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At a glance

A syndrome characterized by multiple intrabuccal/interalveolar synechiae associated with craniofacial malformations.

Synonyms

CPLS Syndrome; Cleft Palate and Congenital Lateral Alveolar Synechia Syndrome.

Incidence

Extremely rare genetic disorder (<20 cases have been described).

Genetic inheritance

Autosomal dominant.

Diagnosis

Based on the typical clinical findings.

Clinical aspects

Patients present with growth and mental retardation and hypotonia. Head and neck anomalies include microcephaly, malar hypoplasia, lacrimal abnormalities, beaked nose, short philtrum, protruding lips, cleft palate, hypoplastic or bifid uvula, micrognathia/retrognathia, and multiple, “cord-like,” intraoral adhesions. These synechiae are variable with regard to their anatomical location, thickness, and tissue composition (from thin muscosal to bony). They may connect the maxillary with the mandibular alveolar ridge, the edges of the palatal cleft with the lateral parts of the tongue, the hard palate with the floor of the mouth, and/or the hard palate with the lower lip. Synechiae from the soft palate to the tongue with loss of the continuity between oral cavity and pharynx have also been described. The adhesions restrict fetal facial growth and result in elevation of the floor of the mouth and tongue.

Precautions before anesthesia

Assess if there is any mouth opening and try to localize the synechiae with regards to direct laryngoscopy (most likely impossible) and fiberoptic intubation. However, nasal intubation is most likely the approach of choice, also because of enoral surgery, thus try to assess patency of the nasal passage. The child may suffer from breathing and/or feeding difficulties. An anticholinergic drug should be given before induction of anesthesia to decrease oral secretions and ease airway management and nasal application of a vasoconstrictor will help reducing the risk of bleeding during nasal intubation attempts. Developmental delay may cause agitation and stress in the perioperative period, thus anxiolytic and sedative premedication and/or presence of a parent (or primary caregiver) for induction of anesthesia may be beneficial.

Anesthetic considerations

The described features suggest that airway management (direct laryngoscopy and tracheal intubation) will most likely be very difficult or impossible due to severely restricted mouth opening and obstructed pathway for the laryngoscope blade. Maintenance of spontaneous ventilation is recommended until the airway has been secured. Alternative airway management options should be immediately available (eg, supraglottic airway device, fiberoptic bronchoscope) and adherence to an established difficult airway algorithm is advised. Emergent release of the synechiae or tracheostomy may be required in cases with breathing/ventilation difficulties. Depending on the extent of the adhesions, local anesthesia with mild sedation should also be considered. A surgeon familiar with surgical airway management and the necessary equipment should easily be available in the operating room. Awake nasal fiberoptic intubation seems to be the technique of choice. Blind nasal intubation has also been ...

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