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This condition is normally discovered at birth. If other symptoms are present, a specific syndrome may be indicated. Diagnosis of a specific syndrome is based on family history, medical history, and a physical exam. Webbed toes are also known as "twin toes," "duck toes," "turkey toes" and "tiger toes."
Severity can vary. Most cases involve the second and third toes but any number of toes can be involved. In some cases the toes are joined part way while in some the webbing can extend right up to the nails. In some cases the entire toes, including the nails and bones, can be fused.
Clinodactyly (from the Ancient Greek κλίνειν ' meaning "to bend" and δάκτυλος ' meaning "digit") is a medical term describing the curvature of a digit (a finger or toe) in the plane of the palm, most commonly the fifth finger (the "little finger") towards the adjacent fourth finger (the "ring finger").
It is a fairly common isolated anomaly which often goes unnoticed, but also occurs in combination with other abnormalities in certain genetic syndromes.
The constriction ring syndrome is a complex collection of asymmetric congenital anomalies, in which no two cases are exactly alike. This is why a classification is difficult to make.
The most widely used classification system was proposed by Patterson. This classification system is based on the severity of the syndrome and is useful because, the different types require different treatments. Other clinicians have amended this scheme by separating the depth of the ring into mild, moderate, severe and amputation and by further defining the presence or absence of lymphedema or soft tissue loss distal to the ring. Expanding over subdivision in depth of the clefts for every classification is not necessary because the principles of treatment and technique for correction are the same
There are four categories:
1. simply constriction rings
2. constriction rings associated with deformity of the distal part with or without lymphedema
3. constriction rings associated with acrosyndactyly
4. uterine amputation
Patterson divided the constriction ring associated with acrosyndactyly into three types:
- Type I: conjoined fingertips with well-formed webs of the proper depth
- Type II: the tips of the digits are joined, but web formation is not complete
- Type III: joined tips, sinus tracts between digits and absent webs
Patterson I;
There are simple constriction rings which are strands most commonly around the distal extremities as fingers and toes. In general, the thumb is not likely to be affected by a constriction ring because the fetus typically holds the thumb in tight adduction flexion, making entanglement with strands less likely. These malformations need to be surgically removed which must be executed in different stages and can done by different techniques (see also treatment).
Patterson II;
The CRS involves strands which obstruct the lymphatic vessels and thus causing fluid retention, distal of the affected extremity. This utters itself with swollen parts distal of the constriction.
Patterson III;
In this form there is a complex form of syndactyly named acrosyndactyly, the fingers (or toes) were initially separated but due to the constriction they are formed back together. Sometimes multiple fingers can be involved. The distal fusion between digits or toes never initially involves a skeletal coalition. The digits are usually hypoplastic if multiple digits are involved. When the constriction cuts of the blood supply to the fingers, the fingers can form a peak with the most palmar digit being the index finger. Normal neurovascular bundles are not present in the distal parts. Hands with fused fingers need to be released in phases to preserve the distal blood supply.
Paterson IV;
One of the most severe consequence of constriction strains is probably intrauterine amputations, this is where the constriction goes as deep as the bone and cuts of the blood supply of the proximal extremity. The result will be that the developing toe or finger will become ischemic and will fall of. Because the end result is a transverse amputation that cuts off the vascular supply to the developing extremity, the actual constriction ring is not seen
This can result in different outcomes;
- complete resorption; this is the most common form of amputation. In this form the amputated digit is completely resorbed during intrauterine development and therefore the amputated digit can’t be found.
- recovery of the digit; the digit is recovered with the placenta during delivery.
- engraftment elsewhere; the amputated digit can be, in rare cases, engrafted somewhere on the fetus.
Intrauterine death;
In extremely rare cases a strain can form around the umbilical cord and cut off the blood supply to the fetus which will result in intrauterine death.
Malformation associated with constriction ring syndrome;
The percentage of associated anomalies varies from 40% to as high as 80%
Constriction ring deformities are as common on the lower extremity as on the upper, almost all of these involve the musculoskeletal system, with clubbed feet being the most common in up to 30% of reported cases
Large reported series reveal an incidence between 5% and 15% of craniofacial malformations with clefting of the lip or palate.
The groove begins on the lower and internal side of the base of the fifth toe, usually according to the plantar-digital fold. The groove becomes gradually deeper and more circular. The rate of spread is variable, and the disease may progress to a full circle in a few months, or still be incomplete after years. In about 75 per cent both feet are affected, though not usually to the same degree. There is no case reported where it begins in any other toe than the fifth, while there is occasionally a groove on the fourth or third toe. The distal part of the toe swells and appears like a small “potato”. The swelling is due to lymphatic edema distal to the constriction. After a time crusts can appear in the groove which can be infected with staphylococcus.
While the groove becomes deeper, compression of tendons, vessels and nerves occurs. Bone is absorbed by pressure, without any evidence of infection. After a certain time all structures distal the stricture are reduced to an avascular cord. The toe’s connection to the foot becomes increasingly slender, and if it is not amputated, it spontaneously drops off without any bleeding. Normally it takes about five years for an autoamputation to occur.
Cole describes four stages of ainhum:
Pain is present in about 78% of cases. Slight pain is present in the earliest stage of ainhum, caused by pressure on the underlying nerves. Fracture of the phalanx or chronic sepsis is accompanied with severe pain.
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.
The cause of in-toeing can be differentiated based on the location of the disalignment. The variants are:
- Curved foot (metatarsus adductus)
- Twisted shin (tibial torsion)
- Twisted thighbone (femoral anteversion)
Webbed toes is the common name for syndactyly affecting the feet. It is characterised by the fusion of two or more digits of the feet. This is normal in many birds, such as ducks; amphibians, such as frogs; and mammals, such as kangaroos. In humans it is considered unusual, occurring in approximately one in 2,000 to 2,500 live births.
There are various levels of webbing, from partial to complete. For example, the rare Hose's civet, a viverrid endemic to northern Borneo, has partially webbed feet. Most commonly the second and third toes are webbed or joined by skin and flexible tissue. This can reach either part way up or nearly all the way up the toe.
Diplopodia is a congenital anomaly in tetrapods that involves duplication of elements of the foot on the hind limb. It comes from the Greek roots diplo = "double" and pod = "foot". Diplopodia is often found in conjunction with other structural abnormalities and can be lethal. It is more extreme than polydactyly, the presence of extra digits.
Onychogryphosis (also known as onychogryposis or ram's horn nails) is a hypertrophy that may produce nails resembling claws or a ram's horn.
The differential diagnosis includes;
- Symbrachydactyly
- Chorionic villus sampling
- Congenital amputations
- Hypoplasias of hand, digit, thumb
- Adams-Oliver syndrome
- ADAM complex
ADAM Complex; CRS is sometimes mislabeled as ADAM complex. ADAM is an abbreviation for Amniotic Deformity, Adhesions Mutilations. CRS is the malformation due to a constriction ring around mostly a limb. ADAM-complex is the association of limb defects (caused by constriction rings) and certain craniofacial clefts
“Adams-Oliver syndrome is often mislabeled as CRS and consists of cutis aplasia of the scalp in which a longitudinal defect can vary in size and can often be associated with full-thickness skullcap loss. The distal digital or toe hypoplasia-aplasia is often confused with CRS. Constriction rings with or without edema are not present. The digital or toe hypoplasia-aplasia usually contains diminutive nails or nail folds”.
Malalignment of the nail plate is a congenital malalignment of the nail of the great toe, and is often misdiagnosed although it is a common condition.
Onychogryphosis may be caused by trauma or peripheral vascular disease, but most often secondary to self-neglect and failure to cut the nails for extended periods of time. This condition is most commonly seen in the elderly.
Ainhum (from Portuguese, pronounced "īn-yoom´", "i´num" or "ān´hum"; also known as dactylolysis spontanea) is a painful constriction of the base of the fifth toe frequently followed by bilateral spontaneous autoamputation a few years later.
The tibia or lower leg slightly or severely twists inward when walking or standing.
Hallux varus, or sandal gap, is a deformity of the great toe joint where the hallux is deviated medially (towards the midline of the body) away from the first metatarsal bone. The hallux usually moves in the transverse plane. Unlike hallux valgus, also known as hallux abducto valgus or bunion, hallux varus is uncommon in the West but it is common in cultures where the population remains unshod.
Radial dysplasia, also known as radial club hand or radial longitudinal deficiency, is a congenital difference occurring in a longitudinal direction resulting in radial deviation of the wrist and shortening of the forearm. It can occur in different ways, from a minor anomaly to complete absence of the radius, radial side of the carpal bones and thumb. Hypoplasia of the distal humerus may be present as well and can lead to stiffnes of the elbow. Radial deviation of the wrist is caused by lack of support to the carpus, radial deviation may be reinforced if forearm muscles are functioning poorly or have abnormal insertions. Although radial longitudinal deficiency is often bilateral, the extent of involvement is most often asymmetric.
The incidence is between 1:30,000 and 1:100,000 and it is more often a sporadic mutation rather than an inherited condition. In case of an inherited condition, several syndromes are known for an association with radial dysplasia, such as the cardiovascular Holt-Oram syndrome, the gastrointestinal VATER syndrome and the hematologic Fanconi anemia and TAR syndrome. Other possible causes are an injury to the apical ectodermal ridge during upper limb development, intrauterine compression, or maternal drug use (thalidomide).
The symptoms of bunions include irritated skin around the bunion, pain when walking, joint redness and pain, and possible shift of the big toe toward the other toes. Blisters may form more easily around the site of the bunion as well.
The presence of bunions can lead to difficulties finding properly fitting footwear and may force a person to buy a larger size shoe to accommodate the width the bunion creates. If the bunion deformity becomes severe enough, the foot can hurt in different places even without the constriction of shoes. It is then considered as being a mechanical function problem of the forefoot.
Navicular syndrome, often called navicular disease, is a syndrome of lameness problems in horses. It most commonly describes an inflammation or degeneration of the navicular bone and its surrounding tissues, usually on the front feet. It can lead to significant and even disabling lameness.
Tailor's bunion, or bunionette, is a condition caused as a result of inflammation of the fifth metatarsal bone at the base of the little toe.
It is mostly similar to a bunion (the same type of ailment affecting the big toe). It is called Tailor's Bunion because in past centuries, tailors sat cross-legged, and this was thought to cause this protrusion on the outside aspect of the foot.
It is usually characterized by inflammation, pain and redness of the little toe.
Often a tailor's bunion is caused by a faulty mechanical structure of the foot. The fifth metatarsal bone starts to protrude outward, while the little toe moves inward. This change in alignment creates an enlargement on the outside of the foot.
Tailor's bunion is easily diagnosed because the protrusion is visually apparent. X-rays may be ordered to help the surgeon find out the severity of the deformity.
Hammer toe most frequently results from wearing poorly fitting shoes that can force the toe into a bent position, such as excessively high heels or shoes that are too short or narrow for the foot. Having the toes bent for long periods of time can cause the muscles in them to shorten, resulting in the hammer toe deformity. This is often found in conjunction with bunions or other foot problems (e.g., a bunion can force the big toe to turn inward and push the other toes). It can also be caused by muscle, nerve, or joint damage resulting from conditions such as osteoarthritis, rheumatoid arthritis, stroke, Charcot–Marie–Tooth disease, complex regional pain syndrome or diabetes. Hammer toe can also be found in Friedreich's ataxia (GAA trinucleotide repeat).
Additional findings that may be present in HFGS according to the latest research are:
- Limited metacarpophalangeal flexion of the thumb or limited ability to oppose the thumb and fifth finger
- Hypoplastic thenar eminences
- Medial deviation of the great toe (hallux varus), a useful diagnostic sign when present
- Small great toenail
- Fifth-finger clinodactyly, secondary to a shortened middle phalanx
- Short feet
- Altered dermatoglyphics of the hands; when present, primarily involving distal placement of the axial triradius, lack of thenar or hypothenar patterning, low arches on the thumbs, thin ulnar loops (deficiency of radial loops and whorls), and a greatly reduced ridge count on the fingers
Radiographic findings
- Hypoplasia of the distal phalanx and first metacarpal of the thumbs and great toes
- Pointed distal phalanges of the thumb
- Lack of normal tufting of the distal phalanges of the great toes
- Fusions of the cuneiform to other tarsal bones or trapezium-scaphoid fusion of the carpals
- Short calcaneus
- Occasional bony fusions of the middle and distal phalanges of the second, third, fourth, or fifth toes
- Delayed carpal or tarsal maturation
- Metacarpophalangeal profile reflecting shortening of the first metacarpal, the first and second phalanges, and the second phalanx of the second and fifth digits
Urogenital Defects
Females may have the following:
- Vesicoureteral reflux secondary to ureteric incompetence
- Ectopic ureteral orifices
- Trigonal hypoplasia
- Hypospadiac urethra
- Subsymphyseal epispadias
- Patulous urethra
- Urinary incontinence (related to structural anomalies and weakness of the bladder sphincter muscle)
- Small hymenal opening
- Various degrees of incomplete Müllerian fusion with or without two cervices or a longitudinal vaginal septum
Males may have the following:
- Retrograde ejaculation (related to structural anomalies and weakness of the bladder sphincter muscle)
A foot deformity is a disorder of the foot that can be congenital or acquired.
Such deformities can include hammer toe, club foot, flat feet, pes cavus, etc.
Sesamoiditis occurs on the bottom of the foot, just behind the big toe. There are normally two sesamoid bones on each foot; sometimes sesamoids can be , which means they each comprise two separate pieces. The sesamoids are roughly the size of jelly beans. The sesamoid bones act as a fulcrum for the flexor tendons, the tendons which bend the big toe downward.
Symptoms include inflammation and pain.
Sometimes a sesamoid bone is fractured. This can be difficult to pick up on X-ray, so a bone scan or MRI is a better alternative.
Among those who are susceptible to the malady are dancers, catchers and pitchers in baseball, soccer players, and football players.
Bunion can be diagnosed and analyzed by plain projectional radiography. The "hallux valgus angle" (HVA) is the angle between the longitudinal axes of the proximal phalanx and the first metatarsal bone of the big toe. It is considered abnormal if greater than 15–18°. The following HVA angles can also be used to grade the severity of hallux valgus:
- Mild: 15–20°
- Moderate: 21–39°
- Severe: ≥ 40°
The "intermetatarsal angle" (IMA) is the angle between the longitudinal axes of the first and second metatarsal bones, and is normally less than 9°. The IMA angle can also grade the severity of hallux valgus as:
- Mild: 9–11°
- Moderate: 12–17°
- Severe: ≥ 18°
Metatarsalgia, literally metatarsal pain and colloquially known as a stone bruise, is any painful foot condition affecting the metatarsal region of the foot. This is a common problem that can affect the joints and bones of the metatarsals.
Metatarsalgia is most often localized to the first metatarsal head – the ball of the foot just behind the big toe. There are two small sesamoid bones under the first metatarsal head. The next most frequent site of metatarsal head pain is under the second metatarsal. This can be due to either too short a first metatarsal bone or to "hypermobility of the first ray" – metatarsal bone and medial cuneiform bone behind it – both of which result in excess pressure being transmitted into the second metatarsal head.