J Korean Assoc Oral Maxillofac Surg.  2022 Aug;48(4):232-236. 10.5125/jkaoms.2022.48.4.232.

A plunging ranula in a child with holoprosencephaly: a case of unique pathophysiology and difficult airway management

• Watanabe T
• Yokoyama A
• Shimizu S
• Bessho K

• KMID: 2532828
• DOI: http://doi.org/10.5125/jkaoms.2022.48.4.232

Abstract

A ranula is a pseudocyst that originates from the sublingual gland after trauma. Acute cases of ranulas that progress rapidly and cause respiratory distress are rare. Holoprosencephaly is a complex brain malformation caused by incomplete cleavage of the prosencephalon. Children with holoprosencephaly may experience upper airway obstruction due to the associated dentoalveolar malformations and oromotor dysfunctions. We present the case of an eight-year-old female patient with holoprosencephaly and a plunging ranula that manifested as an acute course due to difficult airway management. She required gastrostomy for oromotor dysfunctions related to feeding and swallowing and difficulty managing oral secretions. The sublingual gland and ranula were removed under general anesthesia. Postoperatively, urgent reintubation and close monitoring in the intensive care unit were required due to upper airway obstruction. We successfully managed the patient with close cooperation of a pediatrician and an anesthetist, and no recurrence was observed at the one-year follow-up. A ranula can be caused by trauma to the floor of the mouth in association with lingually inclined mandibular teeth, a type of dentoalveolar compensation seen in maxillary hypoplasia associated with holoprosencephaly. Careful consideration is needed in such cases since airway management can be difficult due to postoperative swelling and oromotor dysfunctions.

Keyword

Respiratory distress; Dentoalveolar malformation; Oromotor dysfunction

Figure

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  Fig. 1 A soft, fluctuant, and translucent mass with a diameter of 7 mm located on the left side of the floor of the mouth is seen.

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  Fig. 2 A fluctuant, translucent, and dome-shaped swelling with a diameter of approximately 30 mm located on the left side of the floor of the mouth is shown.

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  Fig. 3 A swelling is located in the left upper neck, and the overlying skin appears normal. The flat nose suggests nasal pyriform aperture stenosis.

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  Fig. 4 The intraoperative view after orotracheal intubation, showing a cleft palate.

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  Fig. 5 The intraoperative view before incising, showing a mass with reduced tension and size and mandibular teeth with lingual inclination.

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  Fig. 6 On the day after surgery, the patient showed remarkable facial swelling and remained intubated.

Reference

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