J Korean Assoc Oral Maxillofac Surg.  2023 Aug;49(4):223-227. 10.5125/jkaoms.2023.49.4.223.

Midline cleft of the upper lip associated with a microform unilateral cleft and a proboscis-like structure: a case report

• Adetayo AM
• Yusuf OA
• Ibeh CP
• Ukaegbu E
• Oginni F
• Adetayo MO

• KMID: 2545589
• DOI: http://doi.org/10.5125/jkaoms.2023.49.4.223

Abstract

A midline or median cleft lip is rare, and a midline cleft associated with a unilateral cleft and a proboscis-like structure is rarer still. We present a case managed at our center in which a 5-year-old male had a median cleft of the upper lip with an associated ‘proboscis’ and a microform unilateral cleft lip.

Keyword

Midline cleft; Atypical cleft; Cutaneous mass

Figure

• 

  Fig. 1 Preoperative clinical photographs showing the midline and microform cleft lip, bifid columella, and bifid frenula.

• 

  Fig. 2 A, B. Computerized tomography (CT) showing the nasal septum and falx cerebri in the patient with a true median cleft of the upper lip. These structures are absent in false median clefts.

• 

  Fig. 3 Schematic diagram showing the patient’s face and intraoperative procedures.

• 

  Fig. 4 Postoperative clinical photographs. A. Two-week follow-up. B. 24-week follow-up.

Reference

References

1. Millard DR Jr, Williams S. 1968; Median lip clefts of the upper lip. Plast Reconstr Surg. 42:4–14. DOI: 10.1097/00006534-196842010-00002. PMID: 5668093.

2. Ichida M, Komuro Y, Yanai A. 2009; Consideration of median cleft lip with frenulum labii superior. J Craniofac Surg. 20:1370–4. https://doi.org/10.1097/scs.0b013e3181ae42bf. DOI: 10.1097/SCS.0b013e3181ae42bf. PMID: 19816259.

3. de Boutray M, Beziat JL, Yachouh J, Bigorre M, Gleizal A, Captier G. 2016; Median cleft of the upper lip: a new classification to guide treatment decisions. J Craniomaxillofac Surg. 44:664–71. https://doi.org/10.1016/j.jcms.2016.02.012. DOI: 10.1016/j.jcms.2016.02.012. PMID: 27075944.

4. Tessier P. 1976; Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg. 4:69–92. https://doi.org/10.1016/s0301-0503(76)80013-6. DOI: 10.1016/S0301-0503(76)80013-6. PMID: 820824.

5. Fearon JA. 2008; Rare craniofacial clefts: a surgical classification. J Craniofac Surg. 19:110–2. https://doi.org/10.1097/scs.0b013e31815ca1ba. DOI: 10.1097/SCS.0b013e31815ca1ba. PMID: 18216674.

6. Tange I. 1965; [12 Cases of median cleft lip]. Jpn J Plast Reconstr Surg. 8:118–31. Japanese.

7. Nakamura J, Tomonari H, Goto S. 1985; True median cleft of the upper lip associated with three pedunculated club-shaped skin masses. Plast Reconstr Surg. 75:727–31. https://doi.org/10.1097/00006534-198505000-00022. DOI: 10.1097/00006534-198505000-00022. PMID: 3983280.

8. Casseler F, Radovich F, Ubiglia GP, Guidetti F. 1991; [A case report of a true median cleft of the upper lip with a pedunculated median skin-mucosa mass]. Minerva Stomatol. 40:505–8. Italian. PMID: 1753932.

9. Kün-Darbois JD, Chatellier A, Paré A, Caillot A, Ambroise B, Bénateau H, et al. 2018; Congenital midline upper lip sinuses: 3 rare cases. Cleft Palate Craniofac J. 55:292–5. https://doi.org/10.1177/1055665617723635. DOI: 10.1177/1055665617723635. PMID: 29351043.

10. Pai GS, Levkoff AH, Leithiser RE Jr. 1987; Median cleft of the upper lip associated with lipomas of the central nervous system and cutaneous polyps. Am J Med Genet. 26:921–4. https://doi.org/10.1002/ajmg.1320260421. DOI: 10.1002/ajmg.1320260421. PMID: 3591835.

11. Moore MH. 1996; Rare craniofacial clefts. J Craniofac Surg. 7:408–11. https://doi.org/10.1097/00001665-199611000-00003. DOI: 10.1097/00001665-199611000-00003. PMID: 10332258.

12. Jain S. 2017; True median cleft of upper lip: review of anatomic changes and surgical management. J Appl Dent Med Sci. 3:74–81.

13. Kundrat H. 1882. [Arhinencephalie als typische Art von Missbildung]. Leuschner & Lubensky;German.

14. DeMyer W, Zeman W, Palmer CG. 1964; The face predicts the brain: diagnostic significance of median facial anomalies for holoprosencephaly (arhinencephaly). Pediatrics. 34:256–63. DOI: 10.1542/peds.34.2.256. PMID: 14211086.

15. Patel NP, Tantri MD. 2010; Median cleft of the upper lip: a rare case. Cleft Palate Craniofac J. 47:642–4. https://doi.org/10.1597/09-065. DOI: 10.1597/09-065. PMID: 20500055.

16. Mazzola RF. 1976; Congenital malformations in the frontonasal area: their pathogenesis and classification. Clin Plast Surg. 3:573–609. PMID: 788998.

17. James O, Sabo VY, Adamson OO, Otoghile B, Adekunle AA, Adeyemo WL, et al. 2023; Presentation and management of atypical orofacial clefts: a single-institution experience for 13 year period. Cleft Palate Craniofac J. 60:133–41. https://doi.org/10.1177/10556656211055012. DOI: 10.1177/10556656211055012. PMID: 34787018.

18. Markus AF, Delaire J, Smith WP. 1992; Facial balance in cleft lip and palate. II. Cleft lip and palate and secondary deformities. Br J Oral Maxillofac Surg. 30:296–304. https://doi.org/10.1016/0266-4356(92)90179-m. DOI: 10.1016/0266-4356(92)90179-M. PMID: 1390561.

19. Kolker AR, Sailon AM, Meara JG, Holmes AD. 2015; Midline cleft lip and bifid nose deformity: description, classification, and treatment. J Craniofac Surg. 26:2304–8. https://doi.org/10.1097/scs.0000000000002229. DOI: 10.1097/SCS.0000000000002229. PMID: 26594965.

20. Jian XC, Zheng L, Xu P, Liu DY. 2014; Median cleft of the upper lip associated with a mass: a rare case. J Craniomaxillofac Surg. 42:1557–61. https://doi.org/10.1016/j.jcms.2014.01.003. DOI: 10.1016/j.jcms.2014.01.003. PMID: 24507931.