Sub mucous cleft palate with fenestration.

Congenital fenestration in sub mucous cleft palate is not a common identity. This patient is a 16-year-old female who presented with nasal speech. On examination, she had associated microform cleft lip with nasal deformity. Successful palatoplasty with intra velar veloplasty was done.

INTRODUCTION

Sub mucous cleft palate is defined as a traid of bifid uvula, velopharyngeal incompetence and muscular diastasis across midline. Sub mucous cleft palate occurs when the palate has mucosal continuity, but the underlying muscle is dis continuous across the midline. In sub mucous cleft palate with fenestration there is a mucosal tag bridging the cleft palate. Sub mucous cleft palate presenting with fenestration is remarkably uncommon. Various causative theories for this have been reported in the literature, common ones being perinatal or postnatal trauma and infections. Congenital malformation of the palate is the rarest type of palatine cleft. Mehendale and Sommerlad have reported 2.17% incidence of acquired fenestration in 184 patients with sub mucous cleft palate. Cheng and Zhou reported 17% incidence of congenital fenestration in sub mucous cleft palate in their study on 875 patients.

This is a case report of 16-year-old female with fenestration in sub mucous cleft palate with microform cleft lip and nose.

CASE REPORT

A 16-year-old female patient presented at our outpatient department with the complaints of nasal twang in speech and nasal regurgitation of liquids since birth. There was no history of perinatal/postnatal trauma or any other obvious infection. She was born as the first child to her parents (conceived normally), delivered at term by spontaneous vaginal delivery and weighed 2.8 kg at birth. Her developmental milestones and intelligence quotient were normal for her age. She had no syndromic association. There was no family history of similar complaints in the past and future. Other environmental factors such as drugs, radiations, etc., were noncontributory.

The patient also had microform right upper lip and nose [Figure 1]. Her audiometery showed bilateral mild conductive deafness. The hematological (hemoglobin, blood count) and growth profile were normal.

Figure 1

Microform right upper lip

On intraoral examination, she had cleft in secondary palate with a posterior tag of thin soft palate and bifid uvula and naso pharyngeal incompetence [Figure 2].

Figure 2

Intraoral examination

Intra operatively, intrinsic muscles were absent in thin soft palate. Langenback's bipedicle palatoplasty with intravelar veloplasty was done under general anesthesia. No intra operative or postoperative complication was encountered. She was allowed liquids after 6 h and discharged after 4 days. Regular follow-up in the outpatient department shows normal healing with no fistula [Figure 3]. The patient is now on postoperative speech therapy.

Figure 3

Postoperative result

DISCUSSION

The etiopathogenesis of so-called “palatal hole” is unclear, the most common reason being acquired either due to infection or due to trauma to sub mucous cleft palate. Trelat[1] presented a neonate with intact sub mucous cleft palate who later developed a midline perforation. Mehendale and Sommerlad[2] also reported four patients with perforated sub mucous cleft palate. Calnan[3] debated that these perforations can occur if central zone is thin. Weijerman and Heybroek[4] thought that the development of apthous ulcer over palate is the primary cause of perforation. In older age group, this kind of perforation can also result from trauma by denture. The congenital origin of this malformation with normal palatal musculature has been confirmed only in five cases until date. Metcalf[5] described a new-born with 3 mm aperture in the hard palate with normal intact hard palate, soft palate, and uvula. A similar case was reported by Lynch et al.[6] These authors concluded that congenital origin of this defect is not a result of disruption in sub mucous cleft palate.

Our case is a congenital defect in sub mucous cleft palate. There is no history suggestive of failure to thrive in childhood. Her speech and hearing abnormality could only be minimally treated at this age, the surgery being of help only for regurgitation.

The operative technique needs no modification in the presence of perforation as also suggested by Fαra.[7] The thin mucosa surrounding perforation is excised and muscle dissection and retro positioning is done with the same technique as would be done in repair of cleft palate or sub mucous cleft palate. Though the results of von Langenbecks repair have not been satisfactory in the study by Cheng and Zhou[8] but our case shows successful healing with no complication (perhaps because of intravelar veloplasty).

CONCLUSION

We conclude that early diagnosis of sub mucous cleft palate and also presence of any perforation can reduce the incidence of abnormal speech and hearing defects in these patients.