1/15. Sporadic congenital infantile cortical hyperostosis (Caffey's disease).prognosis of congenital infantile cortical hyperostosis (Caffey's disease) is poor particularly in premature babies. Two cases are presented of congenital Caffey's disease in premature babies. The first baby was hydropic at birth and had cortical hyperostosis involving the mandible and long bones of right upper limb and both lower limbs. The second baby had cortical hyperostosis of the nasal bones causing severe nasal nonchoanal stenosis that needed surgery, in addition to involvement of long bones of the four extremities. Both babies recovered from the disease and were discharged home well. These cases suggest that the improved outcome of congenital of infantile cortical hyperostosis may reflect improvement of neonatal mechanical ventilation and availability of neonatal total parenteral nutrition.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
2/15. Recurrent Caffey's cortical hyperostosis and persistent deformity.A boy, age 12 years, who had infantile cortical hyperostosis has continued to have occasional aches and new cortical thickenings in his arms and legs. His mandible is undergrown and his ribs have an abnormal slope. recurrence of Caffey's cortical hyperostosis and persistent deformity have been observed in other children and young adults. Some unexplained cases of late cortical thickening and pain may be due to Caffey's disease.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
3/15. Chronic Caffey's disease: an uncommon entity in children.Chronic Caffey's disease in an uncommon condition in children is characterized by an acute inflammatory reaction in the periosteum along with systemic disturbances. A 30 months old boy was reported in the pediatric unit of BSMMU, Dhaka about two and half years back with the complaints of multiple painful soft tissue swelling in different parts of the body since birth and delay in growth and development. The child was found well and alert, moderately pale, febrile with hard, tender swelling of mandible on both sides. There were multiple swellings over the right arm, forearm, both thighs and bowing of the lower limbes. Investigations revealed normal serum calcium and phosphate level with mild elevation of alkaline phosphatase. Radiological findings showed periosteal new bone formation in mandible and long bones. There was diaphyseal expansion of the long bones with expansion of the ribs anteriorly. He was diagnosed as a case of chronic caffey's disease on the basis of history, clinical examination and investigation.- - - - - - - - - - ranking = 2keywords = mandible (Clic here for more details about this article) |
4/15. Prenatal infantile cortical hyperostosis (Caffey's disease): a 'hepatic myeloid hyperplasia-pulmonary hypoplasia sequence' can explain the lethality of early onset cases.BACKGROUND: Infantile cortical hyperostosis (ICH) is benign and self-limiting when it presents near or after birth but is usually lethal when it presents earlier. methods: We present the clinical, ultrasonic, radiographic and pathologic findings in an instructive case of early onset prenatal ICH. RESULTS: A 40-year-old G2P1 woman delivered spontaneously at 22 weeks' gestation. Prenatal ultrasounds showed rib and mandibular abnormalities as well as short humeri. Post-mortem radiographs showed asymmetric hyperostosis in long bones, mandible, scapulae and pelvis with sparing of spine, hands, feet and skull. The affected skeleton showed marked bony sclerosis and ballooning of the diaphyses of the long bones with periosteal sclerosis. A complete autopsy showed characteristic histologic findings in affected bones. Previous reports at 20 weeks have described anasarca, fetal hydrops, hepatomegaly, and pulmonary hypoplasia. In our case, there was no hydrops/anasarca; hepatomegaly, due to massive extramedullary hematopoiesis with marked myeloid hyperplasia, combined with ribcage abnormalities, caused mild pulmonary hypoplasia. CONCLUSION: We hypothesize that early onset fetal ICH is usually lethal because massive hepatic myeloid hyperplasia sequentially causes: (1) hepatomegaly and, in conjunction with rib abnormalities, mild pulmonary hypoplasia, (2) sinusoidal and pre-sinusoidal portal hypertension followed by ascites/hydrops, and (3) ascites/hydrops-induced severe pulmonary hypoplasia.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
5/15. Familial Caffey's disease and late recurrence in a child.Cortical Infantile hyperostosis (Caffey's disease) is a benign and self-limited disorder. Late recurrence or persistence of symptoms with deformity seems exceedingly rare. Its occurrence in isolated cases or in multiple members in families suggests the existence of two different forms, namely a sporadic form and a familial form. The tibia is the predominant bone known to be affected in the familial form, while the mandible is mostly affected in the sporadic form. The different pathological processes found in these two forms add further to the heterogeneity of this disorder. We describe an Arabic-Christian, non-consanguineous family with two affected sibs. In one, recurrent Caffey's disease was diagnosed at the age of 11 years.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
6/15. Autosomal dominant osteosclerosis.Two cases of a craniotubular hyperostosis are presented. The radiographic features closely resemble Van Buchem disease (hyperostosis corticalis generalisata; endosteal hyperostosis, recessive type), including symmetrical and bilateral diaphyseal cortical thickening of the long and short tubular bones as well as sclerosis and thickening of the calvaria, mandible, shoulder and pelvic girdles, and thoracic cage. Unlike Van Buchem disease, no periosteal excresences are observed, alkaline phosphatase is normal, no basal foramina encroachment of the skull is present, and the genetic pattern is dominant inheritance. These cases possibly represent a separate disorder rather than a variant of Van Buchem disease.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
7/15. hyperostosis corticalis generalisata: surgical management and long-term follow-up of one patient.A 24-year-old woman presented for treatment of her distorted facial appearance. She showed marked widening of the face and skull, which had first become noticeable in childhood. Significant thickening of the cortical bone was seen radiographically throughout the skeleton. Routine laboratory and endocrinological tests showed normal results. These findings, together with a family history of bone disorder, led to the diagnosis of hyperostosis corticalis generalisata. The lower border of the mandible was resected, resulting in improved facial appearance. During the 8-year follow-up, no changes were seen with regard to the mandible.- - - - - - - - - - ranking = 2keywords = mandible (Clic here for more details about this article) |
8/15. Infantile cortical hyperostosis with unusual clinical manifestations.Infantile cortical hyperostosis may mimic a variety of serious conditions in early infancy, such as trauma, vitamin deficiencies, infection, or tumors, In a patient in good general condition there are typical roentgenological features often with involvement of the mandible that are diagnostic of the condition. In 3 cases of infantile cortical hyperostosis (Caffey's disease), one had the clinical picture of Erb's palsy and subsequently developed hyperostosis of the scapula. The second, had a "pulled elbow" and radial nerve palsy and later developed hyperostotic changes in the humerus and radius on the same side. The third was referred for treatment of congenital torticollis and found to develop hyperostosis of the clavicle on the same side. The recognition of this condition obviates unnecessary prolonged investigation and treatment.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
9/15. Caffey's disease: a case originating in the first metatarsal and review of a 12 year experience.This paper describes an infant with Caffey's disease who presented with swelling of the first metatarsal shown as hyperostosis histologically. He then developed scapular sclerosis and new bone formation. The mandible became involved 26 days after onset. A review of a 12 year experience with Caffey's disease (33 patients) disclosed a definite decrease in the number and percentage of patients seen since 1968.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
10/15. Infantile cortical hyperostosis of the ribs (Caffey's disease) without mandibular involvement.Infantile cortical hyperostosis (ICH), or Caffey's disease, first reported by Caffey and Silverman in 1945, is a benign condition characterized radiographically by corticoperiosteal thickening of bone with subperiosteal new bone formation. Sites of occurrence vary, with the mandible being involved in 75%-80% of cases. The following is a case report of ICH limited to four contiguous ribs with no evidence of mandibular involvement.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
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