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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.
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 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”.
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.
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.
Onychogryphosis (also known as onychogryposis or ram's horn nails) is a hypertrophy that may produce nails resembling claws or a ram's horn.
Your baby's skull has seven bones. Normally, these bones don't fuse until around age 2, giving your baby's brain time to grow. Joints called cranial sutures, made of strong, fibrous tissue, hold these bones together. In the front of your baby's skull, the sutures intersect in the large soft spot (fontanel) on the top of your baby's head. Normally, the sutures remain flexible until the bones fuse. The signs of craniosynostosis may not be noticeable at birth, but they become apparent during the first few months of your baby's life. The symptoms differs from types of synostosis. First of all there is Sagittal synostosis (scaphocephaly). Premature fusion of the suture at the top of the head (sagittal suture) forces the head to grow long and narrow, rather than wide. Scaphocephaly is the most common type of craniosynostosis. The other one is called Coronal synostosis (anterior plagiocephaly). Premature fusion of a coronal suture — one of the structures that run from each ear to the sagittal suture on top of the head — may force your baby's forehead to flatten on the affected side. It may also raise the eye socket and cause a deviated nose and slanted skull. The Bicoronal synostosis (brachycephaly). When both of the coronal sutures fuse prematurely, your baby may have a flat, elevated forehead and brow.
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.
The accessory nail of the fifth toe is a physical trait of the small toe, where a minuscule "sixth toenail" is present in the outer corner of the nail situated on the smallest toe. It is commonly perceived to be especially prevalent amongst Han Chinese;.
Common signs of Say–Meyer syndrome are trigonocephaly as well as head and neck symptoms. The head and neck symptoms come in the form of craniosynostosis affecting the metopic suture (the dense connective tissue structure that divides the two halves of the skull in children which usually fuse together by the age of six). Symptoms of Say–Meyer syndrome other than developmental delay and short stature include
- Intellectual disability.
- Low-set ears/posteriorly rotated ears
- Intellectual deficit as well as learning disability
- Intrauterine growth retardation (poor growth of a baby while it is in the mother's womb)
- Posterior fontanel
- Premature synostosis of the lambdoid suture (the fusion of the bones to the joint is premature)
- Narrow forehead
- Trigonocephaly (a frontal bone anomaly that is characterized by a premature fusion of the bones which gives the forehead a triangular shape)
- Hypotelorism or hypertelorism (reduced or increased width between the eyes)
- Craniosynostosis (when one or more seam-like junctions between two bones fuses by turning into bone. This changes the growth pattern of the skull)
- Low birth weight and height
The affected patients sometimes show a highly arched palate, clinodactyly (a defect in which toes or fingers are positioned abnormally) and ventricular septal defect (a heart defect that allows blood to pass directly from left to the right ventricle which is caused by an opening in the septum). Overall, Say–Meyer syndrome impairs growth, motor function, and mental state.
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.
McGillivray syndrome is a very rare syndrome which is also known as a Craniosynostosis. It is characterized mainly by heart defects, skull and facial abnormalities and ambiguous genitalia. The symptoms of this syndrome are ventricular septal defect, patent ductus arteriosus, small jaw, undescended testes, and webbed fingers. Beside to these symptoms there are more symptoms which is related with bone structure and misshape.
McGillivray syndrome is a birth defect in which one or more of the joints between the bones of your baby's skull close prematurely, before your baby's brain is fully formed. When your baby has craniosynostosis, his or her brain cannot grow in its natural shape and the head is misshapen. It can affect one or more of the joints in your baby's skull. In some cases, craniosynostosis is associated with an underlying brain abnormality that prevents the brain from growing properly. Treating McGillivray usually involves surgery to separate the fused bones. If there is no underlying brain abnormality, the surgery allows baby’s brain to grow and develop in adequate space.
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.
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.
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)
The most common form of being pigeon toed, when the feet bend inward from the middle part of the foot to the toes.
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 trait can be observed on either one or both feet, where there is a separation of the toenail situated on the smallest toe. The separated part of the nail forms a smaller "sixth toenail" on the corner of the larger, or "main" section of the toenail, on the outermost side of the foot, which protrudes outwards from the corner of the larger nail. The additional "nail" can be cut with a nail clipper, just like any other nail.
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.
Purpura of the nail beds usually result from trauma, with causes of toe involvement including physical pressure on the toes, such as that seen in surfboarding or windsurfing in which one must maintain balance with the toes, or when exogenous pressure is exerted from poorly fitting shoes. Purpura beneath the nails may present similar to a melanoma, a confusion that may result if the patient does not communicate the acuteness of onset.
It is a disorder that is mostly characterized as developmental delay and short stature. Magnetic resonance imaging scans usually reveal that there is a decreased volume of white matter in the bilateral cerebral hemispheres, a brain stem that is smaller in size, and a thin corpus callosum (nerve fibers that connect the two hemispheres of the brain). The syndrome is one of the rare causes of short stature.
Symptoms of an ingrown nail include pain along the margins of the nail (caused by hypergranulation that occurs around the aforementioned margins), worsening of pain when wearing tight footwear, and sensitivity to pressure of any kind, even the weight of bedsheets. Bumping of an affected toe can produce sharp and even excruciating pain as the tissue is punctured further by the nail. By the very nature of the condition, ingrown nails become easily infected unless special care is taken early to treat the condition by keeping the area clean. Signs of infection include redness and swelling of the area around the nail, drainage of pus and watery discharge tinged with blood. The main symptom is swelling at the base of the nail on the ingrowing side (though it may be both sides).
Onychocryptosis should not be confused with a similar nail disorder, convex nail, nor with other painful conditions such as involuted nails, nor with the presence of small corns, callus or debris down the nail sulci (grooves on either side).
The most common and defining features of BGS are craniosynostosis and radial ray deficiency. The observations of these features allow for a diagnosis of BGS to be made, as these symptoms characterize the syndrome. Craniosynostosis involves the pre-mature fusion of bones in the skull. The coronal craniosynostosis that is commonly seen in patients with BGS results in the fusion of the skull along the coronal suture. Because of the changes in how the bones of the skull are connected together, people with BGS will have an abnormally shaped head, known as brachycephaly. Features commonly seen in those with coronal craniosynostosis are bulging eyes, shallow eye pockets, and a prominent forehead. Radial ray deficiency is another clinical characteristic of those with BGS, and results in the under-development (hypoplasia) or the absence (aplasia) of the bones in the arms and the hands. These bones include the radius, the carpal bones associated with the radius and the thumb. Oligodactyly can also result from radial ray deficiency, meaning that someone with BGS may have fewer than five fingers. Radial ray deficiency that is associated with syndromes (such as BGS) occurs bi-laterally, affecting both arms.
Some of the other clinical characteristics sometimes associated with this disorder are growth retardation and poikiloderma. Although the presentation of BGS may differ between individuals, these characteristics are often observed. People with BGS may have stunted growth, short stature and misshapen kneecaps. Poikiloderma may also be present in people with this syndrome, meaning that their skin may have regions of hyperpigmentation and hypopigmentation, or regions where the skin is missing (atrophy).
Hapalonychia, also known as egg-shell nail, is a condition in which the top of a toe or finger nail becomes soft and thin, causing it to bend or break. This condition can manifest as a result of genetic discrepancy and may also occur due to malnutrition and debility. Nails are often a representation of the internal nutritional of health. Although overall well-being is not typically determined by nail health, fissures, breaks and calcium spots are minor indications of inner health. Hapalonychia may also be attributed to myxedema, rheumatoid arthritis, anorexia, bulimia, Hansen's disease, Raynaud phenomenon, oral retinoid therapy, or radiodermatitis.
There is no consensus on what degree of angulation justifies a diagnosis, an incline between 15° and 30° is typical. A similar-sounding term, camptodactyly, is a fixed flexion deformity of a digit.