Cleft Palate Lateral Synechia Research Articles

Cleft palate lateral synechia syndrome: Inhereditary or not?

Cleft palate lateral synechia syndrome (CPLS) is a very rare syndrome characterized by a cleft palate, adhesions in the mouth roof, some parts of the tongue or the mouth floor. This is a report of a 12-week-old female infant presented for evaluation of cleft palate, with lateral synechia that caused the inability to fully open the mouth. Release of the synechia was performed without complications. Although some researchers believe CPLS is inherited in an autosomal recessive pattern, our case indicated that a new mutation may cause this situation. Further research is recommended to clearly determine its inheredited and phenotypic variablities.

Cleft palate lateral synechia syndrome: An opportunity for unique surgical closure

Objectives To report two cases of cleft palate lateral synechia (CPLS) syndrome in a single family and describe surgical closure using the synechia. Study design Case report and literature review. Methods A case report is presented with a review of the literature of cleft palate in conjunction with lateral synechia. Clinical presentation with photographic images of surgical repair is presented as well as a genetic workup with pedigree. Results A 6-week-old male presented for evaluation of a cleft palate. Mucosa-lined, fibromuscular tissue bands were noted connecting the floor of mouth and the free edges of a bilateral complete secondary cleft palate. No other craniofacial, digital, genital or limb defects were noted. The patient's 13-month-old sister had similar synechial bands and cleft palate at birth. Another older sibling had cleft palate without synechia. The patient's mother and maternal great grandmother had cleft palates at birth without synechia. The three children share a common mother but have three different fathers. Genetic analysis failed to reveal chromosomal defects or a mutation in the interferon regulatory factor 6 (IRF6) gene, a locus linked to Van der Woude syndrome. At 2 years of age, the index patient was growing and feeding well. His intra-oral bands remained intact and were incorporated in the surgical repair using a novel approach. Conclusions Since more otolaryngologists are performing cleft surgeries, the awareness of the differential diagnoses associated with a cleft palate is important. CPLS is an extremely rare condition. The report of this family supports the suspected pattern of autosomal dominant inheritance with variable expressivity. The unusual surgical approach will be discussed.

Congenital lateral cleft palate and lateral palatal synechiae

We report a sporadic case of congenital lateral cleft palate and lateral palatal synechiae and suggest that its mechanism is embryonal. This is the first published case that we know of with cleft palate-lateral synechiae syndrome including the lateral cleft palate.

A case report of fiberoptic intubation in neonate with cleft palate lateral synechia syndrome

A case report of fiberoptic intubation in neonate with cleft palate lateral synechia syndrome

Cleft palate lateral synechia syndrome: Review of the literature and case report

Cleft palate lateral synechiae syndrome is a rare syndrome consisting of cord-like interalveolar adhesions and concomitant hypoplasia of the mandible. We have presented an infant with CPLS syndrome and coincident hydrocephalus. Surgical division of the adhesions is necessary to allow normal feeding, avoid upper airway obstruction, and allow normal mandibular function and growth.

Familial occurrence of syngnathia congenita syndrome

A case of familial occurrence of syngnathia congenita in mother and child is described; this syndrome has been seen only sporadically until now. Due to the distinct appearance of the intraoral chords--they were situated on both sides between the ridges of the jaw and also between the left half of the bifid uvula and the retrolingual region--a relationship to the autosomal-dominantly transmitted Cleft Palate Lateral Synechiae Syndrome (CPLS-Syndrome) is suspected. Complete penetrance with different expressivity may explain the different clinical manifestations of the cases seen up to now and the absence of bifid uvula in the mother of our patient. The probable pathogenesis during embryological development is discussed.