Thumb Hypoplasia Research Articles

Bilateral ST-coalition with thumb hypoplasia and scaphoid synchondrosis.

Carpal coalitions are rare wrist anomalies and are most often diagnosed incidentally.Due to their infrequent occurrence, there is a lack of treatment guidelines in the literature. We present a case study of a 13-year-old boy who presented with symptomatic synchondrosis in both scaphoids along with a bilateral osseous coalition between the scaphoid and trapezium bones in combination with bilateral thumb hypoplasia.We initiated a 10-week immobilization of the wrist, followed by gradual increasing weight-bearing. The patient showed significant symptom relieve after immobilization,further supporting the conservative treatment. In conclusion, a definitive treatment recommendation cannot be made. For young patients, we suggest initiating conservative treatment as the first option. A precise analysis of the pathology and wrist kinematics is mandatory to recommend further therapy especially if operative interventions might be considered.

Long-Term Functional and Radiological Results in Tonkin Type 3B Thumb Hypoplasia Treated With Nonvascularized Toe Phalanx Transfer as an Alternative to Pollicization

Parents of children with hypoplastic thumbs often reject the option of pollicization for various reasons and enquire about alternate choices. Our study aimed to assess the outcome in children who underwent nonvascularized toe phalanx transfer for Tonkin type 3B thumb hypoplasia and compare it with a similar cohort of children treated with pollicization. At an average follow-up of 7 years for toe phalanx transfer and 6 years of pollicization, five children from each group were tested for thumb length, stability of the first carpometacarpal (CMC) joint, mobility, opposition, and donor-site morbidity. Parents were asked to report improvements in function and appearance. All cases were Tonkin type 3B thumb hypoplasia. The CMC joint was found to be stable in all children, and the Kapandji score was 6 in 3 children and 5 in 2 children with toe phalanx transfer compared to 9 in all children with pollicization. The average palmar abduction was 24°, and the average radial abduction was 36° in the toe phalanx transfer group compared to 40° and 45°, respectively, in children with pollicization. The average thumb length was 50.8% of the index finger's proximal phalanx in the toe phalanx group compared to 60 % in the pollicization group. The mean visual analog scale scores for the thumb's function and appearance were 6.8 and 6.4, respectively, compared to 9.2 and 8.8, respectively, in the pollicization group. No resorption was noted in the donor phalanx at a mean follow-up of 7 years with no donor-site morbidity other than mild shortening of the toes. We noted increased palmar abduction and radial abduction in the pollicization group. No resorption was noted in the donor toe phalanx at the longest follow-up of 9 years. Toe phalanx can be considered an alternative for those children in whom a five-finger hand is a priority. Therapeutic V.

Radiographic Features of the Metacarpal in Blauth Type III Thumb Hypoplasia

The purpose of this study was to investigate whether radiographs can be used to aid in the determination of Blauth IIIA and IIIB thumbs. Six pediatric hand surgeons were asked to evaluate the radiographs of 77 thumbs and classify the thumb as IIIA or IIIB and indicate which morphologic features influenced their decision. Quantitative measurements and ratios of radiographs were obtained and compared between IIIA and IIIB thumbs. The radiographic features selected for type IIIA thumbs include near-normal length and near-normal width and for type IIIB thumbs, abnormally short, tapered proximal end, and round proximal end. The six surveyed surgeons reached consensus in 82% (63/77) of thumbs, and this matched the enrolling surgeon's classification in 77% (59/77) cases. The ratio of the length of the thumb metacarpal compared with the length of the index metacarpal was different between IIIA and IIIB thumbs (66% ± 0.08% and 46% ± 0.18%, respectively). The ratio of the width of the thumb metacarpal shaft at its narrowest aspect to the width of the thumb metacarpal base was notably different between IIIA and IIIB (68% ± 0.13% and 95% ± 0.28%, respectively). Near-normal length and near-normal width of the metacarpal were used to predict IIIA and abnormally short, abnormally narrow, and a round or tapered base of the metacarpal were used to predict IIIB classification. The length of the thumb metacarpal relative to the index metacarpal is on average 66% of the length of the index metacarpal in IIIA thumbs compared with 46% in IIIB thumbs. The width of the shaft of the thumb metacarpal at its narrowest is 68% of the width of the thumb metacarpal base in IIIA thumbs, indicating a flared base. In IIIB thumbs, the shaft width was on average 95% of the base width, indicating a tapered base. Diagnostic level III.

Stabilization of the hypoplastic thumb type Blauth IIIB using a non-vascularized proximal interphalangeal joint from the toe as an alternative reconstruction when pollicization is not accepted: Description of the surgical technique

Stabilization of the hypoplastic thumb type Blauth IIIB using a non-vascularized proximal interphalangeal joint from the toe as an alternative reconstruction when pollicization is not accepted: Description of the surgical technique

Opponensplasty With Abductor Pollicis Brevis Rerouting for Types II and IIIA Hypoplastic Thumbs

To evaluate the functional results after opponensplasty using an abductor pollicis brevis rerouting technique in type II and IIIA hypoplastic thumbs. Eleven hypoplastic thumbs in nine children with type II and IIIA hypoplastic thumbs were treated with abductor pollicis brevis rerouting. The mean follow-up period was 70 months (range, 12-172 months). We assessed preoperative to postoperative changes in the angles of the first and second metacarpal axes and the longitudinal axis of the first metacarpal and proximal thumb phalanx as well as grip and pinch strengths. The mean angle of the first and second metacarpal axes showed a significant improvement to 64° (range, 47° to 89°), and the mean angle of the first metacarpal and proximal phalanx of the thumb showed a significant reduction to 8° (range, 1° to 21°) after surgery. The mean postoperative grip and pinch strengths were 77% (range, 63% to 106%) and 72.0% (range, 33% to 97%), respectively, relative to the unaffected side. Abductor pollicis brevis rerouting for type II and IIIA hypoplastic thumbs can produce joint stability and a strong pronation effect in addition to the opponens function. Therapeutic V.

The Effects of Radial Longitudinal Deficiency on Long-Term Use of the Thumb in Pediatric Patients Following Index Pollicization

To assess the effect of radial longitudinal deficiency on the function of pollicized digits as determined by the Thumb Grasp and Pinch (T-GAP) assessment. We retrospectively evaluated 25 hands with thumb hypoplasia that underwent index finger pollicization. Patients were followed for an average of 10.4 years. Hands were divided by severity into two groups: no or mild radial longitudinal deficiency (RLD) (Group 1= 16) and moderate to severe RLD (Group 2= 9). We collected demographic information and completed physical examination measures, including hand strength, elbow, wrist, and hand range of motion, the Kapandji opposition score, active grasp span, and T-GAP total score. Patients with moderate to severe forms of RLD had stiffer long fingers, lower Kapandji opposition scores, and limited active and passive range of motion for elbow flexion, wrist ulnar deviation, and pollicized thumb interphalangeal flexion. They had shorter forearms, decreased active grasp span, and fewer thumb creases at the interphalangeal thumb joint. In addition, the T-GAP total score was significantly lower when comparing the two groups. Children with mild dysplasia were able to achieve 32% of age-matched normal grasp strength. Patients with more severe radial dysplasia averaged 17% less grasp strength compared with children with mild dysplasia. Patients with moderate to severe RLD also had lower T-GAP total scores and strength measurements if they had limited wrist ulnar deviation. Individuals with moderate to severe RLD have unique anatomical factors that affect outcomes after pollicization. These individuals use their thumbs for fewer activities, have weaker grasp, and retain more primitive grasp patterns compared with those who have milder forms of RLD. Prognostic IV.

Non-vascularized metatarsal transfer for thumb hypoplasia: a medium- to long-term retrospective study of 41 patients.

III.

Differential Diagnosis of Trigger Thumb in Children

Trigger thumb is the most common hand deformity in young children. The differential diagnoses of trigger thumb are thumb-in-palm deformity, hyperextension deformity of the metacarpophalangeal joint, thumb hypoplasia and congenital stiffness of the interphalangeal joint. The knowledge of the typical clinical signs of these different diseases enables the surgeon to make the correct diagnosis, leading to the right treatment.

Children with Rare Nager Syndrome-Literature Review, Clinical and Physiotherapeutic Management.

Nager syndrome is a rare human developmental disorder characterized by craniofacial defects including the downward slanting of the palpebral fissures, cleft palate, limb deformities, mandibular hypoplasia, hypoplasia or absence of thumbs, microretrognathia, and ankylosis of the temporomandibular joint. The prevalence is very rare and the literature describes only about a hundred cases of Nager syndrome. There is evidence of autosomal dominant and autosomal recessive inheritance for Nager syndrome, suggesting genetic heterogeneity. The majority of the described causes of Nager syndrome include pathogenic variants in the SF3B4 gene, which encodes a component of the spliceosome; therefore, the syndrome belongs to the spliceosomopathy group of diseases. The diagnosis is made on the basis of physical and radiological examination and detection of mutations in the SF3B4 gene. Due to the diversity of defects associated with Nager syndrome, patients require multidisciplinary, complex, and long-lasting treatment. Usually, it starts from birth until the age of twenty years. The surgical procedures vary over a patient's lifetime and are related to the needed function. First, breathing and feeding must be facilitated; then, oral and facial clefts should be addressed, followed by correcting eyelid deformities and cheekbone reconstruction. In later age, a surgery of the nose and external ear is performed. Speech and hearing disorders require specialized logopedic treatment. A defect of the thumb is treated by transplanting a tendon and muscle or transferring the position of the index finger. In addition to surgery, in order to maximize a patient's benefit and to reduce functional insufficiency, complementary treatments such as rehabilitation and physiotherapy are recommended. In our study, we describe eight patients of different ages with various cases of Nager syndrome. The aim of our work was to present the actual genetic knowledge on this disease and its treatment procedures.

Long-term results of index finger pollicization for congenital thumb anomalies: a systematic review.

This systematic review analyses the literature for long-term outcomes of index finger pollicization. This review includes all seven studies that reported outcomes of index finger pollicization for congenital thumb aplasia or hypoplasia for qualitative review. The seven studies included 108 index finger pollicizations with 54% male children. The studies did not describe the preoperative quality and function of the transposed index finger. Grip strength was in the range of 52%-76% of the contralateral hand. Lateral pinch and key grip strength were also approximately 50% of the contralateral hand. The main drawback of the collective literature is the lack of homogeneity of results and a standardized protocol for reporting postoperative outcomes. These factors need to be addressed to compare the long-term results of pollicizations accurately.

Nager Syndrome Co-Harboring Mutation Consistent with Stickler Syndrome: A Rare Case Report

Nager syndrome, or preaxial acrofacial dysostosis, is a rare malformation characterized by abnormalities of the craniofacial skeleton and limbs. Although most cases are sporadic and some cases have been demonstrated to have an autosomal dominant or recessive mode of inheritance, SF3B4 haploinsufficiency is the most common genetic abnormality identified in this, of which only around 100 cases have been reported so far in the literature. Classically characterized by ante-mongoloid slant, retrognathia, midface retrusion and proximal limb abnormalities like thumb aplasia or hypoplasia, arachnodactyly and radioulnar synostosis, the significant morbidity and mortality in this challenging condition is primarily due to airway abnormalities causing respiratory obstruction. We report a case of genetically confirmed Nager syndrome simultaneously harbouring a mutation consistent with Stickler syndrome type II.

Common Pediatric Hand Anomalies

AbstractUpper extremity congenital anomalies in the newborn are second only to congenital heart anomalies. Some of the more commonly encountered upper extremity anomalies are trigger thumb, thumb hypoplasia, polydactyly, syndactyly, and amniotic band syndrome. While some conditions occur in isolation, others are known to commonly occur in association with syndromes. Familiarity with these conditions is important not only to provide adequate evaluation and workup of these patients but also to deliver appropriate surgical intervention and prepare parents with appropriate expectations. In this article, we outline the etiology, classification, surgical management, and outcomes of these five commonly encountered upper extremity congenital anomalies.

An alternative method to reconstruct grade-3 hypoplastic thumb by hemi-metacarpal transfer

We designed a new method using hemi-longitudinal second metacarpal bone to reconstruct grade-3 hypoplastic thumbs. Seven patients were treated in two stages. In the first stage, the second metacarpal was split longitudinally and transferred to reconstruct the first metacarpal. In the second stage, opponensplasty was performed by transferring the FDS tendon. Bone union was achieved in all cases. All patients could oppose to their middle finger at least. They managed to do daily activities such as writing, eating, using smartphones and so on. This is a useful procedure to preserve a 5-digit hand with good function in treating grade-3 hypoplastic thumbs, with no harm to the foot and no need for vascular anastomosis. Level of evidenceIV.

Aesthetic Considerations in Pollicisation Using the Buck-Gramcko Technique.

The Buck-Gramcko (BG) technique of pollicisation has stood the test of time and provides good to excellent prehensile function in thumb hypoplasia. Proponents of the technique favour it because it provides good exposure to the palmar neurovascular structures. However, the skin flap design may occasionally lead to a 'finger-like' appearance with a sharp interdigital cleft and a triphalangeal form. In this report, we describe some of the important aspects of the operative technique so that the outcome is aesthetically pleasing in addition to providing good function. Level of Evidence: Level V (Therapeutic).

An Alternative Treatment Option for Blauth III B Thumb Hypoplasia-Thumb Stabilization with Iliac Crest Bone Graft and Intermetacarpal Arthrodesis.

Thumb hypoplasia modified Blauth III B is usually treated by pollicization or, less commonly, by toe transfer. Both procedures always result in the resection of a body part, but with good cosmesis and acceptable function. We describe an intermetacarpal I/II arthrodesis with autologous bone graft augmentation to lengthen and stabilize the loose thumb. Clinical data were collected from nine patients, median age at surgery 3 years 8 months, with more than 7 years of follow-up. The results showed a grip strength on the Jamar dynamometer of approximately 61% of the unoperated hand. The Quick-DASH score was 11. The reconstructed thumb was 0.8 cm thinner and 1.9 cm shorter. Overall satisfaction on the VAS, with an average of 1.5 out of 10, is excellent with a partially usable thumb on a hand with five rays. The described procedure is a reliable treatment option with satisfactory results. In addition, none of the patients lost pincer grip between the second and third digit, but their thumb gained new function. Especially in environments where physical integrity has a high value, thumb construction instead of replacement could be considered.

Satisfactory thumb metacarpophalangeal joint stability after ligament reconstruction with flexor digitorum superficialis in children with radial longitudinal deficiency.

We investigated thumb joint stability and patient-reported and functional outcomes a minimum of 1 year after flexor digitorum superficialis opponensplasty and ligament reconstruction in 23 thumbs of 20 consecutive children with radial longitudinal deficiency. In total, 15 thumbs had preoperative multidirectional instability in the metacarpophalangeal joint. We reconstructed 22 ulnar and 16 radial collateral ligaments. At follow-up, all the metacarpophalangeal joints were stable ulnarly. Seven metacarpophalangeal joints were unstable radially despite ligament reconstruction but had no related complaints. We recommend the flexor digitorum superficialis opponensplasty as a safe and reliable procedure in hypoplastic thumbs to create stability and augment thumb strength.Level of evidence: IV.

Aesthetic outcomes of and anatomic reconstruction for Wassel type IV-D radial polydactyly using a modified Bilhaut-Cloquet procedure.

Wassel type IV-D thumb duplication is the most complex form and anatomic reconstruction is difficult. The aim of this study was to create an aesthetically satisfactory anatomical reconstruction for Wassel type IV-D radial polydactyly thumbs using the modified Bilhaut-Cloquet procedure. Surgery was performed on 24 thumbs with Wassel type IV-D radial polydactyly. To align the joint surfaces, the proximal phalanxes were unequally joined, primarily on the ulnar, and the distal phalanx was either symmetrically joined or unequally joined via curvature osteotomy. The patients were followed up for 12-91 months. The interphalangeal joint remained stable in all cases. The average functional score was 13.5 points (maximum 14 points). The overall average cosmetic score was 3.3 (maximum 4 points). Our modification of the Bihaut-Cloquet procedure produced good functional results for patients with Wassel type IV-D radial polydactyly. This method is used to correct the alignment and to stabilize the interphalangeal joint in both hypoplastic thumbs.

Clinical evidence of the association between radial longitudinal deficiency and radial polydactyly: a case series.

Radial longitudinal deficiency (RLD) is commonly associated with thumb hypoplasia. The association between RLD and radial polydactyly (RP) is uncommon, but case reports or case series have been reported. We report our experience of managing patients with this association. A total of 97 patients with RLD were seen in our department, of which six were children with concomitant RLD and RP. Four children had both RLD and RP in the same limb; of them, three also had RLD in the contralateral limb. The mean age at presentation was 11.6 months. Awareness of this association alerts the clinician to look for RLD in the presence of RP and vice versa. This case series supports recent experimental and clinical evidence that RP and RLD may be part of the same developmental spectrum. Further studies may guide its inclusion as a possible new category in the Oberg-Manske-Tonkin (OMT) classification of congenital upper-limb anomalies.Level of evidence: IV.

Donor site reconstruction by adjacent half-width metatarsal after full-width metatarsal transfer for metacarpal reconstruction in severe thumb hypoplasia.

Donor site reconstruction by adjacent half-width metatarsal after full-width metatarsal transfer for metacarpal reconstruction in severe thumb hypoplasia.

The Extensor Indicis Proprius Transposition for the Ulnar Collateral Ligament Stabilization in Thumb Hypoplasia Grades II-IIIa.

Congenital upper extremity deformities are rare, the reported incidence is from 0.15% to 0.2%. The thumb is very important for a hand's functionality, as it provides up to 50% of its total performance. Proper pinch grip formation at the age of 12 to 16 months is part of normal development; however, no functional development can affect a child's psychoemotional development. Well-described techniques such as third or fourth superficial flexor transposition can be found in the literature. There is a paucity of studies on using the extensor indicis proprius (EIP) transposition for stabilization of the ulnar collateral ligament for the first metacarpophalangeal joint (MCPJ). Techniques for the usage of EIP tendon for the reconstruction of absent extensor pollicis longus tendon are more commonly practiced as they create abduction and extension for the thumb hypoplasia grade II-IIIa. We performed EIP transposition with subperiosteally fixation for the ulnar collateral ligament stabilization for thumb hypoplasia patients with a mean age of 38 months (11 to 128) and grade II (n=9), grade IIIa (n=4), and grade IIIb (n=1). Long-term follow-up (2 to 10y) for the esthetical and functional study was performed. Patients and parents are satisfied with functional and esthetic outcomes. We believe that the EIP tendon transposition can be an alternative method for thumb hypoplasia patients grade II-IIIa reconstruction.