Summary of Congenital Craniofacial Clefts: Etiology and Treatment
Congenital Craniofacial Clefts: Etiology & Treatment Guide
Introduction
Tessier craniofacial clefts describe uncommon but important patterns of soft-tissue and bony disruption of the face and skull. This material focuses on the clinical features, skeletal and soft-tissue involvement, and practical considerations for specific Tessier cleft numbers discussed in the provided content (numbers 2, 3, 4, 7, and 8). It is written for a Not attending student and emphasizes recognition, complications, and clinical implications.
Definition: Tessier clefts are numbered craniofacial clefts that map along lines from the mouth to the skull; each number corresponds to a reproducible anatomical track of soft-tissue and bony disruption.
How to approach each Tessier cleft
Break down assessment into three parts for each cleft:
- Soft-tissue findings (lip, cheek, eyelid, canthus, globe)
- Skeletal involvement (where the osseous defect runs, which sinuses/orbit/oral cavities communicate)
- Functional consequences and clinical priorities (lacrimal injury, globe exposure, feeding, occlusion)
Practical exam checklist for any suspected Tessier cleft
- Inspect lip and philtrum, note relationship to cupid’s bow and oral commissure
- Check lower and upper eyelids, medial and lateral canthi
- Test lacrimal drainage and look for displaced puncta or cutaneous drainage
- Assess globe position and corneal exposure
- Palpate zygoma, maxilla, infraorbital rim; note asymmetry
- Request CT to map bony defects and sinus/orbital communications
Tessier number 3 (oronaso-ocular cleft)
Soft-tissue features
- Begins through the philtral column and floor of the nose, between patterns similar to numbers 1 and 2
- Tissue deficiency between the alar base and lower eyelid; nasal ala may be displaced
- Medial canthus often inferiorly displaced and may be hypoplastic
- Colobomas of the lower eyelid are medial to the inferior punctum
- Globe often malpositioned inferiorly and laterally; microphthalmia is possible
Lacrimal and ocular consequences
- The nasolacrimal system, especially the lower canaliculus and inferior punctum, is commonly disrupted
- Drainage may exit onto the cheek instead of the nasal cavity, predisposing to recurrent dacryocystitis
- Corneal exposure and erosions can occur; protect the globe early to prevent desiccation and vision loss
Skeletal involvement
- Osseous defect begins between the lateral incisor and canine and extends through the lacrimal groove
- Direct communication may exist among oral, nasal, maxillary sinus, and orbital cavities
- Anterior maxillary arch may be flattened (contrast to numbers 1 and 2)
- Severe cases may be bilateral and can continue cephalad as a cranial number 11 or 12 cleft
Clinical priorities and management implications
- Early imaging (CT) to evaluate bony involvement and plan reconstruction
- Lacrimal system evaluation and surgical planning because of high risk for obstruction and infection
- Protect the globe (lubrication, temporary tarsorrhaphy if needed)
Tessier number 2
Key features
- Begins between the lateral incisor and canine (similar bony start as number 3) but follows a distinct track
- Extends into the pyriform aperture lateral to the septum and medial to the maxillary sinus
- May involve hard and soft palate; nasal septum deviation away from the cleft is common
- Distorts nasal bones as it passes between them and the frontal process of the maxilla
- Ethmoidal sinus involvement can be associated with orbital hypertelorism
Skeletal consequences
- Asymmetry of greater and lesser sphenoid wings and the anterior cranial base may be present
Clinical implications
- Look for palatal clefts and evaluate airway and feeding
- Imaging to assess sphenoid and cranial base asymmetry for surgical planning
Tessier number 4 (meloschisis / oro-o
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Tessier Craniofacial Clefts - Selected Types
Klíčová slova: Craniofacial clefts overview, Craniofacial clefts and midline anomalies, Craniofacial embryology, Craniofacial clefts embryology, Tessier craniofacial clefts, Classification and anatomy, Craniofacial clefts and orbital anomalies, Reconstruction
Klíčové pojmy: Tessier clefts are mapped lines; assess soft tissue, bone, and function for each case, Number 3 cleft commonly disrupts the nasolacrimal system; expect inferiorly displaced medial canthus and risk of dacryocystitis, Number 3 bony defect starts between lateral incisor and canine and can communicate oral, nasal, sinus, and orbital cavities, Number 2 cleft extends into the pyriform aperture lateral to septum and may involve palate and ethmoid, causing hypertelorism, Number 4 cleft begins lateral to cupid’s bow, passes lateral to the ala, and often spares the nasolacrimal system, Number 7 cleft (commissural) causes zygomatic hypoplasia, vertical ramus deficiency, occlusal cant, and possible facial nerve/ear anomalies, Number 8 cleft affects the frontozygomatic suture, often has dermatocele and epibulbar dermoids, and usually coexists with other clefts, Always obtain CT to map bony defects and plan reconstruction in suspected Tessier clefts, Protect the globe early when eyelid or canthal support is deficient to prevent corneal injury, Manage lacrimal disruptions proactively to avoid recurrent infections and chronic drainage