Features included hypoplastic lungs, cleft palate, retrognathia, micrognathism, small thorax, diaphragmatic hernia, distal limb hypoplasia, and early onset of polyhydramnios with premature delivery. Schwyzer et al. (1987) described an affected infant whose parents were second cousins. Moerman et al. (1988) described infant brother and sister with the syndrome of diaphragmatic hernia, abnormal face, and distal limb anomalies. Both died shortly after birth with severe respiratory distress. Ultrasonography demonstrated fetal hydrops, diaphragmatic hernia, and striking dilatation of the cerebral ventricles in both infants. Post-mortem examination showed Dandy–Walker malformation, ventricular septal defect, and renal cystic dysplasia. Cunniff et al. (1990) described affected brothers and 3 other cases, bringing the total reported cases of Fryns syndrome to 25. One of the affected brothers was still alive at the age of 24 months. Bilateral diaphragmatic hernias had been repaired on the first day of life. He required extracorporeal membrane oxygenation therapy for 5 days and oscillatory therapy for 3 months. Ventriculoperitoneal shunt was required because of slowly progressive hydrocephalus. Scoliosis was associated with extranumerary vertebral bodies and 13 ribs. Because of delayed gastric emptying, a gastrostomy tube was inserted. In addition, because of persistent chylothorax, he underwent decortication of the right lung and oversewing of the thoracic duct. Kershisnik et al. (1991) suggested that osteochondrodysplasia is a feature of Fryns syndrome. Willems et al. (1991) suggested that a diaphragmatic hernia is not a necessary feature of Fryns syndrome.
Histology
No irregularities in the dentin below invagination
Strains of vital tissue or fine canals that communicates with the pulp could be found
Enamel lining irregularly structured
External and internal enamel have different structures

Management
Preventative treatment - e.g. oral hygiene instructions, fissure sealant
Intentional replantation
Root canal treatment with mineral trioxde aggregate
Periapical surgery with retrograde filling
Extraction

References


== External links ==