, camptodactyly, first web space contracture, and hypoplasia of the thumb. Because of clinical variability and rarity, there is no standard management protocol. The authors have developed a systematic method of management of the hand in Freeman-Sheldon syndrome using principles commonly applied to other complex hand problems. In 17 years, the authors have evaluated nine patients. Of 18 hands evaluated, the authors have operated on 15. Good results were achieved in five hands and fair results in 10. There were no cases of useless hands. The authors have been most pleased with the results after crossed intrinsic transfers, centralization of extensor tendons, and intrinsic release of the thumb. Although physical deformities remain, functional adaptation is generally good.     

Classification and treatment of symbrachydactyly. A series of 117 cases

In the present study, a modification has been proposed of the Blauth and Gekeler classification, aimed at a more accurate definition of appropriate surgical treatment. An analysis was made of a series of 120 cases of symbrachydactyly (117 patients); however, surgery was only performed in 86 cases (51 toe transfers in 49 patients; mean age at surgery 12 months). Type I included the separation of short and sometimes stiff fingers; type II, the 'pseudo-cleft', could be subdivided into three groups. Type IIA included those hands with more than two long and frequently hypoplastic digits, regarding which a decision had to be made between removal of rudimentary fingers or their stabilization. In type IIB, hand function was good and surgery was rarely needed. Type III (monodactylous) could also be subdivided into two categories, i.e., normal thumb in type IIIA and hypoplasia in IIIB. Finally, in type IVA, toe transfer surgery was performed on condition that wrist mobility was sufficient to compensate for the insufficient mobility of the artificial thumb on the anterior aspect of the radius. In all cases, a weak but useful pincer movement was obtained, with poor cosmetic results. In the case of toe transfers, surgery was advocated before the age of one year; and although mobility was disappointing (35 degrees active motion), good growth and excellent discrimination (5 mm on average) was observed. Symbrachydactyly is a fairly frequent congenital malformation; its diverse clinical features require a precise classification to better determine adequate treatment management.     

Late psychosocial effects of congenital hand anomaly.

Lifetime-standing psychosocial effects of congenital hand anomalies are inevitable in patients who have not received a comprehensive treatment with appropriate timing and approach. Herein, two adult cases of untreated thumb polydactyly are presented. Both of them had hands with striking appearance and late consequent psychosocial problems.     

Congenital Thumb-Index Web Space Deficiency

Background: There are several congenital hand differences that cause thumb-index (TI) web space deficiency. There is a knowledge gap in the literature about the hand differences that are associated with TI web space deficiency. We aimed to identify these congenital differences and the various specific reconstructive surgical procedures that are used for these conditions. Methods: We conducted a retrospective chart review of children treated operatively over a period of 30 years for congenital TI web space deficiency by the senior author (G.M.R.). We gathered data on demographics and associated congenital hand differences and compiled a list of all surgical procedures performed for the web space and the ipsilateral upper extremity. Results: We included 71 patients (77 hands) with 12 congenital hand differences (62 developmental and 9 spastic). The total number of upper extremity operations, (ie), anesthetics performed for these patients was 186, averaging 2.6 settings and 7.5 procedures for each patient. Cutaneous reconstructive procedures included first dorsal metacarpal artery pedicle flaps (49 patients), 4-flap Z-plasties (15), and transposition flaps (13). In addition, 16 different thumb reconstructive procedures were necessary. Ten patients required revision of their TI web space procedures for recurrence. Conclusions: The prevalence of TI web space deficiency is underappreciated. These patients often have multiple musculoskeletal anomalies of the hand and upper extremity that should be ruled out and require surgical treatment to optimize hand function. Consideration should be given to performing more than one procedure in one setting when possible.     

Treatment alternatives of congenital hand differences with thumb hypoplasia involvement

Congenital thumb hypoplasia is a complex and heterogeneous congenital difference that is detrimental to hand function. Apart from its' classic form, which is now considered to be part of radial dysplasia, it can occur as part of other congenital anomalies including, syndactyly, symbrachydactyly, atypical cleft hand, bifid thumb, triphalangeal thumb, mirror hand, constriction band syndrome, as well as generalized anomalies and syndromes. Management is aimed primarily at restoring basic hand function, specifically, power grasp and precision pinch, and secondarily to improve cosmoses, which inevitably is going to be impaired. Several treatment alternatives have been proposed to manage the specific disabling condition and include, 1st web space reconstruction and opponensplasty, pollicization, toe-to-hand transfer, distraction lengthening, free toe phalangeal transfer or the use of allograft, stabilization of the metacarpophalangeal joint, and surgery of "spare parts." The purpose of this study is to evaluate the various alternatives available today and propose an algorithm applicable for the appropriate management of thumb deficiency, based on their specific characteristics.     

Surgical treatment of the clasped thumb

We report a series of 12 patients (19 hands) with clasped thumb who underwent surgical treatment. The series is divided into three groups. In the first group, the prominent pathological feature was hypoplasia of the extensor tendons which was treated by tendon transfer. The second group, the arthrogryphotic type, had contracture of the intrinsic muscles of the thumb and shortening of the skin; these needed mainly release operations. In only three hands out of nine was tendon transfer performed. The third group was defined by a combination of skeletal, muscular and tendon hypoplasia. Instability of the M.P. joint and adduction contracture of the first ray were found in all hands of this group. The adduction deformity was corrected by skin-plasty of the first web and the extension was improved by tendon transfers. Only one case needed stabilisation of the M.P. joint. The final results were related to the extent of the contractures and to the age of the patient when treatment started.     

Long-term results of digital reconstruction using toe-to-finger transfers

In the period 1987–2002, 147 toe-to-hand transfers were performed in 111 patients. Traumatic amputation was reconstructed by transfer of 131 toes in 101 patients; congenital anomaly was treated in 10 patients by transplantation of 16 toes. In this study, 60 reconstructed hands were evaluated in 59 patients. Patients were divided into 12 groups according to the type of reconstruction. Group A includes patients with thumb reconstruction using the great toe, wrap-around flap, or second toe transfer. Group B involves patients with thumb and second finger reconstruction; group C comprises cases of second and third finger reconstruction. Group D involves patients with claw hand type of reconstruction, group E includes patients with metacarpal hand reconstruction, and finally group F comprises cases with congenital anomalies. Post-transfer total range of motion and sensitivity were measured on transplanted toes. Functional ability was evaluated by the performance of 20 selected activities of daily life. Successful rate of transfer was 94.6% in post-traumatic transplantation and 100% in congenital anomalies reconstruction. The average total range of motion and two-point discrimination sensitivity was 53° and 11 mm, respectively. The best results, judged by grasping ability, were achieved in group B (thumb and second finger reconstruction), group C (second and third finger reconstruction), and, surprisingly, group E (metatarsal hand). Grasping ability was significantly lower in group D (claw hand type of reconstruction) and group F (congenital hand anomalies). The highest patient satisfaction was achieved in cases of thumb and metacarpal hand reconstruction.