Congenital double lip associated to hemangiomas:
report of a case

COSTA-HANEMANN JA, TOSTES-OLIVEIRA D, FERNANDES-GOMES M, JAMES DA SILVADOS ANJOS M, SANT'ANA E. CONGENITAL DOUBLE LIP ASSOCIATED TO HEMANGIOMAS: REPORT OF A CASE. MED ORAL 2004;9:155-8.

SUMMARY

Double lip is a developmental abnormality which may occur either isolated or as a component of Ascher's syndrome. A case of congenital double lip associated to hemangiomas and enlargement of the thyroid is reported. The oral and facial hemangiomas were excised under local anesthesia. Surgical reconstruction of the upper lip was done in order to reduce the interference with speech and mastication. The recognition and treatment of these abnormalities are discussed.

Key words: double lip, Ascher's syndrome, hemangioma.

INTRODUCCION

The occurrence of double lip has been described as uncommon congenital or acquired oral anomaly without gender or race predilection (1-5). Frequently, this development abnormality is reported as a component of Ascher's syndrome, which is usually characterized by concurrent presence of double lip, blepharocalasis and nontoxic thyroid enlargement (6-8). It has been suggested that double lip may be present as an isolated anomaly acquired with oral habits or after trauma to the lip (9-10). The association of congenital double lip with other abnormalities as bifid uvula (1), cleft palate (11) and cheilitis glandularis has been described (2). Hemangiomas are common congenital vascular lesions in the head and neck region, generally considered to be hamartomatous rather than truly neoplasms (12). They are usually solitary although multiple lesions may occur, sometimes as part of a syndrome (13). Double lip and hemangiomas may each occur as isolated anomalies. We reported an unique case of the concurrent congenital double lip, oral and facial hemangiomas and thyroid enlargement. The recognition and surgical treatment of these abnormalities are discussed.

CASE REPORT

A 65-year-old man was evaluated at Bauru Dentistry School for dark lesion in the lower lip, which the patient stated that had a slowly growing in the last years. Clinical examination revealed the presence of a slightly elevated reddish-purple mass, with approximately 2cm in diameter, extending from vermilion border to inner surface of the lower labial mucosa. A similar well circumscribed, asymptomatic and sessile purple lesion was detected in the facial area near to the right subauricular region. Moreover, it was noted that the upper lip presented an additional fold of redundant mucous membrane with midline constriction that became quite evident when the patient talked or smiled (Fig. 1).

Fig. 1. Características clínicas del doble labio superior y hemangioma en labio inferior.
Clinical features of upper double lip and hemangioma of lower lip.

According to the patient, the double lip had been present as long as he could remember. Family history did not reveal any evidence for hereditary or congenital malformations. The thyroid hormones levels were normal but an enlargement of this gland, without other pathologic alterations, was detected by computerized tomography. None eye abnormality as blepharochalasis was detected. The clinical diagnosis was congenital upper double lip associated to hemangiomas and enlargement of thyroid. The oral and facial hemangiomas were surgically excised under local anesthesia and the tissues were submitted to histopathological examination. Microscopic appearance of the both lesions showed larger vascular spaces lined by endothelium and containing red blood cells. The histopathological diagnosis were cavernous hemangiomas. The patient was informed that the removal of a double lip for cosmetic purpose was not indicated but he stated that the congenital anomaly was interfering with speech and mastication. Subsequently, the surgical treatment of double lip was done. Under local anesthesia, the redundant mucosal tissue was removed through transverse elliptical incisions, without resected the maxillary labial frenum avoiding to modify the lip form. The histopathological analysis of the specimens excised showed sections of labial mucosa covering numerous hypertrophied mucous glands. A six-months follow up showed satisfactory functional and aesthetic results in the lip, without evidence of the recurrence.

DISCUSSION

The occurrence of congenital anomalies in the lip, as in the present case, has been described associated with syndromes. Concurrent upper double lip and blepharocalasis are components of Ascher's syndrome. Nontoxic thyroid enlargement is another abnormality that has been described as part of this syndrome (14). It was quite interesting to observe that in our case report the thyroid gland was slightly enlarged.

The incidence of acquired or congenital double lip is probably much higher than reported in the literature (9). The congenital form has been associated to the persistence of the horizontal sulcus between the pars glabrosa and the pars villosa of the lip (5). Although the enlargement of the lip may exist at birth, sometimes, it usually becomes apparent after the eruption of the teeth (3,5). Moreover, it has been suggested that original double lip may be enhanced by a reactive component subsequently to a "sucking-in" of the tissue between the teeth or maloccluding dentures (1,10).

The differential diagnosis of the double lip should include other types of chronic enlargements of the lip as hemangioma, lymphangioma, angioedema, cheilitis glandularis and cheilitis granulomatosis (3-5). Such lesions, frequently, are associated with an uniformly enlarged lip without a midline constriction dividing the lip as in the case reported.

Surgery is the usual treatment for this congenital abnormality (15) and becomes necessary if it interferes with speech or mastication or it is of esthetic concern to the patient (2,3). In the present case, the patient had double lip during 65 years and he did not worry about it before. Then, the option for surgical reconstruction of the upper lip was done in order to reduce the interference with speech and mastication. Some different surgical techniques have been described for treatment of double lip. The patient is operated under general or local anesthesia and the redundant mucosal tissue is removed using transverse elliptical incisions (11,16) or W-plasty (17,18). In the case reported double lip was excised by elliptical incision and there were no postoperative complications.

Numerous treatment modalities as injections of corticosteroids and sclerosants (19), surgery (12,20), cryotherapy (21) and laser therapy (22) have been recommended for the management of the head and neck hemangiomas. However, the choice of the treatment depends of the several factors as size, location, degree of invasion and anatomic structures in the region of the lesion (23). The hemangiomas in the case reported were present at birth, never involuted and had a growing in the last years. Most of the superficial hemangiomas as showed in the present case are treated by surgical excision without risk of hemorrhage or of causing cosmetic/functional deficiency (20). The professional's ability and preoperative surgical planning including size and depth delimitation of the lesion are important factors for success of surgical therapy as showed in the present case.

REFERENCES

1. Barnett ML, Bosshardt LL, Morgan F. Double lip and double lip with blepharocalasis (Ascher's syndrome). Oral Surg Oral Med Oral Pathol 1972; 34:727-33.         [ Links ]

2. Cohen DM, Green JG, Diekmann SL. Concurrent anomalies: cheilitis glandularis and double lip. Oral Surg Oral Med Oral Pathol 1988; 66: 397-9.         [ Links ]

3. Kenny KF, Hreha JP, Dent CD. Bilateral redundant mucosal tissue of the upper lip. J Am Dent Assoc 1990;120:193-4.         [ Links ]

4. Greenfield MF, Icochea R, Hoffman C, Gropper C. Double lip: an unusual presentation. Cutis 2000;66:253-6.         [ Links ]

5. Alkan A, Metin M. Maxillary double lip: Report of two cases. J Oral Sci 2001;4369-72.         [ Links ]

6. Gorlin RJ, Cohen MM, Levin LS, eds. Syndromes of the head and neck. 3rd Ed. New York: Oxford University Press; 1990. p. 420-1.         [ Links ]

7. Papanayotou PH, Hatziotis JC. Ascher's syndrome- report of a case. Oral Surg Oral Med Oral Pathol 1973;35:467-71.         [ Links ]

8. Gomez-Duaso AJ, Seoane J, Vazquez-Garcia J, Arjona C. Ascher syndrome: report of two cases. J Oral Maxillofac Surg 1997;55:88-90.         [ Links ]

9. Rintala AE. Congenital double lip and Ascher syndrome:II. Relationship to the lower lip sinus syndrome. Br J Plast Surg 1981;34:31-4.         [ Links ]

10. Swerdloff G. Double lip- report of a case. Oral Surg Oral Med Oral Pathol 1960;15:627-9.         [ Links ]

11. Calnan J. Congenital double lip: Record of a case with a note on the embryology. Br J Plast Surg 1952;5:197-202.         [ Links ]

12. Ogunsalu C, Fray D, Lewis A. Surgery combined with copper wire implantation in the management of cavernous orofacial haemangiomas. Aust Dent J 2000;45:55-60.         [ Links ]

13. Kaplan I, Mass E, Littner M. A study of small superficial capillary hemangiomas on the lips in children. Pediatr Dent 1998;20:188-91.         [ Links ]

14. Kara IG, Kara CO. Ascher syndrome. Otolaryngol Head Neck Surg 2001; 124:236-7.         [ Links ]

15. Schwimmer A, Dym H, Barr C. Surgical repair of a double lip. J Am Dent Assoc 1979;99:993-4.         [ Links ]

16. Reddy KA, Rao AK. Congenital double lip: A review of seven cases. Plast Reconstr Surg 1989;84:420-3.         [ Links ]

17. Guerrero-Santos J, Altamirano JT. The use of W-plasty for the correction of double lip deformity. Plast Reconstr Surg 1967;39:478-81.         [ Links ]

18. Benmeir P, Weinberg A, Neuman A, Eldad A, Lusthaus S, Rotem M et al. Congenital double lip: Report of five cases and a review of the literature. Ann Plast Surg 1992;28:180-2.         [ Links ]

19. Muto T, Kinehara M, Takahara M, Sato K. Therapeutic embolization of oral hemangiomas with absolute ethanol. J Oral Maxillofac Surg 1990;48:85-8.         [ Links ]

20. Salins PC, Kumar S, Rao CB. Management of large vascular lesions of the lip: case reports. Int J Oral Maxillofac Surg 1997;26:45-8.         [ Links ]

21. Tal H. Cryosurgical treatment of hemangiomas of the lip. Oral Surg Oral Med Oral Pathol 1992;73:650-4.         [ Links ]

22. Sexton J, O'Hare D. Simplified treatment of vascular lesions using the argon laser. J Oral Maxillofac Surg 1993;51:12-6.         [ Links ]

23. Gampper TJ, Morgan RF. Vascular anomalies: hemangiomas. Plast Reconst Surg 2002;110:572-85.         [ Links ]