Journal of Cranio-Maxillo-Facial Surgery xxx (2014) 1e5

Contents lists available at ScienceDirect

Journal of Cranio-Maxillo-Facial Surgery journal homepage: www.jcmfs.com

Case report

Median cleft of the upper lip associated with a mass: A rare case Xin-chun Jian a, b, *, Lian Zheng a, b, Pu Xu a, De-yu Liu a a b

Department of Oral and Maxillofacial Surgery, Affiliated Haikou Hospital, Central South University, Haikou 520208, Hainan, People’s Republic of China Department of Oral and Maxillofacial Surgery, Xiangya Hospital, Central South University, Changsha 410008, Hunan, People’s Republic of China

a r t i c l e i n f o

a b s t r a c t

Article history: Paper received 5 January 2013 Accepted 3 January 2014

Median cleft lip is a midline vertical cleft through the upper lip. This is a very rare anomaly described in the literature. Median cleft lip is caused by the failure of fusion of the medial nasal prominences. In this case report, a 4-month-old boy with a median cleft associated with a mass of the upper lip is presented. The patient has no other anomalies of the nose or alveolus. The patient has normotelorism. A Z-plasty technique was used on the skin of the base of the columella. A vertical excision of the cleft with muscle approximation was performed on the white roll and the wet-dry border of either side of the defect of the upper lip. Postoperatively, the patient had a satisfactory result. The incisive scars were not visible. Cupid’s bow was appropriately aligned, and the height of the upper lip was equal on both sides. Ó 2014 Published by Elsevier Ltd on behalf of European Association for Cranio-Maxillo-Facial Surgery.

Keywords: Median cleft lip Teratoid polyp Upper lip

1. Introduction Median cleft lip is a midline vertical cleft through the upper lip. Within cranio-maxillofacial anomalies (Hou et al., 2011; Laure et al., 2010; Mohamed et al., 2011), this is a very rare anomaly described in the literature (Ghildiyal et al., 2003; Kawamoto, 1990). In 1935, Davis (Davis, 1935) found five oblique clefts of the face and four median clefts of the lip in a group of 945 patients. Fogh-Andersen’s (FoghAndersen,1965) study included 3988 cleft patients seen over 30 years of which 15 had a median cleft of the upper lip. The exact incidence is not known, but estimates range from 1.4 to 4.9 per 100,000 live births (Kawamoto, 1990). At the 1976 Interdisciplinary Workshop Conference in Chicago, Tessier (Tessier, 1976) presented a comprehensive classification of craniofacial clefts based on his personal experience with 336 patients. In the Tessier 0 through 14 cleft, the location of the median or midline cleft of the upper lip has a spectrum of dysmorphic gradation. This varied from a simple central vermilion notch to a wide cleft accompanied by a bifid nose and hypertelorbitism. Median cleft lip is caused by the failure of fusion of the medial nasal prominences (Mazzola, 1976). In this case report, a 4-month-old boy with a median cleft associated with a mass of the upper lip is presented.

2. Case report A 4-month-old boy presented to the Xiangya Hospital of the Central South University with median cleft of the upper lip * Corresponding author. Department of Oral and Maxillofacial Surgery, Xiangya Hospital, Central South University, Changsha 410008, Hunan, People’s Republic of China. Tel.: þ86 (0) 731 84327493. E-mail address: [email protected] (X.-c. Jian).

associated with a mass. The pregnancy was full-term with no complications. The mother did not have any diseases during the pregnancy. His mother did not smoke or drink alcohol, nor was she exposed to any environmental risk factors during the pregnancy. The patient was the first-born male to healthy parents, delivered at 39 weeks gestation via spontaneous vaginal delivery with outlet forceps. The patient’s birth weight was normal and presented with no other anomalies. There was no family history of clefts. On examination, there was a median cleft of the upper lip. There was a mass in median cleft between the base of the columella and the vermilion of the upper lip, this small mass was actually a ‘blob-like’ piece of tissue between the cleft hemilabellum of the upper lip (Fig. 1A and Fig. 2A). The vermilion was separated in the midline, but the total length of the vermilion and the vertical height of the upper lip were normal. There were no bony abnormalities, and the alveolus was normal. The nose and the intercanthal distance were normal (Fig. 1A). Hematological and biochemical investigations revealed normal values, and hormonal studies were also within normal limits. Magnetic resonance imaging indicated normal position of the carotid and vertebral vessels. The median cleft lip was repaired using the following steps. First, on the skin of the base of two nostril, the point 4 and the point 5 were marked, on the skin of the dry border of either side of the defect of the upper lip, the point 1 and point 6 were marked; then, a vertical line linked the point 4 with point 1, another vertical line linked the point 5 with point 6. The distance of the point 4 to the point 1 equaled to the distance of the point 5 to the point 6. The distance of the point 4 to the point 1 or the distance of the point 5 to the point 6 is actually the vertical height of two philtral columella. On the skin of the base of the columella, a point 3 was marked, on the dry vermilion of either side of the defect of the upper lip, the

1010-5182/$ e see front matter Ó 2014 Published by Elsevier Ltd on behalf of European Association for Cranio-Maxillo-Facial Surgery. http://dx.doi.org/10.1016/j.jcms.2014.01.003

Please cite this article in press as: Jian X-c, et al., Median cleft of the upper lip associated with a mass: A rare case, Journal of Cranio-MaxilloFacial Surgery (2014), http://dx.doi.org/10.1016/j.jcms.2014.01.003

2

X. Jian et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2014) 1e5

Fig. 1. A 4-month-old boy with a median cleft lip with a mass in the center of the lip separating the vermilion. A. Preoperative frontal view; B. Postoperative frontal view.

Fig. 2. Surgical plan. A. Preoperative design; B. Postoperative condition.

point 2 and the point 20 were marked, length of the point 3 to point 2 equaled to the length of point 3 to point 20 . The two side limbs were marked on the two side of the point 3 to point 2 and point 3 to point 20 , the three lines of the Z must be equal in length. The angles of Z two flaps were 60 (Fig. 2A). These incisive lines were marked with methylene blue. Next, a Z-plasty incision was made, the tissue in the midline and mass were excised via a vertical incision. The skin of the upper lip was dissected from the orbicularis oris muscle on both sides of the defect via the same incision. Next, a 5-0 Vicryl was used to close the muscle layer. The positions of the Z-plasty flaps of the skin of the base of the columella of the upper lip were exchanged and were first closed by reapproximation, then the white roll and the wetedry border were sutured (Fig. 2B and Fig. 1B). The surgical pathology report was “a teratoid polyp with skin appendages, fibrous connective tissue, skeletal muscle, bone”. No evidence of malignancy noted. Postoperatively, the patient had a satisfactory result. The incisive scars were not visible. Cupid’s bow was appropriately aligned, and the height of the upper lip was equal on both sides (Fig. 3).

3. Discussion A median cleft of the lip is defined as a vertical cleft through the centre of the upper lip. This is a rare anomaly, which have been divided into two categories: true and false. Millard and Williams (1968) stressed that any congenital vertical cleft through the centre of the upper lip, no matter to what extent, in the absence of a prolabial remnant should be classified as a median cleft of the upper lip. These clefts are best explained by the failed mesodermal migration into fused frontonasal and maxillary process (Stark, 1954). It is a rather rare condition among craniofacial anomalies. At present, the exact incidence is not known. There have been a few reports in the literature describing different variations of the median cleft lip (Table 1). Veau (Veau, 1937) classified three varieties of median clefts: notch of the lip, median cleft extending to the columella, and a defect due to atrophy of midline facial structures. Our case may be a secondary type of Veau classification, namely median cleft extending to the columella. DeMyer (DeMyer, 1967) described two groups of syndromes associated with the

Please cite this article in press as: Jian X-c, et al., Median cleft of the upper lip associated with a mass: A rare case, Journal of Cranio-MaxilloFacial Surgery (2014), http://dx.doi.org/10.1016/j.jcms.2014.01.003

X. Jian et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2014) 1e5

3

Fig. 3. Postoperative 7 years. A. Postoperative anterior view at 7 years after surgery; B. Postoperative lateral view at 7 years after surgery; C. Postoperative oblique view at 7 years after surgery; D. Postoperative chin-up view at 7 years after surgery.

median cleft lip. The first is associated with orbital hypotelorism and the second with hypertelorism. Ghildiyal et al. (Ghildiyal et al., 2003) reported that a 3-year-old boy was admitted for primary repair of his median cleft of the upper lip. The patient’s deformity consisted of a median cleft in the lower half of the upper lip continuing as a depression up to the base of the columella. The orbicularis oris fibres were completely interrupted with the absence of the characteristic philtrum complex. Eversion of the lip revealed a double frenulum (one on either side of the midline) to the alveolus and a gap between the central incisors. An aberrant

mucosal tag was seen projecting from the median defect of the lip. Hypertelorism and flattened nose were associated with median cleft lip. Fogh-Andersen (Fogh-Andersen, 1965) reported on 15 cases in a survey of 3988 facial cleft patients seen at one hospital over a 30-year period. Stewart et al. (Stewart et al., 2007) reported a case of an apparent duplication of the mouth in a patient with midline dysraphism and a teratoid polyp; A 15-year postsurgical follow-up. In this case report, they reported that surgical pathology report was “a teratoid polyp” with skin appendages, fibrous connective tissue, skeletal muscle, bone and numerous teeth. In our

Please cite this article in press as: Jian X-c, et al., Median cleft of the upper lip associated with a mass: A rare case, Journal of Cranio-MaxilloFacial Surgery (2014), http://dx.doi.org/10.1016/j.jcms.2014.01.003

4

X. Jian et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2014) 1e5

Table 1 Clinical characteristics and operative outcomes of the patients. Authors

Age, y

CL

Starck and Epker (1994) Akoz et al. (1999) Ghildiyal et al. (2003)

3 4 1.5 3

True True True True

Urata and Kawamoto (1999) Springer et al. (2004)

1.3 5

Stewart et al. (2007) Freitas et al. (2008)

15 2 0 0

True med An island of skin of upper vermilion True med False med False med False med

0 0 0

Patel and Tantri (2010)

Alveolar

Nose

Septum

Association

Surgical Procedure

Normal Clefted Clefted Double frenulum Notched Normal

Columella short Bifid nose Bifid nose Flattened nose

Normal Normal Normal Normal

No No No No

Lip Lip Lip Lip

Slight bifidity Normal

Normal Normal

No No

Lip repair Lip repair

Normal Absence Absence Absence

Bifid nose Columella absence Columella absence Columella absence

Normal Absence Absence Absence

Lip repair Lip repair No No

False med False med False med

Absence Absence Absence

Columella absence Columella absence Columella absence

Absence Absence Absence

0 0 0 4

False med False med False med True med

Absence Absence Absence Clefted

Columella absence Columella absence Columella absence Intronasal Tumor

Absence Absence Absence Normal

Double and teratoma Holoprosen Holoprosen Holoprosen þ hydrocephalla Holoprosen Holoprosen Holoprosen þ hydrocephalla Holoprosen Holoprosen Holoprosen ¼ Pai syndrome

6 0 3 0 3 3 13 0 9-months 4.5

True med True med True med True med True med True med True med True med True med Med cleft

Clefted Clefted Clefted Clefted Clefted Clefted Clefted Clefted Normal Bifid frenulum

Normal Normal Normal Normal Normal Normal Normal Normal Columella short Normal

Normal Normal Normal Normal Normal Normal Normal Normal Normal Normal

No No No Cleft palate No No No No No No

med med med med

repair repair repair repair

No No No No No No Lip repair þ resection of tumor Lip repair Lip repair Lip repair Lip repair þ No Lip repair Lip repair Lip repair Lip repair Lip repair

Note: True med ﹦ True median cleft; False med ﹦ False median cleft; Med cleft ﹦ Median cleft.

case report, the patient described had a median cleft of the upper lip associated with a mass without any deformities of the nose, philtrum, or alveolus. In our case, a median cleft of the upper lip associated with a mass was actually also a teratoid polyp with skin appendages, fibrous connective tissue, skeletal muscle, bone and numerous teeth. The acknowledged rarity of median cleft lip is our reason for publishing this case. The technique that was chosen to repair the median cleft lip involved a vertical incision to remove the epithelium in the middle of the lip. There has been a report of using an inverted V excision. However, this leads to a visible scar in the upper lip (Springer et al., 2004). 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. This lengthens the skin in the centre of Cupid’s bow (Millard and Williams, 1968). In our case, we used Z-plasty on the skin of the base of the columella, therefore, the skin of the upper lip was released. Our operative technique produced adequate lengthening of the lip and a normal-looking philtrum and tubercle of the lip (Fig. 3). 4. Conclusion In this case report we present, a 4-month-old boy with a median cleft associated with a teratoid polyp of the upper lip. The patient had no other anomalies of the nose or alveolus. A Z-plasty technique was used on the skin of the base of the columella. A vertical excision of the cleft with muscle approximation is performed on the white roll and the wet-dry border of either side of the defect of the upper lip. Postoperatively, the patient had a satisfactory result. The incisive scars were not visible. Cupid’s bow was appropriately aligned, and the height of the upper lip was equal on both sides.

Role of funding source No funding was needed. Conflict of interest statement The authors declare no conflict of interest. A written photo release permission form has been signed by the patient’s father. References Akoz T, Kapucu MR, Erdogan B: The use of “excess” soft tissue in the repair of median facial cleft; a report of two cases. Ann Plast Surg 43: 195e198, 1999 Davis WB: Congenital deformities of the face. Surg Gynecol Obstet 61: 201e209, 1935 DeMyer W: The median cleft face syndrome: differential diagnosis of cranium bifidum occultum, hypertelorism, and median cleft nose, lip and palate. Neurology 17: 961e971, 1967 Fogh-Andersen P: Rare clefts of the face. Acta Chir Scan 29: 275e281, 1965 Freitas SR da, Alonso N, Shin JH, Busato L, Ono MCC, Oliveriae Cruz GA: Surgical correction of Tessier number 0 cleft. J Craniofac Surg 19: 1348e1352, 2008 Ghildiyal HC, Misra T, Keswani NK: Median cleft of the upper lip. Plast Reconstr Surg 112: 1175e1177, 2003 Hou R, Feng X, Zhang J, Lu B, Liu G, Wang L, et al: A rare bilateral Tessier no. 6 and 7 clefts. J Craniomaxillofac Surg 39: 93e95, 2011 Kawamoto H: Rare craniofacial clefts. In: McCarthy J (ed.), Plastic surgery. Philadelphia: WB Saunders, 2922e2973, 1990 Laure B, Picard A, Bonin-Goga B, Letouze A, Petraud A, Goga D: Tessier number 4 bilateral orbito-facial cleft: a 26-year follow-up. J Craniomaxillofac Surg 38: 245e247, 2010 Mazzola RF: Congenital malformations in the frontonasal area: their pathogenesis and classification. Clin Plast Surg 3: 573e609, 1976 Millard Jr DR, Williams S: Median lip clefts of the upper lip. Plast Reconstr Surg 42: 4e14, 1968 Mohamed El-Massry MA, Ali TS, Hussain AI, Dashty F: Duplicated, translocated maxilla and upper lip: a case report of a rare congenital anomaly. J Craniomaxillofac Surg 39: 96e98, 2011 Patel NP, Tantri DP: Median cleft of the upper lip: a rare case. Cleft Plate Craniofac J 47: 642e644, 2010

Please cite this article in press as: Jian X-c, et al., Median cleft of the upper lip associated with a mass: A rare case, Journal of Cranio-MaxilloFacial Surgery (2014), http://dx.doi.org/10.1016/j.jcms.2014.01.003

X. Jian et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2014) 1e5 Springer IN, Sprengel M, Terheyden H, Suhr MA, Harle F, Warnke PH: Wedge excision: treatment of choice in minimal median clefts of the upper lip. Plast Reconstr Surg: 812e814, 2004 Starck WJ, Epker BN: Surgical repair of a median cleft of the upper lip. J Oral Maxillofac Surg 52: 1217e1219, 1994 Stark RB: The pathogenesis of harelip and cleft palate. Plast Reconstr Surg 13: 20e 24, 1954 Stewart C, Hughes LA, Thomson HG, Armstrong D, Forte V: An apparent duplication of the mouth in a patient with midline dysraphism and a teratoid polyp: a 15-year postsurgical follow-up. Can J Plast Surg 15: 227e229, 2007

5

Tessier P: Anatomical classification of facial, craniofacial, and latero-facial clefts. J Maxillofac Surg 14: 69, 1976 Urata MM, Kawamoto Jr HK: Median cleft of the upper lip; A review and surgical management of a minor manifestation. J Craniofac Surg 14: 749e755, 1999 Veau V: Hasencharten menschlicher Keimlinge auf der Stufe 21e23mm SSZ. Anat Entwiclungsgesch 108: 459, 1937

Please cite this article in press as: Jian X-c, et al., Median cleft of the upper lip associated with a mass: A rare case, Journal of Cranio-MaxilloFacial Surgery (2014), http://dx.doi.org/10.1016/j.jcms.2014.01.003

Median cleft of the upper lip associated with a mass: a rare case.

Median cleft lip is a midline vertical cleft through the upper lip. This is a very rare anomaly described in the literature. Median cleft lip is cause...
1MB Sizes 2 Downloads 0 Views