A case report of acampomelic campomelic dysplasia and operative difficulties in cleft palate reconstruction.

Acampomelic campomelic dysplasia (CD) is a type of CD (CD; OMIM #114290), a rare form of congenital short-limbed dwarfism and is due to mutations in SOX9 gene family. Characteristic phenotypes of CD include bowing of the lower limbs, a narrow thoracic cage, 11 pairs of ribs, hypoplastic scapulae, macrocephaly, flattened supraorbital ridges and nasal bridge, cleft palate and micrognathia. The bending of the long bones is not an obligatory feature and is absent in about 10% of cases, referred to as acampomelic CD. A child previously diagnosed with acampomelic CD was brought to our outpatient clinic for cleft palate reconstruction. Our neurosurgeon cautioned us against performing surgery with extension of the neck in view of the possibility of producing quadriparesis, due to narrowing of the spinal canal as part of the osseous anomaly noted in the magnetic resonance imaging study of the spine, thus making the anaesthesia, surgical and post-operative procedures difficult. The cleft palate reconstruction was performed with all precautions and was uneventful.

Alternative approaches to managing the cleft alveolus.

The cleft alveolus component of the oral cleft deformity is addressed with a separate surgical stage. Several host and operator related factors affect the surgical outcome. When factors that increase the likelihood of secondary alveolar bone graft failure are identified, alterative methods like dentoalveolar distraction (DAD) may be employed. In infants, molding of the alveolar segments is possible and when a synergistic surgical approach is used, the possibility of successful alveolar cleft repair is increased. The authors present two case reports wherein the use of nasoalveolar molding (NAM) and DAD helped to tackle the alveolar cleft deformity.

Presurgical nasoalveolar molding assisted primary reconstruction in complete unilateral cleft lip palate infants.

BACKGROUND: Various methods have been described for the primary surgical reconstruction of the unilateral cleft lip and palate deformity (UCLP) in infants. There have been several attempts at restoring the normal anatomy of the nose at the time of lip repair in the affected individuals with varying degrees of success. Presurgical nasoalveolar molding (PNAM) is a presurgical infant orthopedic procedure that attempts to target the nasal deformity leading to a more esthetic surgical repair. OBJECTIVE: At our center we aimed to use PNAM to help in providing the surgical team with a better foundation for an easier and more esthetic single stage repair at the level of nose in addition to the lip and alveolus. METHOD: The infant nasal cartilages are amenable to correction in the first few weeks of life when they retain their plasticity. Three infants with complete unilateral cleft lip palate (CUCLP) were operated upon after a course of PNAM. No nasal stents were use after repair to retain the results. RESULTS: PNAM reduced the extent of the cleft deformity and improved the anatomic relationship between the affected structures. Postoperative recovery was uneventful. Subjective evaluation immediate post surgery and at the time of palate repair reveals adequate nasolabial esthetics. Long-term results of PNAM assisted repair are to be ascertained. CONCLUSIONS: The use of PNAM enables in reducing the severity of the deformity the surgical team has to tackle thereby enabling in a better and esthetic primary repair.