Characteristics of patients with hypoplastic thumb: a prospective study of 51 patients with the results of surgical treatment

The aim of this study was to characterize a group of patients with hypoplasia of the thumb, classifying them, describing the associated anomalies and to evaluate the results of surgical treatment of such cases. Thumb hypoplasia is a complex and heterogeneous congenital disorder that is detrimental to hand functions. The characteristics of patients with these anomalies are not well described in the literature. A prospective study on 51 patients with 82 hypoplastic 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 examination with genetic assessment and radiological examination including abdominal ultrasonography and echocardiography when requested by the paediatrician. The cases were classified using the modified Blauth classification into five types and we added the five-fingered hand. Surgical treatment was performed for 26 hands in 18 patients and the postoperative results were recorded with an average duration of follow-up of 38.6 months. Of the 51 patients, there was a positive consanguinity in 23.5%. Eighty-six per cent were found to have associated anomalies. Type V thumb hypoplasia was the most common type followed by type IV, with type I being the least common. All the patients' parents were satisfied with the results of surgical treatment and noticed improvement of the performance of the operated hands in the daily activities. We detected some anomalies that have never been described before or described as case reports only; such as lacunar skull, congenital facial palsy and toe amputation; we also described familial radial side dysplasia with variable presentation of congenital anomalies of the thumb in the families. In addition, we think that the five-fingered hand should be added to the classification of thumb hypoplasia. Our study agrees with the literature regarding the distribution of different types of hypoplasia of the thumb, the incidence of associated anomalies and the results of surgical treatment.     

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.     

Bifid thumb type IV in children: transferring an epiphyseal segment of the proximal phalanx with insertion of the abductor pollicis brevis tendon

Objective

To evaluate the clinical and functional results of the surgical treatment of bifid thumb type IV in children.

Materials and methods

A retrospective study was undertaken from January 1995 to December 2006. Clinical and radiographic evaluations were made according to Wassel’s classification. The patients were performed by transferring an epiphyseal segment of the proximal phalanx with insertion of the abductor pollicis brevis tendon into the radial side of the epiphyseal proximal phalanx of the ulnar thumb. All patients were operated using one of five surgical procedures for bicephalous metacarpus, cartilaginous connection between the radial and ulnar proximal phalanges, the angular deformity of the metacarpophalangeal joint (MPJ) is >20°, and zigzag deformities. The postoperative results of the patients were evaluated for both function and cosmesis according to Tien’s modified Tada scoring system.

Results

One hundred and sixty-four patients (102 females, 62 males) were included in this study. The MPJ was stable in 170 thumbs, 15 thumbs had 10° of radial instability, and new collateral ligaments were augmented in 27 thumbs. The alignment was normal in 75 thumbs, with alignment of the interphalangeal joint (IPJ) in 101 thumbs and alignment of the MPJ in 75 thumbs. Postoperatively, there were zigzag deformities in four thumbs (developed zigzag in two thumbs, recurrent zigzag in two thumbs); there was no first web space in those hands. There were four of 185 thumbs with thumb stiffness. The abductor function of 185 thumbs was as follows: >70° in 158 thumbs (85.4%), 50°–70° in 21 thumbs (11.4%), and <50° in six thumbs (3.2%). At the latest follow-up evaluation, no evidence of physeal growth injury or growth arrest was observed in any patient. Overall, we attained good results in 140 thumbs (75.7%), fair results in 36 thumbs (19.4%), and poor results in nine thumbs (4.9%).

Conclusion

We recommend the use of an epiphyseal segment of the proximal phalanx with insertion of the abductor pollicis brevis tendon into the radial side of the epiphyseal proximal phalanx of the ulnar thumb and to restore anatomical insertion of the abductor pollicis brevis muscle. The technique is simple, safe, and effective for thumb abductor function in the treatment of bifid thumb type IV in children.     

Flexion deformities of the thumb: clasped thumb and trigger thumb

Congenital clasped thumb in palm corresponds to a spectrum of anomalies leading to a loss of thumb extension and abduction. Intrinsic muscles and skin shortening are not infrequent. Conservative orthopedic treatment should be undergone as soon as possible. When this treatment fails, or when patients are seen late, surgical correction has to be customized according to the involved structures. The trigger thumb is 10 times more frequent than the trigger finger. It is bilateral in 30% of the cases. The term "congenital" remains unclear as several investigations on newborns have not evidenced trigger thumb. In children, it is very rare to find a real trigger and presentation consists in a fixed flexion deformity of the interphalangeal joint. Diagnosis is clinical, with a palpable nodule at the level of T1 pulley. Conservative treatment, consisting in a nocturnal splint, is indicated before the age of two years old. After this age, or in case of failure of splinting, surgery will be needed.     

Déformation en flexion du pouce de l’enfant : pouce flexus adductus et pouce à ressaut

Congenital clasped thumb in palm corresponds to a spectrum of anomalies leading to a loss of thumb extension and abduction. Intrinsic muscles and skin shortening are not infrequent. Conservative orthopedic treatment should be undergone as soon as possible. When this treatment fails, or when patients are seen late, surgical correction has to be customized according to the involved structures. The trigger thumb is 10 times more frequent than the trigger finger. It is bilateral in 30% of the cases. The term “congenital” remains unclear as several investigations on newborns have not evidenced trigger thumb. In children, it is very rare to find a real trigger and presentation consists in a fixed flexion deformity of the interphalangeal joint. Diagnosis is clinical, with a palpable nodule at the level of T1 pulley. Conservative treatment, consisting in a nocturnal splint, is indicated before the age of two years old. After this age, or in case of failure of splinting, surgery will be needed.     

Congenital clasped thumb combined with Waardenburg syndrome in three generations of one family: an undescribed congenital anomalies complex

Four cases of congenital clasped thumb with Waardenburg syndrome in three generations of one family (three males and one female) are presented. The combination of these congenital anomalies has not been reported previously in the literature and appears to be transmitted as an autosomal dominant trait with variable expressivity.     

Congenital Absence of Flexor Pollicis Longus Tendon Without Associated Anomalies of Thumb Hypoplasia: A Case Report and Review of the Literature

In this paper, we report a case of a 14-year-old girl with congenital aplasia of the flexor pollicis longus tendon who had no other associated anomalies of thumb hypoplasia and no trauma history. Flexor pollicis longus tendon anomalies are rare; several types of this congenital anomaly have been reported in the literature. The diagnosis should be considered if a patient is unable to flex the interphalangeal joint of the thumb. A hypoplastic thumb or an absent interphalangeal joint crease may be a diagnostic feature in such cases. Besides physical examination, we also used direct radiography and magnetic resonance imaging to diagnose this rare congenital anomaly in our patient.     

A simple splinting method for correction of supple congenital clasped thumbs in infants

A splint has been designed to correct the congenital clasped thumb. It is like a short opponens splint that can keep the thumb in a position of abduction and extension without limiting wrist movement. The application of the splint was easy and adjustment for fit could be made at each visit if necessary. The device has been used in 11 infant patients (17 thumbs) with congenital clasped thumb of the supple type. The functional results were excellent in 15 of 17 thumbs, and the other two were good according to the grading system of Weckesser et al. (1968).     

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.     

Aspergillus osteomyelitis of the thumb in a 5-year-old child

Aspergillus osteomyelitis is a rare condition and is a recognized infection of the immunosuppressed. The pediatric cases that were documented suggest that in children, chronic granulomatous disease is the major underlying disease [Tack et al.1982 73(2):295–300, Baez-Escudero et al. 2000 Case report—primary sternal Aspergillus osteomyelitis. Infect Med 17(7):505–516]. We report an interesting case of Aspergillus osteomyelitis of the thumb in a 5-year-old boy with aplastic anemia. The infection progressed despite a combination of antifungal therapy with Voriconazole and surgical debridement. The thumb was amputated and the child recovered. This case highlights the difficulty in diagnosing Aspergillus osteomyelitis and also the failure of conventional management in this child, which resulted in the amputation of the thumb as a life-saving measure. We believe this to be the first case report of Aspergillus osteomyelitis in the thumb.     

Congenital flexion deformities of the thumb

A congenital flexion deformity of the IP joint of the thumb is usually due to a trigger finger. The diagnosis is based on the thumb being normal except for a fixed, flexed posture of the IP joint and a palpable nodule in the FPL tendon at the level of the A-1 pulley. Treatment can be postponed until after age 1, as spontaneous regression probably occurs in about 30 per cent of cases. After that age, surgical correction is the best approach to treatment and usually results in normal thumb function. Congenital soft tissue flexion deformities of the MP joint of the thumb vary from passively correctable deformities due to loss of extensor power to severe, fixed contractures involving multiple thumb structures and associated with significant anomalies of the wrists and fingers. The passively correctable deformities will frequently respond to cast immobilization in the very young but can otherwise be treated by an appropriate tendon transfer to re-institute active extension. Complex clasped thumb deformities require elucidation of the specific pathologic features of the deformity, based both on clinical evaluation and surgical exploration. These digits will require not only an extensive release to correct the fixed contracture but also reconstructive procedures to correct lax ligaments and to substitute for absent muscles and skin as appropriate. The latter deformities are often associated with a syndrome affecting numerous body structures. It is hoped that recognition of these syndromes and documentation of the pattern of pathology present in these thumbs will result in a better method of classification.     

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.     

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 clasped thumb: a review of forty-three cases

Over a 10-year period, 43 patients (75 hands) with congenital clasped thumb were seen in our institution. Three groups were identified: group I, 14 patients (24 hands) without contracture; group II, 14 patients (21 hands) with contractures of the palmar side; and group III, 15 patients (30 hands) with arthrogryposis multiplex congenita. Forty-two hands were treated with splinting alone and 16 hands with surgery. The remaining 17 hands were followed conservatively without splinting or surgery. The mean follow-up was 32 months. The results were evaluated by active abduction of the carpometacarpal joint and extension of metacarpophalangeal joint. All patients in group I showed good response to splinting, and the cause of the deformity appeared to be the predominance of the flexor muscles. In groups II and III, 10 patients (16 hands) who had severe deformity or no response to splinting were treated by release of the palmar soft tissues, skin grafts, and reconstruction of the extensors. Satisfactory results were obtained in 12 of 16 hands.     

After thumb duplication: a proposal for evaluation of the result

Thumb duplication is the second most common congenital hand anomaly after syndactyly [Cohen MS (1998) Hand Clin 14(1):17; Dobyns JH, Wood VE, Bayne LG (1993) In: Green DP (ed) Operative Hand Surgery. Churchill and Livingstone, New York, pp 251–549]. To gain a functional and aesthetically acceptable thumb, surgical treatment is necessary in almost all cases. Many articles have been written on classification systems, surgical treatment and factors influencing outcome. However, literature on evaluating the results of surgical treatment is scarce. Using a mixture of criteria as described by Cheng et al., Townsend et al. and Tada et al., we developed a scoring system that enables an objective comparison of treatment results. We performed surgical correction of the duplicated thumb on 15 patients with various anomalies. Eleven had good results immediately, three had good outcomes after secondary surgery, and one patient had a normal result. It was not possible to compare our results with the results described in other studies due to a lack of similar criteria.     

先天性拇指疾病的疗效分析

目的：介绍先天性拇指疾病的治疗效果。方法：1988年起,对56例先天性拇多指畸形,先天性拇板机指及先天性拇巨指症进行手术治疗。采用拇指赘生指切除、副指皮瓣转移,多拇残端修整术治疗拇多指畸形;巨指截指及整形术治疗巨指症;增厚狭窄腱鞘切除术治疗先天性拇板机指。结果：术后随访2－10年。（1）先天性拇指多指畸形：主干拇指外形改善,拇外展充分,对指功能良好。（2）拇指板机指：拇指伸屈自如,发育满意。（3）先天性巨指症1例：示、中指巨指切除,拇指远节截除,近节缩短整形,术后外形欠佳。结论：先天性拇指多指畸形应根据具体情况选择手术方式,以改善畸形、恢复拇指功能。先天性板机指明确诊断手术疗效佳。先天性巨指症采用截指方法较为理想。     

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.     

大多角骨切除加肌腱固定术治疗第一腕掌关节炎的疗效观察

目的介绍大多角骨切除加肌腱固定术治疗第一腕掌关节炎的手术方法及疗效。方法以大多角骨完全切除加部分桡侧腕屈肌腱固定第一掌骨基底部的方法治疗第一腕掌关节炎11例。测量患侧手术前后及健侧腕关节和拇指的活动度、握力和捏持力,进行统计学分析。结果11例中的10例随访1～3年,平均2年3个月,比较术后和术前腕关节及拇指活动范围和握力及捏持力,术后有明显的增加,术前、术后相比差异有统计学意义(P<0.01)。术后与健侧对比差异无统计学意义(P>0.05)。结论大多角骨切除加桡侧腕屈肌腱固定第一掌骨基底部治疗第一腕掌关节炎是一种可行的方法值得临床推广。