Dental Management of a Rare Case of Cornelia de Lange Syndrome
ZJ03-ZJ04
Correspondence
Dr. R Krishna Kumar,
Professor, Department of Paediatric and Preventive Dentistry, Annoor Dental College and Hospital, Muvattupuzha-686673, Kerala, India.
E-mail: drkrishnapedo@gmail.com
A six-year-old female patient with Cornelia de Lange Syndrome (CdLS) presented to the Department of Paediatric and Preventive Dentistry with multiple mutilated teeth and an inability to chew, resulting in low food intake. The patient complained of nocturnal pain and disturbed sleep. A behavioural analysis of the child showed that she was introverted, had difficulty in interacting with other children or strangers, and exhibited distinctly negative behaviour, as per Frankel’s behaviour rating scale.
Extraoral findings included facial dysmorphism, a triangular face, low frontal hairline, thick and highly arched eyebrows, widely spaced eyes (hypertelorism) with mild epicanthal fold, a shallow and protruded philtrum, and a wide mouth with downward-slanting corners (Table/Fig 1),(Table/Fig 2). No skeletal deformities were noted. These facial findings were suggestive of CdLS.
Intraoral findings included narrowed mandibular and maxillary arches, a high-arched palate, and crowding of teeth (Table/Fig 3),(Table/Fig 4). Dental caries were observed in relation to 52, 62, and 63, with deep dental caries in relation to 55, 65, 64, 51, 52, 74, 75, 84, and 85. Due to the child’s inability to cooperate during dental treatment because of her mental disability and difficulties in managing her behaviour, it was decided that full mouth rehabilitation would be done under General Anaesthesia (GA). The major challenge in performing the procedure under GA was the child’s airway features, specifically micrognathia, restricted mouth opening, receding chin, and stiff neck. These features hindered the visualisation of the vocal cords during laryngoscopy and Endotracheal Tube (ETT) placement. To manage the narrow airway of the patient, a modification in the Ryles tube was performed. An Oral/Nasal Endotracheal Tube (ONETT) was used in the present study. The family was fully informed about all clinical procedures, and the parents signed an informed consent form before the treatment.
Pulpectomy was performed on teeth 55 and 65, followed by the placement of Stainless Steel Crowns (SSCs) (Table/Fig 5). Silver Diamine Fluoride (SDF) was applied, followed by the Hall technique in relation to teeth 54 and 64. Additionally, SDF application was done with respect to teeth 52, 53, 62, and 63, and extractions were performed for teeth 51, 61, 74, 75, 84, and 85. Immediate post-operative images of the mandibular arch could not be taken due to profuse bleeding. A follow-up image has been added as (Table/Fig 6). The patient was followed-up after six months and one year, showing an improvement in dental hygiene and no new caries. Thus, there was an overall improvement in the quality of life and general health of the patient.