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 Table of Contents  
REVIEW ARTICLE
Year : 2021  |  Volume : 13  |  Issue : 2  |  Page : 139-142

Congenital midline labial sinus of the upper lip – A review of literature


1 Department of Oral Medicine and Radiology, Government Dental College and Hospital, Aurangabad, Maharashtra, India
2 Department of Dentistry, Jag Pravesh Chandra Hospital, New Delhi, India

Date of Submission08-Apr-2020
Date of Decision20-Jan-2021
Date of Acceptance10-Feb-2021
Date of Web Publication22-Jun-2021

Correspondence Address:
Anka Sharma
Department of Oral Medicine and Radiology, Government Dental College and Hospital, Government Medical Campus, Panchakki Road, Aurangabad - 431 001, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jorr.jorr_10_20

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  Abstract 


Congenital midline labial sinus is a rare condition known to affect both the upper and the lower lip. This anomaly has been reported in both genders, but with a slightly increased predilection in females. It can occur either in isolation or in association with a wide array of congenital malformations such as cleft lip, cleft palate, Van der Woude syndrome, median lip pit, and brachydactylia. Usually, the patient presents with swelling of the upper lip with or without associated discharge. A history of recurrent cellulitis of the lip is almost always present. In this manuscript, a review of the literature (spanning across almost six decades) has been attempted for this rare condition. Search engines such as PubMed, Medscape, and Google Scholar were searched for keywords such as “recurrent cellulitis of the upper lip,” “sinus of the upper lip,” “fistula of the upper lip,” and “congenital midline labial sinus.” All the available full text in the English language was compiled and thoroughly scrutinized. The observations such as age, sex, clinical feature, associated anomaly, and other relevant information were noted and tabulated in the chronological order.

Keywords: Congenital midline labial sinus of the upper lip, recurrent cellulitis of the upper lip, upper lip fistula


How to cite this article:
Sharma A, Parate AR, Kasat VO, Upmanyu A. Congenital midline labial sinus of the upper lip – A review of literature. J Oral Res Rev 2021;13:139-42

How to cite this URL:
Sharma A, Parate AR, Kasat VO, Upmanyu A. Congenital midline labial sinus of the upper lip – A review of literature. J Oral Res Rev [serial online] 2021 [cited 2021 Dec 5];13:139-42. Available from: https://www.jorr.org/text.asp?2021/13/2/139/319001




  Introduction Top


Congenital midline labial sinus of the lip is a rare condition. This anomaly occurs either in isolation or in association with other congenital malformations such as cleft lip, cleft palate, and Van der Woude syndrome.[1] It can occur in either upper or lower lip. In the White population, the estimated prevalence of the lower lip sinuses is about 0.00001%, but upper sinuses are even rarer.[2],[3] Till date, only three cases have been reported in the Indian population.[4],[5],[6] The exact etiology is not understood, but three hypotheses have been put forth as possible explanation.

  1. The Invagination theory suggests that the upper lip sinuses are formed by the failure of ectodermal invagination of nasal placodes during the formation of frontonasal process[7],[8],[9]
  2. The Merging theory[10],[11] states that the midline sinuses are formed secondary to aberrations in the normal mesodermal merging process
  3. The Fusion theory[12],[13],[14] considers the failure of complete fusion between the frontonasal and the maxillary process as the etiology.



  Materials and Methods Top


The literature was reviewed from 1965 to 2019 exclusively for case reports and series using the keywords: recurrent cellulitis of upper lip, sinus of upper lip, fistula of upper lip, and congenital midline labial sinus of upper lip. Search engines such as PubMed Central, Google Scholar, and Embase were used by applying the Boolean operator “AND” or “OR.” A total of 1084 articles appeared in the search. Based on the exclusion criteria (articles in language other than English, case reports on the lateral labial sinus and/or lower lip sinus, review articles, and grey literature), 1043 articles were excluded. One article was rejected as full text was not available. Thus, only 40 articles met the inclusion criteria. All the available full text in the English language was compiled and thoroughly scrutinized. The observations such as age, sex, clinical feature, associated anomaly, and other relevant information were noted and tabulated in the chronological order [Table 1] and [Table 2].
Table 1: Summary of the previously reported cases in the literature (1965-1999)

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Table 2: Summary of the previously reported cases in the literature (2000-2019)

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  Discussion Top


Congenital midline labial sinus of the upper lip is a rare condition, first reported in 1879 by Lannelongue and Menard.[15] In 2011, Aoki et al.[7] proposed a classification for upper lip sinuses. They divided them as:

  1. Type 1: Midline sinus without accompanying anomalies
  2. Type 2: Midline sinus with accompanying anomalies
  3. Type 3: Lateral sinus with or without accompanying abnormalities.


Of the 40 cases reported, 25 cases were females and 15 were males. The youngest case, a 7-month-old male, was reported by Watanabe et al.,[20] while the oldest was a 47-year-old female reported by Sen and Agir.[32] Twenty-one cases were in the first decade of life. Out of 27 cases reported in the Asian subcontinent, only three were from India.[4],[5],[6],[38]

Nineteen cases were asymptomatic, while 15 cases presented with a history of discharge (clear, mucus, or pus). Recurrent cellulitis or intermittent swelling was noted as the presenting feature in 14 cases. In 35 cases, the sinus tract was found on clinical examination, whereas fistulas were found in just five cases. Only in one case, a sinus tract ending in a fistula, as well as a cyst, was noted.[13] Twenty-six cases were not associated with any congenital abnormality, while 14 showed an association with one or other kinds of abnormality. Median cleft of the lip was the most reported associated anomaly. Other reported anomalies were partial midline clefting of maxillary alveolar process, bifid uvula, notching of posterior nasal spine,[31] median sinus of the frenulum of the upper lip,[13] and congenital hydronephrosis.[11] One patient was reported with Pierre Robin syndrome.[11]

The sinus tract was commonly diagnosed using a probe. It was confirmed using a sinogram[5] or a gutta percha point and an intraoral periapical film.[6] All the patients were managed by surgical excision of the sinus tract. The excised specimen was almost invariably a sinus tract lined by stratified squamous epithelium with sebaceous glands. Recurrence was not reported in any of the operated cases.

The findings of this review article can be summarized as follows:

  1. The condition is most prevalent in the first decade of life
  2. Females are mostly affected
  3. Asian subcontinent has most of the reported cases
  4. Median cleft of the upper lip is the most common congenital anomaly associated with this condition
  5. Surgical excision is the mainstay of treatment.



  Conclusion Top


The diagnosis of congenital midline sinus of the upper lip is difficult not only because of its inconspicuous appearance but also because of a dearth of knowledge, regarding this unusual condition. Patients with a history of recurrent cellulitis or intermittent swelling of the lip should be carefully examined for evidence of a pit or a draining sinus and any congenital abnormality must be ruled out. In the suspected cases, the sinus tract can be detected using a probe. Antibiotic coverage can simply suppress the infection and inflammation for some period of time, causing recurrence of symptoms at a later stage. Early and prompt diagnosis followed by surgical excision of the sinus tract remains the mainstay treatment.

Acknowledgment

The authors extend their gratitude to Dr. Ashita Kalaskar, Associate Professor, Department of Oral Medicine and Radiology, for her valuable inputs.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Fok D, Kua E. Congenital midline sinus of the upper lip. Singapore Med J 2014;56:107-9.  Back to cited text no. 1
    
2.
Coccia CT, Bixler D. Cleft lip, cleft palate, and congenital fistulas of the lower lip. Report of a familial occurrence. Oral Surg Oral Med Oral Pathol 1967;24:246-9.  Back to cited text no. 2
    
3.
Cervenka J, Gorlin RJ, Anderson VE. The syndrome of pits of the lower lip and cleft lip and/or palate. Genetic considerations. Am J Hum Genet 1967;19:416-32.  Back to cited text no. 3
    
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Bakshi S, Shankar M. Midline upper lip sinus: A case report and review of literature. Oral Maxillofac Surg 2015;19:217-9.  Back to cited text no. 4
    
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Chowdhary F, Chowdhary A, Chowdhary Z, Chowdhary A. Midline congenital upper lip sinus: A rare case. Indian J Otolaryngol Head Neck Surg 2019;71:553-7.  Back to cited text no. 5
    
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Sharma A, Kumbhare SP, Kalaskar A. Congenital midline labial sinus of the upper lip. SRM J Res Dent Sci 2019;10:53-6.  Back to cited text no. 6
  [Full text]  
7.
Aoki M, Sakamoto Y. Classification of congenital midline upper lip sinuses: A case report and review of the literature. Cleft Palate Craniof J 2012;51:154-7.  Back to cited text no. 7
    
8.
Miller CJ, Smith JM. Midline sinus of the upper lip and a theory concerning etiology. Plast Reconstr Surg 1980;65:674-5.  Back to cited text no. 8
    
9.
Eppley BL, Sadove AM, Goldenberg J. Philtral fistula in median cleft lip: Cause and effect or coincidence? Ann Plast Surg 1992;29:263-5.  Back to cited text no. 9
    
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Bartels RJ, Howard RC. Congenital midline sinus of the upper lip. Case report. Plast Reconstr Surg 1973;52:665-8.  Back to cited text no. 14
    
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Lannelongue OM, Menard V. Affection congenitales. Asselin et Houzeau, Libraries de Faculte de Medicine; 1891.  Back to cited text no. 15
    
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Tange I. Median cleft of upper lip and its related malformations: Report of 12 cases. Keisei Geka 1965;8:118-31.  Back to cited text no. 17
    
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Menendez OR. Congenital pit of the upper lip. Oral Surg Oral Med Oral Pathol 1969;27:441-4.  Back to cited text no. 18
    
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Watanabe T, Katsuki T, Tashiro H. Three cases of congenital dimples of the upper tip. Jpn J C P A 1981:6:50-4.  Back to cited text no. 20
    
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26.
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28.
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37.
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  [Table 1], [Table 2]



 

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