|Year : 2021 | Volume
| Issue : 2 | Page : 139-142
Congenital midline labial sinus of the upper lip – A review of literature
Anka Sharma1, Amit R Parate1, Vikrant O Kasat1, Anirudh Upmanyu2
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 Submission||08-Apr-2020|
|Date of Decision||20-Jan-2021|
|Date of Acceptance||10-Feb-2021|
|Date of Web Publication||22-Jun-2021|
Department of Oral Medicine and Radiology, Government Dental College and Hospital, Government Medical Campus, Panchakki Road, Aurangabad - 431 001, Maharashtra
Source of Support: None, Conflict of Interest: None
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 Sep 26];13:139-42. Available from: https://www.jorr.org/text.asp?2021/13/2/139/319001
| Introduction|| |
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. 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., Till date, only three cases have been reported in the Indian population.,, The exact etiology is not understood, but three hypotheses have been put forth as possible explanation.
- 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,,
- The Merging theory, states that the midline sinuses are formed secondary to aberrations in the normal mesodermal merging process
- The Fusion theory,, considers the failure of complete fusion between the frontonasal and the maxillary process as the etiology.
| Materials and Methods|| |
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|| |
Congenital midline labial sinus of the upper lip is a rare condition, first reported in 1879 by Lannelongue and Menard. In 2011, Aoki et al. proposed a classification for upper lip sinuses. They divided them as:
- Type 1: Midline sinus without accompanying anomalies
- Type 2: Midline sinus with accompanying anomalies
- 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., while the oldest was a 47-year-old female reported by Sen and Agir. Twenty-one cases were in the first decade of life. Out of 27 cases reported in the Asian subcontinent, only three were from India.,,,
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. 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, median sinus of the frenulum of the upper lip, and congenital hydronephrosis. One patient was reported with Pierre Robin syndrome.
The sinus tract was commonly diagnosed using a probe. It was confirmed using a sinogram or a gutta percha point and an intraoral periapical film. 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:
- The condition is most prevalent in the first decade of life
- Females are mostly affected
- Asian subcontinent has most of the reported cases
- Median cleft of the upper lip is the most common congenital anomaly associated with this condition
- Surgical excision is the mainstay of treatment.
| Conclusion|| |
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.
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
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2]