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| CASE REPORT |
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| Year : 2021 | Volume
: 8
| Issue : 1 | Page : 65-68 |
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Tessier cleft type 5 craniofacial anomaly – A rare oblique facial cleft
Zynul Ali Sirsmith John1, Sunita S Shrivastav1, Nitin Bhola2
1 Department of Orthodontics and Dentofacial Orthopedics, Sharad Pawar Dental College, Wardha, Maharshtra, India
2 Department of Oral and Maxillofacial Surgery, Sharad Pawar Dental College, Wardha, Maharshtra, India
| Date of Submission | 09-Jun-2020 |
| Date of Acceptance | 22-Sep-2020 |
| Date of Web Publication | 13-Jan-2021 |
Correspondence Address:
Dr. Zynul Ali Sirsmith John
Department of Orthodontics and Dentofacial Orthopedics, Sharad Pawar Dental College, Wardha, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jclpca.jclpca_17_20
One of the rarest types of craniofacial malformations is Tessier cleft type 5. These are oblique facial clefts with bony defect representing an alveolar cleft in the premolar region. This extends to the lateral surface of the maxilla up to the infraorbital rim and orbital floor. The cleft begins in the upper lip, just medial to the commissure, and extends up to the eyelid. They may also show deficient maxillary growth on the side of the cleft, clinically represented as constriction in the maxilla and occlusal cant. It becomes very challenging to treat such cases and requires a team approach. The goals of such cases include surgical repair of the lower eyelid and outer canthus, expansion of the arch and alignment, Bone grafting for the alveolar cleft, zygomatic region, and orbital rim to restore the continuity. The following is a case report of Tessier type 5 facial cleft describing the clinical features, diagnosis, and treatment approach for the case.
Keywords: Facial cleft, lateral maxillary dysplasia, Tessier cleft type 5
How to cite this article:
John ZA, Shrivastav SS, Bhola N. Tessier cleft type 5 craniofacial anomaly – A rare oblique facial cleft. J Cleft Lip Palate Craniofac Anomal 2021;8:65-8 |
How to cite this URL:
John ZA, Shrivastav SS, Bhola N. Tessier cleft type 5 craniofacial anomaly – A rare oblique facial cleft. J Cleft Lip Palate Craniofac Anomal [serial online] 2021 [cited 2021 Apr 11];8:65-8. Available from: https://www.jclpca.org/text.asp?2021/8/1/65/306769 |
| Introduction | |  |
Oblique facial clefts are among the rare anomalies that constitute a broad spectrum of anomalies. They represent 0.24% of all the craniofacial clefts.[1] In 1976, Tessier gave a classification for craniofacial clefts, which is the most accepted one. They are numbered from 0 to 14. They can be grouped into four groups of midline (0.14), paramedical (1, 2, 12, 13), orbital (3, 4, 5, 9, 10, 11), and lateral clefts (6, 7, 8). Among the oblique facial clefts, Tessier facial cleft type 5 is very rare.[2],[3] The cases that are known are sporadic and did not possess genetic or syndromic involvement. The typical representation of the cleft begins from commissure up to the eyelid. They also show lateral alveolar cleft, most commonly in the premolar region, along with maxillary hypoplasia. They are also associated with coloboma and canting of the orbital floor on the affected side.[4]
Craniofacial clefts have complex involvement of hard and soft tissues and require complex diagnostic and treatment modalities. The common treatment modalities include a combination of soft- and hard-tissue repair followed by orthognathic surgery, facial bipartition, or orbital box osteotomy. A multidisciplinary approach is vital in success and optimum care for such cases.
| Case Report | | |
A 11-year-old female child reported after the previous repair of the oblique cleft on the left side running from commissure up to the eyelid giving an appearance of macrostomia. No history of consanguinity or congenital anomaly in other siblings could be elicited. The general and ENT examination were normal. Respiratory and central nervous examination revealed no abnormality.
Examination of the face revealed an oblique scar on the left side from the commissure up to the center of the lower eyelid [Figure 1]. The lower eyelid repair was incomplete, representing an eyelid coloboma and had a loss of eyelash hairs [Figure 2]. Epiphora was absent. The nasolacrimal duct was patent on both sides. The left orbital margin was lower than the right and the left commissure was superior as compared to the right representing cant and deficient growth. The nose was deviated toward the right side. The zygomatic region showed depression on both the sides, but severe on the left side. Intraoral examination revealed macroglossia with a lateral alveolar cleft in the premolar region with missing the first premolar on the left side. The bilateral posterior open bite was present with tongue thrusting and constricted upper jaw [Figure 3] and [Figure 4].
Both chest X-ray and electrocardiogram were normal. Computed tomography (CT) scan revealed an alveolar cleft on the right side and hypoplastic maxilla showing significant deficiency in the buttress region [Figure 5]. Both the supra-orbital and infra-orbital rims on the left side were lowered. Slight canting of the maxilla was seen due to deficient growth on the left side. No other cephalic disorders were present. Based on the examination, a diagnosis of Tessier type 5 facial cleft was given with Angles Class III malocclusion subdivision on the left side and Skeletal Class III malocclusion.
| Discussion | | |
Tessier type 5 facial cleft is among the rare craniofacial anomalies and requires a team approach for its management.[1] The American Association of Cleft Palate Rehabilitation has classified Tessier type 5 as orofacial canthal cleft.[5] The embryology of such clefts has been ascribed to the failure of fusion between the nasomedial, nasolateral and maxillary process.[6] Some experimental work has suggested neurovascular deficiency, migration of tissue such as amniotic bands, and local pressure causing ischemia as a cause for these malformations. Some authors have also identified oblique cleft to be occurring in the late developmental period rather than primary morphogenesis.[7]
The diagnostic approach included clinical examination, which revealed facial asymmetry with depressed malar prominence. It is very important to examine malar prominence on both sides in such cases to plan its reconstruction to achieve symmetry [Figure 2]. Also, the eyelid examination has to be done in worm's view in both open and closed eyelid positions to assess the amount of closure required as well as its prognosis. The soft tissue in the vicinity of the eyelid is critical in planning their closure. The soft tissue was deficient due to previous surgery; hence, a direct closure was done to preserve the soft tissue. There was a lateral cleft, which was quite different from the defect we see in unilateral or bilateral cleft cases. The defect extended up to 3mm below the orbital margin which was confirmed by a CT scan [Figure 5]. The scan also revealed collapse on the left side, evident by cant in the palatal and orbital plane. Such cases have altered growth at the site of the defect, which was evident by the tipping of these planes. This helps us in planning grafting requirements in the defect as well as planning reconstruction for the malar. The goals for this case were to correct the eyelid coloboma, expansion of the maxillary arch to correct the crossbite and eliminate the premature contacts, and leveling and alignment of the arches so that after bone grafting on the lateral cleft, the second premolar in the cleft vicinity could be extruded. Bilateral grafting would be required in the buttress region to treat the asymmetry. The case was started with expansion [Figure 6] and alignment of the upper arch to full slot alignment to correct the crossbite. After 5 months, direct closure of eyelid coloboma was done [Figure 7], and at the same time, lateral alveolar bone grafting was done [Figure 8] taking graft from the iliac crest. [Figure 9] shows post-operative images showing expansion achieved and stable results of bone graft in an orthopentomogram. After 3 months, the case will be revaluated whether to go for bilateral bone grafting in the zygomatic region or premolar extraction in the upper arch to create negative overjet for midface advancement and correction of maxillary cant. After the surgical reconstruction phase and completion of orthodontic treatment, the deviation of nose and macroglossia has to be addressed. | Figure 9: Postoperative (a) occlusal intraoral photograph, (b) occlusal radiograph, (c) orthopentomogram
Click here to view |
Cases with Tessier type 5 facial cleft should be thoroughly examined for the lateral cleft, the soft tissue in the vicinity of the eyelid, dental prematurity to eliminate the development of future temporomandibular disorders as well as the requirement of reconstruction of the malar prominence.
| Conclusion | | |
Tessier facial clefts are rare anomalies that constitute severe esthetic and functional disturbances. Clinical presentations are varied, and hence, they require individualized treatment planning. A team approach is required for the optimum care of the patient. Accurate diagnosis and treatment planning is the key to success in such cases.
Informed consent
A prior consent was obtained from the patients parents regarding publication of photographs and radiographs related to the case.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | da Silva Freitas R, Alonso N, Shin JH, Busato L, Dall'Oglio Tolazzi AR, de Oliveria e Cruz GA. The tessier number 5 facial cleft: Surgical strategies and outcomes in six patients. Cleft Palate Craniofac J 2009;46:179-86.  |
| 2. | Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg 1976;4:69-92. |
| 3. | Nayak BB, Lopamudra M. A rare case of a combination of tessier cleft 0 and 3 in a 4-year-old child-a case report. Indian J Plast Surg 2019;52:250-1. |
| 4. | Reddy SG, Reddy RR, Obwegeser J, Mommaerts MY. Options for the nasal repair of non-syndromic unilateral Tessier no. 2 and 3 facial clefts. Indian J Plast Surg 2014;47:340-5. [ PUBMED] [Full text] |
| 5. | Abdollahifakhim S, Shahidi N, Bayazian G. A bilateral tessier number 4 and 5 facial cleft and surgical strategy: A case report. Iran J Otorhinolaryngol 2013;25:259-62. |
| 6. | Prasad V, Ahmed R, Singh AK, Kumar V. A rare case of bilateral oblique facial cleft and accessory maxilla with repaired unilateral cleft lip and palate. Natl J Maxillofac Surg 2019;10:241-4. [ PUBMED] [Full text] |
| 7. | Kalantar-Hormozi A, Abbaszadeh-Kasbi A, Goravanchi F, Davai NR. Prevalence of Rare Craniofacial Clefts. J Craniofac Surg 2017;28:e467-70. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]
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