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.     

Restoration of Five Digit Hand in Type III B & C Thumb Hypoplasia–A Game Changer in Surgical Management

Background  Hypoplasia of thumb is the second common congenital difference of the thumb, next only to duplication. It may occur as an isolated hand difference or as a part of radial longitudinal deficiency. In approximately 60% of these children, the radius shows hypoplasia. The incidence of thumb hypoplasia is one in 100,000 live births. In 50% of these children, the other hand will also have similar deficiency, although variable in severity. Hypoplasia of thumb has been classified into five major categories, according to the increasing severity of hypoplasia. Type III hypoplasia of thumb is characterized by skeletal hypoplasia involving the first metacarpal and carpometacarpal joint, absent intrinsic muscles and rudimentary extrinsic muscles. It was further subclassified into types A, B & C. Type III B, described by Manske and McCarroll, involves extensive deficiency of extrinsic and intrinsic musculature with aplasia of the metacarpal base. Type III C, described by Buck-Gramcko, has hypoplastic metacarpal head. Methods  It is widely believed that reconstruction of Type III B & C hypoplastic thumb will not be functionally useful, and they are often included in the indications for pollicization in thumb hypoplasia. In India, we frequently come across parents, who are not willing to remove the hypoplastic digit. This forced us to find out a way to reconstruct the hypoplastic thumb into a functionally useful digit. We describe our surgical technique of reconstruction of hypoplastic thumbs and our experience in utilization of the technique in five children with Type III B & C hypoplasia of thumb. Carpometacarpal joint of thumb was reconstructed and stabilized with a toe phalangeal transfer in the first stage and an opponensplasty was done in the second stage to improve movement. Results  In all the five operated children, our surgical technique yielded a stable thumb which was functional. The donor site morbidity was acceptable. The parents were satisfied with the appearance and functional improvement. Conclusion  Surgical reconstruction of hypoplastic thumbs of Type III B & C is possible, and conversion of these poorly developed remnants into a useful digit by our surgical technique is a gamechanger in the management of thumb hypoplasia.     

Pollex abductus in hypoplasia and duplication of the thumb

Pollex abductus is an anomaly in which the flexor pollicis longus attaches not only at its customary insertion, but also into the extensor by a tendon that passes around the radial aspect of the thumb. Contraction of the anomalous flexor abducts the thumb. In the original report, the anomaly occurred in thumb hypoplasia. A similar anomaly is reported in 11 hypoplastic thumbs (35.5% of all cases) and in 9 cases of radial polydactyly (21.4%). The common feature was poor definition of the interphalangeal skin crease, with complete absence of active flexion of that joint. All patients were treated surgically; 13 before the age of 10 months, with correction of the duplication and the hypoplasia, and in the latter with elimination of the abduction deformity and restoration of opposition. The anomalous connection was excised in all patients. Active interphalangeal joint motion after reconstruction of the hypoplastic thumbs averaged 21 degrees.     

Blauth II thumb hypoplasia: a management algorithm for the unstable metacarpophalangeal joint

Blauth Type II thumb hypoplasia is defined by first web space narrowing, deficiency of thenar musculature, and instability of the metacarpophalangeal joint (MCPJ). This instability can be uni-axial (type IIA) or multi-axial (type IIB). The aim of this study was to assess the results of treating type II thumb hypoplasia using an algorithm based on the type of instability present. Cases of uni-axial MCPJ laxity (type IIA) underwent stabilization as part of a flexor digitorum superficialis opposition transfer. Type IIB cases with multi-axial instability were treated with an MCPJ chondrodesis and an abductor digit minimi transfer for opposition. First web space release was achieved using a z-plasty approach in all patients. An analysis was carried out of all cases of type II thumb hypoplasia treated by the senior author within the setting of a tertiary referral children's hospital over a 9 year period. Using our management algorithm, equivalent functional results were seen in each subgroup in terms of first web space release, MCPJ stabilization, and opposition.     

采用掌骨部分移位重建改良Blauth 3B及3C型发育不良拇指的疗效观察

目的 观察第2掌骨纵向半侧骨干移位和二期拇对掌功能重建治疗改良Blauth 3B和3C型拇指发育不良的疗效.方法 2013年12月至2018年6月,复旦大学附属华山医院手外科收治的改良Blauth3B和3C型患儿资料12例.将第2掌骨纵向半侧移位延长第1掌骨,稳定第一腕掌关节.确认骨折愈合后,二期行肌腱移位拇对掌功能重...     

Treatment of a type 3B hypoplastic thumb using extra phalanges from the contralateral duplicated thumb: case report

A boy was born with a right duplicated thumb (Wassel type 6) and a left radial club hand (type 3) associated with a hypoplastic thumb (type 3B). He underwent surgical centralization of the left wrist when he was 13 months old. At age 38 months, he underwent reconstruction of the carpometacarpal joint of the hypoplastic left thumb. This procedure involved transplantation of the radial ray of the right duplicated thumb to the base of the left thumb. When he was 6 years old, the patient underwent an abductor digiti minimi muscle transfer to create opposition for the left thumb and deepening of the first web. At the final follow-up, he could grasp items in the first web space and pick up small items between the thumb and other digits of the left hand. The treatment represents a method of using otherwise discarded tissues for effective reconstruction.     

Connective tissue structures in the first intermetacarpal space in cases of malformations of the hand with and without syndactyly

Summary In three clinical cases of cutaneous syndactyly and varying degrees of serious disorders to the skeleton of the hand and in one case of hypoplasia of the thumb, hypoplastic carpometacarpal and metacarpophalangeal joints we were struck by the narrowness of the first intermetacarpal space. At the same time a lack of thumb opposition was clinically evident. Our own previous anatomical investigations and embryological considerations on a series of hands with cutaneous and osseous syndactylies lead us to suspect that abnormal ligaments or connective tissue bands might be responsible for the functional restrictions mentioned above [9]. The findings concerning both the bone structure and the soft tissue can be traced back to disorders in the distribution of mesenchymal material of the handplate and to a failure in resorption. Our procedure consisted of making a Z-shaped incision in the first intermetacarpal space and in radically extirpating abnormal ligaments and connective tissue bands therefrom. The freeing of the thumb thus obtained was completed by exchanging the triangular skin flaps in accordance with the technic of Z-plasty. In the four cases an improvement in the whole functioning of the malformed hands was attained.     

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.     

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.     

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     

Abductor digiti minimi musculocutaneous island flap as an opposition transfer: a case report

We described the use of an abductor digiti minimi musculocutaneous (ADM) island flap as an opposition transfer. This procedure is easy and safe, and provides more of a bulge in the thenar area than the Huber transfer. This procedure can be used in the treatment of Blauth grade 2 and 3A hypoplastic thumb.     

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.     

Congenital absence of flexor pollicis longus without hypoplasia of the thenar muscles.

We report a rare case of absence of the flexor pollicis longus in a hypoplastic right thumb, without hypoplasia of the thenar muscles in an 11-year-old girl, who was treated by transfer of flexor digitorum superficialis of the ring finger to the thumb to achieve active flexion at the interphalangeal joint of the thumb.     

Reconstruction of the type IIIA hypoplastic thumb

The management of the hypoplastic thumb depends on the degree of bony malformation, soft tissue anomaly, and deficiencies. Type IIIA thumbs have thumb-index web-space narrowing, hypoplasia of the thenar musculature, metacarpophalangeal joint instability, and extrinsic tendon abnormalities. They also have a hypoplastic metacarpal with a stable carpometacarpal joint, differentiating them from type IIIB thumbs which have an unstable carpometacarpal joint due to partial metacarpal aplasia. Whereas type IIIB thumbs are best treated with ablation and index pollicization, type IIIA thumbs are amenable to reconstruction. Many techniques have been described for correction of the variety of deformities found in theses thumbs. The purpose of this article is to present our preferred technique for comprehensive reconstruction of type IIIA thumbs.     

Reconstruction of the congenitally deficient thumb.

Hand function in patients with congenitally deficient thumbs can usually be improved by operative procedures. The specific operation depends on the type of hypoplasia present. It is important for the surgeon to determine whether the thumb can be retained and made more functional by various reconstructive procedures, or whether it should be ablated in favor of a single-stage index finger pollicization. This decision is particularly difficult in patients with type III hypoplastic thumbs, in whom both the osseous and musculotendinous structures may be significantly affected. It is nearly impossible to perform pollicization as a salvage procedure after attempts to reconstruct a retained thumb have failed. The principles presented may assist the surgeon in this decision-making process. Pollicization itself is an exacting procedure requiring attention to numerous details. The surgeon and parents must realize that additional operative procedures may be required to enhance the function of the pollicized digit.     

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.     

Precision grip with congenital hypoplasty or hypofunction of the thumb

We tested the grip in four patients with congenital defects of the hand and either a hypoplastic thumb or a thumb with impaired inervation. Small objects were taken by a scissors grip between the fingers. In a hand with radial duction in the manus vara congenita, during strengthening of the wrist, the grip from the ulnar side between the fourth and fifth fingers was changed to the radial side between the second and third fingers. Large objects were gripped by all the three-phalanx fingers into the palm in a horizontal position. In case 4 with hypoplasia of the thumb grade IIIC by the classification of Blauth and Buck-Gramcko, we describe a transposition of the index finger to the site of the thumb and the hypoplastic thumb to the site of the index finger. It is obvious that the precision grip is affected by the thumb length and strengthening of the ulnar side of the wrist. We assume that the scissors grip is the earliest precision grip in the evolution of the primate hand.     

Bilateral carpal tunnel syndrome after abductor digiti minimi opposition transfer: a case report

We present a case of bilateral, delayed-onset, median nerve compression at the wrist after abductor digiti minimi opposition transfer for thumb hypoplasia. The symptoms resolved on each side after transverse carpal ligament release without disruption of the opposition transfers.     

Combination of fillet and triangular flaps for the web reconstruction in pollicization: a case report

Thumb hypoplasia represents a major disability requiring sophisticated reconstructive procedures. One of the basic criteria of the pollicization to construct a functional thumb is scar free web reconstruction. In this article, a technique to avoid excessive scarring in the first web space reconstruction by filleting out the hypoplasic thumb combining with a triangular flap is presented. A 5-year-old girl was presented. She had a Back-Gramcko type IV thumb hypoplasia. A triangular flap was planned on radial border of the long finger. Floating thumb was filleted out with preservation of the neurovascular bundle and transferred to the first web space while cooperating with triangular flap. Patient was followed up for 18 months. No early or late major complication was seen. Flap healed without any necrosis. The ratio of active motion in pollicized finger was measured 60% after cerebral reorientation. A satisfactory grip and pinch were seen in pollicized finger. Patient as well as her parents was satisfied with the results. Filleting out the floating thumb and combining with a triangular flap harvested by a V-Y flap design is a good option to avoid excessive scarring in reconstruction of the first web space in pollicization. It is strongly recommended that floating thumb should be preserved for future pollicization, and any attempt to amputate it in early years of life should be postponed till the time of definitive surgery.     

Poland's syndrome.

Forty-three consecutive cases of Poland's syndrome were analyzed and the relevant literature was reviewed. The syndrome is not hereditary and is of unknown origin. It affects males more frequently than females. The clinical features are variable but always include congenital aplasia and syndactyly. The middle phalanges are hypoplastic or absent so that effectively there is only one interphalangeal joint. The syndactyly is usually incomplete and simple. It may involve all fingers and frequently includes the thumb, which then lies in the same plane as the fingers. Poland's syndrome may also include hypoplasia of the nipple and breast, hypoplasia of the upper ribs, herniation of the lung, contracture of the anterior axillary web, and elevated scapula. The arm and more frequently the forearm are hypoplastic. The right side is more often affected than the left. Surgical treatment by separating the syndactyly is recommended. In some cases a digit is removed to produce a three-fingered hand. Surgery is initiated by the age of one year and is completed by the time the child enters school, although periodic revisions may be necessary. Although the hand remains hypoplastic and functional capacity is limited by the inherent skeletal anomalies, surgical treatment improves functional capacity and cosmetic appearance in the majority of patients.