**3. Craniofacial pathology**

Pathology includes any deviations from normal function and structure. The pathologic conditions show themselves as aplastic, hypoplastic, hyperplastic, neoplastic, traumatic or developmental entities. In craniofacial pathology a good access to the lesion and preservation of vital structures are important factors in a successful operation. Most operations in this field should be carried out in a team work manner engaging both the maxillofacial surgeon and the neurosurgeon.

## **3.1. Fibrous dysplasia**

**2. Craniofacial anomalies**

840 A Textbook of Advanced Oral and Maxillofacial Surgery Volume 2

treatment of patients.

is affected more frequently.

synostosis.

girls.

Craniofacial anomalies (CFA) include different dysmorphogenic conditions in this region of the body. People with these anomalies generally have problems not only in function but also with their ordinary life in the society. These people, especially children with CFA have less social competence and lack of self-esteem. The aim of craniofacial surgery is to give a normal appearance to the patients. Specialized centers would be a great help in treating patients with CFA. Accurate data of epidemiology of CFA is of utmost importance for managing and

**Cleft lip and palate** are the most common congenital anomalies which affect the orofacial region. The incidence of oral clefts in United States is 1 in 700 births.[4] Orofacial clefts are more common in boys but cleft palate without cleft lip have a slight tendency to involve girls. One fourth of oral clefts are bilateral and the rest are unilateral. In unilateral cases the left side

**Craniosynostosis** means premature fusion of cranial vault sutures. There are six major cranial vault sutures. Any of these sutures can be affected in craniosynostosis, alone or in combination with other sutures. In this section we discuss the prevalence of nonsyndromic single suture

**Sagittal suture synostosis** or scaphocephaly is the most common single suture synostosis with the prevalence of 1 in 5000 live births. [5] Boys are affected three times more frequently than

**Coronal suture synostosis** or anterior plagiocephaly is the second most common single suture

**Metopic suture craniosynostosis** or trigoncephaly is an unusual type of synostosis with an

**Lambdoid suture synostosis** or posterior plagiocephaly is a rare entity with a prevalence of 1

Pathology includes any deviations from normal function and structure. The pathologic conditions show themselves as aplastic, hypoplastic, hyperplastic, neoplastic, traumatic or developmental entities. In craniofacial pathology a good access to the lesion and preservation of vital structures are important factors in a successful operation. Most operations in this field should be carried out in a team work manner engaging both the maxillofacial surgeon and the

synostosis. The prevalence is approximately 1 in 10000 births. [6]

**Bilateral coronal suture craniosynostosis** or brachycephaly is also rare.

approximate prevalence of 1 in 15000. [7]

in 150000 live births. [8]

neurosurgeon.

**3. Craniofacial pathology**

Fibrous dysplasia is a benign fibro-osseous lesion. Fibro-osseous lesions are a category of entities in which normal bone tissue is replaced with fibrous and mineralized tissue. In fibrous dysplasia normal bone is substituted with cellular fibrous tissue and immature bone.[9]

In most of cases it is monostotic involving a single bone.[10] The rate of growth is very slow. The maxilla and frontal bone are the most affected sites.[11] The most common feature is painless swelling. Radiographic feature of ground glass is of significant importance for diagnosis. This pattern is due to superimposition of disorganized poorly calcified bone trabecular.

Polyostotic Fibrous dysplasia is uncommon. In syndromic conditions like Jaffe-Lichtenstein, McCune-Albright and Mazabraud syndromes, polyostotic fibrous dysplasia forms an impor‐ tant part of these syndromes.

Treatment of fibrous dysplasia depends on the degree of functional or cosmetic impairment from shaving of involved area to resection. The aim is not to remove the entire lesion but to have an acceptable appearance. Regrowth after surgical reduction is unpredictable.

#### **3.2. Sarcoma**

One of the most important issues in morbidity and mortality of children is malignant neo‐ plasms.[12] One third of malignant solid tissue tumors during infancy and childhood are caused by sarcomas.[13] The most common sarcoma in children is rhabdomyosarcoma and after that fibrosarcoma.[14] For management of rhabdomyosarcoma, radiotherapy and chemotherapy in combination with surgery are recommended in accessible tumors by many authors.

#### **3.3. Lymphoma**

Malignant lymphoid tissue tumors are common in head and neck region. There are different types of classification for lymphoma but separation to Hodgkin and non- Hodgkin types is the most common. Both have manifestations in the head and neck area. In Africa, another type of this condition known as Burkitt's lymphoma is common in children. Surgery is usually not indicated and chemotherapy should be done by an oncologist.

#### **3.4. Melanoma**

Approximately 20% of melanomas occur in head and neck regions.[15] An important etiologic factor is excessive exposure to ultraviolet light but many risk factors have been proposed in development of this lethal entity. Most of them arise from pigmented lesions. Melanoma can be categorized to superficial spreading, nodular, lentigo maligna, acral lentigenous and desmoplastic type according to clinical and histological evaluations. Excision of the lesion is the treatment of choice. Elective lymph node dissection is a controversial matter.
