Treatment of Birth Defects by Plastic Surgery

The birth of a child is much awaited and celebrated event in the lift of every couple and family. The joy and celebrations of this unique creation of nature are eclipsed by a mixed feeling of Guilt, Depression and Confusion if the new born is found to have a birth defect.

Birth defects can involve any and every part and system of the body. All such defects that are visible on the surface of body are treated by the Plastic Surgeons. These Abnormalities can involve, The Skin The Face, The Hand, & The Genital Organs. It is not possible to give detail account of all the abnormalities; therefore, the common abnormalities are their management shall be discussed.

Skin provides the external appearance of the body and face. It is like a make-up that highlights the contours and shapes. Its quality is the most valued thing in life by most of us. Defects and abnormalities of the skin are one of the most distressing abnormalities. There can be defective development of the various components of the skin. The commonest problem is Too much Pigment (Melanin) or its absence in localized area. The other common abnormality is the excessive amount of Blood Vessels of skin and deeper tissues, called Hemangiomas.

a) Common Mole (Pigmented Nevus) : is too much melanin at a given spot. In every body there are average of 12-13, such moles. These moles are present since birth but all area not seen. They become visible by the age of 13 to 14 years. A mole at an unwanted place is best removed by Electric Desiccation, or may be excised surgically. Removal is necessary only if it suddenly starts growing, breaks-up and forms an ulcer or there is itching or bleeding.

b) Giant Nevus : This is a patch of dark colored skin over a large area of skin, it can have light brown, dark brown or black color or can even have thick hairs (Giant Hairy Nevus). Removal of these Nevi is mainly for improving appearance.

Removal by plastic surgery is possible. Best result are achieved by a technique called Tissue Expansion. Use of full thickness skin graft gives reasonably good results.

The abnormalities of the skin vessels are many and are commonly seen. These are called Hemangiomas, their appearance and behavious depend upon the size of blood vessels that are abnormal ranging from capillaries to larger vessels like arteries and veins. Lesions of Capillaties are called, Capillary hemangiomas, those invoving veins are called Cavernous hemangiomas. Some times there is an arteriovenous communication resulting in Cirsoid anneurism

Cavernous Hemangiomas
These have large abnormal sinuses filled with blood or lymph and are connected with venous system. Cavernous Hemangiomas, Lymphangiomas and mixed cavernous and capillary hemangiomas. These are treated by combination of Excision and Sclerosing agent Injections

Cavernous Hemangiomas These are large raised swellings present over any part of body, commonly seen over the face neck and hands or upper limp. They may have bluish color if the lesion is close to the skin. These are compressible swellings. Always require excision mainly due to cosmetic reasons. If the cavernous hemangioma is very large it may lead to increase growth of the organ and some times can produce gross bony deformities.

These can be treated by a combination of sclerosing agents and surgical excision. The removal of whole of the lesion may require several stages.

Lymphangiomas and mixed lesions These have similar presentation Lymphangiomas are not reducible and will not have bluish color. Treatment is for cosmetic reasons, and involves careful dissection and surgical removal.

Capillary Hemangiomas
These are abnormalities of the finest blood vessels called capillaries; the examples are Port-wine Stain, Salmon Patch, and Strawberry Hemangioma.

Port-wine Stain This is bluish red discoloration of skin, Needs removal like the pigmented lesions of skin. Use of laser has some role in very large lesions but some scarring can occur. Removal by plastic surgery is possible. Best result are achieved by a technique called Tissue Expansion. Use of full thickness skin graft gives reasonably good results.

Salmon Patch is more Blue than Red Generally present over the hair bearing scalp. If present over the face needs excision similar to pigmented lesions of skin.

Strawberry Hemangioma one of the most common type of hemangioma, appear red like a strawberry and is raised over the skin. Not always present sine birth, appear at few months after birth and gradually grow in size for two to three months. These remain of constant size for two to three years and then reduce and disappear by the age of 6 to 7 years.

Treatment is essential if :

• A very large lesion is obstructing vision.
• If they do not stop growing or grow rapidly over normal areas of skin.
• If there is recurrent bleeding or formation of ulcer.
• If the lesions are present even after 7 years of age.

Bleeding from hemangiomas will stop by firm pressure for five minutes
Treatment is done by injecting Sclerosing agents. Some times systemic steroids have to be given. If the lesion is large it has to be removed. Use of full thickness skin is essential to have good cosmetic result. Local flaps or Tissue expansion gives best results.

Development of face is complex and it occurs in the first eight weeks of Pregnancy, Due to its complexity most common defects are seen over the face. The commonest defect is Harelip (Cleft Lip) a condition in which the lip is cut since birth. The other common defect of face is abnormal shape of ear. Abnormal shapes or similar cuts (Clefts) can occur any where on the face upper lip lower lip nose over the head or eyelids.

Most of these defects are correctable by Plastic Surgery and the child can lead a normal life. Consultation with Plastic Surgeon is essential immediately after birth. Each type of defect is treated at different time of life depending on its severity.

Cleft Lip
Most common Birth Defects of face are Cleft Lip and Palate also called Harelip. It is a defect in the upper lip either on the one side or both sides. The defect may run in to the gums and some times even in to the roof of mouth called palate. All these children need special method of Feeding as the child is unable to produce suction An Early consultation with plastic Surgeon is essential for proper feeding. The surgery to correct this problem is done around 2-3 months of age, the child may require more than one surgery, and constant supervision so that he can lead a normal life.

Deformity of Ear
The commonest abnormality of ear is Bat ear or prominent ear. The other common defect is Microtia (Small cup shaped ear or presence of a small crumpled piece of cartilage. All the abnormalities of Ear are corrected when the child is about six years old.

The shape of ear can be greatly improved. In cases of microtia the ear has to be created either from the child’s ribs or by the use of synthetic implants that are placed under the skin pocket created at the place of ear. The surgery to make ear can take two or three stages.

Development of hand takes place during the 4 th to the 8 th week of pregnancy. The abnormalities of hand are occur very often. These are mostly genetic and have a tendency to run in families. Presence of extra digit (Polydactyly) is genetic and it so common that in one of Arabian tribes a child that has only ten fingers is considered evil. Removal of extra digit should be done early in life and careful restoration of bony contour and joints are important.

Syndactyly (Joined/webbed fingers). Commonly occurs between the middle & ring finger separation of fingers by plastic surgery is required to prevent abnormal development of fingers. Some times several fingers or all the fingers are joined together. Such conditions require to be treated very early in life. Abnormality of the Thumb absence of thumb or duplication Uncommon but is correctable by PlasticSurgery.

Timing and Technique :
All the defects of hand should have consultation with plastic Surgeon early in life preferably in the first year of life. The treatment is so planned that the child has a normal hand by the time he / she is 5 years of age. Development of hand completes at the age of five years

All Surgical procedures over hand will have better results and less chance of damage to other structures if performed by Microsurgery . All Hand surgery in Max is performed by Plastic Surgeons Trained in Microsurgery.

The development of external and internal genitalia in both male and female sexes is dependent on the hormones produced by the fetus it self and that of the mother. Male genitalia develops till the 8 th month of pregnancy and so is very prone to abnormalities. One out of every 600 male children born one will have an abnormal opening for urine. When present abnormally on the under side is called Hypospadias. Similarly if the opening is on the upper side it is termed Epispadias. Incomplete decent of testis in to the scrotum is another common abnormality. There are abnormalities associated with female genitalia, the commonest being the non development Vagina but this is only detected when the child grows up.

Abnormal curvature & low opening of urinary passage

Hypospadias,The abnormal opening for urine below the penis a small curved penis some times difficulty in passing urine. Consultation by Plastic surgeon early in life is very important. There should be free flow of urine to prevent damage to kidneys. Abnormality of kidney and other structures need to be investigated. Plastic Surgery of Penis is aimed to have a normal looking penis with normal passage for urine by the time the child is 5 years of age. One or two stage surgery are performed. Details can be discussed at the time of consultation. Vaginal Agenesis (Absence of Vagina)There can be absence of vagina alone with normal uterus or there may be total absence of uterus and vagina. It is important to understand that this abnormality is present since birth but are detected only when the child attains puberty. Total absence of menstruation leads to investigations. These girls externally appear normal and have normal development of breast and other female secondary sexual characteristics. An unltrasound is sufficient to give the final diagnosis.

The absence of vagina is best treated by the Mclndoe technique using a split skin graft to line the neovagina created by careful dissection. If the patient is very particular about not having a scar over the thigh, Horton’s method is used and full thickness skin is taken from the groin crease. It is important to understand that if there is absence of Uterus, it cannot be reconstructed. These women who have no uterus, after vaginal reconstruction can have normal sexual activity but cannot have children.

Non development of breast is also a birth defect. But it is detected only when the child reaches puberty. One or both breast may fail to develop, or may have abnormal shape or may be small in size. Initial Breast development stops after the age of 18 yrs. These operations are therefore performed after the age of 15 years to 18 years the purpose of surgery is to have both side breasts almost equal in size, of normal shape and of desired size.

Some times one breast may be bigger than the other. Minor difference in size is normal but if it is gross then either larger breast is reduced or the smaller breast is made bigger. Breast size is increased by use of Breast Implants. These implants are introduced through small cuts made in the breast crease. Reduction in size is done by use of special lipoplasty techniques by which fat is removed. There is almost no scar.

When there is complete non-development of breast there is inadequate skin so reconstruction of breast has to be done in two stages. A special device called Tissue expander is used to create extra skin. In the next stage the Breast is created by use of Beast Implants.