Wednesday, June 1, 2011

Sindactilie

Sindactilia is the most common congenital malformation of limbs with an incidence of 1 in 2,000 births. Sindactilia can be classified as simple when only involve soft tissue and bone involve complex or when the adjacent finger nail. Sindactilia a common feature of more than 28 syndromes including Poland syndrome, and Holt-Oram Aperta. Sindactilia is a lack of differentiation in the fingers is not separated into individual appendix. This separation usually occurs during the 6-8 week of gestation. The term derives from the Greek word syn which means together and representing dactyly fingers.
Sindactilia is a cosmetic condition and does not affect walking, running or manipulate objects. Appears isolated or associated with several congenital syndromes. It is considered the most common anomaly of the hand and occurs in approximately 1 in 2,500 births.It is more common in boys and in Caucasians compared to Asians or Africans. From the cameraman and the annular space between the middle finger is most affected, and two interdigital foot space is the most involved.
Sindactilia can be treated surgically by separating fingers together.Generally the skin is split equally between the two fingers through zigzag incisions and skin grafts will be used to overcome the lack of skin. Grafts are taken from the lower abdomen. Separation procedure is performed between 6 months and 2 years old.Separate only one side of the finger to determine the ischemic complications. If more fingers are involved will require more surgery.After surgery tomorrow is put in a cast up by side to protect and immobilize the graft member. After removing gypsum interdigital wear for six weeks apart. child will follow a program of therapeutic gymnastics. To improve the hand function.
Pathogenesis and causes sindactilieCase sindactiliei is no separation of the skin or bone tissue between the fingers during development. At the molecular level is the suspected involvement of a protein called sentrina that is produced when cells die. During embryonic development sentrina absence not cause apoptosis of epithelial cells controlled fingers.The condition follows an autosomal dominant pattern of inheritance. Children of a parent with sindactilie shows 50% risk to inherit the anomaly.
Signs and symptoms of sindactilieSindactilia is a congenital anomaly in which two or more fingers of the hand or feet are fused. The phenomenon occurs in different degrees of severity which may include sindactilia incomplete, complete and complex. In some cases the disease process includes the bones and nails.Sindactiliei classification is based on severity of clinical presentation:- Mildest form is simple sindactilia referring to fingers united only Princess Soft- Sindactilia simple incomplete union occurs when soft tissue is only partial and does not extend to the tops fingers- When the union tops soft tissue extends to condition your fingers just to name Full sindactilie- Sindactilia complex refers to the fingers united by bone or cartilage- The most severe form is classified as complicated sindactilia referring to the fingers united by the merger and include bone abnormalities such as phalanges extranumerare, missing or duplicated and abnormally shaped bones, such as delta falagele; anomalies musculotendinoase and neurovascular structures may be present.
In simple sindactilia interdigital space between thumb and ring finger three is the area most affected areas followed by four, two and rarely initially. Bilateral impairment is found in 50% of patients.Sindactilia may be an isolated incident or in combination with other anomalies such as polydactyly, cleft his hand, building annular or craniofacial syndromes.Syndromes associated with sindactilia:Sindactilia symptoms may be associated with other craniofacial syndromes especially syndrome Sintra which Alpert is best known.Poland syndrome is another anomaly in the pectoral muscle is found in association with simbrahidactilia and / or other abnormalities of the ipsilateral upper extremity. Syndrome may be associated with amniotic bands, but etiology sindactiliei sindactilia is different.
Aperta syndrome:Aperta or acrocefalosindactilia syndrome is a rare anomaly. It is defined by facial characteristics and associated anomalies of the upper and lower limbs. Anomalies of the hand are mirror images and are characterized by the following four characteristics:Radial deviation of thumb, a short proximal phalanx as a result of faultIndex-sindactilia complex, large and ring fingersCentral segments of the finger-simbrahiofalangismul-Sindactilia simple interdigital space between the ring and little finger.
Poland syndrome:Poland syndrome is a sporadic congenital anomaly characterized by the absence of the tip of sternum large pectoral muscle hypoplasia of the breast or nipple, lack of subcutaneous fat and axillary hair. These are associated with abnormalities of the thoracic cage and ipsilateral upper extremity. The abnormalities consist simfalangism fingers, sindactilie with hypoplasia, or aplasia brahidactilie fingers.
Amniotic bands syndrome:Sindactilia found in this syndrome is the result of lack of differentiation during embryogenesis. In this condition your fingers are already formed, and injuries due to constriction bands together forcing them to heal sindactilie postlezionala. Extending damage can be mild with only a rudimentary skin bridge to connect the two fingers. Expansion may be even more severe damage with a complete soft tissue fusion in combination with amputated fingers.Occasionally epithelizant sinuses or fistula can be found proximal to the sindactiliei. Constriction distal fingers are amputated in utero with atrophic or ischemic. These elements can be isolated or in combination with other building of the arm, leg or face.Evolution sindactiliei:Sindactilia not released can significantly affect the function of the hand and fingers. Damage is worse if sindactilia is complex, involving complete or different size fingers as the little finger and ring finger or thumb and index.
Treatment sindactilieSindactiliei complex treatment is surgical release of the fingers to increase the functionality of the hand. Time of operation is critical because many children require surgery for other abnormalities of the skull, face syndrome associated with middle and orbits Aperta.
If there is an incomplete sindactilii minor surgical indication if the only problem is cosmetic appearance. However, a movement that affects the fingers involved sindactilie require surgical release to increase the functionality of your fingers.In people with complex sindactilie that are functional but hypoplasia fingers united individual fingers may result in separation of two individual fingers nonfunctionale because one set of tendons and neurovascular pedicle may be present.
Sindactilia time in children may indicate surgical procedures at the age of 5-6 months. Intervention before that age increases the risk of anesthesia. Before that age are not required intervention if there are no problems. If you associate a complex sindactilia paronychia occurring, children should wash hands thoroughly with soap and water and will apply a topical antibacterial solution. Oral antibiotics are needed.Sindactiliei operated complications include:Sindactiliei-appellant as a result of synechiae between the incision edges and thin skin graft incorporationFinger-ischemia can occur if an abnormality is present or if the development of the vasculature is destroyed-Contracture develops along the incision, the incision can cause scarring and stifle articulation of finger.

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