*3.1.3 Clinical assessment*

*Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications*

permanent loss of function.

**3. Corrective surgery**

tional conditions and quality of life.

**3.1 Pre-surgical assessment & preparation**

specific problem prior to any surgical decision.

*3.1.1 Multi-disciplinary approach*

ogy, and oculoplastic, among others.

*3.1.2 General assessment*

the pathognomonic features of broad thumbs and great toes with variable soft tissue syndactyly. Other associated features include hypertelorism, strabismus, downslanting palpebral fissures, class III malocclusion and beaked nasal deformity. Again, the majority of cases involve FGFR-2 mutations, 5% of patients express an FGFR-1 mutation and demonstrate less severe phenotype [17, 18]. It is an autosomal dominant inheritance pattern with the incidence of 1 in 100,000 births. A classification system proposed that patients are categorized into three types based upon clinical findings and severity. Type 1 is the classic Pfeiffer syndrome clinical pattern. Type 2 is more severe and associated with the cloverleaf skull and type 3 Pfeiffer syndrome is the most severely affected. In one institution, a review of 28 patients has been conducted and the Cohen subtypes dissemination is 61% type 1, 25% type 2 and 14% type 3 [19]. All patients have undergone numerous corrective surgery. This study recommends aggressive treatment and monitoring on patients' functional conditions to prevent further damage to the vital organ that leads to

Therefore, syndromic craniosynostosis is a condition of multiple associated clinical problems with the same pattern of treatment strategies, expected difficulties and pathologic identifications. Profound knowledge of the disease process, pathognomonic findings and clinical situations of each syndrome is essential. Therefore, multidisciplinary approach in total management of the clinical problems is important and must be detected and treated earlier to improve patient's func-

In syndromic craniosynostosis, surgical intervention is often functionally indicated with the primary aim is to treat the pressing functional discrepancy or to salvage vital structures such as the brain and eyes [4, 20]. The three main functional issues secondary to the severe skull deformity are increased intracranial pressure, severe exopthalmos and obliterated nasopharyngeal airway. As such, comprehensive assessment to the brain, eyes and upper airway is paramount to determine the

Taking the multiple functional issues into consideration, multi-disciplinary approach has become the trend in syndromic craniosynostosis management. A craniofacial centre or unit may consist of various specialties such as neurosurgery, oral and maxillofacial surgery, plastic surgery, otorhinolaryngology, ophthalmol-

Patient growth progress and development should be assessed and properly documented as it provides valuable baseline and comparative data before and after surgery. This includes objective data such as head circumference, height, weight, gross and fine motor, as well as speech development, among others. These parameters are important as it may determine whether any corrective surgery should be

indicated as early as possible or performed at a later stage [21].

**132**

Specifically on the craniofacial region, assessment can be focused on patient's initial head shape, the degree of exopthalmos, ability for eyelid closure, nasal airflow, midfacial projection, jaw relationship and intraoral condition. Clinical picture documentation is very useful as it can be used for serial comparison.
