Reconstructive

Neurofibromatosis

2009-06-23T00:51:00.001+07:00

Neurofibromatosis is a neurogenic tumors, arising from the neural sheath cells, are located along peripheral and cranial nerves. In children, neurofibromatosis presentation may be either solitary or multiple. Multiple neurofibromatosis is most familiar as von Recklinghausen disease. This syndrome of multiple neurofibromas, abnormal skin pigmentation, bone abnormalities, and multiple associated congenital anomalies is inherited in an autosomal dominant pattern with variable penetrance.

The incidence neurofibromatosis is 0.03% of live births. Half of children born with von Recklinghausen disease will be diagnosed at birth by characteristic patterns of pigmentation (cafe au lait spots), skin tumors, and associated anomalies.

Intracranial, abdominal, and thoracic location of tumors may delay diagnosis.

The natural history of the syndrome is one of slow progression in the size and number of tumors.

Although neurofibromatosis is initially benign, there is a significant risk for malignant transformation of 8% to 16%.

Plastic surgical involvement may be requested for neurofibromatosis; for tumors involving cranial nerves presenting with facial palsies; and in patients with cranio-orbital neurofibromatosis, in which orbital bone erosion and absence of portions of the sphenoid bone present significant challenges. Children may present with solitary lesions that may be cutaneous or subcutaneous in origin.

The patient with elephantiasis neuromatosa will develop severe distortion of the face that may include proptosis. These hanging pedunculated lesions require complete excision for prevention of progressive facial deformity. Because of the extensive nature of neurofibromatosis, multiple debulking procedures are more commonly performed.

Plexiform lesions arise from major nerve trunks and therefore tend to be deeply located within tissues. In patients with plexiform neurofibroma involving the craniofacial region, computed tomographic scan is essential because these tumors may spread extensively and cause gross deformity of the skeleton.

Blepharoptosis

2009-06-22T00:27:00.001+07:00

Ptosis is the drooping or sagging of a body part. Ptosis of the upper eyelid (upper lid ptosis, blepharoptosis, drooping upper eyelid, droopy-lid syndrome) is defined as an abnormally low-lying upper eyelid margin in the primary gaze, resulting in narrowing of the palpebral opening and fissure and a covering of part of the eye. Blepharoptosis is a condition of inadequate upper eyelid position. The descended upper eyelid margin commonly results in partial or complete obstruction of the superior visual field. The causes are varied but may be categorized as either congenital or acquired.

Ptosis can either be present at birth (congenital ptosis) or develop later in life (acquired ptosis). Acquired blepharoptosis may further be differentiated based on anatomic abnormalities, namely neurogenic, mechanical, traumatic, and myogenic.

The normal adult upper lid margin is 0.5 to 2 mm below the superior corneal limbus and is highest just nasal to the pupil (upper lid height). A deficient function may be caused by weakness of the muscle responsible for lid retraction (either the levator palpebrae superioris muscle and its aponeurosis or Muller's muscle) or damage to the nerves that control these muscles. Laxity of the upper eyelid skin leads at best to mechanical ptosis. Ptosis may affect one or both eyes. Sometimes, ptosis is an isolated problem that changes the patient's appearance without otherwise affecting the health. In other patients, ptosis can be a warning that a more severe condition affecting the muscle, nerve, brain, or the eye socket is present. In particular, ptosis that develops within hours or days may indicate a serious medical problem.

Most commonly, elderly patients present with involutional or senile ptosis from a dehiscence of the levator aponeurosis insertion. The resting eyelid position insidiously migrates inferiorly with a concomitant superior positioning of the supratarsal crease in the presence of normal (8 mm) levator muscle function.

Congenital blepharoptosis most frequently results from an embryologic failure of development of the levator muscle. In congenital dysmyogenic ptosis, the levator muscle fibers are dystrophic and replaced by fibrous tissue. Surgical treatment is often indicated for congenital, traumatic, and involutional causes of ptosis.

Traditional approaches focus on frontalis sling techniques incorporating either autologous or alloplastic materials to link the eyelid to the frontalis muscle. Modifications of this technique and frontalis sling materials have recently been described.

Treacher Collins Syndrome

2009-06-14T23:11:00.001+07:00

Treacher Collins syndrome, also known as mandibulofacial dysostosis and Franceschetti-Zwahlen-Klein syndrome, has a distinctive facial appearance that is well known. This bilateral facial deformity has etiology in anomalies of the structures derived from the first branchial arch, groove, and pouch. The deformity is centered around the middle portion of the face as opposed to hemifacial microsomia, which is secondary to abnormalities of the first and second branchial arches and therefore usually centered around the lower face and mandible.

Treacher Collins syndrome is generally considered to be autosomal dominant in its inheritance with variable expressivity. The characteristic appearance revolves around the deformity in the lower lateral orbit and medial zygoma. There is frequently an absence of the bone in this region, which many think is consistent with a craniofacial cleft. However, the deformity extends outward from this area to the maxilla and mandible in such a way that it is much more severe than the malformation seen even in complex facial clefts. This suggests a more extensive underlying pathophysiologic process. Basic science animal investigation has produced similar malformations in animals with teratogenic doses of vitamin A and isotretinoin. The histologic findings demonstrated neurocrest development abnormalities.

The maxilla, from the nasomaxillary region, is caudally rotated. This results in clockwise rotation of the mandible, which is also retrognathic and micrognathic. These abnormalities in the facial skeletal framework displace the associated soft tissues, resulting in many other deformities. The palpebral fissures are short and slanted downward. The lateral canthus is inferiorly displaced, and there is usually an associated coloboma of the lower eyelid. The nose has a parrot beak deformity made more noticeable by its small size. The underlying nasal, oral, and pharyngeal airway is narrowed and compromised, resulting in airway obstruction and the need for a tracheostomy in patients with more severe disease. Other malformations include cleft palate in 35% of patients and macrostomia in 15%. Finally, there frequently are auricular abnormalities that are both internal and external in nature. Because the ear anomaly is bilateral, most patients require a bone conduction hearing aid. Cochlear hearing devices that are anchored to bone have been introduced. The patient usually undergoes costochondral external ear reconstructions for complete microtia.

Nager syndrome is similar to Treacher Collins syndrome but with hypoplastic or absent thumbs, radius and ulna fusion, or radial and metacarpal hypoplasia or aplasia. Other aspects in the differential diagnosis of Nager syndrome are a lower incidence of coloboma, a higher incidence of cleft palate, and more severe hypoplasia of the mandible.

Vascular Marlformations

2009-05-31T05:20:00.001+07:00

Vascular malformations have been recognized throughout history as birthmarks. Vascular malformations have been categorized using confusing, inaccurate, and inconsistent terminology. A significant number of patients with vascular lesions receive ineffective and potentially harmful treatment based on misclassification.

Mulliken classified vascular malformations using understandable and accurate nomenclature. Vascular malformations are subdivided into low-flow (capillary, venous, lymphatic, or a combination thereof) and high-flow (arterial) anomalies. Clinical, histological, histochemical, and biochemical differences and radiographic imaging findings support this classification.

What other Problems in Baby with Cleft Lip and Palate ?

2009-05-24T15:08:00.001+07:00

Children with clefts can have ear infections more often than other children. The cleft allows fluid and germs to enter child's ear more easily than normal. Many children require a special kind of surgery by an ear, nose and throat doctor. Tubes are put in the eardrum to drain fluid and stop some infections. All children with clefts need to have their ears checked regularly by their doctor.

Your child will also need to see a dentist often, because children with clefts tend to have more cavities and other dental problems than other children. Fluoride treatment, toothbrushing and careful dental care will be a big help.

Infants and children with clefts may become withdrawn from both family and friends. It is very important to spend as much time as possible with your baby, cuddling, talking, hugging and so on. If you are worried about any problems with growth and making friends, you should talk to your doctor. You may also want to talk to a psychologist or a psychiatrist.

Neonatal Care for CLP

2009-05-16T10:18:00.001+07:00

When a neonate with a cleft is born, a pediatrician has 3 major concerns:

Risk of aspiration because of communication between oral and nasal cavities
Airway obstruction (in addition to sequelae of aspiration, especially in Robin sequence in which the cleft palate [CP] is combined with micrognathia and the tongue has a normal size)
Difficulties with feeding of a child with a cleft and nasal regurgitation

These 3 factors are influenced by the presence of other major or minor anomalies that may, in association with a cleft, represent 1 of 300 known cleft syndromes.

Therefore, a neonate with an orofacial cleft should be seen by a medical geneticist as soon as possible. A pediatrician/neonatologist is usually the first person to take care of a neonate born with a cleft and the first to talk to the parents. As soon as possible, refer each baby born with orofacial cleft to the cleft palate or craniofacial center, where each specialist evaluates the baby, delineates the best management options and treatment plan, and continuously revises individual procedures and treatment during follow-up visits.

Cleft Lip types

2009-05-09T23:25:00.001+07:00

Because clefting causes specific visible symptoms, it's easy to diagnose. It can be detected through a prenatal ultrasound. If the clefting has not been detected prior to the baby's birth, it's identified immediately afterward. Some kids have clefts that extend through both the front and rear part of the palates, while others have only partial clefting.

The three common kinds of clefts are:

1. cleft lip without a cleft palate

2. cleft palate without a cleft lip

3. cleft lip and cleft palate together

In addition to classification as unilateral or bilateral, cleft lips are further classified as complete or incomplete. A complete cleft involves the entire lip, and typically the alveolar arch. An incomplete cleft involves only part of the lip.

The Iowa system (which also classifies cleft palate) classifies cleft lip in five groups:

* Group I—clefts of the lip only

* Group II—clefts of palate only

* Group III—clefts of lip, alveolus, and palate

* Group IV—clefts of lip and alveolus

* Group miscellaneous

Another widely accepted cleft lip classification is based on recommendations of the American Cleft Palate Association. This classification divides cleft lip into unilateral or bilateral (right, left or extent) in thirds—(i.e., one-third, two-thirds, three-thirds), or median cleft lip, the extent of which is also measured in thirds.

Tattoo removal

2009-05-07T02:33:00.002+07:00

No one knows when the practice of tattooing the skin began, but Egyptian mummies dating back to 1300 B.C. have shown evidence of blue tattoo marks. Tattooing is accomplished by injecting colored pigment into small deep holes made in the skin.

In the United States, about 16% of people with tattoos experience some regret. According to a poll done in 2008, the most common reasons for regret are "too young when I got the tattoo (20%)," it's "permanent" and I'm "marked for life (19%)," and I just "don't like it (18%)." An earlier poll showed that 19% of British people with tattoos suffered regret, as did 11% of Italian people with tattoos.

A new job, problems with clothes, and a significant life event (wedding, divorce, baby) were also commonly cited as motivations.

Verruca

2009-05-03T00:12:00.001+07:00

A wart (also known as verruca) is generally a small, rough tumor, typically on hands and feet but often other locations, that can resemble a cauliflower or a solid blister. Verrucas are slightly raised and circular in shape, with a thickened rim of surrounding skin.

There may be several verrucas or just one. Warts are common, and are caused by a viral infection, specifically by the human papillomavirus (HPV) and are contagious when in contact with the skin of an infected person.

It is also possible to get warts from using towels or other objects used by an infected person. HPV is associated with oral cancer, laryngeal cancers, tracheal and lung cancers.

In children, verrucas tend to come and go quite quickly, but in adults they can persist for several years if they are not treated.

Nevi

2009-04-27T12:55:00.001+07:00

Nevus (or naevus, plural nevi, from nævus, Latin for birthmark) is the medical term for sharply-circumscribed and chronic lesions of the skin. These lesions are commonly named birthmarks and moles.

By definition, nevi are benign. Using the term nevus and nevi loosely, most physicians and dermatologists are actually referring to a variant of nevus called the "melanocytic nevus", which are composed of melanocytes. Histologically, melanocytic nevi are differentiated from lentigines (also a type of benign pigmented macule) by the presence of nests of melanocytes, which lentigines (plural form of lentigo) lack.

What is Clavus ?

2009-04-25T12:58:00.001+07:00

Clavus is a thickening of the skin due to intermittent pressure and frictional forces.

These forces result in hyperkeratosis, clinically and histologically. This extensive thickening of the skin may result in chronic pain, particularly in the forefoot; in certain situations, this thickening may result in ulcer formation.

Syndactyly

2009-04-19T00:42:00.001+07:00

A condition in which two or more of the fingers or toes are joined together. This joining can involve the bones or just the skin between the digits.

Joining of the bones is called bony syndactyly. Webbing of the skin between the fingers without any joining of the bones is called cutaneous syndactyly.

In complete syndactyly, the fingers or toes are completely joined together, with the connection extending from the base to the tip of the involved digits. In partial syndactyly, the connection extends only part of the way up from the base of the involved digits.

Polydactyly

2009-04-14T23:58:00.002+07:00

Polydactyly, a congenital abnormality, is the presence of more than the normal number of fingers or toes. the condition is usually inherited as an autosomal dominant characteristic and can usually be corected by surgery.

If recommended, the surgery is usually done while the child is about 1 year of age. Also called polydactylia, polydactylism or hyperdactyly. Polydactyly can vary from an unnoticeable rudimentary finger or toe to fully developed extra digits.

Polydactyly and syndactyly (fusion of the fingers or toes) can occur simultaneously when extra digits are fused. This condition is known as polysyndactyly.

Facial Cleft

2009-04-13T00:59:00.004+07:00

Cranio-facial clefts are a wide spectrum of malformations affecting the face and cranium in a great variety of forms. The low frequency of most of them has made its study, statistics and classification complex and uncertain for many, many years.

Clefts in the cranio-facial region range from the most commonly known cleft lip and palate to extensive cranio-facial clefts that can be dramatically disfiguring. Facial clefts constitute the most challenging malformation as they are never the same.

The surgeon must be skilful in cranio-facial surgery, maxillofacial techniques, soft tissues procedures for soft tissue reconstruction, and no less important, have a solid background in management of cranio-facial anomalies.

Despite all this, restoring, functional and aesthetically, a clefted face is one of the most rewarding surgeries.

What is cleft palate?

2009-04-11T14:28:00.001+07:00

Cleft palate is a treatable birth defect in which the baby's roof of the mouth (palate) does not develop normally during pregnancy, leaving an opening (cleft) that may go through to the nasal cavity.

Cleft palate may involve any part of the palate, including the front part of the roof of the mouth (hard palate) or the small tag of tissue that hangs down from the soft palate (uvula). It may also occur by itself or along with other birth defects of the face and skull, particularly cleft lip.

Cleft palate and cleft lip are the most common congenital defects of the head and neck. Until treated surgically, cleft palate can interfere with feeding, speech development, and hearing.

Microtia (Small Ear)

2009-04-09T12:46:00.001+07:00

Microtia by definition means ‘small ear’. This term is applied to anyone who is born with an underdeveloped ear. Microtia occurs in approximately 1:6000 - 10000 births. Microtia occurs more often on the right side than the left and more often in males. The condition can affect one ear (unilateral) or both ears (bilateral). It is bilateral in approximately 10% of cases.

Children born with microtia have a varying degree of ear malformation. In most cases there is a vertical skin appendage with a small lobule (ear lobe) on the lower end. In others the hollow of the ear may partly be formed with the ear lobe. The most severe form of microtia is complete absence of the ear, known as anotia.

Microtia can occur as an isolated deformity or present as part of a spectrum of other defects. Any child born with microtia should be evaluated to rule out other conditions. Microtia is known to occur as part of a several of syndromes, especially hemifacial microsomia, Goldenhar syndrome and Treacher-Collins syndrome.

Cleft Lip

2009-04-07T23:36:00.003+07:00

Cleft lip, with or without cleft palate, is a congenital malformation. Generally, facial clefting results when medial, lateral, and maxillary nasal processes on either left, right or both sides of the forming craniofacial complex do not fuse completely.

Early embryonic changes (during the fourth and tenth weeks of gestation) may result in clefting.

Suspected causes include:

1) environmental insults (i.e. maternal diseases, chemotherapy, radiation, alcohol, excess retinoic acid and anticonvulsant medications); or

2) genetic factors.

Contracture

2009-03-30T12:12:00.004+07:00

A contracture scar is a permanent tightening of skin that may affect the underlying muscles and tendons that limit mobility and possible damage or degeneration of the nerves. Contractures develop when normal elastic connective tissues are replaced with inelastic fibrous tissue. This makes the tissues resistant to stretching and prevents normal movement of the affected area. Physical Therapy, pressure and exercise in many cases can aid in controlling contracture burn scars. If these treatments do not control the effects of contracture scars, surgery may be required. A skin graft or a flap procedure may be performed. Additionally your doctor may recommend a new technique such as Z-Plasty or tissue expansion.

What is a scar?

2009-03-29T19:48:00.006+07:00

The skin is the largest organ of the body, with an average surface area of 1.8 square metres in adults. Our skin helps to keep us cool in hot weather and warm in cold weather and it also prevents us from dehydrating. Our skin is constantly shedding old cells and creating new ones, enabling it to repair itself if damaged. It is the repair process that can result in a scar.

A scar is an essential part of this natural healing process following an injury to the thick layer of skin (the dermis) or the outer layer of skin (the epidermis).

Damage to the outer layer of skin is healed by rebuilding the tissue, and in these instances, scarring is slight. When we damage the thick layer of tissue beneath the skin, rebuilding is more complicated.

Our bodies lay down collagen fibres (a protein which is naturally produced by the body) and this usually results in a noticeable scar. After the wound has healed, the scar continues to alter as new collagen is formed and the blood vessels return to normal. This is the reason why most scars will fade and improve in appearance over the two years following an injury.

However, there will always be some visible evidence of the injury and hair follicles and sweat glands do not grow back.

What is Reconstruction Surgery?

2009-03-28T22:38:00.004+07:00

Definition

Reconstructive surgery is a type of plastic surgery performed to reshape abnormal structures of the body to improve function and appearance. Reconstructive surgery is different from cosmetic surgery, which is performed to reshape normal structures of the body to improve a patient's appearance and self-esteem.

Purpose

The goals of reconstructive surgery are to reshape abnormal structures of the body, to improve function, and/or to allow a person to have a more normal appearance. Abnormal structures of the body that are corrected during reconstructive surgery may be the result of birth defects, developmental abnormalities, trauma or injury, infection, tumors, or disease.

Description

The three most commonly performed reconstructive surgeries in the United States are tumor ablation (removal) and reconstruction, hand surgery, and breast reconstruction.

2009-03-10T03:19:00.007+07:00

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