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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 2  |  Issue : 1  |  Page : 63-65

Incomplete median cleft of lower lip with ankyloglossia


Department of Craniofacial Surgery and Research Centre, SDM College of Dental Science and Hospital, Dharwad, Karnataka, India

Date of Web Publication4-Feb-2015

Correspondence Address:
Dr. Anil Kumar Desai
Department of Craniofacial Surgery and Research Centre, SDM College of Dental Sciences and Hospital, Sattur, Dharwad - 580 009, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2348-2125.150755

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  Abstract 

Median cleft of lower lip and mandible is a rare congenital anomaly and is also described as Tessier's no 30 cleft. It is also associated with other congenital abnormalities with varying degree of severity. The condition was first described by Couronine in 1819 and since then very few cases have been reported. We report a case of median cleft of lower lip and ankyloglossia, which was surgically treated. The etiology, clinical feature and treatment are discussed.

Keywords: Ankyloglossia, lower lip, mandible, median cleft


How to cite this article:
Desai AK, Kumar N, Pavate SS, Ramarathinam D. Incomplete median cleft of lower lip with ankyloglossia. J Cleft Lip Palate Craniofac Anomal 2015;2:63-5

How to cite this URL:
Desai AK, Kumar N, Pavate SS, Ramarathinam D. Incomplete median cleft of lower lip with ankyloglossia. J Cleft Lip Palate Craniofac Anomal [serial online] 2015 [cited 2019 Jul 19];2:63-5. Available from: http://www.jclpca.org/text.asp?2015/2/1/63/150755


  Introduction Top


Median cleft of lower lip and mandible is a rare deformity. It is derived from the lower half of the first brachial arch and is very rare. They present with varying degree of severity ranging from incomplete cleft of lower lip, mandible, tongue, at times associated with cleft of hyoid bone, thyroid cartilage, atrophic strap muscles and absent manubrium sterni. [2] The rarity and variation in severity of the condition are responsible for the lack of consensus on the mode of management. We report a case of median cleft of lower lip with ankyloglossia, the etiology; clinical features and treatment are discussed briefly.


  Case Report Top


A 6-month-old child reported to our unit with a deformity of the lower lip. This was the first child of the parents with no family history of cleft lip or palate. The child was delivered at term and pregnancy was uneventful.

On physical examination, the child had an incomplete cleft of the lower lip extending up to the mentolabial sulcus [Figure 1]. There was no vermillion show along the cleft margin and lip appeared as being pulled inwards. The tip of the tongue had adhered to the floor of the mouth with restricted movements. On palpation, there was a small notching noted over the alveolar aspect of mandible in the midline, but there was no cleft of the mandible, and it was intact. Radiographs were not done because clinically there was no cleft of the mandible and no mobility. Furthermore, examination of the upper limbs revealed syndactyly of the left hand [Figure 2] and constriction band syndrome noted of the right hand [Figure 3].
Figure 1: Preoperative picture of cleft lip

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Figure 2: Syndactyly

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Figure 3: Constriction band syndrome

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The child was operated at the age of 6 month with 6 kg of weight for median cleft lip. "V" marking was done along cleft lip and excision of the tissue along the cleft margin was carried out and closed in layers. The straight line incision was used over the vermilion. As the vermilion had turned toward the lingual side and hence Intra orally vestibular incision was placed and closed in the form of "V-Y" technique and a good amount of vermilion exposure was achieved [Figure 4]. The tip of the tongue had completely adhered to the mandible [Figure 4] and hence vestibular attachment towards the lingual vestibular side of mandible was released using dissecting forceps. Then the mucosa was undermined from the base of the tongue till the tip and closure were carried in the form of "Z" plasty, thereby achieving good release of the tip. During the follow up for 6 months, the lip length was maintained, and there was no scarring with good vermillion show. Also, the child was able to protrude the tongue with no residual attachment left to the sulcus [Figure 5].
Figure 4: Intraoperative picture of lip

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Figure 5: Postoperative picture of lip and tongue

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  Discussion Top


Atypical craniofacial clefts are rare, the incidence being estimated at 1.4-4.9/100,000 live births. [3] The median cleft of the lower lip was first reported by Couronine in 1819. [1] This is a developmental anomaly of first brachial arch, and It may also be associated with other congenital anomalies. The frequency is about is 4-5 cases/million births. [1] A review of the literature by Erdogan et al. found 48 patients with a median cleft of the lower lip and of these, 37 also involved the mandible. [4] Oostrom et al. in 1996 reported about 65 cases in his study. [5] Armstrong AP and Waterhouse N (1996) reported 67 cases with this anomaly. [3]

There are many hypotheses regarding the development of median mandibular cleft, but the hypothesis proposed by Oostrom et al. in 1996 is most widely acknowledged. Any hypoplasia of the mandibular process during early embryonic period can lead to severe cleft that may involve entire mandible. If the failure of development occurs in the late embryonic period, it will lead to less severe median cleft. [5]

The severity of the deformity can range from minor to major and with broad variations. In minor forms the cleft of the lip is present, which may extend into the mandibular symphysis, also the tongue may be bifid and attached to the alveolar process or at times the tongue may not be developed adequately or absent. [6]

In the severe forms the mandible is bifid, epiglottis or hyoid may be absent and underdeveloped thyroid cartilage may be present. The midline cervical cord, strap muscles may be atrophic. There may be midline dermoid in the neck, the clavicles may be spaced and manubrium sterni may be bifid. [2] Our patient exhibited a cleft of lower lip, notching of the alveolus in the mandible and ankyloglossia.

The common associated anomalies related to this condition are congenital heart diseases, hand anomalies (Syndactyiy and Brachydactly) and foot anomalies like club foot, ectrodactyly and constriction band syndrome have been reported. [3] Our patient exhibited syndactyly, absence of digits, constriction band syndrome.

Common and most readily visible of the associated anomalies are those of the craniofacial structures and limbs. Three major theories have been suggested to explain this association: Early amnion rupture theory (operating through uterine pressure and/or disruption by amniotic band), vascular compromise theory (primarily vascular hypoperfusion) for the spectrum of anomalies observed and lastly early intrinsic defect of the developing embryo theory. [7] With the review of literature, we can conclude that this association of malformation originates as early as embryonic disc stage and that some of the observed associated anomalies are secondary complications of the primary disturbance in embryogenesis.

There is a lack of consensus on the timing of surgery and mode of management. Majority of literatures review suggested correction of the soft tissue structures as soon as possible, such as lower cleft lip and tongue repair at early age. However, the timing of mandibular cleft treatment remains debatable.


  Summary and Conclusion Top


Lower midline cleft lip is a rare facial deformity and has a high incidence of association with the other congenital defects. Thorough evaluation of these deformities is necessary to define aberrant anatomy, to plan surgical procedure, evaluate the effect of craniofacial growth and surgical reconstruction. We can conclude that mode of management is mainly dependent on the rarity and variation in severity of the condition. However, the timing for treatment should depend on the severity of the cleft.

Correction of the soft tissue structures should be done as soon as possible to facilitate feeding and speaking. If the mandibular segments are mobile, an early attempt should be made to stabilize the mandible to achieve functional movement of mandible at an early stage.

 
  References Top

1.
Arshad AR. Incomplete midline cleft of lower lip. Cleft Palate Craniofac J 1995;32:167-9.  Back to cited text no. 1
    
2.
Rana RE, Puri VA, Thakkur RK, Baliarsing AS. Median cleft of mandible and lower lip with ankyloglossia and ectopic minor salivary gland on tongue. Indian J Plast Surg 2004;37:67-70.  Back to cited text no. 2
    
3.
Ladani P, Sailer HF, Sabnis R. Tessier 30 symphyseal mandibular cleft: Early simultaneous soft and hard tissue correction - A case report. J Craniomaxillofac Surg 2013;41:735-9.  Back to cited text no. 3
    
4.
Erdogan B, Karaca C, Gorgu M, Bozkurt MA. Incomplete midline cleft of lower lip. Eur J Plast Surg 1989;12:279-80.  Back to cited text no. 4
    
5.
Oostrom CA, Vermeij-Keers C, Gilbert PM, van der Meulen JC. Median cleft of the lower lip and mandible: Case reports, a new embryologic hypothesis, and subdivision. Plast Reconstr Surg 1996;97:313-20.  Back to cited text no. 5
    
6.
Armstrong AP, Waterhouse N. Tessier 30 median mandibular cleft: Case report and literature review. Br J Plast Surg 1996;49:536-8.  Back to cited text no. 6
    
7.
Hunter AG, Seaver LH, Stevenson RE. Limb-body wall defect. Is there a defensible hypothesis and can it explain all the associated anomalies? Am J Med Genet A 2011;155A:2045-59.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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