Ankyloglossia with cleft lip: A rare case report.

Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman syndrome or Simpson-Golabi-Behmel syndrome. This article presents a rare case of ankyloglossia associated with cleft lip treated with diode laser in a 12-year-old Indian boy who had undergone surgical correction of associated cleft lip soon after birth. Correction of ankyloglossia at a young age would lead to enhanced phonetics, improved oral hygiene, and overall personality development.

INTRODUCTION

The term ankyloglossia is derived from the Greek word “agkilos” and “glossa” meaning curved and tongue respectively. Also known as tongue-tie, it is a developmental abnormality in which the inferior lingual frenum is short and attached at or close to the tip of the tongue. This causes limitations in the movement of the tongue. The tongue cannot be protruded beyond the lower incisor teeth. The prime oral and general concern of this congenital abnormality is speech (articulation of words), swallowing, suckling (leads to breastfeeding problems) and poor oral hygiene. The child's articulation of sound may lead to embarrassment in social groups.

Ankyloglossia may or may not be associated with other congenital abnormalities like orofacial clefts (i.e., cleft lip, cleft palate) and other craniofacial syndromes. Our present case report arrays the occurrence of ankyloglossia along with cleft lip in a 12-year-old boy who was treated with a diode laser.

Tongue tie can be considered a relatively common anomaly with a prevalence of <1–10.7%. A descriptive analysis of the prevalence of tongue lesions by Patil et al. in 2013 showed 21 patients out of 4926 Indian patients were affected with ankyloglossia, which accounts for 3.5% of the tongue lesions.[1] For unknown reasons, the abnormality seems to be more common in males with a male to female ratio of 2.5:1.0.[2] Among South Indian population, the occurrence of tongue tie is relatively rare with an incidence of 0.2%.[3]

CASE REPORT

A 12-year-old boy of Indian ethnicity reported to the Department of Periodontics with a chief complaint of inability to move his tongue like all his other school friends. He also complained of slurred speech. History revealed that he had congenital cleft lip (not associated with cleft palate) and was surgically treated for the same soon after birth [Figure 1].

Figure 1

Associated cleft lip

On oral examination, the patient had thick inferior lingual frenum attached 3 mm from the tip of the tongue [Figure 2]. Restricted tongue movements like protrusion, lateral movements and inability to touch the palate with the tip of the tongue were observed. On protrusion, a bifid or heart shaped tip [Figure 3] of the tongue was observed. According to Kotlow's classification, he was allocated under Class III “severe ankyloglossia” which accounts for the movement of the tongue between 3 mm and 7 mm. According to Hazelbaker's assessment tool, the appearance score was 2 (which was <8) and the functional score was 5 (which was <11) hence was indicated for frenectomy.[4]

Figure 2

Ankyloglossia (tongue tie)

Figure 3

Heart shaped tongue

A routine hematological examination of hemoglobin percentage, bleeding time, clotting time, random blood sugar, total leukocyte count and differential count was taken and was found to be within the normal range hence was planned for surgery.

After application of topical anesthetic gel, adequate local anesthesia (2 ml of 0.2% lignocaine hydrochloride in 1:200,000 adrenaline) was given as infiltration around the lingual frenum. A tissue forceps was used to clamp the frenum. A soft tissue diode laser of power 1 W was used to relieve the lingual frenum approaching from either side of the tissue forceps [Figure 4]. No bleeding was observed on the surgical site. A 3–0 silk suture was used to approximate the dorsum of the tongue [Figure 5]. The patient was given adequate antibiotics and analgesics (amoxicillin 250 mg TDS and paracetamol 250 mg BD) along with postoperative instructions and was asked to report after 1-week for suture removal and review.

Figure 4

Frenectomy using diode laser

Figure 5

Immediate postoperative view

The Hazelbaker's appearance score after suture removal post 1-week was 10 and the function score was 12 [Figures 6 and 7]. The parents were instructed to consult a speech therapist for improvement in the articulation of speech.

Figure 6

1-week postoperative view

Figure 7

“U” shaped tongue

The patient was called after 1-month for a review [Figure 8]. The Hazelbaker's appearance score and function score were 10 and 13 respectively.

Figure 8

One-month postoperative view

DISCUSSION

Ankyloglossia leads to various consequences that could be categorized under “oral” and “general”. The oral consequences of ankyloglossia include restricted movement of the tongue leading to the impaired articulation of speech. Articulation of the sounds “h”, “l”, “r”, “t”, “d”, “n”, “th”, “sh”, “w” and “z” is impaired. Lack of sweeping action of the tongue especially in the lower lingual region may lead to oral halitosis and periodontal problems. It can also lead to early childhood caries. In these cases, the gingival biotype is thin due to the excessive frenal pull and may lead to midline diastema of mandibular incisors in severe cases. It may also lead to lingual recession of mandibular anterior teeth with subsequent sensitivity.

General consequences of ankyloglossia include social embarrassment due to disharmony of the speech, which may build up a complex within the child. Tongue tie has been suggested to cause breast-feeding difficulties for both the mother and the child. Suckling inability due to improper seal leads to poor infant weight gain and early weaning. Mothers may experience nipple pain and/or sore nipples due to the excessive suckling pressure by the infant.

Various criteria have been used to classify ankyloglossia. However there is no standard worldwide definitive criterion used to assess the severity of ankyloglossia. Clinical features of ankyloglossia are the tongue tip that cannot be protruded beyond the lower gum line, abnormally short and thick frenulum and heart shaped tongue on protrusion. Hazelbaker in 1993 gave an assessment tool for lingual frenum function with a scoring criteria based on which the necessity of frenectomy is decided.[4] It measures two scores-the appearance score and the function score. The appearance score assesses the appearance of the tongue and frenum. The perfect appearance score is given as 10 and if the appearance score is <8, it is indicated for frenectomy. The function score assesses the lateral movement of the tongue, extension, lifting, cupping, peristalsis and the spread of the anterior tongue. The perfect function score is given as 14 and a score of <11 is indicated for frenectomy. Kotlow in 1999 categorized ankyloglossia into five classes based on the severity.[5] Clinically acceptable movement of the tongue is >16 mm. Class 1 or mild ankyloglossia shows movement of the tongue in between 12 mm and 16 mm. Class II or moderate ankyloglossia shows tongue movement of 8–10 mm. Class III or severe ankyloglossia shows tongue movement of 3–7 mm. Class IV or complete ankyloglossia has a tongue movement of <3 mm. Hogen et al. in 2005 explained ankyloglossia as a frenulum extending along 25–100% of tongue's total length.[6]

Various treatment protocols for the management of ankyloglossia have been reported in the literature. Frenectomy, that is, incising the frenum or frenuloplasty, that is, is the surgical alteration of the frenulum can be performed in mild Class 1 (Kotlow's classification) cases. Frenectomy, that is, the complete removal of the frenum and its attachment to the underlying structure has to be performed in Class II, III, IV cases, that is, moderate, severe and complete ankyloglossia.

All these procedures can be performed using conventional scalpels or lasers. The advantage of lasers over the traditional techniques has been reported in the literature. The benefits of laser treatment include reduced bleeding during surgery with consequent reduced operating time and rapid hemostasis, thus reducing the need for sutures. The reduced need for local anesthetics and sutures, as well as intra-operative comfort makes this technique particularly useful for very young patients.[7]

After surgical correction, patients should be referred to a speech therapist for speech modulation.

The absolute pathogenesis of ankyloglossia remains unknown.[3] While most cases of ankyloglossia are sporadic, mutations in the T-box transcription factor TBX22 may lead to heritable (X-linked) ankyloglossia with or without cleft lip, cleft palate and/or hypodontia.[8] Reports suggest that there is a genetic basis for the microvariation in the attachment of genioglossus muscle due to the mutations present in the TBX22 gene. In case of cleft palate with ankyloglossia (CPX) patients, TBX22 expression is seen in early human development, where the expression is found in the palatal shelves and is highest prior to elevation to a horizontal position above the tongue. TBX22 mRNA is also detected in the base of the tongue in the region of the frenulum that corresponds to the ankyloglossia seen in CPX patients.[9] A case report on familial ankyloglossia by Morowati et al. in 2010 reported a family of five generations in which five individuals had ankyloglossia inherited as an autosomal dominant or recessive trait.[10] Another case report in 1952 describes a Dutch family in which 13 persons in three generations had ankyloglossia and there were many instances of male to male transmissions.[11]

CONCLUSION

Ankyloglossia or tongue-tie which may cause oral and general complications can be easily treated using frenotomy/frenuloplasty/frenectomy based on the severity of lingual attachment. Laser frenotomy/frenectomy provides remarkable results as management therapy of tongue tie. The patient, however, should be referred to a speech therapist after surgical correction. It may be noted that although ankyloglossia is a sporadic developmental abnormality, it can be associated with other congenital defects such as cleft lip, both of which should be treated effectively for the general well-being of the patient.

CLINICAL SIGNIFICANCE

Ankyloglossia has mechanical as well as social effects. Early intervention would prevent feeding problems which leads to enhanced general health, improved the oral hygiene status, accurate articulation of words and overall personality development.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.