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In medicine, split hand syndrome is a neurological syndrome in which the hand muscles on the side of the thumb (lateral, thenar eminence) appear wasted, whereas the muscles on the side of the little finger (medial, hypothenar eminence) are spared. Anatomically, the abductor pollicis brevis and first dorsal interosseous muscle are more wasted than the abductor digiti minimi.
If lesions affecting the branches of the ulnar nerve that run to the wasted muscles are excluded, the lesion is almost sure to be located in the anterior horn of the spinal cord at the C8-T1 level. It has been proposed as a relatively specific sign for amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease). It can also occur in other disorders affecting the anterior horn, such as spinal muscular atrophy, Charcot-Marie-Tooth disease, poliomyelitis and progressive muscular atrophy. A slow onset and a lack of pain or sensorial symptoms are arguments against a lesion of the spinal root or plexus brachialis. To an extent, these features can also be seen in normal aging (although technically, the apparent muscle wasting is sarcopenia rather than atrophy).
The term split hand syndrome was first coined in 1994 by a researcher from the Cleveland Clinic called Asa J. Wilbourn.
In general, ulnar neuropathy will result in symptoms in a specific anatomic distribution, affecting the little finger, the ulnar half of the ring finger, as well as the intrinsic muscles of the hand.
The specific symptoms experienced in the characteristic distribution depend on the specific location of ulnar nerve impingement. Symptoms of ulnar neuropathy may be motor, sensory, or both depending on the location of injury. Motor symptoms consistent of muscle weakness; sensory symptoms or paresthesias consist of numbness or tingling in the areas innervated by the ulnar nerve.
- Proximal impingement is associated with mixed symptoms, as the proximal nerve consists of mixed sensory and motor innervation.
- Distal impingement is associated with variable symptoms, as the ulnar nerve separates near the hand into distinct motor and sensory branches.
In cubital tunnel syndrome (a proximal impingement), sensory and motor symptoms tend to occur in a certain sequence. Initially, there may be numbness of the small and ulnar fourth finger which may be transient. If the impingement is not corrected, the numbness may become constant and progress to hand weakness. A characteristic resting hand position of "ulnar claw," where the small and ring fingers curl up, occurs late in the disease and is a sign of severe neuropathy.
By contrast, in Guyon's canal syndrome (distal impingement) motor symptoms and claw hand may be more pronounced, a phenomenon known as the ulnar paradox. Also the back of the hand will have normal sensation.
Symptoms include intrinsic minus hand deformity, paralysis of intrinsic hand muscles, and C8/T1 Dermatome distribution numbness. Involvement of T1 may result in Horner's syndrome, with ptosis, and miosis. Weakness or lack of ability to use specific muscles of the shoulder or arm.It can be contrasted to Erb-Duchenne's palsy, which affects C5 and C6.
Most patients diagnosed with cubital tunnel syndrome have advanced disease (atrophy, static numbness, weakness) that might reflect permanent nerve damage that will not recover after surgery. When diagnosed prior to atrophy, weakness or static numbness, the disease can be arrested with treatment. Mild and intermittent symptoms often resolve spontaneously.
Diagnosing the congenital clasped thumb is difficult in the first three to four months of life, as it is normal when the thumb is clutched into the palm in these first months.
Diagnoses that cause the same flexion or adduction abnormalities of the thumb are:
- Congenital clasped thumb
- Congenital Trigger thumb (flexion of the interphalangeal joint) - Trigger finger
- Spasticity: overstimulation of muscles
Syndrome associated flexion-adduction of the thumb:
- Freeman-Sheldon syndrome (a congenital, heritable affection of the face, the hands, the feet and some joints)
- Distal arthrogryposis
- MASA syndrome
- X-linked hydrocephalus
- Adducted thumb syndrome
- Waardenburg syndrome
- Whistling face syndrome (Freeman-Sheldon syndrome)
- Digitotalar dysmorphism
- Multiple pterygium syndrome
Klumpke's paralysis is a form of paralysis involving the muscles of the forearm and hand, resulting from a brachial plexus injury in which the eighth cervical (C8) and first thoracic (T1) nerves are injured either before or after they have joined to form the lower trunk. The subsequent paralysis affects, principally, the intrinsic muscles of the hand (notably the interossei, thenar and hypothenar muscles) and the flexors of the wrist and fingers (notably flexor carpi ulnaris and ulnar half of the flexor digitorum profundus). Forearm pronators and wrist flexors may be involved, as may dilators of the iris and elevators of the eyelid (both of which may be seen in the case of associated Horner's syndrome). The classic presentation of Klumpke's palsy is the “claw hand” where the forearm is supinated and the wrist and fingers are flexed. If Horner syndrome is present, there is miosis (constriction of the pupils) in the affected eye.
The injury can result from difficulties in childbirth. The most common aetiological mechanism is caused by a traumatic vaginal delivery. The risk is greater when the mother is small or when the infant is of large weight. Risk of injury to the lower brachial plexus results from traction on an abducted arm, as with an infant being pulled from the birth canal by an extended arm above the head or with someone catching himself by a branch as he falls from a tree. Lower brachial plexus injuries should be distinguished from upper brachial plexus injuries, which can also result from birth trauma but give a different syndrome of weakness known as Erb's palsy.
Other trauma, such as motorcycle accidents, that have similar spinal cord injuries to C-8 & T-1, also show the same symptom's of Klumpke's paralysis.
Very frequent signs
- Abnormal gastrointestinal tract
- Absent pectoral muscles
- Brachydactyly (Short fingers)
- Dextrocardia
- Diaphragmatic hernia/defect
- Humerus absent/abnormal
- Liver/biliary tract anomalies
- Maternal diabetes
- Oligodactyly/missing fingers
- Radius absent/abnormal
- Rhizomelic micromelia (relatively shorter proximal segment of the limbs compared to the middle and the distal segments)
- Sparsity or abnormality of axillary hair on affected side
- Syndactyly of fingers (webbing)
- Ulna absent/abnormal
- Upper limb asymmetry
- Abnormal rib
- Simian crease on affected side
Frequent signs
- Hypoplastic/absent nipples
- Scapula anomaly
Occasional signs
- Agenesis/hypoplasia of kidneys
- Encephalocele/exencephaly
- Abnormal morphology of hypothalamic-hypophyseal axis
- Abnormal function of hypothalamic-hypophyseal axis
- Microcephaly
- Preaxial polydactyly
- Ureteric anomalies (reflux/duplex system)
- Vertebral segmentation anomaly
"Infant’s persistent thumb-clutched hand, flexion-adduction deformity of the thumb, pollex varus, thumb in the hand deformity."
Congenital clasped thumb describes an anomaly which is characterized by a fixed thumb into the palm at the metacarpophalangeal joint in one or both hands.
The incidence and genetic background are unknown. A study of Weckesser et al. showed that boys are twice as often affected with congenital clasped thumb compared to girls. The anomaly is in most cases bilateral (present in both hands).
A congenital clasped thumb can be an isolated anomaly, but can also be attributed to several syndromes.
Tandem gait is a gait (method of walking or running) where the toes of the back foot touch the heel of the front foot at each step. Neurologists sometimes ask patients to walk in a straight line using tandem gait as a test to help diagnose ataxia, especially truncal ataxia, because sufferers of these disorders will have an unsteady gait. However, the results are not definitive, because many disorders or problems can cause unsteady gait (such as vision difficulties and problems with the motor neurons or associative cortex). Therefore, inability to walk correctly in tandem gait does not prove the presence of ataxia.
Profoundly affected tandem gait with no other perceptible deficits is a defining feature of posterior vermal split syndrome.
Suspects may also be asked to perform a tandem gait walk during the "walk and turn" part of a field sobriety test.
Symptoms are pain at the radial side of the wrist, spasms, tenderness, occasional burning sensation in the hand, and swelling over the thumb side of the wrist, and difficulty gripping with the affected side of the hand. The onset is often gradual. Pain is made worse by movement of the thumb and wrist, and may radiate to the thumb or the forearm.
The triphalangeal thumb has a different appearance than normal thumbs. The appearance can differ widely; the thumb can be a longer thumb, it can be deviated in the radio-ulnar plane (clinodactyly), thumb strength can be diminished. In the case of a five fingered-hand it has a finger-like appearance, with the position in the plane of the four fingers, thenar muscle deficiency, and additional length. There is often a combination with radial polydactyly.
Generally, triphalangeal thumbs are non-opposable. In contrast to most people with opposable thumbs, a person suffering from TPT cannot easily place his or her thumb opposite the other four digits of the same hand. The opposable thumb's ability to effortlessly utilize fingers in a "pinch" formation is critical in precision gripping. For the thumb to adequately grip, certain thumb criteria must be met (e.g. suitable position and length, stable joints and good thenar muscle strength). Because triphalangeal thumbs cannot easily oppose and do not possess many of the optimal qualities found in most opposable thumbs, they tend to cause the hand to be less effective in use and, therefore, prove to be more problematic in daily life.
Poland syndrome, named after British surgeon Alfred Poland, is a rare birth defect characterized by underdevelopment or absence of the chest muscle (pectoralis) on one side of the body, and usually also webbing of the fingers (cutaneous syndactyly) of the hand on the same side (the ipsilateral hand). In most affected individuals, the missing part is the large section of the muscle that normally attaches to the upper arm on one side and the breastbone (sternum) on the other. Other abnormalities may occur on the affected side of the torso. In some cases, additional muscles in the chest wall, side, and shoulder are missing or underdeveloped.
There may also be rib cage abnormalities, such as shortened ribs, and the ribs may be noticeable due to less fat under the skin (subcutaneous fat). Breast and nipple abnormalities may also occur, and underarm (axillary) hair is sometimes sparse or abnormally placed. In most cases, the abnormalities in the chest area do not cause health problems or affect movement. Poland syndrome most often affects the right side of the body, and occurs more often in males than in females.
It is usually considered a unilateral condition. Some have claimed that the term can be applied in bilateral presentation, but others recommend using alternate terminology in those cases.
It is characterized by a nearly symmetrical presence of a spoon hand (classical type) or, more frequently, an oligodactylous hand. Individuals with this syndrome present the following symptoms: carpal, metacarpal and digital synostoses, disorganization of carpal bones, numeric reduction of digital rays and toe syndactyly. Additionally, other symptoms may include radioulnar synostosis, brachymesomelia, radius head dislocation, metatarsal synostoses and numeric reduction of rays.
Any fracture in elbow region or upper arm may lead to Volkmann's ischemic contracture, but it is especially associated with supracondylar fracture of the humerus.
Volkmann's contracture results from acute ischaemia and necrosis of the muscle fibres of the flexor group of muscles of the forearm, especially the flexor digitorum profundus and flexor pollicis longus. The muscles become fibrotic and shortened.
The condition is caused by obstruction on the brachial artery near the elbow, possibly from improper use of a tourniquet, improper use of a plaster cast, or compartment syndrome. It is also caused by fractures of the forearm bones if they cause bleeding from the major blood vessels of the forearm.
Injuries to the arm, forearm or wrist area can lead to various nerve disorders. One such disorder is median nerve palsy. The median nerve controls the majority of the muscles in the forearm. It controls abduction of the thumb, flexion of hand at wrist, flexion of digital phalanx of the fingers, is the sensory nerve for the first three fingers, etc. Because of this major role of the median nerve, it is also called the eye of the hand. If the median nerve is damaged, the ability to abduct and oppose the thumb may be lost due to paralysis of the thenar muscles. Various other symptoms can occur which may be repaired through surgery and tendon transfers. Tendon transfers have been very successful in restoring motor function and improving functional outcomes in patients with median nerve palsy.
Volkmann's contracture is a permanent flexion contracture of the hand at the wrist, resulting in a claw-like deformity of the hand and fingers. Passive extension of fingers is restricted and painful.
De Quervain syndrome is diagnosed clinically, based on history and physical examination, though diagnostic imaging such as x-ray may be used to rule out fracture, arthritis, or other causes, based on the patient's history and presentation. Finkelstein's test is a physical exam maneuver used to diagnose de Quervain syndrome. To perform the test, the examiner grasps the thumb and sharply deviates the hand toward the ulnar side. If sharp pain occurs along the distal radius (top of forearm, about an inch below the wrist), de Quervain's syndrome is likely. While a positive Finkelstein's test is often considered pathognomonic for de Quervain syndrome, the maneuver can also cause pain in those with osteoarthritis at the base of the thumb.
Differential diagnoses include:
1. Osteoarthritis of the first carpo-metacarpal joint
2. Intersection syndrome—pain will be more towards the middle of the back of the forearm and about 2–3 inches below the wrist
3. Wartenberg's syndrome
In general there are five types of thumb hypoplasia, originally described by Muller in 1937 and improved by Blauth, Buck-Gramcko and Manske.
- Type I: the thumb is small, normal components are present but undersized. Two muscles of the thumb, the abductor pollicis brevis and opponens pollicis, are not fully developed
. This type requires no surgical treatment in most cases.
- Type II is characterized by a tight web space between the thumb and index finger which restricts movement, poor thenar muscles and an unstable middle joint of the thumb metacarpophalangeal joint. This unstable thumb is best treated with reconstruction of the mentioned structures.
- Type III thumbs are subclassified into two subtypes by Manske. Both involve a less developed first metacarpal and a nearly absent thenar musculature. Type III-A has a fairly stable carpometacarpal joint and type III-B does not. The function of the thumb is poor. Children with type III are the most difficult patients to treat because there is not one specific treatment for the hypoplastic thumb. The limit between pollicization and reconstruction varies. Some surgeons have said that type IIIA is amenable to reconstruction and not type IIIB. Others say type IIIA is not suitable for reconstruction too. Based on the diagnosis the doctor has to decide what is needed to be done to obtain a more functional thumb, i.e. reconstruction or pollicization. In this group careful attention should be paid to anomalous tendons coming from the forearm (extrinsic muscles, like an aberrant long thumb flexor – flexor pollicis longus).
- Type IV is called a pouce flottant, floating thumb. This thumb has a neurovascular bundle which connects it to the skin of the hand. There’s no evidence of thenar muscles and rarely functioning tendons. It has a few rudimentary bones. Children with type IV are difficult to reconstruct. This type is nearly always treated with an index finger pollicization to improve hand function.
- Type V is no thumb at all and requires pollicization.
Arthritis of the hand is common in females. Osteoarthritis of the hand joints is much less common than rheumatoid arthritis. As the arthritis progresses, the finger gets deformed and lose its functions. Moreover, many patients with rheumatoid arthritis have this dysfunction present in both hands and become disabled due to chronic pain. Osteoarthritis is most common at the base of thumb and is usually treated with pain pills, splinting or steroid injections.
Carpal tunnel syndrome is a common disorder of the hand. This disorder results from compression of an important nerve in the wrist. Disorders like diabetes mellitus, thyroid or rheumatoid arthritis can narrow the tunnel and cause impingement of the nerve. Carpal tunnel syndrome also occurs in people who overuse their hand or perform repetitive actions like using a computer key board, a cashiers machine or a musical instrument. When the nerve is compressed, it can result in disabling symptoms like numbness, tingling, or pain in the middle three fingers. As the condition progresses, it can lead to muscle weakness and inability to hold objects. The pain frequently occurs at night and can even radiate to the shoulder. Even though the diagnosis is straightforward, the treatment is not satisfactory.
Dupuytren's contracture is another disorder of the fingers that is due to thickening of the underlying skin tissues of the palm. The disorder results in a deformed finger which appears thin and has small bumps on the surface. Dupuytren's contracture does run in families, but is also associated with diabetes, smoking, seizure recurrence and other vascular disorders. Dupuytren's does not need any treatment as the condition can resolve on its own. However, if finger function is compromised, then surgery may be required.
Ganglion cysts are soft globular structures that occur on the back of the hand usually near the junction of the wrist joint. These small swellings are usually painless when small but can affect hand motion when they become large. The cysts contain a jelly like substance and usually do disappear on their own. If the ganglion cyst is not bothersome, it should be left alone. Just removing the fluid from the cyst is not curative because fluid will come back in less than a week. Surgery is often done for large cysts but the results are poor. Recurrences are common, and there is always the possibility of nerve or joint damage.
Tendinitis is disorder when tendons of the hands become inflamed. Tendons are thick fibrous cords that attach small muscles of the hand to bones. A Tendon is useful for generation of power to bend or extend the finger. When repetitive action is performed, tendons often get inflamed and present with pain and difficulty for moving the finger. In most cases, tendinitis can be treated with rest, ice and wearing splints. In some cases, an injection of corticosteroid may help. Tendinitis is primarily a disorder from overuse but if not treated properly, can become chronic.
Trigger finger is a common disorder which occurs when the sheath through which tendons pass, become swollen or irritated. Initially, the finger may catch during movement but symptoms like pain, swelling and a snap may occur with time. The finger often gets locked in one position and it may be difficult to straighten or bend the finger. Trigger finger has been found to be associated with diabetes, gout and rheumatoid arthritis.
Thumb hypoplasia is a spectrum of congenital abnormalities of the thumb varying from small defects to absolute retardation of the thumb. It can be isolated, when only the thumb is affected, and in 60% of the cases it is associated with radial dysplasia (or radial club, radius dysplasia, longitudinal radial deficiency). Radial dysplasia is the condition in which the forearm bone and the soft tissues on the thumb side are underdeveloped or absent.
In an embryo the upper extremities develop from week four of the gestation. During the fifth to eighth week the thumb will further develop. In this period something goes wrong with the growth of the thumb but the exact cause of thumb hypoplasia is unknown.
One out of every 100,000 live births shows thumb hypoplasia. In more than 50% of the cases both hands are affected, otherwise mainly the right hand is affected.
About 86% of the children with hypoplastic thumb have associated abnormalities. Embryological hand development occurs simultaneously with growth and development of the cardiovascular, neurologic and hematopoietic systems. Thumb hypoplasia has been described in 30 syndromes wherein those abnormalities have been seen. A syndrome is a combination of three or more abnormalities. Examples of syndromes with an hypoplastic thumb are Holt-Oram syndrome, VACTERL association and thrombocytopenia absent radius (TAR syndrome).
Finger injuries are usually diagnosed with x-ray and can get to be considerably painful. The majority of finger injuries can be dealt with conservative care and splints. However, if the bone presents an abnormal angularity or if it is displaced, one may need surgery and pins to hold the bones in place.
Ectodermal dysplasia is characterized by absent sweat glands resulting in dry (hypohydrotic), often scale-like skin, sparse and usually coarse scalp hair that is often blonde, sparse eyebrows and eyelashes, and small brittle nails. In addition, abnormalities of ectodermal derivatives, neuroectodermal derivatives, and mesectodermal derivatives are often found. The ectodermal derivative abnormalities can affect the epidermis including mammary, pituitary and sweat glands, as well as hairs, dental enamel, nails, lens, and the internal ear. Neuroectodermal derivatives that can be affected include sensory placodes, cutaneous pigmental cells, and hair buds. Mesectodermal derivatives affected can include the dermis, hypodermis, dentin, head muscles and conjunctival cells, cervicofacial vascular endothelial cells, and part of the maxillofacial skeleton.
The hypohydrotic symptoms of ectodermal dysplasia described above are evidenced not only in the skin of affected individuals, but also in their phonation and voice production. Because the vocal folds may not be as hydrated as is necessary during the adduction phase of vocal fold vibration (due to lack of lubrication), a complete seal may not be accomplished between the folds and mucosal wave movement may be disrupted. This results in air escapement between the folds and the production of breathy voice, which often accompanies the skin abnormalities of ectodermal dysplasia.
Because lesions to different areas of the median nerve produce similar symptoms, clinicians perform a complete motor and sensory diagnosis along the nerve course. Decreased values of nerve conduction studies are used as indicators of nerve compression and may aid in determining the localization of compression.
Palpation above the elbow joint may reveal a bony consistency. Radiography images may show an abnormal bony spur outgrowth (supracondyloid process) just proximal to the elbow joint. Attached fibrous tissue (Struthers' ligament) may compress the median nerve as it passes underneath the process. This is also known as supracondylar process syndrome. Compression at this point may also occur without the bony spur; in this case, aponeurotic tissue found at the location of where Struthers' ligament should be is responsible for the compression.
If patients mention reproduction of symptoms to the forearm during elbow flexion of 120–130 degrees with the forearm in maximal supination, then the lesion may be localized to the area underneath the lacertus fibrosus (also known as bicipital aponeurosis). This is sometimes misdiagnosed as elbow strain and medial or lateral epicondylitis.
A lesion to the upper arm area, just proximal to where motor branches of forearm flexors originate, is diagnosed if the patient is unable to make a fist. More specifically, the patient's index and middle finger cannot flex at the MCP joint, while the thumb usually is unable to oppose. This is known as hand of benediction or Pope’s blessing hand. Another test is the bottle sign—the patient is unable to close all their fingers around a cylindrical object.
Carpal tunnel syndrome (CTS) is caused by compression of the median nerve as it passes under the carpal tunnel. Nerve conduction velocity tests through the hand are used to diagnosis CTS. Physical diagnostic tests include the Phalen maneuver or Phalen test and Tinel's sign. To relieve symptoms, patients may describe a motion similar to "shaking a thermometer", another indication of CTS.
Pronator teres syndrome (also known as pronator syndrome) is compression of the median nerve between the two heads of the pronator teres muscle. The Pronator teres test is an indication of the syndrome—the patient reports pain when attempting to pronate the forearm against resistance while extending the elbow simultaneously. The physician may notice an enlarged pronator teres muscle. Tinel's sign the area around the pronator teres heads should be positive. The key to discerning this syndrome from carpal tunnel syndrome is the absence of pain while sleeping. More recent literature collectively diagnose median nerve palsy occurring from the elbow to the forearm as pronator teres syndrome.
In uncooperative patients, the skin wrinkle test offers a pain-free way to identify denervation of the fingers. After submersion in water for 5 minutes, normal fingers will become wrinkled, whereas denervated fingers will not.
In "Ape hand deformity", the thenar muscles become paralyzed due to impingement and are subsequently flattened. This hand deformity is not by itself an individual diagnosis; it is seen only after the thenar muscles have atrophied. While the adductor pollicis remains intact, the flattening of the muscles causes the thumb to become adducted and laterally rotated. The opponens pollicis causes the thumb to flex and rotate medially, leaving the thumb unable to oppose. Carpal tunnel syndrome can result in thenar muscle paralysis which can then lead to ape hand deformity if left untreated. Ape hand deformity can also be seen in the hand of benediction deformity.
The Anterior Interosseus Nerve (AIN), a branch of the median nerve, only accounts for the movement of the fingers in hand and does not have any sensory capabilities. Therefore, the AIN syndrome is purely neuropathic. AINS is considered as an extremely rare condition because it accounts for less than 1% of neuropathies in the upper limb. Patients suffering from this syndrome have impaired distal interphalangeal joint, because of which they are unable to pinch anything or make and "OK" sign with their index finger and thumb. The syndrome can either happen from pinched nerve, or even dislocation of the elbow.
Classification of radial dysplasia is practised through different models. Some only include the different deformities or absences of the radius, where others also include anomalies of the thumb and carpal bones. The Bayne and Klug classification discriminates four different types of radial dysplasia. A fifth type was added by Goldfarb et al. describing a radial dysplasia with participation of the humerus. In this classification only anomalies of the radius and the humerus are taken in consideration. James and colleagues expanded this classification by including deficiencies of the carpal bones with a normal distal radius length as type 0 and isolated thumb anomalies as type N.
Type N: Isolated thumb anomaly
Type 0: Deficiency of the carpal bones
Type I: Short distal radius
Type II: Hypoplastic radius in miniature
Type III: Absent distal radius
Type IV: Complete absent radius
Type V: Complete absent radius and manifestations in the proximal humerus
The term absent radius can refer to the last 3 types.