Bifid Uvula in three members of a family

Uvula is a key organ in functions like speech, deglutition and mastication. The majority of the world population has a uvula that is conical in shape, hanging upside down. However, there are times when the uvula is split, the condition is called a bifi d or bifurcated uvula. Sometimes it is also called a cleft uvula. Three male blood relatives, father and his two sons reported to outpatient department of Oral Medicine and Radiology at Sharad Pawar Dental College, Wardha for odontogenic complaint. But, their intraoral examination revealed interesting fi nding, that was presence of bifi d uvula and cleft palate in three of them, who were of course fi rst relatives of each other. The presentation of the condition in three members of the same family is a unique feature of this article. Case Report Bifi d Uvula in three members of a family Suwarna Dangore Khasbage* Oral Medicine and Radiology, Sharad Pawar Dental College, Datta Meghe institute of Medical Sciences Wardha, Maharashtra, India Dates: Received: 29 May, 2017; Accepted: 15 July, 2017; Published: 17 July, 2017 *Corresponding author: Suwarna DangoreKhasbage, Oral Medicine and Radiology, Sharad Pawar Dental College, Datta Meghe institute of Medical Sciences Wardha, Maharashtra, India, Email


Introduction
Bifi d uvula means a cleft in uvula. It is often considered as a marker for sub mucous cleft palate. Compared to the normal one, it has fewer amounts of muscular tissues. It is commonly noticed in infants and is rarely found in adult. A bifi d or bifurcated uvula exists in two percent of the general population. The prevalence of cleft uvula is much higher than that of cleft palate. Cleft uvula is more common in whites (1 in every 80 white individuals) as compared to blacks (1 in every 250 individuals) [1]. It can cause problems in ear. Sometimes it is unable to reach the posterior pharyngeal wall during swallowing, causing regurgitation. It may produce velopharyngeal insuffi ciency and nasal intonation. Sometimes it is associated with major systemic problems like aneurysm in different vascular bed like coronary and abdominal aortic aneurysm. But, it does not cause problems in view of airway management.

Case report 1
A 58 years old male had reported to outpatient department of Oral Medicine and Radiology at Sharad Pawar Dental College, Wardha with a complain of toothache in left posterior region of lower jaw. His past medical history and past dental history was not contributory. Clinical examination revealed food lodgment and initial proximal caries with 36. Soft tissue examination revealed presence of approximately 1X 1.5cm, oval palatal perforation suggestive of cleft palate and short bifi d uvula ( Figure 1). He had no local problems like speech diffi culty or nasal regurgitation etc., and not ready for further evaluation regarding cleft palate and bifi d uvula. Routine treatment protocol was advised for the carious tooth.

Case report 2
A 28 years old male who was an elder son of the patient described in fi rst case report, had reported to outpatient department of Oral Medicine and Radiology at Sharad Pawar Dental College, Wardha with a complaint of decayed tooth in posterior region of right side of lower jaw. His past medical and dental history was not contributory. Clinical examination revealed deep caries with mandibular fi rst molar. Examination of palate showed presence of approximately 1X 1cm, round palatal perforation suggestive of cleft palate and short bifi d uvula as shown in (Figure 2). There was no history of speech diffi culty, nasal twang or nasal regurgitation. He was also not ready for further evaluation regarding cleft palate and bifi d uvula. The carious tooth was treated by conservative approach.

Case report 3
A 26 years old male who was a younger son of the patient Various symptoms associated with bifi d uvula and cleft palate may be inability to breastfeed, diffi culty in bottlefeeding, nasal regurgitation and recurrent otitis media, delay in speech development etc. The speech had a characteristic hyper-nasal resonance with nasal air emission and an abnormal speech pattern with compensatory articulations [2].
Bifi d uvula, although looks apparently benign, sometimes may be associated with anomalies leading to catastrophic complications. Cornelia de Lange syndrome is a rare congenital syndrome associated with bifi d uvula and sub mucous cleft palate that causes problems in airway due anatomical distortion [3]. Bifi d uvula may be associated with increased risk of schizophrenia, mild mental retardation, and chromosomal disorder, diagnosed by fl uorescent in situ hybridization technique [4]. Loeys-Dietz syndrome (autosomal dominant) is a genetic syndrome with clinical features overlapped with Marfan syndrome, but etiology due to mutations in the genes encoding transforming growth factor beta receptor 1.
Hypertelorism, cleft palate, or bifi d uvula are the major fi ndings.
Arterial aneurysms/dissections, arterial tortuosity involving aortic and its branches, carotid, vertebral, extracranial artery, abdominal aorta and its branches, common iliac, and popliteal arteries are reported in this syndrome [5,6,7]. It is stated that bifi d uvula may have been a warning sign of the syndrome with internal anatomical or functional changes without any external manifestation akin to the tip of an iceberg. Although cerebral aneurysm is very rare with bifi d uvula, it may be a part of the above mentioned syndromes [8].
Thus, whenever anesthetists plans to conduct a case with bifi d uvula (even though non-syndromic), they must ask for detailed family and genetical history, clinical examination relevant investigations, and specialty consultation [8].
Adequate preoperative preparation and, accordingly, intra- to be alert to SMCP because SMCP may account for these persistent mild complaints. Therefore, early detecting of SMCP can yield profi ts [10].
The possible treatments for bifi d uvula depend on the severity of problem. In asymptomatic cases, as such no treatment required which was the situation in the patients described in the present article and thus they were not ready for any investigations or management. If the symptom includes speech diffi culties, then a speech therapist could possibly help the patient learn how to talk well. Swallowing and feeding problems may also be addressed through appropriate therapy.
Some patients may opt for the removal of the bifurcated uvula but others would opt for the surgical reconstruction of these abnormal tissues.
Treatment of SMCP is surgical repair which includes a V-Y palatal pushback and simultaneous transposition of a superiorly based pharyngeal fl ap. Similarly the interrelated problems of chronic otitis media and faulty speech production may be diminished by functional reorientation of the palatal muscles and simultaneous revision of the velopharyngeal portal [11].