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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 4  |  Issue : 3  |  Page : 198-200

Median cleft lip: A case series from a rural cleft center


1 Department of Plastic Surgery, Shriram Hospital, Akola, Maharashtra, India
2 Department of Plastic Surgery, KLE University, Belgaum, Karnataka, India
3 Department of Plastic Surgery, VMMC, Safdarjung Hospital, New Delhi, India

Date of Web Publication21-Nov-2017

Correspondence Address:
N R Salampuria
Department of Plastic Surgery, Shriram Hospital, Aman Khan Plots, Shastri Nagar, Akola, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jclpca.jclpca_70_17

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  Abstract 

Median cleft lip is a midline craniofacial cleft through upper lip. The incidence of craniofacial clefts is about 1:1,000,000 of live births. It indicates the rarity of this anomaly. Hence, this series is being presented. From January 2010 to December 2017, eight cases of the median cleft of upper lip attended the cleft clinic based in rural India. All eight patients had partial midline cleft lip. One patient had abnormal soft tissue mass and one patient had hypertelorism with nasal deformity. All of them underwent surgical repair of median cleft lip using inverted-U incision along with repair of orbicularis oris muscle in the midline. The postoperative symmetry was achieved in 100% of patients with good reconstruction of the philtrum. The associated deformities were left to be managed in second stage. A series of eight cases of partial midline cleft lip has been presented with good surgical outcome.

Keywords: Median cleft lip, midline cleft lip, partial median cleft lip, Tessier's 0 cleft, Tessier's cleft


How to cite this article:
Salampuria N R, Agrawal MB, Chugh A. Median cleft lip: A case series from a rural cleft center. J Cleft Lip Palate Craniofac Anomal 2017;4, Suppl S1:198-200

How to cite this URL:
Salampuria N R, Agrawal MB, Chugh A. Median cleft lip: A case series from a rural cleft center. J Cleft Lip Palate Craniofac Anomal [serial online] 2017 [cited 2021 Sep 17];4, Suppl S1:198-200. Available from: https://www.jclpca.org/text.asp?2017/4/3/198/218887


  Introduction Top


Craniofacial clefts are a rare type of clefts. Incidence of median cleft of the lip or Tessier number 0 cleft is reported to be about 0.43% to 0.73% of the craniofacial cleft population, and it occurs in 1:1,000,000 live births.[1],[2] Median cleft lip is defined as congenital vertical craniofacial cleft through the center of the upper lip. These are classified as Tessier's 0 craniofacial clefts. Median cleft lip defects appear to occur when medial nasal prominences are deficient or absent.[3]

In a rural cleft care center, eight patients with midline cleft lip attended the clinic over a period of 8 years. Although it is a rare anomaly in literature, we encountered median cleft lip in significant number. Hence, this series is being presented.


  Case Report Top


Eight patients with median cleft lip reported to our rural health-care clinic from January 2010 to December 2017 [Figure 1], [Figure 2], [Figure 3], [Figure 4]. Out of eight patients, three of them had microform cleft with small notching and tenting of white line [Figure 2]. All of these cases had a partial median cleft lip and underwent surgical repair. Out of these patients, one patient had associated abnormal soft tissue mass, nasal deformity, and epibulbar dermoid in the right eye [Figure 4] and another had hypertelorism [Figure 3] with nasal deformity. In these two patients, secondary surgeries were deferred due to want of facilities in a rural setup. All patients underwent preoperative examination and available investigations to rule out any associated cardiac or neurological abnormalities. None of them had any cardiac or neurological abnormalities.
Figure 1: Median cleft lip: (a) preoperative and (b) postoperative

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Figure 2: A young female with median cleft lip: (a) preoperative and (b) postoperative

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Figure 3: Median cleft lip with soft tissue mass: (a) preoperative and (b) postoperative

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Figure 4: Tessier 0 cleft lip with hypertelorism and nasal deformity: (a) preoperative and (b) postoperative

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The surgical technique consisted of inverted-U incision in the upper lip. All the surgeries were performed under general anesthesia with oral intubation. An inverted-U incision was used over the mucocutaneous white roll. The thin vermilion and redundant mucosa along the cleft edge were excised. The orbicularis oris muscle was identified, separated from skin and mucosa, and repaired, followed by mucosa and skin closure. Care was taken to achieve symmetrical white line and good vermilion bulk on both sides. Special attention was paid to the reconstruction of vermilion tubercle in midline using the muscle from both sides. The vermilion tubercle was reconstructed equidistant from the lateral philtral points. The white line was matched and aligned well under magnification. Any dog ear in the region of columellar base was addressed primarily. No complication was observed in any case.


  Discussion Top


A case series of partial median cleft lip is being presented because of its rarity. Median cleft lip can be treated effectively with good esthetic outcomes even at a rural health-care clinic with limited resources.

This anomaly occurs during the 3rd week of gestation. At this age of gestation, bilateral thickening of the frontonasal process occurs and forms the medial and lateral nasal prominences that contribute to the formation of the nasal philtrum and upper lip. Fusion of the globular processes is responsible for the formation of the anterior part of the hard palate, the central dentoalveolar ridge, and the central upper lip. The fusion of these prominences results in the normal development of the upper lip.[4]

Demyer et al. classified three varieties of median clefts that included notch of the lip, median cleft extending to the columella, and a defect due to atrophy of midline facial structures.[5] Veau described two groups of syndromes associated with the median cleft lip, one with orbital hypertelorism and the second with hypotelorism.[6]

Millard and Williams classified median cleft of the lip as any congenital or vertical cleft through the center of the upper lip, regardless of the extent and divided into two groups. The first group consists of agenesis of the frontonasal process and the second group associated with various degrees of bifurcation of the nose, hypertelorism, and cranial malformations.[7]

Due to the wide spectrum of anomalies, various techniques are available for individual cases. In this case series of eight cases, we have used an inverted U-shaped incision technique and repaired orbicularis oris muscle in all the cases. Springer et al. used inverted V excision although it leads to a visible scar in the upper lip.[8] Millard recommended an inverted V excision and 90° angle in the excision, 2 mm above the mucocutaneous white roll on each side of the cleft which lengthened the skin in the center of Cupid's bow.[9] Although the type of incision might differ from case to case, proper muscle repair is key to success. In our all cases, good muscle repair was achieved with satisfactory esthetic outcomes with respect to the philtrum and Cupid's bow.


  Conclusion Top


Median cleft lip is a rare presentation. This case series of eight patients treated in a rural cleft care center in India highlights the diagnosis, associated conditions, and surgical technique used for the management of this anomaly. To achieve good esthetic results in this rare entity, repair of the orbicularis oris muscle is recommended.

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.

 
  References Top

1.
Urata MM, Kawamoto HK Jr., Median clefts of the upper lip: A review and surgical management of a minor manifestation. J Craniofac Surg 2003;14:749-55.  Back to cited text no. 1
    
2.
Koh KS, Kim do Y, Oh TS. Clinical features and management of a median cleft lip. Arch Plast Surg 2016;43:242-7.  Back to cited text no. 2
[PUBMED]    
3.
Johnston MC, Sulik KK. Some abnormal patterns of development in the craniofacial region. Birth Defects Orig Artic Ser 1979;15:23-42.  Back to cited text no. 3
[PUBMED]    
4.
DeMyer W. Median facial malformations and their implications for brain malformations. Birth Defects Orig Artic Ser 1975;11:155-81.  Back to cited text no. 4
[PUBMED]    
5.
Demyer W, Zeman W, Palmer CG. The face predicts the brain: Diagnostic significance of median facial anomalies for holoprosencephaly (arhinencephaly). Pediatrics 1964;34:256-63.  Back to cited text no. 5
    
6.
Veau V. Harelings of human seedlings on the stage 21-23 mm S. St.L. Z Anat. Develop. 1937;108:459.  Back to cited text no. 6
    
7.
Millard DR Jr., Williams S. Median lip clefts of the upper lip. Plast Reconstr Surg 1968;42:4-14.  Back to cited text no. 7
    
8.
Springer IN, Sprengel M, Terheyden H, Suhr MA, Härle F, Warnke PH, et al. Wedge excision: Treatment of choice in minimal median clefts of the upper lip. Plast Reconstr Surg 2004;114:812-4.  Back to cited text no. 8
    
9.
Millard DR Jr., editors. Median cleft lip with hypertelorism. In: Cleft Craft: The Evolution of Its Surgery. Vol. 2. Boston: Little, Brown; 1977. p. 727-68.  Back to cited text no. 9
    


    Figures

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


This article has been cited by
1 True Median Cleft Lipó15 Years of Review and Prevalence
Muhammad Izzuddin Hamzan,Wan Azman Wan Sulaiman
The Cleft Palate-Craniofacial Journal. 2020; 57(8): 1051
[Pubmed] | [DOI]



 

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