Article Type : Research Article
Authors : Shunmugavelu K
Keywords : Bifid Tongue; Cleft tongue; Developmental abnormality; Tongue; Case report
Bifid
tongue is a rare condition that can be syndromic or nonsyndromic and usually
occurs in conjunction with other oral abnormalities. In oro-facial-digital
syndrome and Tessier type 30 craniofacial cleft, bifid tongue has been
frequently reported as an associated finding. Although rare, it has been found
in conjunction with other syndromes and in nonsyndromic cases. A 51 year old
female patient presented with a bifid tongue tip involving the anterior
one-third of the tongue and found the patient unusually with no other intraoral
abnormalities. The patient was in good health, his vital signs were within
normal ranges, and he had no relevant family or medical background. Any other
related congenital orofacial defects were ruled out by extra oral and intraoral
analysis. No associated clinical features suggesting any syndrome was evident
in this case. With this article, the patient clinical features on examination
suggests that bifid tongue need not always be associated with a syndrome,
nonsyndromic bifid tongue can also be found on ruling out possible syndromic
association.
During the fourth week of
pregnancy, the tongue grows from a central swelling, the tuberculum impar on
the pharyngeal surface, and two lateral lingual swellings that join this
central structure. These lateral lingual structures quickly develop to cover
the tuberculum impar and form the tongue's anterior two-thirds. First the tip
of the tongue is separated longitudinally for a certain distance when this
mechanism is disrupted, resulting in cleft tongue/bifid tongue [1]. Bifid
tongue in the absence of other orofacial disorders is an unusual condition, and
it normally gets much less attention than those seen in conjunction with
certain syndromes [2]. Human development is a process that must be meticulously
planned and executed. Even a minor ailment can lead to clinically significant
consequences later on [3,4]. As seen in the case report presented here, ancient
beliefs and practices continue to override/delay a patient's desire to seek
treatment. Congenital bifid tongue has been linked to a number of orofacial
anomalies [5]. A differential diagnosis must be made when a patient presents
with a bifid tongue in order to arrive at a definitive diagnosis that will aid
in identification, classification, management, and documentation. We present the patient’s history, clinical
features, and make differential diagnosis of syndromes associated with
syndromes or non-syndromic and further aid in identifying cases which can be
non-syndromic.
A 51 year old Indian female patient, visited the clinic with a chief complaint of difficulty in speech from childhood. Patient revealed the history of consanguineous marriage. Her mother did not have any previous abortion or drug during pregnancy. Patient had no relevant medical and surgical history. Patient had no other difficulties other than aphasia. On physical examination at that time, she was noted to have obesity and hypertension. Her body mass index was 29kg/m2. Lab testing revealed an abnormal glucose loading test and abnormal 3-hour glucose tolerance test. On clinical examination of the oral cavity revealed the tongue was bifid at the tip which was slightly towards the right (Figure 1). In both the upper and lower vestibules, there were several frenal attachments. The upper frenii were hypertrophic and bilaterally clefted the upper alveolus. The clefts were found between the upper lateral incisors and canines, though the deciduous canines were found in the premaxillary segment. On the left side of the lower lip, there was a single mucous pit. Extra oral examination showed slanting palpebral fissures, alopecia, and a large tip to the nose, excessive facial hair, and dry coarse hair all over the scalp. Examination of the rest of the body revealed normal fingers and toes. Systemic examination did not reveal any abnormality. Our case defies categorization into any well-defined syndrome. There were no orofacial defects, hereditary predispositions, tongue piercings, or postnatal trauma histories found. Surgical correction of the defect was planned under local anaesthesia. The median parts of the defect were freshened and reconstructed by suturing the muscles in layers. The patient was not willing for surgical management.
Figure 1: Picture representing
bifid tongue in anterior 2/3rd of the tongue.
The appearance of the median
tongue bud at the end of the fourth embryonic week is the first sign of tongue
formation. On either side of the median tongue bud, lateral lingual swellings,
also known as distal tongue buds, appear shortly after. These swellings are
caused by mesenchymal proliferation of the pharyngeal arch's first pair. The
distal buds grow in size, merge in the midline, and form the tongue's anterior
two-thirds, also known as the oral segment. The fusion is marked by a middle
groove on the tongue called the median sulcus.
A tongue with a groove or
split running lengthwise along the tip (glossoschissis) is known as a bifid or
cleft tongue. It's caused by a lack of fusion between the distal tongue buds. A
bifid tongue may be a one-of-a-kind condition, but it has also been linked to
maternal diabetes. Two babies with a bifid tongue were born to diabetic mothers
[6]. Only orofacial digital syndromes type I, II, IV, and VI have been linked
to median tongue clefts in the literature. All of these syndromes are linked to
clefts in the median lip and/or mandible, as well as digital variations. Some
malformations discovered in those babies included a cleft palate and
polydactyly. Various variants of the heterogeneous group of oral-facial-digital
syndromes are believed to manifest as combined deformities of the palate and
tongue [7]. Vandenhaute et al reported a child with the Pierre-Robin sequence,
epignathus teratoma, and a bifid tongue died at the age of two months. In
around 6% of cases, epignathus teratomas of the oropharyngeal region are
associated with other malformations, such as a bifid tongue or even a bifid
nose. Other syndromes with a bifid tongue have been identified, including short
rib syndrome, short rib polydactyly syndrome, median cleft syndrome, and the
Klippel-Feil anomaly [8].
The diagnostic criteria
of any well-defined condition are not fully met in our case. While minor
digital bone abnormalities can be missed on a propositus radiogram, the
diagnosis of an oral-facial-digital syndrome is doubtful due to the lack of
characteristic digital features [5]. Due to the absence of characteristic
stigmata such as hypertelorism and a deep nasal root, it was also difficult to
diagnose a median cleft syndrome [9]. The lack of fused cervical vertebrae
ruled out the Klippel-Feil anomaly. Nonetheless, the Pierre-Robin series can be
classified based on the combination of abnormalities found in our case.
Micrognathia, a cleft palate, posterior retraction of the tongue, and neonatal
breathing problems are the most common characteristics of this congenital
malformation, which has a heterogeneous and largely unknown etiology [4].
Bifid tongue is a rare
condition which is mostly encountered as a developmental abnormality.
Non-syndromic bifid tongue is reported in this article which suggests that
bifid tongue need not always be found associated with syndromes. Proper patient
history and clinical examination must be ensured before misdiagnosing it as a
syndromic.