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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 1  |  Page : 73-75

Management of median cleft lip


1 Center for Cleft Services, St. Joseph's Hospital; Department of Oral and Maxillofacial Surgery, Farooqia Dental College and Hospital, Mysore, Karnataka, India
2 Center for Cleft Services, St. Joseph's Hospital, Mysore, Karnataka; Kannur Dental College, Kannur, Kerala, India
3 Center for Cleft Services, St. Joseph's Hospital, Mysore, Karnataka, India

Date of Submission02-Oct-2020
Date of Acceptance13-Nov-2020
Date of Web Publication13-Jan-2021

Correspondence Address:
Dr. K Harish
Center for Cleft Services, St. Joseph's Hospital, Mysore, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jclpca.jclpca_34_20

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  Abstract 


Median cleft or midline cleft is a congenital vertical cleft through the center of the upper lip. Median facial anomalies are broadly divided into those with a deficiency of tissue and those with normal or excessive amount of tissue. The main objective of concern during surgery is both functional and esthetics.

Keywords: Median cleft lip, midline cleft, rare cleft anomalies, triangular flap


How to cite this article:
Prasad S M, Nair P A, Anushree N, Harish K. Management of median cleft lip. J Cleft Lip Palate Craniofac Anomal 2021;8:73-5

How to cite this URL:
Prasad S M, Nair P A, Anushree N, Harish K. Management of median cleft lip. J Cleft Lip Palate Craniofac Anomal [serial online] 2021 [cited 2021 Jan 26];8:73-5. Available from: https://www.jclpca.org/text.asp?2021/8/1/73/306774




  Introduction Top


Median cleft or midline cleft, first described by Bechard in 1823, is a congenital vertical cleft through the center of the upper lip. The exact cause is unknown, but this rare anomaly occurs with an incidence of 0.43%–0.73% in the cleft population and is frequently associated with hypertelorism, midline cleft, and bifid nose.[1] Embryologically, the failure of the mesoderm migration or incomplete merging of the naso-medial prominence which forms the intermaxillary segment is the reason for the anomaly. The incidence ratio is about 1:1,000,000.[2]


  Case Report Top


A 17-year-old male patient reported to our center with midline cleft of the upper lip and philtrum, extending one-third the distance of the base of the nose. Flattening and widening of the nose was noted [Figure 1]. There was no family history of cleft lip or facial deformity; consanguineous marriage; and maternal drug, alcohol, or medicine use. General and physical examination revealed no systemic abnormalities. Intraoral examination revealed a normal central alveolar segment with midline diastema; the primary and secondary palates were intact but high arched. Movement of the soft palate was normal. The patient underwent thorough routine investigations; computed tomography (CT) scan of the head was normal and evaluated by a physician. After obtaining the consent, the patient was taken for surgery.
Figure 1: Preoperative photograph

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Surgical technique

The procedure was performed under general anesthesia. Surgical markings were outlined in an inverted V fashion after injecting lignocaine with adrenaline [Figure 2a and b]. Incision was made along the markings. The orbicularis oris muscle was dissected superficially and in deeper planes. On either side of the midline, the muscle fibers attached abnormally into anterior nasal spine and to base of the nose was dissected and rotated downwards to achieve the adequate length of the lip. The labial frenum was released to get further lengthening and rotation of the lip. The median fibrotic band was excised completely, and the free end of the muscle was approximated and closed with V-Y technique. The main goal of the surgery was to achieve adequate lip length [Figure 3].
Figure 2: (a) Intraoperative surgical marking photograph. (b) Intraoperative photograph after final closure

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Figure 3: 10 days postoperative photograph

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  Discussion Top


In 1971, Pinto and Gloria from Mumbai divided median cleft into two groups. The first type is characterized by the gross deficiency of tissue development from the median nasal process and the second type is a result of the failure of fusion which may vary from diastema to median lip muscle gaps to median cleft associated with broadening and duplication of the philtrum, columella, nose tip, and nasal septum.[3]

Two major categories of dysplasia of fronto-nasal process associated with cleft lip have been described. They are:

  1. DeMyer sequence: Fronto-nasal deformity associated with hypotelorism, holoprosencephaly, and facial deformity, which can range from cyclopia to midline facial cleft with premaxillary agenesis
  2. Median cleft face syndrome: Median cleft associated with nasal deformity, hypertelorism, and corpus colloquial agenesis (Millard and Williams, 1968; Weimer et al., 1978).[1]


Ortiz-Monasterio reviewed 6500 craniofacial clefts over a 20-year period. In their study, out of 345 atypical clefts 92 (66.7%) cases had median cleft corresponding to Tessier 0 classification.

Classification

There is no precise classification system for median cleft. Median facial anomalies are broadly divided into those with a deficiency of tissue and those with normal or excessive amount of tissue. DeMyer and Zeman[4] initiated the well-known classification of the midline clefts based on the morphology of the brain. A modification in this classification was put forth by Cohen[5] [Table 1] and subsequently by Elias, Wilson, and Kawamoto [Table 2].
Table 1: Holoprosencephaly (DeMyer, Cohen et al.)

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Table 2: Median cerebrofacial dysmorphogenesis (after Kavamoto and Patel)

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The mechanism of cleft formation appears to involve either failure of mesoderm migration or inadequate consolidation of median nasal processes, which arises from the fronto-nasal process. The DeMyer sequence is associated with holoprosencephaly, due to agenesis of the midline facial elements, premaxilla, nasal bone, and nasal septum. Orbital hypotelorism is present because of agenesis of the ethmoid bone. Amentia is always present, and these patients rarely survive infancy. The second classification system is the median cleft face syndrome. Here, the patient displays mild clefting of the nose and the lip with orbital hypotelorism. Intelligence and longevity is not affected.[6],[7],[8]

This article intends to address the patho-morphogenesis, classification, and surgical technique used to treat a rare entity and the subsequent follow-up for other multidisciplinary procedures such as speech therapy and orthodontic treatment for correction of midline diastema. The main objective of concern during surgery is both functional and esthetics. Several techniques have been described, such as Francesconi triangular flap,[9] Pinto and Goleria's modified Z-plasty,[7] and V excision technique. Repair of an existing shortened or widened columella may be incorporated into the initial incision design.[10] Adequate width of the alar base, sufficient length of the lip and columella, is achieved by downward rotation of the flap. Functioning of the orbicularis oris muscle is achieved by proper muscle suturing. The incision design should also address the release of the labial frenum, which helps in achieving lip length and reorient it from the ectopic position to proper position. The associated nasal clefting should be treated at the same time.

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.

Acknowledgment

We would like to thank DCKH (Germany) and ABMSS (India) for funding cleft surgeries.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Apesos J, Anigian GM. Median cleft of the lip: Its signi?cance and surgical repair. Cleft Palate Craniofac J 1993;30:94-6.  Back to cited text no. 1
    
2.
Kawamoto HK, Patel PK. Atypical facial clefts. In: Bentz M, editor. Pediatric Plastic Surgery. Stamford: Appleton and Lange; 1998.  Back to cited text no. 2
    
3.
Millard DR. Median clefts of the upper lip. In: Millard DR, editor. Cleft Craft. Boston, MA: Little, Brown and Company; 1976. p. 729.  Back to cited text no. 3
    
4.
DeMyer W, Zeman W. Alobar holoprosencephaly (arrhinencephaly) with median cleft lip and palate: Clinical electroencephalographic and sonologic considerations. Conf Neurol 1963;23:1-36.  Back to cited text no. 4
    
5.
Cohen MM Jr. An update on the holoprosencephalic disorders. J Pediatr 1982;101:865-9.  Back to cited text no. 5
    
6.
DeMyer W, Zeman W, Palmar CG. The face depicts the brain: Diagnostic significance of median facial anomalies for holoprosencephaly (arrhinencephaly) with median cleft lip and palate. Pediatrics 1964;34:256-63.  Back to cited text no. 6
    
7.
Pinto CJ, Goleria KS. The median cleft lip. In: Hueston JT, editor. Transactions of the 5th International Congress of Plastic and Reconstructive Surgery. Melbourne: Butterworth's; 1971. p. 206-13.  Back to cited text no. 7
    
8.
Millard DR. Median cleft lip with hypotelorism. In: Millard DR, editor. Cleft Craft. Boston: Little, Brown and Company; 1976. p. 735.  Back to cited text no. 8
    
9.
Francesconi G, Fortunato G. Median dysraphia of the face. Plast Reconstr Surg 1969;43:481-91.  Back to cited text no. 9
    
10.
Millard DR Jr., Williams S. Median lip clefts of the upper lip. Plast Reconstr Surg 1968;42:4-14.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2]



 

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