Trifid Epiglottis with Midline Cleft Lip: A Rare Craniofacial Anomaly—First Case Report

Abstract

Congenital anomalies involving the epiglottis are an uncommon occurrence and are seldom reported as isolated anomalies. Bifid epiglottis, by itself, is also a rare occurrence. Trifid epiglottis has only been reported once in literature to the best of our knowledge. Epiglottic anomalies are observed commonly as part of a syndrome or a sequence, mostly in association with facial dysmorphic features, cleft lip and palate, polydactyly, and skeletal abnormalities among others. We report to you the case of a 10 year-old child having a triad of clinical features consisting of a trifid epiglottis, and a median cleft lip with associated polydactyly.

Introduction

A true bifid epiglottis is defined as a cleft in the epiglottis that extends at least two third of its length. An accurate diagnosis of the bifid or trifid epiglottis can definitively be made only when this criterion is met [1]. Patients generally present with symptoms of laryngomalacia with inspiratory stridor and airway obstruction in association with other features of a syndrome. Other malformations usually observed along with cleft in epiglottis are digital anomalies, hypopituitarism, and hypothalamic hamartoma. These combinations can be explained by the concurrent development of epiglottis, digital bud and hypothalamus that is between the gestation periods of 32–46 days [2]. Development of the lip also takes place during the same period of intrauterine life (6th week). Our patient was posted for surgical correction of the cleft lip. The epiglottic anomaly was discovered during laryngoscopy for tracheal intubation. We therefore present a case with a triad of features, including a trifid epiglottis, polydactyly, and a midline cleft of lip.

Case Report

A ten-year-old male patient presented with a median cleft of the upper lip (Tessier #0 cleft) (Fig. 1). His younger brother, aged 8 years, also had a midline cleft lip but had none of the other anomalies that were observed in our patient. The parents had a second-degree consanguineous marriage. The patient was born of full-term normal delivery.

Fig. 1.

Ten-year-old boy presented with a median cleft of the upper lip

He had no history of previous surgeries. He was of an average build and was well-nourished. On examination, the patient exhibited normal gait. He had postaxial polydactyly (Type A) in the upper and lower left limbs (Fig. 2) and a bony deformity at the right wrist and also on the left rib. The midline cleft in the upper lip extended to half of the length of the lip. No other intra-oral anomaly or facial deformity was observed.

Fig. 2.

Bony deformities in limbs

Correction of the cleft lip under general anaesthesia was planned. After induction of anaesthesia, direct laryngoscopy was performed for tracheal intubation.The abnormality in the epiglottis was noticed at this point (Fig. 3). The epiglottis was divided into three folds, two prominent folds on either side of one bulbous structure in the middle, making it a trifid epiglottis. Ventilation was maintained with bag and mask for some time and Video laryngoscopy was performed after return of spontaneous respiratory efforts to study and record the epiglottic anatomy more clearly and to assess any possible difficulty in intubation.

Fig. 3.

Abnormal epiglottis

The epiglottis movements were within normal limits and showed no stridor. Tracheal intubation was performed after deepening the plane of anesthesia. The surgical correction of the cleft lip was done after administration of local anesthesia consisting of Lignocaine 2% with adrenaline 1:100000, with an inverted “V” incision. Orbicularis oris was then dissected on a superficial and deep aspect which was extended laterally approximately 1.5–1 cm on either side of the midline. The abnormal muscular attachment was separated from the base of the nose. The median fibrotic band was excised, all bleeding points cauterized and muscle back cuts were done at the vermillion area to rotate both sides of the muscles to achieve a more favourable length and position, subsequently, the free end of the muscles was approximated using 4–0 vicryl. An inverted V–Y closure of the skin was planned for the case as this would help achieve the desired length of the repaired lip and accommodate the skin defect created after muscle closure. The skin markings were done with the base at the lowest point of the cupid's bow on with sides, and the marking was joined at a point approximately 4 mm below the columella making it an inverted V. The vertical limb from the V marking extended to the base of the columella. The skin closure was done using 6–0 Prolene enabling the broad base of the V to fit into the defect. No intraoperative or post-operative surgical complications were noticed and the wound healed well with satisfactory cosmetic results. There was no anesthetic complication during the surgery or during extubation, and the patient recovered uneventfully.

Discussion

The epiglottis is a flap of soft tissues and cartilage located just above the vocal cords to help prevent food and irritants from entering the lungs. Hence, any anomaly in the epiglottis is usually presented with other problems such as inspiratory stridor, obstructive sleep apnea, feeding difficulties or respiratory compromise [7]. Epiglottic anomalies are also mostly reported as a clinical presentation of a syndrome such as Bardet-Biedl syndrome [3], Pallister-Hall syndrome [4], and Joubert’s syndrome [5]. A cleft in the epiglottis has been an enigma, primarily because of its rare occurrence but also due to the debate in the literature over embryology. Embryologists agree that the main predecessor to the epiglottis is the hypobranchial eminence, although there are few questions related to the aryepiglottic folds [6].

Soulie and Bradier in 1907 put forth that the “hypobranchial eminence is of bilateral origin”, while Frazer proposed it was a midline structure and was the only source of the epiglottis. Roth proposed an idea that “the epiglottis originates both from a medial swelling (hypobranchial eminence) and two lateral swellings (fourth brachial arches)”; However, Kallius suggested that “the lateral swellings give rise only to the aryepiglottic folds”. Thus, the exact origin of the epiglottis remained in question [7].

Yukoi Katori et al., in a study, investigated 18 fetuses in their mid-term at around 7–25 weeks of gestation. They suggested an epithelial-mesenchymal interaction in the development of the epiglottic cartilage. The thyroid-epiglottic ligament seems to develop from the glial fibrillary acidic protein (GFAP)—positive mesenchymal condensation, and the hyo-epiglottic ligament originates from the lingual muscle fasciae [6]. Common syndromes associated with bifid epiglottis are Pallister-Hall syndrome [4], Bardet-Biedl syndrome [3], and Joubert’s syndrome [5]. Although none of these syndromes have a feature of Cleft lip or palate, which is present in our case.

Bifid epiglottis is more commonly reported than trifid epiglottis. Trifid epiglottis, to the best of our knowledge, has been reported only once before in the literature [1]. The patient in their case presented with mild stridor soon after extubation which was not in the case of our patient. Abnormality in our patient was detected accidentally during intubation for the surgical correction of cleft lip and palate. Graham et al. in their report on three cases with the bifid epiglottis, out of which two cases were suspected as having Pallister-Hall Syndrome (PHS) out of these two cases one case had both Poly(syn) dactyly and cleft palate. The other clinical features in above mentioned case are hypothalamic hamartoma, micrognathia, kidney disease, flat nasal bridge, dislocated hips, short nose and limbs, simian creases, and pulmonary hypoplasia [8, 10]. Goldenberg et al. reported a case with bifid epiglottis, cleft palate, and poly(syn)-dactyly also showing other clinical features such as hypopituitarism, micropenis, midfacial hypoplasia, and cryptorchidism [9, 10]. Bamforth et al.also reported a case of two brothers with cleft palate and bifid epiglottis [11].

The same symptoms and features as seen with patients with a bifid epiglottis [1] could also be observed with a trifid epiglottis, could be even worse because of the wider gaps between the three parts of the epiglottis. The patient in this report was posted for surgery requiring general anesthesia and tracheal intubation. All cleft lip and palate cases pose a challenge to the anaesthesiologist because of the airway sharing with the surgeon. An epiglottic anomaly could add to these challenges. This could include stridor pre-intubation or post-extubation, a risk of aspirated gastric contents, trauma to the abnormal epiglottic structures during direct laryngoscopy for tracheal intubation, etc. If the diagnosis of the epiglottic anomaly is made before the commencement of anesthesia, the anaesthesiologist could be prepared for these above incidents and be able to manage them better, should they occur. In this case report, we have found a trifid epiglottis in association with a midline cleft of the lip and polydactly. If all patients showing these two other features together are evaluated for an epiglottic anomaly as a part of pre-surgical/pre-anaesthetic investigations, then the possible ill-effects of an incidental discovery and complications arising thereof could be mitigated.

Conclusion

A trifid epiglottis has been documented only once in the literature, making this the second known case and the first associated with both a midline cleft lip and polydactyly. While our patient had no symptoms and the anaesthesia process, including intubation and extubation, was uneventful, the potential for complications like aspiration or respiratory issues cannot be ruled out in similar cases. Managing anaesthesia in patients with cleft lip or palate is already challenging due to the altered anatomy, and the presence of a trifid epiglottis could add further complexity. Therefore, patients presenting with polydactyly and a cleft lip should be carefully evaluated for a trifid epiglottis to mitigate the risk of airway complications during or after anaesthesia.

Funding

None.

Declarations

Conflicts of interests

None.

Informed Consent

Informed consent was obtained from the participant in the study.