Turkish Journal of Plastic Surgery

ORIGINAL ARTICLE
Year
: 2018  |  Volume : 26  |  Issue : 1  |  Page : 12--19

Tessier number 30 facial cleft: A rare maxillofacial anomaly


Elif Sari 
 Department of Plastic, Reconstructive and Aesthetic Surgery, Faculty of Medicine, Kirikkale University, Kirikkale, Turkey

Correspondence Address:
Dr. Elif Sari
Department of Plastic, Reconstructive and Aesthetic Surgery, Kirikkale
Turkey

Abstract

Introduction: In number 30 facial cleft patients, the deformities vary in their presentation, ranging from minor clefting of the lower lip (2) to complete clefts of the mandible with absence of the hyoid bone, thyroid cartilage, and manibrium. Materials and Methods: A case of Tessier number 30 facial cleft with only lower lip deformity is reported in this paper. Results: The pathognomonic features of the facial clefts were reviewed in terms of the related literature. Conclusion: We aimed to remind the pathology and review the literature in this report.



How to cite this article:
Sari E. Tessier number 30 facial cleft: A rare maxillofacial anomaly.Turk J Plast Surg 2018;26:12-19


How to cite this URL:
Sari E. Tessier number 30 facial cleft: A rare maxillofacial anomaly. Turk J Plast Surg [serial online] 2018 [cited 2019 Sep 17 ];26:12-19
Available from: http://www.turkjplastsurg.org/text.asp?2018/26/1/12/228009


Full Text



 Introduction



Number 30 facial cleft was first reported as a caudal extension of number 14 and number 0 facial clefts by Tessier in 1976.[1] The deformities vary in their presentation, ranging from minor clefting of the lower lip [2] to complete clefts of the mandible with the absence of the hyoid bone, thyroid cartilage, and manibrium.[3] In this article, we present a case of midline lower lip cleft that was repaired in late adolescent period and a review of the literature.

A 20-year-old male patient was referred to our clinic with a midline cleft and ulceration of the lower lip without any other involvement [Figure 1]. Several treatment methods such as antibiotic ointments and antihistaminic drugs had been tried for the ulcer by dermatologists; however, the pathology was not resolved.{Figure 1}

In physical examination, the upper lip, tongue, nose, and frontal region were found normal. The mandible and the soft tissue of the neck were not involved. Radiographic confirmation was done. Written consent was obtained from the patient, and the surgical approach was planned.

Under local anesthesia, an elliptic incision was performed, ulcer was debrided, and the orbicularis oris muscle was identified. Free edges of the muscle were dissected and sutured together in the midline. Mucosa and the vermillion line were connected meticulously. No complications were observed in the early postoperative period. At 10-month follow-up, the patient was satisfied with the result [Figure 2].{Figure 2}

 Discussion



The incidence of craniofacial clefts is estimated between 1.4 and 4.9/100,000 live births.[4] Although two genders are being affected by craniofacial clefts, girls are more commonly affected. The pathology can develop due to large doses of radiation, infection, vitamin deficiencies, metabolic syndromes, and Vitamin A toxicity.[5],[6],[7]

In Tessier Classification, clefts 0–14 radiate around the orbital bone rims [Figure 3] and [Table 1]. Tessier number 0 and number 3 are seen to be the most commonly reported cleft types in both genders in the literature.[8],[9] Tessier number 30 cleft of the face is a rare congenital anomaly. The deformity occurs as insufficient fusion of first branchial arc or the failure of mesodermal penetration in midline.[10] Anomaly can range from a lower vermillion notching to the involvement of the teeth, tongue, mandible, and neck.[11],[12],[13],[14] Tafreshi et al. reported a Tessier number 30 cleft case with congenital heart defects.[13] One patient was reported by Adetayo and Martin [15] with a Tessier number 30 cleft concomitant with levocardia, ventricular septal defect, a patent foramen ovale, double outlet right ventricle, intestinal malrotation, and bilateral undescended testicles. Vendramini-Pittoli et al. published two Tessier number 30 cleft cases. One of them was accompanied by a Tessier number 7 cleft, and the other one had developed together with Tessier number 4, 7, and 9 clefts.[16] Moreover, nasal anomalies and cleft lip and palate can also be seen with Tessier number 30 facial clefts.[17],[18],[19]{Figure 3}{Table 1}

Midline cleft of the lower lip and mandible can also be a sign of a syndrome, such as the Robinow syndrome which presents itself with extremity, genitalia, costa and vertebra anomalies,[20] or the Richieri-Costa syndrome which involves limb defects.[21]

The prenatal diagnosis of the Tessier number 30 cleft can be made through ultrasonography at 15–18 weeks of the pregnancy.[22]

Reports about this pathology are rare in the literature.[10] Less than 80 cases have been reported since the first article by Couronné.[10],[23] There are a few cases of craniofacial clefts reported from Turkey;[24],[25],[26],[27] moreover, only two papers reporting cases from Turkey that are accompanied by a simple lower lip defect.[24],[28] Due to this rarity, it can be said that there is no consensus on the management of this deformity. Therefore, we decided to prepare an algorithm for the treatment of the pathology.

Simple notching of the lower lip can be treated with wedge excision or Z-plasty.[29] Lip deformity and bifid tongue should be corrected as early as possible due to functional deficits. Duplication of the tongue can be corrected with wedge excision or Z-plasty. In addition, the tongue should be released from the floor of the mouth.[30]

Clefts of the mandible can be treated with one- or two-stage procedure depending on the range of the defect.[31] Most surgeons begin the procedure when the child is about 10-year-old to avoid tooth bud damage and use interosseous wires or plates.[32] On the other hand, Oostrom et al. report that a carefully planned osteosynthesis of the base of the mandible does not harm the buds and may provide better occlusion.[33] If the mandibular cleft is too wide, the bones should be stabilized at an early age to avoid obstructing eating and breathing.[34] A bone graft harvested from the costa, calvarium, or iliac crest can be inserted around the age of 10 in the second stage.[28],[35],[36]

One of the largest case series was reported by Freitas et al. with 17 patients aged from 1 month to 30 years.[36] Early surgery was performed in two patients with sleep apnea and impaired nutrition using distraction osteogenesis to achieve better tongue movement. They used Z-plasty for mucosal bands, muscle repair for the contraction, and straight closure of the skin in a few patients. Early bone grafting was recommended in this report to establish mandibular continuity.

The strap muscles of the neck are often replaced with dense scar tissue and cause flexion contracture bands and can be repaired with multiple Z-plasties.[1] Facial anomalies can extend to the widened interclavicular space, bifid or absent manubrium, presternal skin tags, cleft lip and palate, hemifacial microsomia, dermoid cyst of the face, extremity anomalies, and cardiac deformities.[10] Treatment choices for all these anomalies should be determined according to the type of the deformity.

 Conclusion



The lower lip notching was corrected with wedge resection and three-layer closure in our patient. No complications occurred. Functional and esthetic results were good in the postoperative 10-month follow-up period. Tessier number 30 cleft should be kept in mind in lower lip anomalies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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