> ## Content Index > Fetch the complete content index at: https://www.theplasticsfella.com/llms.txt > Use this file to discover other available public pages before exploring further. # Craniofacial Clefts: Tessier Classification, Types, & Management - URL: https://www.theplasticsfella.com/craniofacial-clefts/ - Published: 2024-05-22T11:06:00.000Z - Updated: 2024-05-23T13:32:42.000Z - Description: Craniofacial clefts are skeletal and soft-tissue abnormalities. This article details the Tessier Classification, types of clefts and their management. - Author: PlasticsFella - Tags: Head and Neck #### Summary Card [****Definition**](https://www.theplasticsfella.com/craniofacial-clefts/#definition-of-craniofacial-cleft) A skeletal and soft-tissue facial pathology that can be described as cleft numbers from 0-14 and can form part of syndromes. [****Pathogenesis**](https://www.theplasticsfella.com/craniofacial-clefts/#pathogenesis-of-craniofacial-cleft) Failure of fusion and dehiscence. They occur sporadically and have been linked to exposure and maternal risks. [****Tessier Classification**](https://www.theplasticsfella.com/craniofacial-clefts/#tessier-classification-of-craniofacial-clefts) Assigns a number to a craniofacial cleft based on its position relative to the midline. Clefts 0-7 are facial, and 8-14 are cranial. [****Types**](https://www.theplasticsfella.com/craniofacial-clefts/#types-of-craniofacial-clefts) Numbered 0-14 and can be grouped into oral-nasal, oral-ocular, lateral facial, and cranial clefts. [****Management**](https://www.theplasticsfella.com/craniofacial-clefts/#management-of-craniofacial-clefts) A multidisciplinary team to treat function, cosmesis, and reconstruct anatomical structures and cavities. --- ## Definition of Craniofacial Cleft ****Key Point** Craniofacial clefts encompass various soft-tissue and bone deformities, classified from 0-14 based on anatomical location. Craniofacial clefts is an umbrella term that encompasses a wide of soft-tissue and bone deformities. It is a type of craniofacial deformity defined by the American Society of Cleft Lip and Palate. They can be sub-defined based on the anatomical location: - **Oral-Nasal:** Clefts 0-3 - **Oral-Ocular:** Clefts 4-6 - **Lateral Facial:** Cleft 7-9 - **Cranial:** Clefts 10-14 ###### --- ## Craniofacial Cleft Pathogenesis ****Key Point** Craniofacial Clefts are caused by a failure of fusion and dehiscence. They occur sporadically and have been linked to exposure and maternal risks. Craniofacial clefts are "atypical clefts" that occur due to two theories: 1. **"Classic":** failure of fusions of the facial prominences. 2. **Mesodermal penetration:** failure of mesodermal penetration results in unsupported epithelial walls that dehisce. These occur sporadically, but specific environmental factors have been identified: - **Exposure:** radiation, vitamin A derivatives - **Maternal:** infection (CMV, toxoplasmosis), diabetes, weight, folic acid deficiency. 💡 ****Tip:** Facial development occurs during months 1 and 2\. The frontonasal prominence creates nasal and olfactory placodes that become medial and lateral processes. --- ## Tessier Classification of Craniofacial Clefts ****Key Point** Tessier Classification assigns a number to a craniofacial cleft based on its position relative to the midline. Clefts 0-7 are facial, and 8-14 are cranial. ### **Tessier Classification** is an **anatomical** classification of craniofacial clefts. A number is assigned to each malformation according to its **position relative to the midline.** Tessier classification is the most commonly used for Craniofacial clefts. - **Benefit:** easily correlate anatomical defects and required reconstruction. - **Limitation:** purely descriptive and does not denote causation. The image below further describes the Tessier classification for craniofacial clefts. ![Tessier Classification for Craniofacial Clefts](https://storage.ghost.io/c/a0/9f/a09fd7b4-d396-4b28-aed3-b87e20483292/content/images/2022/07/Tessier-Classification.png) Tessier Classification for Craniofacial Clefts 1. **All clefts** are numbered from 0-14. 2. **Midline clefts** are number 0. 3. **Facial clefts** are numbered out laterally from 1-7 inferior to the orbit. 4. **Cranial clefts** are numbered in medially from 8-14 superior to the orbit. 5. **Facial** and **Cranial** clefts can be connected. The table below illustrates some tips to remember Tessier Classification. ![Tips for Tessier Classification, Classification for Craniofacial Clefts](https://storage.ghost.io/c/a0/9f/a09fd7b4-d396-4b28-aed3-b87e20483292/content/images/2022/07/Tessier-Tips-Craniofacial-Clefts.png) Tips for Tessier Classification Craniofacial clefts can be grouped based on their location. There are 4 main groups: - **Oral-nasal clefts** are Tessier Clefts 0-3 between the midline and cupid's bow. This results in clefts involving the midline structures (lips and nose). - **Oral-Ocular clefts** are Tessier Clefts 4-6 that occur between oral and orbital cavities without disrupting the nose. - **Lateral Facial clefts** are Tessier Clefts 7-9, resulting in Treacher Collins Syndrome, hemifacial microsomal, and necrotic facial dysplasia. - **Cranial Clefts** are Tessier clefts 10-14 that occur in the frontal and cranial vault. 😎 ****Fun Fact:** Van der Meulen's embryological classification is less common: craniofacial skeleton develops along a helical course symbolised by the letter **S*. --- ## Types of Craniofacial Clefts ****Key Point** Craniofacial clefts are numbered 0-14 and involve soft tissue and bone. They can be grouped into oral-nasal, oral-ocular, lateral facial, and cranial clefts. **Cleft Number 0** - A cleft spectrum of **deficient** **or** **excess** midline structures. - Deficiency leads to **hypo**tolerism; excess leads to **hypertelorism**. - Can continue as cleft number 14. - Incomplete merging of the median nasal prominences in the midline. **Cleft Number 1** - Nasal dysplasia has a pattern similar to cleft lip and palate. - Lateral margin of cupid's bow and between the central and lateral incisors. - Can continue as cleft number 13. **Cleft Number 2 (Rare)** - Hypoplastic nasal ala, flattened nose, displaced medial canthus. - The lacrimal duct is not involved. - Can continue as cleft number 12. **Cleft Number 3 ("Common")** - Between the lateral incisors and canine. - Superiorly based alar and short nose. - The lacrimal system involved + **colobomas** of the lower eyelid. - Can continue as cleft 10 or 11. **Cleft Number 4** - Lateral to cupid's bowel and nasal ala. - Ascend **around** the alar base, along the nasomaxillary junction, and across the tear duct and medial orbital tissues. - Unilateral or bilateral. - **Colobomas** but intact medial canthal ligament. 😎 ****Fun Fact:** Coloboma is a ****congenital** ocular defect of the eyelid, iris, retina, choroid, or optic disk. The defects can range in size, from a small notch to a large structural cleft. **Cleft Number 5 (Rarest)** - Lateral to canine and infraorbital foramen. - Medial to the oral commissure. - Extends through the maxillary sinus to the orbital floor. - Abnormal **sphenoid wing** (**agenesis** linked to neurofibromatosis). **Cleft Number 6** - Forms part of **Treacher Collins Syndrome.** - **Normal Ear**. - Zygomaticomaxillary cleft. **Cleft Number 7 (Most Common)** - The middle ear, zygoma, maxilla, and mandible affected. - Variable expression of macrostomia and hypoplasia of the zygoma. - **Trigeminal** and **facial** nerves are involved. - Caused by disruption of the **stapedial artery** during embryogenesis. - Centred in the region of the zygomaticotemporal suture. - Associated with a duplicate maxilla, supernumerary teeth. - Occur in isolation or more commonly with craniofacial/[hemifacial microsomia](https://www.theplasticsfella.com/hemifacial-microsomia/). **Cleft 8 (Rare in Isolation)** - Primarily involves the orbital area. - Lateral commissure **coloboma** and lateral canthus **absence**. - Associated with **Goldenhaar Syndrome**. **Cleft Number 9 (Rare)** - Involves the upper eyelid and brow. - The temporal branch of facial nerve palsy. - Associated with encephaloceles. **Cleft Number 10** - Involvement of middle orbital area and cranial base. - Link to hypertelorism. - Proptosis with front-orbital encephalocele may be present. - Can be an extension of cleft number 4. **Cleft Number 11** - Involvement of upper medial eyelid (not cranial base). - Link to encephalocele & hypertelorism (pneumatisation of ethmoid cells). - Can be an extension of cleft number 4. **Cleft Number 12** - Laterally displaced medial canthus and frontal hairline projected downward. - Hypertelorism and telecanthus. - Involved frontal and sphenoid sinuses. **Cleft Number 13** - Paramedian frontal encephalocele. - V-shaped hair. - Dystopia and hypertelorism. **Cleft Number 14** - A spectrum of deficiency to excess. - Involves CNS abnormalities. - Can see herniation of intracranial contents. **Cleft Number 30** - Bifid tongue. - A notch on the lower lip. - Issues with hyoid bone and thyroid cartilage. 💡 ****Tip**: Clefts do not pass through bony foramina that are the site of neurovascular structures. --- ## Management of Craniofacial Clefts ****Key Point** The management is based on a multi-disciplinary team focusing on treatment function, cavity separation, and cosmesis. ### Principles The management of craniofacial clefts is centred on a multi-disciplinary team. The goal of treatment is to restore function, cosmesis, and normal anatomical alignment of structures and cavities. Critical aspects of the management focus on: 1. **Eyes:** Eyelid reconstruction to prevent globe exposure. 2. **Mouth**: functional correction of macrostoma. 3. **Cavities:** recreate separations between nose, mouth, and orbits. 💡 ****Tip:** Lateral clefts affect bone more, and medial clefts affect soft tissue more. ### Techniques To achieve these goals, a myriad of techniques is available. These include: - Distraction Osteogenesis - [Bone grafting ](https://www.theplasticsfella.com/bone-grafts/) - Alloplastic implants - [Tissue Expansion](https://www.theplasticsfella.com/tissue-expansion/) - [Flap Reconstruction](https://www.theplasticsfella.com/classification-of-flaps/) 💡 ****Tip**: Bilateral cases may present different clefts on each side. --- ## Conclusion Upon completing this article, you will have accomplished the following: ****1\. Craniofacial Clefts:** You've understood the definition and classification of craniofacial clefts, ranging from numbers 0 to 14, based on the Tessier classification. ****2\. Pathogenesis:** You understand the developmental disruptions that lead to craniofacial clefts, including the failure of fusion and dehiscence, influenced by genetic and environmental factors. ****3\. Types of Clefts:** You've learned about the different types of craniofacial clefts categorized under oral-nasal, oral-ocular, lateral facial, and cranial groups, along with their specific anatomical impacts. ****4\. Management:** You have become familiar with the multidisciplinary approach to treating craniofacial clefts, focusing on restoring function and cosmesis through various surgical and non-surgical techniques. ****5\. Early Intervention:** You understand the importance of early and accurate diagnosis and intervention in managing craniofacial clefts to improve functional and aesthetic outcomes for patients. ---