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Paediatric Upper Limb Conditions

69 citationsUpdated Sep 2026

Overview

Congenital upper limb anomalies encompass a spectrum of conditions including radial longitudinal deficiency, symbrachydactyly, and cleft hand. The updated Oberg-Manske-Tonkin (OMT) classification provides a straightforward framework for these anomalies, though limitations persist for hands with fewer than five skeletal digits, syndactyly, and diagnostic overlap between ulnar longitudinal deficiency and cleft hand [1]. Nomenclature remains an evolving challenge influenced by surgeon subjectivity [11], with future updates anticipated as understanding of limb formation progresses [1]. Epidemiological data from the Stockholm region align with comparable total population studies from Western Australia [13]. Assessment utilizes valid and reliable tools such as the Assisting Hand Assessment and the Prosthetic Upper Extremity Functional Index for radial deficiencies [21], while contralateral limbs serve as references for length and circumference in unilateral cases [3].

Children with these differences experience significant functional impairment closely linked to psychosocial wellbeing [7]. Caregivers report significant impacts on family life, with parental wellbeing and family impact scores significantly lower than population norms [8, 18]. Despite these challenges, children demonstrate good peer relationships and marked adaptability [9]. Surgical intervention can improve cosmesis, and most respondents prefer an appearance as close to normality as possible [40]. Long-term treatment plans for radial longitudinal deficiency are guided by the child’s overall health and deformity severity, aiming to realign the forearm, wrist, and hand while providing a functional thumb [2]. Although treatment remains controversial [9], radialization has shown promise in early follow-up for severe deformities [9].

The overall complication rate following congenital upper limb surgery is approximately 5.3%, with most complications being minor and not requiring additional surgery [6]. In obstetric brachial plexus palsy, patients with better functional recoveries exhibit smaller differences in limb length [4]. Microsurgical reconstruction is indicated for symbrachydactyly, radial-side deficiency, and long bone disorders such as congenital pseudarthrosis of the forearm [32]. Specific procedures include toe-to-hand transfers defined by the presence or absence of the thumb and fingers [16], pseudo-pollicization of the little finger to facilitate web opening and pinch [28], and thumb reconstruction without formal pollicization for mirror hand deformity [39]. The one-per-mil tumescent technique has proven safe and effective for a wide range of upper extremity surgery indications [52].

Anatomy & Pathophysiology

Embryology and Classification

The Oberg-Manske-Tonkin (OMT) classification, proposed in 2010 as a replacement for the Swanson International Federation of Societies for Surgery of the Hand system, reflects current knowledge on upper-extremity anomalies and is designed for straightforward clinical application [1]. It is accepted for classifying congenital upper limb anomalies based on the developmental biology of the upper limb [33]. The OMT system separates malformations from deformations and dysplasias [48]. Malformations are subdivided according to the primary axis of formation and differentiation affected and whether the anomalies involve the whole limb or the hand plate [48]. A prospective review of 101 patients confirmed that all diagnoses could be classified within the OMT system [48].

Current concepts in upper limb development include limb patterning along three spatial axes and the embryogenesis of various congenital hand anomalies with an emphasis on their pathogenetic basis [12]. Variation in longitudinal deficiencies is likely related to the timing and duration of an insult during early limb development [55]. In experimental models, teratogenic insults induce ulnar deficiencies earlier in gestation than radial deficiencies [55]. A child with longitudinal cleavage of the upper extremity may represent a proximal manifestation of central longitudinal deficiency with early limb bud cleavage affecting the apical ectodermal ridge and underlying mesoderm [30].

Normal Anatomy and Biomechanics

The skeleton of the hand and wrist consists of 27 bones, of which 19 are long bones [45]. The skeleton is divided into five rays, each forming a polyarticulated chain comprising the metacarpals and phalanges [45]. The radial ray or first ray is the shortest, made up of only three bones—a metacarpal and two phalanges [45]. The trapezium is clearly angled out in front of the carpal plane so that the first metacarpal makes an angle of about 45 degrees with the second metacarpal in the sagittal plane [45]. The thumb metacarpal is the shortest, the index finger metacarpal is the longest, and the others decrease in length from the third to the fifth digits [45]. The proximal and particularly the middle phalanges of the middle and ring fingers are longer than those of the index finger [45]. The transverse axis of the palm is oblique, more distal at the metacarpophalangeal joint of the index finger and more proximal at the fifth metacarpophalangeal joint [45]. This transverse axis forms an acute angle of approximately 75 degrees with the longitudinal axis [45]. Epiphyseal plates are located at the proximal ends of the phalanges and the first metacarpal, whereas they are located at the distal ends of the other metacarpals [45].

The hand consists of 19 bones, 17 articulations, and 19 muscles situated entirely within the hand, and about the same number of tendons activated by the forearm muscles [41]. The radioulnocarpal articulation has two axes of movement to which is added a third—pronation and supination from the forearm [45]. The wrist has three axes of movement, permitting the hand to be positioned in any spatial configuration and allowing it to be placed as needed for grasping [45]. The metacarpal arch is endowed with a great deal of adaptability because of the mobility of the peripheral metacarpals [59]. The index metacarpal is the most firmly fixed [59]. The ring metacarpal has about 10 degrees of mobility in flexion and extension [59]. The fifth metacarpal has a range of flexion–extension of approximately 20 degrees [59]. The second to fifth metacarpals are all bound together by various fibrous structures, the most distal of which is the deep transverse intermetacarpal ligament [59]. The deep transverse intermetacarpal ligament is better named the interglenoid ligament, because it ties together the anterior “glenoid ligaments” of the metacarpophalangeal articulations, known as the “volar plates” [59]. The keystones of the longitudinal arches are the metacarpophalangeal articulations, whose thick anterior glenoid capsules, the volar plates, prevent hyperextension [59].

The extrinsic extensors run through six different fibroosseous retinacular compartments at the wrist level [44]. Compartment contents: * First: Abductor pollicis longus and extensor pollicis brevis [44]. * Second: Extensor carpi radialis longus and extensor carpi radialis brevis [44]. * Third: Extensor pollicis longus, which turns abruptly radialward about Lister tubercle [44]. * Fourth: Extensor indicis proprius lying deep to the four tendons of the extensor digitorum communis [44]. * Fifth: Extensor digiti quinti [44]. * Sixth: Extensor carpi ulnaris tendon [44].

The extensor digitorum communis tendons of the middle, ring, and little fingers are tethered together by juncturae tendinum over the dorsum of the hand proximal to the metacarpophalangeal joint [44]. The extrinsic finger flexors are the flexor digitorum profundus and the flexor digitorum superficialis [44]. The flexor digitorum profundus inserts on the proximal volar aspect of the distal phalanx [44]. The flexor digitorum superficialis acts as a flexor of the proximal interphalangeal and metacarpophalangeal joints [44].

There are seven interosseous muscles, four dorsal and three volar [57]. The dorsal interossei are abductors [57]. The volar interossei are adductors [57]. The middle finger has two dorsal interossei (abductors) and no volar interossei (adductors) because the central axis of the hand lies within it [57].

The dorsal skin of the hand is thin and lined by a horny layer that is only 0.02 mm thick [65]. The dorsal skin possesses a normal pilosebaceous system [65]. In the middle finger, the distance between the wrist and the ungual fold shows an average increase of 3 cm as the finger goes from extension to full flexion [65]. Flexion at the metacarpophalangeal joint alone requires an average skin lengthening of 1.25 cm [65]. The dorsal integument must be supple, elastic, and malleable [64]. The skin of the back of the hand slides distally to allow metacarpophalangeal joint flexion [64]. Interphalangeal flexion is accomplished by means of a special arrangement of skin folds on the dorsum of each articulation [64]. The vessels and nerves adapt themselves to differences in length and are surrounded by loose fibroadipose connective tissue [64].

The palm forms a cutaneous unit extending from the distal transverse crease of the wrist up to the transverse crease at the base of the digits [56]. The skin of the radial portion of the palm covers the thenar eminence and the external part of the palm and is the mobile portion [56]. The skin of the ulnar and distal portion covers the hypothenar eminence where the skin has poor mobility [56]. The central triangular part of the palm has skin that is fixed and poorly vascularized, covering almost directly the superficial palmar aponeurosis [56]. The web spaces are formed from the union of two nonsymmetrical cutaneous surfaces [56]. The dorsal slope of the web space has a gradual incline and its supple skin is not adherent to the subjacent region [56]. The palmar surface of the web space is flat and precipitously interrupted, and the skin is densely adherent to the commissural skeleton [56]. The commissural skeleton is formed by the interdigital palmar (natatory) ligament between the fingers and by the distal transverse ligament at the level of the thumb web [56].

The superficial palmar fascia lies in a coronal plane beneath the palmar subcutaneous tissue [66]. The palmaris longus tendon, when present, terminates in continuity with the fibers of the proximal corner of the superficial palmar fascia [66]. Four central bands of fascia extend distally from the proximal corner of the superficial palmar fascia toward each of the fingers [66]. There is no central band for the thumb in the superficial palmar fascia [66]. The digital neurovascular structures are surrounded by a diffuse network of thin transverse oblique fibers [66]. Fibers dorsal to the neurovascular bundle are collectively called Cleland ligament [66]. Fibers palmar to the neurovascular bundle are called Grayson ligament [66].

The most common variations of the palmar arteries can be schematized by dividing the thumb into three segments, defined by the metacarpophalangeal and interphalangeal flexion creases [60]. The “princeps pollicis” artery, the terminal branch of the radial artery, crosses the first intermetacarpal space and runs along the ulnar side of the first metacarpal bone [60]. The princeps pollicis divides into two terminal rami, namely the collateral palmar arteries of the thumb, which run along the digital tunnel symmetrically and are of equal caliber [60]. An arcade located deep in the flexor tendon joins together the two arteries at the level of the distal metaphysis of the first phalanx [60]. Only 15% of dissections fall into the category of the classical description of the palmar arteries of the thumb [60]. In the second segment of the thumb, the main artery is the ulnar collateral artery [60]. The subtendinous anastomosis situated at the level of the neck of the first phalanx acts as “moderator” between the two arteries in the second segment [60]. The dorsal arteries of the thumb are vascularized by two arteries which originate from the palmar arteries at the level of the first metacarpal [60].

The extrinsic flexors of the finger consist of the flexor digitorum profundus and the flexor digitorum superficialis [63]. The flexor digitorum profundus originates from the proximal ulna and the interosseous membrane [63]. The tenosynovial sheath of the flexor pollicis longus is continuous with the radial bursa [63]. The tenosynovial sheath to the little finger is continuous with the ulnar digital bursa [63]. In some patients, the radial and ulnar bursae communicate, allowing a so-called horseshoe abscess to spread between the thumb and little finger if infection occurs in the flexor tendon sheath of either one of these digits [63]. The lumbricals originate from the radial side of the index, middle, ring, and little fingers in the palm [63]. The innervation of the flexor digitorum profundus of the index and middle fingers is through the anterior interosseous branch of the median nerve [63]. The profundus of the ring and little fingers is innervated by the ulnar nerve [63]. The flexor digitorum superficialis has two heads: The radial head originates from the proximal shaft of the radius, and the humeral ulnar head originates from the medial humeral epicondyle and coronoid process of the ulna [63]. The entire flexor digitorum superficialis muscle receives innervation from the median nerve [63]. The flexor pollicis longus originates from two heads: The radial head takes origin from the proximal radius and interosseous membrane, and an accessory head originates from the coronoid process of the ulna and from the medial epicondyle of the humerus [63]. The flexor pollicis longus inserts into the proximal base of the thumb distal phalanx and is innervated by the anterior interosseous branch of the median nerve [63].

The fibroosseous tunnel, or digital flexor sheath, extends distally to the proximal aspect of the distal phalanx [63]. The tendinous sheath consists of annular pulleys, which provide mechanical stability, and cruciate pulleys, which provide flexibility [63]. The first, third, and fifth annular pulleys (A1, A3, and A5) are located over the metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints, respectively [63]. The second and fourth pulleys (A2 and A4) are situated over the middle portion of the proximal and middle phalanges [63]. The A2 and A4 pulleys are the most essential in maintaining the mechanical advantage of the flexor tendons [63]. The tenosynovium that lines the fibroosseous tunnel supplies both nutrition and lubrication to the poorly vascularized flexor tendons [63]. Within the sheath, tendon vascularity is supplied via the vincula system: the vinculum longus and brevis [63].

In unrestricted areas, where the tendon has a straight trajectory, it is surrounded by the paratenon, areolar connective tissue arranged in layers [64]. In narrow crowded areas, the gliding mechanism is assured by the synovial sheath, which allows a considerable amplitude of movement [64]. The synovial sheaths at specific sites are surrounded by fibrous sheaths that keep the tendon close to the skeleton [64]. The fibrous sheath assumes the role of a pulley when the tendon changes direction [64]. The gliding mechanism represented by the synovial sheaths is much more developed on the palmar aspect [64]. There are three digitopalmar synovial sheaths for the flexor tendons of the index finger, long finger, and ring finger [64]. The superficial and deep flexor tendons of the digits also glide over each other [64]. On the dorsal aspect, the extensor synovial tendon sheaths are present only at the level of the wrist [64]. Each synovial sheath has a visceral and parietal component separated by a potential synovial cavity containing a very thin layer of synovial fluid [64].

The articulations of the hand form functional groups arranged in kinetic chains [64]. The position of each articulation depends on the equilibrium of forces acting at that level, and this equilibrium is subject to the position of the immediately proximal articulation [64]. The wrist influences the position of the metacarpophalangeal joint; the metacarpophalangeal joint also affects the position of the proximal interphalangeal joint, which in turn affects the distal interphalangeal joint [64]. The active factor in articular equilibrium is the dynamic balance between antagonist muscles [64]. The passive factors in articular equilibrium include the restraining action of ligaments and muscular “viscoelasticity” [64]. Single articular movements around a fixed perpendicular axis simply do not exist in the hand [64]. Almost all the movements are around oblique and variable axes, resulting in combined movements permitting optimal orientation of the phalanges at the time of prehension [64].

The sarcomere is the basic unit of muscle tissue in the muscle fiber [64]. Sarcomeres are composed of interacting molecules of myosin and actin [64]. With sarcolemmal contraction, there is overlap and interdigitation of these molecules and physical shortening of the sarcomere [64]. With maximum interdigitation, there is a maximal force of contraction [64]. The force of active contracture is diminished if the molecules are pulled apart or pushed too close together, thus losing the interdigitations by “bunching up” the molecules [64]. Sarcomeres are arranged in series to determine the length of a muscle fiber, which directly affects the distance through which a muscle can contract [64]. Sarcomeres are positioned next to one another in parallel, thus establishing the cross-sectional area of the muscle [64].

Pathophysiology of Specific Conditions

Trigger thumb is not present at birth but develops with postnatal growth [23]. Trigger finger developed earlier in life than trigger thumb [24]. The spontaneous recovery rate was higher in trigger finger than trigger thumb [24]. Patients with better functional recoveries of the affected upper extremities showed smaller differences in limb length in obstetric brachial palsy [4]. Upper extremity size differences in brachial plexus birth palsy do not correlate with patient age or clinical measurements of active movement [37].

Classification

Oberg-Manske-Tonkin (OMT): Proposed in 2010 as a replacement for the Swanson International Federation of Societies for Surgery of the Hand (IFSSH) classification, which had been the accepted international standard since 1976 [48]. The OMT system distinguishes malformations from deformations and dysplasias [48]. Malformations are subdivided according to the primary axis of formation and differentiation affected, and further categorized by whether anomalies involve the whole limb or the hand plate [48]. The system demonstrates acceptable inter- and intraobserver reliability [48] and is regarded as a needed and appropriate replacement for the IFSSH classification [82]. Designed to improve categorization based on modern understanding of limb development, the OMT classification allows categorization by narrow groups [84].

2020 OMT Update: This update reflects the latest knowledge on upper-extremity anomalies and is designed to be as straightforward as possible to employ [1]. It reclassified cleft hand from an 'unspecified' axis to the 'proximal distal axis' [84]. In a review of 150 consecutive patients with congenital upper limb anomalies, five surgeons assessed the reliability of the updated classification, which demonstrated substantial overall reliability [84]. Reliability for Group 1 ('common and easily distinguished anomaly') was near perfect, while reliability for Group 2 (all other anomalies) was moderate [84]. The updated system is reasonably practical for precise classification of congenital upper limb anomalies [79].

Swanson IFSSH: This system has been the accepted classification for the international surgical community since 1976 [48]. Its categories "Duplication," "Overgrowth," "Undergrowth," and "Constriction ring syndrome" reflect appearance [86]. The category "Generalized skeletal abnormalities" has a radiological basis [86]. The classification includes a final group labeled "Others" and uses different criteria for classification within groups [86].

HWP Severity Grading: This system scores hand, wrist, and proximal defects [20]. It allows better comparison of radially deficient limbs and treatment results between patient groups [20].

Congenital Thumb Duplication Type C3: A proposed classification based on radiographic pathoanatomy complements the system of Wu et al. by identifying four distinct subtypes of deformity [50].

Other Considerations: Limitations and controversies remain in the OMT classification for hands with less than 5 skeletal digits, syndactyly, and cases with diagnostic overlap between ulnar longitudinal deficiency and cleft hand [15]. Nomenclature for congenital upper limb anomalies has evolved over the last few decades, and naming these conditions remains an ongoing challenge influenced by surgeon subjectivity and experience [11]. The emergence of congenital upper limb anomaly registries, such as CULA North and CoULD, increases the importance of reliable classification [84]. Genetic investigations into congenital upper limb conditions leverage phenotypic data and categorization from classification systems [84].

Clinical Presentation

Classification and Nomenclature

The updated Oberg-Manske-Tonkin (OMT) classification reflects the latest knowledge on upper-extremity anomalies and is designed to be straightforward to employ [1]. For radially deficient limbs, the HWP severity grading system scores hand, wrist, and proximal defects to allow better comparison of treatment results between patient groups [20].

Epidemiology and Incidence

The incidence of congenital hand and upper limb differences generally occurs in 19.2–27.2 per 10,000 live births [47]. In patients with congenital thumb duplication, the condition is often unilateral and frequently radial and right-sided [51]. Triphalangeal thumb in these patients is more common in boys [51]. The extra thumb is often duplicated at the level of the proximal phalanx and articulated to the main thumb by a joint [51].

Physical Examination and Assessment

Perceived upper extremity and global function was significantly decreased in patients with congenital differences compared with normal individuals [17].

Specific Clinical Presentations

Freeman-Sheldon syndrome is characterized by a striking mask-like facies, ulnar deviation, flexion contractures of the fingers, resistant talipes equinovarus, and short stature [49]. In the case of longitudinal cleavage of the upper extremity, the patient had normal shoulder motion but no other function in the more superior extremity, while the inferior extremity had active flexion and extension of two fingers [30]. A 4-year-old boy presented with a normal right upper extremity and two left upper extremities, one with radial components and a rudimentary thumb ray extended from the humerus, and another with the ulna and ulnar hand elements attached to the thorax [30]. None of the 13 positive Fanconi Anaemia patients had an isolated upper limb anomaly in the absence of other Fanconi Anaemia features [31].

Psychosocial and Family Impact

Parents of children with congenital upper limb differences have unique psychological experiences and needs, with wellbeing and family impact scores significantly lower than population norms [18]. Observation of a congenital upper limb difference at birth or on an ultrasound scan is a traumatic experience for a parent [47]. Parents of children with congenital upper limb differences have feelings of guilt relating to the cause of the anomaly [47].

Investigations

Plain radiography: Radiographic assessment is central to the classification and surgical planning of congenital upper extremity anomalies. The Oberg-Manske-Tonkin (OMT) classification is designed to be straightforward to employ for these conditions [1]. In cases of congenital thumb duplication, a proposed classification system for type C3 is based on radiographic pathoanatomy and identifies four distinct subtypes of deformity [50]. For triphalangeal thumb, a distance of more than 2 mm between the abnormal bone and the distal phalanx suggests the diagnosis [54]. Radiographic findings also correlate with functional outcomes, as patients with better functional recoveries of the affected upper extremities showed smaller differences in limb length [4].

Other Considerations: A careful physical examination is essential to direct care and future testing if indicated [42]. In patients with congenital thumb duplication, triphalangeal thumb is frequently radial and right-sided [51]. The extra thumb is often articulated to the main thumb by a joint [51] and is often duplicated at the level of the proximal phalanx [51].

Treatment

General Principles and Classification

The Oberg-Manske-Tonkin (OMT) classification is the accepted framework for categorizing congenital upper limb anomalies, grounded in the developmental biology of the upper limb [33]. This updated classification reflects current knowledge of upper-extremity anomalies and is designed for straightforward clinical application [1]. Effective management requires a thorough understanding of this system to educate patients and families regarding anatomic differences and potential treatment options [34]. A careful history and stepwise examination of the affected extremity are mandatory before establishing a definitive treatment plan [34]. Not all deformities require surgery; each child’s function must be evaluated individually, often involving observation during growth before a definitive plan is created [34]. Surgical intervention depends on the specific needs of the child and the perspective of their family [33]. The surgeon may be the first to identify a specific congenital anomaly and must recognize associated conditions and syndromes to recommend appropriate testing or referral [33].

The goal of pediatric upper extremity reconstruction is to maximize patient independence while avoiding dependence on the medical team [71]. Determining which children benefit from surgical reconstruction, the optimal age for intervention, and the appropriate operative procedure represents a critical challenge for pediatric hand surgeons [71]. Guidelines for reconstruction must account for the child’s variable ability to cooperate postoperatively, anesthesia risks in early life, the size of structures, the absence of critical parts, and the need for growth [71]. The overall health of the child and the severity of deformities guide long-term plans aimed at realigning the forearm, wrist, and hand while providing a functional thumb [2]. Advances in understanding the anatomy and pathophysiology of these disorders allow for the restoration of aesthetics, function, and quality of life [34]. Continuing education, knowledge of new surgical techniques, and collaboration with an interdisciplinary team equip the orthopedic surgeon to provide optimal care [34].

Assessment and Outcomes: The HWP severity grading system, which scores hand, wrist, and proximal defects, facilitates better comparison of radially deficient limbs and treatment results between patient groups [20]. Instruments such as the Assisting Hand Assessment and the Prosthetic Upper Extremity Functional Index provide valid and reliable results in children with radial deficiencies [21]. Perceived upper extremity and global function are significantly decreased in patients with congenital differences compared with normal individuals [17]. Children with congenital upper limb differences experience significant functional impairment, and physical function is closely linked with psychosocial wellbeing [7]. Research on congenital hand anomalies continues to advance via retrospective investigations and surgical technique improvements, though most publications are retrospective reviews or smaller case series, typically level IV evidence [14]. The overall complication rate following congenital upper limb surgery is approximately 5.3%, with most events being minor and not requiring additional surgery [6]. Surgery can improve cosmesis in congenitally different hands, and most respondents prefer an appearance as close as possible to normality [40]. Early corrective surgery is recommended for progressive or significant deformities [38].

Terminology and Development: The care of children with congenital upper limb differences involves changing landscapes in research, treatment, civil rights protection, disability awareness, and accepted terminology to guide clinical and nonclinical conversations [10]. Historical case note studies observe an evolution of nomenclature for congenital upper limb anomalies over recent decades, highlighting that naming these conditions remains an ongoing challenge influenced by surgeon subjectivity and experience [11]. Current concepts in upper limb development, limb patterning along three spatial axes, and the embryogenesis of various congenital hand anomalies emphasize their pathogenetic basis [12]. Occupational therapy and/or hand therapy are often vital to the function and postoperative recovery of the child’s upper extremity [34].

Specific Procedures and Techniques

Radial Deficiency and Reconstruction: Treatment for radial longitudinal deficiency remains controversial, but radialization has shown promise in early follow-up for severe deformities [9]. Primary indications for microsurgical reconstruction of congenital hand anomalies include the treatment of symbrachydactyly, radial-side deficiency, and long bone disorders such as congenital pseudarthrosis of the forearm [32]. Specific indications for toe transfers are defined based on the presence or absence of the thumb and fingers [16]. In a case of longitudinal cleavage of the upper extremity, excision of the nonfunctioning radius and thumb and an "on-top-plasty" by mobilizing the inferior extremity on its neurovascular pedicle and fusing the ulna to the distal aspect of the humerus was performed [30].

Thumb and Finger Procedures: The procedure of pseudo-pollicization of the little finger facilitates opening of the web and pinch between the little and ring finger tips, continuing to be of functional value to children as they grow [28]. The technique of thumb reconstruction without formal pollicization is an acceptable option for mirror hand deformity [39]. Surgery is effective in correcting the deformity of the thumb delta phalanx in Rubinstein–Taybi Syndrome, although there is a risk of incomplete correction or recurrence [87]. There is no need to delay surgery for the small extra phalanx in young children with triphalangeal thumb, as removal can be performed and procedures are exchangeable [90]. Postoperative rehabilitation could not fully compensate for anatomical abnormalities in pollicization for congenital thumb malformations, suggesting the need to evaluate more specific functional tasks [19].

Forearm and Bone Lengthening: The one-bone forearm procedure improves malformed forearm functions, increasing the possibility of carrying out daily life activities in congenital ulnar club hand [29]. The Partial Excision Greenstick (PEG) Osteotomy is a simple and effective method for correcting malalignment in the small bones of children's hands [25]. Lengthening via callus distraction in the hand and upper extremity ranged between 15 and 135 mm between the phalanges and the humerus [88]. The degree of lengthening via callus distraction ranged between 20% and 400% of the specific skeletal segment lengthened [88]. Poor bone regeneration in callus distraction occurs with either inadequate fixation or lengthening through dysvascular bone [88]. Premature consolidation occurs because of a delay in the onset of the lengthening process after an early healing period of more than a week [88]. Soft-tissue injury can be avoided by ensuring adequate soft-tissue coverage, exposure and release of dense scar tissue, and avoiding the use of "through-and-through" pins [88]. Angular deformity can result from inadequate control of the distal segment, choice of an unstable device, and unrecognized soft-tissue tightness [88].

Adjuncts: The one-per-mil tumescent technique proved safe and effective for a wide range of indications in upper extremity surgeries [52].

Cerebral Palsy and Spasticity

Non-Operative Management: Nonsurgical treatment is the first-line approach for upper extremity cerebral palsy and includes a combination of hand therapy, splinting, casting, bracing treatment, and neuromuscular medications [72]. Management should include a multidisciplinary approach with a thorough family/caregiver discussion regarding functional needs and surgical outcome expectations [72].

Operative Indications and Goals: Surgical treatment options involve a combination of tendon lengthenings, transfers, and/or releases and joint arthrodesis [72]. Care must be taken to fully evaluate each area of the upper extremity to correctly indicate patients for surgical treatment [72]. Goals of surgical intervention include improving hygiene, function of the affected extremity, and aesthetics as appropriate [72].

Evidence and Outcomes: Tendon transfer surgery in upper-extremity cerebral palsy is more effective than botulinum toxin injections or regular ongoing therapy [74]. Reduction in upper-extremity tone after lumbar selective rhizotomy in children with spastic cerebral palsy has been reported [74]. Early results of anterior elbow release with and without biceps lengthening in patients with cerebral palsy have been reported [74]. A randomized, double-blind, placebo-controlled study of the short-term outcomes of treatment with botulinum A toxin for upper extremity spasticity in children with cerebral palsy has been conducted [74]. Management of the spastic wrist and hand in cerebral palsy has been described [74]. Effects of upper-extremity surgery on manual performance of children and adolescents with cerebral palsy using a multidisciplinary approach with shared decision-making have been reported [74]. Prevalence and pattern of upper limb involvement in cerebral palsy have been described [74]. Shoulder arthroplasty in patients with cerebral palsy has been compared to patients with osteoarthritis in a matched cohort study [74]. The effect of upper limb deformities on gross motor and upper limb functions in children with spastic cerebral palsy has been reported [74]. The outcome of hand surgery in children with spasticity—a 9-year follow-up study has been reported [74]. Functional outcomes following single-event multilevel surgery of the upper extremity for children with hemiplegic cerebral palsy have been reported [74]. Preferred options and evidence for upper limb surgery for spasticity in cerebral palsy, stroke, and brain injury have been reviewed [74].

Arthrogryposis

General Principles: Treatment of arthrogryposis of any type can be challenging, but improvement can be seen in many patients with early intervention and proper nonsurgical and surgical management [73]. The goal for any treatment strategy is to maximize function with the minimal amount of hospitalization necessary [73]. Physical and occupational therapy in conjunction with bracing can help improve function in many children, so that surgical management can be mitigated or avoided [73]. Surgical treatment should be reserved for enhancing function, not just improving the appearance of the deformity [73]. Orthotic devices and surgical treatment should focus on improving function, not necessarily on correcting deformity [73]. Both the surgical and nonsurgical management of upper extremity amyoplasia should focus on enhancing the patient’s ability to complete hand-to-mouth activities [73]. The goal of any treatment plan for a child with arthrogryposis is to maximize function and independence [76]. Basic upper limb function is equally as important to the well-being of children with arthrogryposis as ambulation [76].

Surgical Indications and Goals: If the child does possess limbs and joints in optimal position for function by the first or second year of life, surgery should be performed to reorient and/or increase joint motion [76]. The primary goals for the upper extremity are to have “one hand to eat and one to wipe” [76]. The rate-limiting step in optimizing the child’s independence is uniformly finger and thumb function [76]. The child must be able to acquire the food, grip firmly enough to bring food to the mouth, and release the food by opening the digits [76]. The creation or maintenance of an appropriate thumb-index web space is important for single-handed manipulation of objects [73].

Joint-Specific Management: For nearly all children with arthrogryposis, shoulder rotation should be centered at 15 to 30 degrees of internal rotation [76]. If the optimal shoulder position is not present, the surgeon should consider a humeral rotational osteotomy [76]. If elbow flexion is insufficient to reach the mouth, a release of the posterior elbow should be performed [76]. Elbows should be released within the first 2 years of life to provide maximal passive motion, with an optimal range between 30 degrees of extension and at least 100 degrees of flexion [76]. For the minority of children that lack sufficient extension, soft tissue releases, osteotomies, and tendon transfers may be required [76]. The wrist and the elbow combined must have enough extension to reach the plate or the table and sufficient flexion to allow the hand to reach the mouth [76]. Forearm and shoulder rotation need to allow the hand to face the plate and then the mouth [76].

Complications

Functional Outcomes: Most complications following congenital upper limb surgery were minor and did not require additional surgery [6]. In patients undergoing pollicization for congenital thumb malformations, postoperative rehabilitation could not fully compensate for anatomical abnormalities [19].

Other Considerations: Individuals with moderate to severe radial longitudinal deficiency exhibit weaker grasp compared with those who have milder forms of radial longitudinal deficiency [26]. These patients also retain more primitive grasp patterns compared with those who have milder forms of radial longitudinal deficiency [26]. Furthermore, individuals with moderate to severe radial longitudinal deficiency use their thumbs for fewer activities compared with those who have milder forms of radial longitudinal deficiency [26].

Recovery

Functional milestones: Perceived upper extremity function and perceived global function are significantly decreased in patients with congenital differences compared with normal individuals [17]. In radial longitudinal deficiency, functional outcomes after index pollicization are stratified by severity; individuals with moderate to severe deficiency use their thumbs for fewer activities, exhibit weaker grasp, and retain more primitive grasp patterns compared with those who have milder forms of the condition [26].

Other Considerations: Postoperative rehabilitation cannot fully compensate for anatomical abnormalities following pollicization for congenital thumb malformations [19]. For congenital ulnar club hand, the one-bone forearm procedure improves malformed forearm functions and increases the possibility of carrying out daily life activities [29]. In radial polydactyly, the development of the retained digit is not influenced by the ablation of the extra digit [36].

Key Evidence

  • [L5] The updated OMT classification reflects the latest knowledge on upper-extremity anomalies and is designed to be as straightforward as possible to employ, with future updates anticipated as understanding of limb formation progresses. [1] (10.1016/j.jhsa.2020.01.002)
  • [L5] The overall health of the child and the severity of deformities guide long-term treatment plans aimed at realigning the forearm, wrist, and hand while providing a functional thumb. [2] (10.5435/00124635-200701000-00005)
  • [L4] Contralateral limbs can be used for comparison of length and circumference of the arm and forearm in cases of unilateral upper extremity abnormality. [3] (10.1177/1558944718824706)
  • [L3] Patients with better functional recoveries of the affected upper extremities showed smaller differences in limb length. [4] (10.1007/s11552-010-9270-z)
  • [L5] Findings report the status of outcome measure utility and use in pediatric UE function. [5] (10.1016/j.jht.2014.09.004)
  • [L2] The overall complication rate following congenital upper limb surgery was approximately 5.3%, with most events being minor and not requiring additional surgery. [6] (10.1016/j.jhsa.2025.11.004)
  • [L3] Children with congenital upper limb differences experience significant upper extremity functional impairment, and physical function is closely linked with psychosocial wellbeing. [7] (10.1177/17531934251355084)
  • [L2] Caregivers of children with congenital upper extremity differences report a significant impact on family life. [8] (10.1016/j.jhsa.2024.02.011)
  • [L4] Treatment for radial longitudinal deficiency remains controversial, but radialization has shown promise in early follow-up for severe deformities, while children with congenital upper-limb differences demonstrate good peer relationships and marked adaptability. [9] (10.2106/jbjs.22.01323)
  • [L5] The article provides information on the changing landscapes in research, treatment, civil rights' protection, disability awareness, and accepted terminology in the care of children with congenital upper limb differences to guide clinical and nonclinical conversations between patients and their families. [10] (10.1016/j.jhsa.2021.07.001)
  • [L4] This historical case note study observed an evolution of nomenclature for congenital upper limb anomalies over the last few decades and highlighted that naming these conditions remains an ongoing challenge influenced by surgeon subjectivity and experience. [11] (10.1177/17531934231160400)
  • [L5] The paper reviews current concepts in the steps of upper limb development, limb patterning along three spatial axes, and the embryogenesis of various congenital hand anomalies with an emphasis on their pathogenetic basis. [12] (10.1016/j.jhsa.2013.03.018)
  • [L3] The incidence of congenital upper limb anomalies in the Stockholm region was similar to the only previously comparable total population study from Western Australia. [13] (10.1016/j.jhsa.2010.07.007)
  • [L5] [14] (10.1016/j.jhsa.2013.03.023)
  • [L4] Limitations and controversies remain, particularly for hands with less than 5 skeletal digits, syndactyly, and cases with diagnostic overlap between ulnar longitudinal deficiency and cleft hand. [15] (10.1016/j.jhsa.2022.07.007)
  • [L4] The study defines specific indications for toe transfers based on the presence or absence of the thumb and fingers. [16] (10.1007/s11552-013-9534-5)
  • [L4] In contrast, the perceived upper extremity and global function was significantly decreased in patients with congenital differences compared with normal individuals. [17] (10.1016/j.jhsa.2015.12.004)
  • [L3] Parents of children with congenital upper limb differences have unique psychological experiences and needs, with wellbeing and family impact scores significantly lower than population norms. [18] (10.1177/17531934241256793)
  • [L4] The study suggests evaluating more specific functional tasks and notes that postoperative rehabilitation could not fully compensate for anatomical abnormalities. [19] (10.1177/17531934251375812)
  • [L4] The HWP severity grading system, which scores hand, wrist, and proximal defects, allows better comparison of radially deficient limbs and treatment results between patient groups. [20] (10.1177/1753193413518709)
  • [L1] The current results contribute to the evidence that the instruments, especially the Assisting Hand Assessment and the Prosthetic Upper Extremity Functional Index, provide valid and reliable results in children with RDs. [21] (10.1016/j.jhsa.2007.01.011)
  • [L3] The results suggest that trigger thumb is not present at birth but develops with postnatal growth. [23] (10.1016/j.jhsa.2005.12.024)
  • [L3] Trigger finger developed earlier in life than trigger thumb and the spontaneous recovery rate was higher in trigger finger than trigger thumb. [24] (10.1054/jhsb.2000.0417)
  • [L4] This approach is a simple and effective way of correcting malalignment in the small bones of children's hands. [25] (10.1177/1753193409103246)
  • [L4] Individuals with moderate to severe radial longitudinal deficiency have unique anatomical factors that affect outcomes after pollicization, using their thumbs for fewer activities, having weaker grasp, and retaining more primitive grasp patterns compared with those who have milder forms of radial longitudinal deficiency. [26] (10.1016/j.jhsa.2024.02.010)
  • [L4] The procedure facilitates opening of the web and pinch between the little and ring finger tips, and continues to be of functional value to the children as they grow. [28] (10.1177/1753193413498191)
  • [L4] The one-bone forearm procedure improves malformed forearm functions, thus increasing the possibility of carrying out daily life activities. [29] (10.1016/j.jhsa.2011.10.027)
  • [L4] [30] (10.1016/j.jhsa.2010.07.017)
  • [L3] None of the 13 positive Fanconi Anaemia patients had an isolated upper limb anomaly in the absence of other Fanconi Anaemia features. [31] (10.1016/j.jhsa.2013.08.029)
  • [L5] Primary indications remain the treatment of symbrachydactyly, radial-side deficiency, and long bone disorders such as congenital pseudarthrosis of the forearm. [32] (10.1016/s0749-0712(21)00147-5)
  • [L4] The development of the retained digit is not influenced by the ablation of the extra digit. [36] (10.1016/j.jhsa.2016.06.012)
  • [L4] These differences do not correlate with patient age or clinical measurements of active movement. [37] (10.1007/s11552-008-9103-5)
  • [L4] The authors recommend early corrective surgery for progressive or significant deformities. [38] (10.1054/jhsb.2001.0643)
  • [L4] The technique of thumb reconstruction used in the current series is an acceptable option. [39] (10.1177/1753193412475129)
  • [L4] This study demonstrated that surgery could improve cosmesis in congenitally different hands and overall, most respondents prefer an appearance that is as close as possible to normality. [40] (10.1177/17531934221139698)
  • [L4] [47] (10.1177/17531934241249014)
  • [L4] [48] (10.1016/j.jhsa.2013.03.019)
  • [L4] The syndrome is characterized by a striking mask-like facies, ulnar deviation, flexion contractures of the fingers, resistant talipes equinovarus, and short stature. [49] (10.2106/00004623-197658010-00033)
  • [L3] The proposed system is based on radiographic pathoanatomy and complements that of Wu et al. by identifying four distinct subtypes of deformity. [50] (10.1186/s12891-024-07916-x)
  • [L3] Triphalangeal thumb in patients with congenital thumb duplication is more common in boys, often duplicated at the level of the proximal phalanx, with the extra thumb articulated to the main thumb by a joint, often unilateral, and frequently radial and right-sided. [51] (10.1016/j.jhsa.2024.11.004)
  • [L4] It proved safe and effective for a wide range of indications. [52] (10.1016/j.jhsa.2013.09.034)
  • [L4] A distance of more than 2 mm between the abnormal bone and the distal phalanx suggests a triphalangeal thumb. [54] (10.1016/j.jhsa.2019.04.013)
  • [L4] [55] (10.1016/j.jhsa.2009.04.042)
  • [L4] The updated OMT classification system is reasonably practical for precise classification of these anomalies. [79] (10.1016/j.jhsa.2023.05.016)
  • [L3] With further refinements, we regard the OMT classification as a needed and appropriate replacement for the IFSSH classification. [82] (10.1016/j.jhsa.2013.11.014)
  • [Commentary] [84] (10.1177/17531934221123447)
  • [Commentary] [86] (10.1016/j.jhsa.2017.02.019)
  • [L4] Surgery is effective in correcting the deformity, but there is a risk of incomplete correction or recurrence. [87] (10.1177/1753193409354523)
  • [L4] [88] (10.2106/jbjs.j.01106)
  • [L3] There is no need for delaying surgery for the small extra phalanx in young children, as removal of the extra phalanx can be performed and procedures are exchangeable. [90] (10.1177/1753193415576459)

See Also

  • Trigger Finger

References

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a. UNLESS OTHERWISE SEPARATELY UNDERTAKEN BY THE LICENSOR, TO THE EXTENT POSSIBLE, THE LICENSOR OFFERS THE LICENSED MATERIAL AS-IS AND AS-AVAILABLE, AND MAKES NO REPRESENTATIONS OR WARRANTIES OF ANY KIND CONCERNING THE LICENSED MATERIAL, WHETHER EXPRESS, IMPLIED, STATUTORY, OR OTHER. THIS INCLUDES, WITHOUT LIMITATION, WARRANTIES OF TITLE, MERCHANTABILITY, FITNESS FOR A PARTICULAR PURPOSE, NON-INFRINGEMENT, ABSENCE OF LATENT OR OTHER DEFECTS, ACCURACY, OR THE PRESENCE OR ABSENCE OF ERRORS, WHETHER OR NOT KNOWN OR DISCOVERABLE. WHERE DISCLAIMERS OF WARRANTIES ARE NOT ALLOWED IN FULL OR IN PART, THIS DISCLAIMER MAY NOT APPLY TO YOU.

b. TO THE EXTENT POSSIBLE, IN NO EVENT WILL THE LICENSOR BE LIABLE TO YOU ON ANY LEGAL THEORY (INCLUDING, WITHOUT LIMITATION, NEGLIGENCE) OR OTHERWISE FOR ANY DIRECT, SPECIAL, INDIRECT, INCIDENTAL, CONSEQUENTIAL, PUNITIVE, EXEMPLARY, OR OTHER LOSSES, COSTS, EXPENSES, OR DAMAGES ARISING OUT OF THIS PUBLIC LICENSE OR USE OF THE LICENSED MATERIAL, EVEN IF THE LICENSOR HAS BEEN ADVISED OF THE POSSIBILITY OF SUCH LOSSES, COSTS, EXPENSES, OR DAMAGES. WHERE A LIMITATION OF LIABILITY IS NOT ALLOWED IN FULL OR IN PART, THIS LIMITATION MAY NOT APPLY TO YOU.

c. The disclaimer of warranties and limitation of liability provided above shall be interpreted in a manner that, to the extent possible, most closely approximates an absolute disclaimer and waiver of all liability.

Section 6 -- Term and Termination.

a. This Public License applies for the term of the Copyright and Similar Rights licensed here. However, if You fail to comply with this Public License, then Your rights under this Public License terminate automatically.

b. Where Your right to use the Licensed Material has terminated under Section 6(a), it reinstates:

1. automatically as of the date the violation is cured, provided it is cured within 30 days of Your discovery of the violation; or

2. upon express reinstatement by the Licensor.

For the avoidance of doubt, this Section 6(b) does not affect any right the Licensor may have to seek remedies for Your violations of this Public License.

c. For the avoidance of doubt, the Licensor may also offer the Licensed Material under separate terms or conditions or stop distributing the Licensed Material at any time; however, doing so will not terminate this Public License.

d. Sections 1, 5, 6, 7, and 8 survive termination of this Public License.

Section 7 -- Other Terms and Conditions.

a. The Licensor shall not be bound by any additional or different terms or conditions communicated by You unless expressly agreed.

b. Any arrangements, understandings, or agreements regarding the Licensed Material not stated herein are separate from and independent of the terms and conditions of this Public License.

Section 8 -- Interpretation.

a. For the avoidance of doubt, this Public License does not, and shall not be interpreted to, reduce, limit, restrict, or impose conditions on any use of the Licensed Material that could lawfully be made without permission under this Public License.

b. To the extent possible, if any provision of this Public License is deemed unenforceable, it shall be automatically reformed to the minimum extent necessary to make it enforceable. If the provision cannot be reformed, it shall be severed from this Public License without affecting the enforceability of the remaining terms and conditions.

c. No term or condition of this Public License will be waived and no failure to comply consented to unless expressly agreed to by the Licensor.

d. Nothing in this Public License constitutes or may be interpreted as a limitation upon, or waiver of, any privileges and immunities that apply to the Licensor or You, including from the legal processes of any jurisdiction or authority.

Creative Commons is not a party to its public licenses. Notwithstanding, Creative Commons may elect to apply one of its public licenses to material it publishes and in those instances will be considered the “Licensor.” The text of the Creative Commons public licenses is dedicated to the public domain under the CC0 Public Domain Dedication. Except for the limited purpose of indicating that material is shared under a Creative Commons public license or as otherwise permitted by the Creative Commons policies published at creativecommons.org/policies, Creative Commons does not authorize the use of the trademark "Creative Commons" or any other trademark or logo of Creative Commons without its prior written consent including, without limitation, in connection with any unauthorized modifications to any of its public licenses or any other arrangements, understandings, or agreements concerning use of licensed material. For the avoidance of doubt, this paragraph does not form part of the public licenses.

Creative Commons may be contacted at creativecommons.org.