Congenital palatal fistula: Case report and review of literature

Sneha Gulati; Aditya Shivaji Yadav; Ramesh Kumar Sharma

doi:10.4103/jclpca.jclpca_20_22

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CASE REPORT

Year : 2023 | Volume : 10 | Issue : 1 | Page : 37-39

Congenital palatal fistula: Case report and review of literature

Sneha Gulati, Aditya Shivaji Yadav, Ramesh Kumar Sharma
Department of Plastic Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission	15-Aug-2022
Date of Acceptance	12-Oct-2022
Date of Web Publication	14-Mar-2023

Correspondence Address:
Dr. Sneha Gulati
Department of Plastic Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jclpca.jclpca_20_22

Abstract

Congenital palatal fistula (CPF) is the rarest phenotypic variation of cleft palate, commonly associated with submucous cleft palate (SCP). Isolated palatal fistula without SCP has been reported only rarely. We report here the case of a 4-year-old child, with a 10 mm × 5 mm CPF, presenting with nasal regurgitation and speech abnormalities. The patient had unilateral palatal muscular malposition without classical features of SCP. The repair was performed using turnover flaps and Bardach's two-long flap palatoplasty and intravelar veloplasty. Healing occurred without any complications.

Keywords: Congenital palatal fistula, hard palate, palatal muscles, palatoplasty, soft palate, submucous palate

How to cite this article:
Gulati S, Yadav AS, Sharma RK. Congenital palatal fistula: Case report and review of literature. J Cleft Lip Palate Craniofac Anomal 2023;10:37-9

How to cite this URL:
Gulati S, Yadav AS, Sharma RK. Congenital palatal fistula: Case report and review of literature. J Cleft Lip Palate Craniofac Anomal [serial online] 2023 [cited 2023 Mar 30];10:37-9. Available from: https://www.jclpca.org/text.asp?2023/10/1/37/371641

Introduction

Clefts of the lip, alveolus, and palate are the most common congenital craniofacial anomalies.^[1] Congenital palatal fistula (CPF) is seen in 0.17%–0.45% of cleft palate and in 6%–17% of submucous cleft palate (SCP).^[2],[3] True CPF is the rarest phenotypic variation of the cleft palate as noted by von Bergmann.^[2] Acquired palatal fistulae occur more frequently as complications of palatoplasty or due to infection or trauma to SCP.^[3],[4]

Embryology

Based on theories explaining the formation, the types of CPF are as follows:

CPF with SCP: Fistulae present at birth (majority), occurring due to prenatal rupture of the submucous cleft (Veau)^[3],[4]
CPF without SCP (rarest): With normal soft palatal musculature (Lynch)^{[2],[5],[6],[7]}
Palatal fistulae associated with SCP, develop after birth by thinning out of mucosa overlying SCP, in areas of maximal tension or due to trauma or infection.^[3],[6],[7]

Mesenchymal migration and proliferation into maxillary processes lead to the approximation and fusion of the two processes from which the palate develops. DNA synthesis in the epithelium at the medial edges ceases before contact and fusion occur by mesenchymal penetration. Cleft formation results from failure of the epithelium at medial edges to apoptosis, failed mesenchymal migration, or rupture after fusion.^[3],[8]

In SCP, there is a separation of soft palate musculature with intact overlying mucosa. Rupture of this mucosa before or after birth can result in CPF.^[4]

Anatomy of cleft

In a normal soft palate, the levator veli palatini occupies the middle 40%, and spreads toward the midline, meeting its counterpart. The tensor muscle fans out to form an elastic aponeurosis at the anterior third of the palate. In cases of cleft palate, palatal muscles insert at the margin of the cleft and posterior border of the hard palate, occupying the anterior two-thirds of velum.^[9]

Case Report

A 4-year-old boy, born at term by institutional normal vaginal delivery, presented to our outpatient department with a history of nasal regurgitation of feeds since birth and speech difficulties. Parents noticed a small hole in the palate, a few weeks after birth, which progressively increased in size. He had normal developmental milestones, no other anomalies, and no history of trauma to the palate or previous surgeries. There was no family history of cleft lip, palate, palatal fistulae, or other congenital anomalies, and no history of maternal exposure to alcohol or antiepileptics.

On examination, a 10 mm × 5 mm full-thickness fistula was present at the junction of the hard and soft palate to the left of the midline. It was larger on the oral surface, with an absent muscular layer and a small defect in the nasal mucosa. No bifid uvula is seen [Figure 1] and [Figure 2].

Figure 1: (a) Left Palatal Fistula with full thickness palatal defect with larger oral mucosal defect, 5x5mm nasal mucosal defect and absent muscle layer; (b) Marking of the anatomical midline

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Figure 2: Incision markings. Hinge flaps have been marked around the fistula for nasal lining

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The patient underwent closure of the fistula using Bardach's two-flap palatoplasty. Mucoperiosteal flaps were raised using incisions along the alveolar margin, midline, and margin of the fistula. The orientation of palatine muscles on the right was normal (transverse), whereas that on side of the fistula was longitudinal [Figure 3]. These muscles were dissected and separated from the posterior margin of the hard palate. The repair was completed in three layers: nasal, muscular, and palatal. The nasal layer was repaired using turnover flaps from cleft margins. Intravelar veloplasty was done to create a palatal sling. The postoperative course was uneventful. [Figure 4] shows a well-healed palate at 1 month.

Figure 3: (a) Mucoperiosteal flaps elevated bilaterally. Turnover flaps raised for nasal layer repair. Soft palatal muscles oriented vertically on the cleft side (left) and horizontally on the normal right side, (b) closure of the fistula and reconstituted muscle sling, (c) diagrammatic representation of muscles intraoperatively, and (d) diagrammatic representation of reconstructed sling

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Figure 4: (a) Final closure at the end of the surgery, (b) well-healed palate is seen at 1-month postoperative follow-up

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Discussion

CPF, first reported by Veau and Borel (1931),^[2] is the rarest of cleft deformities (25 cases reported). It is commonly, but not always, associated with SCP. Presentation is similar to the cleft palate – nasal regurgitation, failure to thrive, and speech abnormalities.

Cheng and Zhou reported four cases of CPF with SCP. Three were repaired using the von Langenbeck technique.^[7] Fara described five cases of CPF with SCP, proposing they resulted from intrauterine rupture of the hard palate in areas of maximum tension. He used a primary pharyngeal flap for repair.^[10] Eshete et al. reported two cases, both with SCP, repaired using von Langenbeck and Furlow's techniques.^[2]

All these cases, however, had a midline fistula. There has never been mention of a unilateral CPF. This case is unique as it is not associated with SCP and there is unilateral malpositioning of soft palatal musculature contributing to speech abnormalities. We found a full-thickness defect at the junction of the hard and soft palate, to the left of the midline [Figure 2]. On dissection, left-sided muscles were malpositioned and inserted into the posterior margin of the hard palate, whereas those on the right were normal [Figure 3].

Lynch in 1966 described a CPF of the hard palate with an intact soft palate, which could not be explained by known embryological mechanisms.^[5] Karacan et al. reported a CPF without SCP, which healed spontaneously.^[6] Similar cases have been described by Jagannathan et al. and Rogers et al.^[3],[8]

Treatment is usually surgical; nonsurgical options like an obturator can be used when parents refuse surgery.^[8] The aims of surgery remain the separation of nasal and oral cavities, rearrangement of palatal muscles, and lengthening of the soft palate.^[2],[6] Reported repair techniques are variable and dependent on the surgeon's choice. They include closure with a unilateral mucoperiosteal flap and inferior pharyngeal flap,^[7] von Langenbeck's, and Furlow's repair techniques. Owing to its rarity, there is scant literature with respect to ideal repair. When associated with SCP, the recreation of the palatal sling is important to achieve velopharyngeal sufficiency.^[11]

We report the use of Bardach's palatoplasty technique for repair of CPF, to achieve straightforward closure of the fistula, along with intravelar veloplasty to reconstitute a soft palatal muscular sling.

Heterogeneity in the regulation of palatine fusion anteroposteriorly has been documented, resulting in focal alterations in the degeneration of epithelium and its adhesion, resulting in a cleft only in a part of the palate.^[3] Since there is abnormal musculature on one side, this fistula could not have occurred due to postfusion rupture, rather, is likely to be due to failure of mesodermal penetration. It is possible that mesenchymal penetration occurs earlier on one side, compared to the other. However, more research is needed to answer that question.

Conclusion

CPF is a rare entity. This case is even rarer given the unilaterality of muscular malpositioning. Repair in such cases can be performed using turnover flaps for nasal layer repair, intravelar veloplasty, and Bardach's repair for the oral layer.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2.	Eshete M, Camison L, Abate F, Hailu T, Demissie Y, Mohammed I, et al. Congenital palatal fistula associated with submucous cleft palate. Plast Reconstr Surg Glob Open 2016;4:e613.
3.	Rogers GF, Murthy A, Mulliken JB. Congenital fenestration of the palate: A case of embryologic syzygy. Cleft Palate Craniofac J 2006;43:363-6.
4.	Holenarasipurrangegowda NK. A rare case of Sub mucous cleft palate wit Palatal fistula - Case report and Literature review. University Journal of Surgery and Surgical Specialties. 2016;2.
5.	Lynch JB, Lewis SR, Blocker TG Jr. Cleft palate not explained by embryology. Plast Reconstr Surg 1966;38:552-4.
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