NEUROFIBROMATOSIS

Neurofibromatosis (NF) is a generalized form of the benign tumor of the peripheral nerves derived from fibrous element of the nerve sheath. It is neuroectodermal in origin with varying involvement of the skin, subcutaneous tissue, and bone. It is an autosomal dominant inherited disorder with approximately 50% of patients having a positive family history of the disease. There are two types; neurofibromatosis type 1 (NF-1, Plexiform Neurofibroma), known as Von Recklinghausen disease. It is more common, occurring with a frequency of 1 in 3000 live birth, which accounts for 96% to 97% of all cases of NF. The type 2 (NF-2) accounts for approximately 3% of all cases.

 

The incidence of head and neck involvement with neurofibromatosis ranges from 1% to 22% in the published literature. NF-1 manifests in the craniofacial complex as varying involvement of the soft tissue and bone of the orbitocranial and jaw regions usually resulting in hemifacial deformities. Due to the extensive and destructive manifestation of craniofacial neurofibromatosis and the consequent functional and cosmetic disabilities imposed by the disease, sophisticated radio-diagnostic facilities are required for effective treatment planning. The surgical management of the disease can be highly technical because of the involvement of vital structures of the craniofacial complex.

 

HAEMANGIOMA AND VASCULAR MALFORMATION

Hemangiomas are benign (non-cancerous) tumors of the blood vessels. They may or may not appear at birth, but always become visible within one to four weeks after birth. Hemangiomas have rapid growth phase that begins between four and eight months of age and plateaus between six and 12 months of age. After the plateau period, they begin to involute (shrink). This stage can last anywhere from three to 10 years. They usually occur on the head or neck, but they can occur anywhere, including the internal organs. Hemangiomas typically appear as one of the following:

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  • Herald spot − a pale white or gray-blue flat spot
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  • Telangiectatic spot − a flat patch of blood vessels under the skin’s surface
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  • A raised, red patch of varying size and shape

Vascular malformations are benign (non-cancerous) lesions that are present at birth, but which may not be visible for weeks or months after birth. Unlike hemangiomas, vascular malformations do not have a growth cycle and then regress — they continue to grow slowly throughout life. There are several types of vascular malformations:

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  • Capillary (port wine stains) − always present at birth as pink or purple skin patches
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  • Venous − often confused with a hemangioma, these malformations are soft to the touch and the color disappears when compressed. They are most commonly found on the jaw, cheek, tongue and lips
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  • Lymphatic − formed when excess fluid accumulates within the lymphatic vessels
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  • Arteriovenous − abnormal connections between arteries and veins, resulting in a high flow, pulsating collections of blood vessels
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  • Mixed − a combination of any of the other four types
 

Many hemangiomas do not require intervention. They should, however, be examined frequently to indentify noticeable changes or problems. Early treatment can slow the development of a vascular malformation, and prevent long-term effects. Left untreated, many will darken and thicken as a child grows.We offer several treatment options depending on the location and severity of the haemangioma and vascular malformation:

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  • Oral steroid therapy
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  • Intralesional (injected) steroid therapy
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  • Laser therapy
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  • Surgical removal

 

 

CYST AND TUMORS OF JAW

Because the mouth and jaws are composed of many different types of tissues, such as bone, muscle, glands and mucosa, they are more susceptible to developing abnormal growths than other parts of the body. Lesions developing within the jaws can arise from thedental elements, bone, nerves, or blood vessels.Primary jaw cyst and tumors are generally considered either odontogenic (from the tooth developing tissues) or non-odontogenic. Examples of odontogenic tumors include ameloblastomas and odontomas and odontogenic cyst such as Radicular cyst, dentigerous cyst and odontogenickeratocysts. Non-odontogenic tumors may be considered either benign or malignant. Examples of benign tumors include fibro-osseous lesions, giant cell lesions and fibromyxomas. Malignant tumors include osteosarcoma and fibrosarcoma. Tumors may develop from the blood cell forming bone marrow including multiple myeloma.

 

Many primary jaw tumors are found incidentally on x-rays that may be taken for other reasons including sinusitis and dental exams. Jaw tumors may initially have no symptoms until much larger and involving other structures. Other early symptoms may include pain, swelling, numbness, jaw asymmetry, changes in occlusion and even tooth shifting. Rarely, un-erupted or impacted teeth are associated with development of lesions.

 

Many jaw tumors require surgery for management. The recommended treatment is dictated based on the biologic behavior of the tumor. Tumors that infiltrate into local tissues with a high rate of recurrence may require resection of a region of the jaw for eradication. This may require reconstruction to replace the lost tissue. Other tumors can be enucleated or "scooped out" since they have a low rate of recurrence following removal. Occasionally teeth will have to be removed in location to these tumors which can be replaced later with dental prosthetics and possibly dental implants. Depending on the pathology of the tumor, radiation and chemotherapy may be necessary. A thorough dental evaluation and management of dental problems prior to treatment is crucial to health after therapy.

Dentigerous Cyst

 

Craniofacial Fibrous Dysplasia

 

Radicular Cyst

 

 

Calcifying Epithelial Odontogenic Tumour of Maxilla

 

Ameloblastoma of Mandible and Reconstruction with Iliac Crest Bone

TEMPORO MANDIBULAR JOINT (TMJ) SURGERY

The temporomandibular joint (TMJ) is a small joint located in front of the ear where the skull and lower jaw meet. It permits the lower jaw (mandible) to move and function.

 

TMJ disorders are not uncommon and have a variety of symptoms. Patients may complain of earaches, headaches and limited ability to open their mouth. They may also complain of clicking or grating sounds in the joint and feel pain when opening and closing their mouth. Arthritis is one cause of TMJ symptoms. It can result from an injury or from grinding the teeth at night. Another common cause involves displacement or dislocation of the disk that is located between the jawbone and the socket. A displaced disk may produce clicking or popping sounds, limit jaw movement and cause pain when opening and closing the mouth.

 

The disk can also develop a hole or perforation, which can produce a grating sound with joint movement. There are also conditions such as trauma or rheumatoid arthritis that can cause the parts of the TMJ to fuse, preventing jaw movement altogether.

 

TMJ treatment may range from conservative dental and medical care to complex surgery. Depending on the diagnosis, treatment may include short-term non-steroidal anti-inflammatory drugs for pain and muscle relaxation, bite plate or splint therapy, and even stress management counseling.

 

Generally, if non-surgical treatment is unsuccessful or if there is clear joint damage, surgery may be indicated. Surgery can involve either arthroscopy (the method identical to the orthopaedic procedures used to inspect and treat larger joints such as the knee) or repair of damaged tissue by a direct surgical approach.

Temporo − mandibular Joint Ankylosis

 

Temporo − mandibular Joint Ankylosis

 

Temporo − mandibular Joint Ankylosis

 

Temporo − mandibular Joint Ankylosis

OTOPLASTY

Otoplasty denotes the surgical and non−surgical procedures for correcting the deformities and defects of the pinna (external ear); and for reconstructing a defective, or deformed, or absent external ear, consequent to congenital conditions (e.g. microtia, anotia, etc.) and trauma (blunt, penetrating, blast). Correction of the defect or deformity by creating an external ear that is of natural proportions, contour, and appearance is usually achieved by the reshaping, the moving, and the augmenting of the cartilaginous support framework of the pinna.

Otoplasty denotes the surgical and non−surgical procedures for correcting the deformities and defects of the pinna (external ear); and for reconstructing a defective, or deformed, or absent external ear, consequent to congenital conditions (e.g. microtia, anotia, etc.) and trauma (blunt, penetrating, blast). Correction of the defect or deformity by creating an external ear that is of natural proportions, contour, and appearance is usually achieved by the reshaping, the moving, and the augmenting of the cartilaginous support framework of the pinna.

Unilateral Microtia: Ear Reconstruction with Autogenous Cartilage

 

Bilateral Microtia: Ear Reconstruction with Autogenous Cartilage

 

Unilateral Microtia: Ear Reconstruction with Autogenous Cartilage

 

Correction of Prominent Ear

 

Correction of Lop Ear

OCULOPLASTY

Oculoplastic Surgery, also known as Ocular Plastic and Reconstructive Surgery, is the cosmetic, corrective, and reconstructive surgery of the eye structures to treat a broad range of conditions. These include the eyelids, the eye socket, the soft tissues that surround the eyes and the tear system. These structures can be affected by injuries, infections, tumours, by birth or by problems related to aging.

Excision of Dermoid Cyst from Eyebrow

 

Excision of Cyst of Moll´s gland from Orbit

 

Excision of Lymphangioma from Orbit

 

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