Congenital fibrous fusion of the jaws (synechiae) with cleft palate: A case report

1 Background

Congenital maxillomandibular fusion is a rare disorder. This fusion defect can present with a wide range of severities, ranging from mucosal band (synechiae) to complete bony fusion (syngnathia) [1].

Synechiae is a rare yet challenging defect- having cosmetic and functional implications. The fusion is usually due to soft tissue adhesions [1–3]. It is generally observed together with other anomalies as a part of syndromes, such as Van der Woude syndrome [4]. Around 50 cases have been documented in the literature so far. Early division of fibrous bands not only allows effective feeding but also prevents the development of facial deformities [5].

Here we present a case of congenital maxillomandibular fusion with a cleft palate in a male neonate.

2 Case report

A 3-day-old full term male neonate (2760 grams) was referred to our unit with a complaint of inability to feed and restricted mouth opening following an unremarkable pregnancy and normal vaginal delivery. Apgar scores were 8 and 9 at 1 and 5 minutes after birth with normal cord gas. He was being fed orally via a small orogastric tube. There was no significant family history of structural or functional abnormalities in the family. However, the parents did have a third-degree consanguineous marriage.

The neonate was presented with a orogastric tube, breathing in room air with stable vital signs.

Intraorally, there was soft tissue fusion of maxillary and mandibular gum pads with multiple fibrous bands present in the posterior region; the thickness of the bands was approximately between 2 and 4 mm. (Fig. 1). A detailed intraoral examination could not be carried out due to the restricted mouth opening. However, on limited examination, all other oral structures seemed to be normal except a very small posterior narrow cleft palate.

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Fig. 1

Restricted mouth opening with fibrous maxillomandibular synechiae.

The baby was thoroughly evaluated for other anomalies- no other anomalies noted. Routine investigations including full blood count were normal. Brain and abdominal ultrasounds were normal, skeletal survey and 2D Echo were normal.

Excision of soft tissue bands was planned under local anesthesia and sedation. General anaesthesia was eliminated by anaesthesia team due limited mouth openings.

The patient was sedated by an anaesthetist using midazolam intravenously, then local anesthesia was infiltrated in the area of fibrous bands, and all the fibrous bands were released. High-pressure suction was attached to prevent blood aspiration (Fig. 2).

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Fig. 2

Excision of bands under high power suction (during excision).

After incision of the fibrous bands, the index fingers of both hands were inserted in between the alveolar ridges, and mouth opening was achieved. A thorough intraoral examination revealed all structures, including the uvula and tongue, to be normal with a very small posterior cleft palate. Complete hemostasis was obtained by applying finger pressure for a few minutes, then a gauze piece was placed in between the alveolar ridges and secured in place to ensure hemostasis in the immediate post-operative period and to prevent reattachment of the raw surfaces.

The patient was monitored in the NICU for 48 hours, then discharged with the advice to use a pacifier for 10 minutes every hour for the next 4 days to avoid reattachment of the cut surfaces until complete epithelization could take place, patient achieved adequate mouth opening and was able to breastfeed, and cry normally. The posterior cleft palate was very small and narrow. it was seen by paediatric surgeon and was planned to follow up in outpatient clinic. Uneventful complete healing occurred in one week (Fig. 3). The patient was given a follow-up appointment with paediatrics, paediatric surgery and occupational therapist clinic, he was re-evaluated two weeks later, and examination and feeding were normal.

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Fig. 3

Picture of the patient (after excision).

Congenital adhesions (synechiae) of the oral cavity are rare. They arise between the upper and lower alveolar ridges (syngnathism) or between the tongue and palatal margins (glossopalatal ankylosis). These adhesions consist of membrane bands of epithelium supported by various amounts of connective tissue, muscle, or bone [5, 6]. Alveolar adhesions are rarely seen in isolation and are often accompanied by additional congenital defects, such as a cleft lip or palate, microglossia, micrognathia, A cleft palate is frequently seen with alveolar fusion [7].

In a review of 50 cases of alveolar synechiae between 1990 and 1993, Gartlan et al. [7] found only 7 cases of isolated alveolar fusion; the remainder were associated with syndromes such as Van der Woude, cleft palate alveolar synechiae, and oromandibular limb hypogenesis syndromes [8, 9].

The etiology of synechiae is unknown. It is postulated that during the 7th to 8th weeks of embryological alveolar ridge development, when the tongue and palatal shelves are in close contact, the ensuing palatal closure depends on downward and forward contraction of the tongue; failure of the tongue to protrude predisposes to alveolar fusion. Genetic, teratogenic, or physical insults during this period may also lead to prolonged contact between oral structures, thereby leading to abnormal fusion.

Others feel that adhesions may be remnants of the buccopharyngeal membrane. Fhurmann et al. [10], 1972, found a hereditary link in five family members with cleft palates and synechiae.

In all cases, surgical division of adhesions was necessary for normal feeding, preventing airway obstruction, and allowing normal mandibular function and growth. The sooner treatment is rendered, the less the possibility of temporomandibular joint ankylosis; ankylosis can lead to a lack of mandibular growth and facial deformities. It should be noted that, although surgery is simple, general anesthesia is often difficult [1].

Fine fiberoptic laryngoscopes are required for nasoendotracheal intubation. Another point to be mentioned is that normal-range mouth opening often cannot be obtained immediately after incising the adhesions and forceful manipulation. The temporomandibular joints are stiffened due to disuse, the alveolar ridges are soft, and infantile bone is fragile; thus, excessive force may easily lead to jaw fracture and should be avoided. Normal mouth opening will automatically be regained within 1-2 weeks postoperatively [3].

4 Conclusion

Isolated fusion of the gums is a very rare congenital anomaly. Early surgical treatment is necessary to avert feeding problems. However, anesthesia for this procedure is challenging.

Ethical approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the parents of the newborn for the publication of this case and the accompanying images. A copy of the written consent is available for review.

Data availability

Data were collected from medical files and are not publicly available due to patient confidentiality but are available through the corresponding author under clearly justified academic requests.

Conflict of interest

The authors declare that they have no competing interests.

Funding

None received.