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1/55. 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.
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2/55. 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.
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3/55. A case of infantile cortical hyperostosis.

    Infantile cortical hyperosteosis (ICH) is usually a self-limited disease of infancy with bony changes, soft tissue swelling, fever, irritability, decreased appetite, and decreased movement of the affected bones. Its description in isolated patients or in multiple members of families suggests the existence of two different forms, namely a sporadic form and a familial form with incomplete penetrance. In this article, we report a 2.5-month-old girl with ICH of sporadic form, due to unusual presentation.
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4/55. Lethal prenatal onset infantile cortical hyperostosis (Caffey disease).

    We describe a sporadic case of lethal prenatal onset infantile cortical hyperostosis (Caffey disease), which resulted in early postnatal death at 30 weeks gestation. The mother presented with antepartum haemorrhage and preterm labour. She was found to have polyhydramnios. The infant showed extensive symmetrical diaphyseal subperiosteal cortical thickening throughout the skeleton with short extremities. hepatomegaly and lung hypoplasia were present. Currently, in the absence of a specific marker, diagnostic ultrasound offers the only prospect of prenatal diagnosis. This diagnosis should be considered in infants with short angulated long bones, where the diaphyses are irregular and echodense, and where there is no sign of fractures.
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5/55. Antenatal onset of cortical hyperostosis (Caffey disease): case report and review.

    We report on a fetus of 27 weeks of gestation whose clinical, radiological, and histopathological findings are compatible with the prenatal form of Caffey disease (cortical hyperostosis). Prenatal ultrasound examination showed polyhydramnios and markedly short and angulated long bones, which had led to the incorrect diagnosis of lethal osteogenesis imperfecta. We found 43 published descriptions on the antenatal onset of cortical hyperostosis symptomatic in utero or at birth. Two groups of prenatal cortical hyperostosis (PCH) can be distinguished: (1) severe (25 reports and the fetus presented here), with onset before 35 weeks of gestation and generally associated with polyhydramnios, lung disease, prematurity, and high lethality; (2) mild (18 reports), with onset after 35 weeks of gestation and without complications. Autosomal recessive inheritance has been suggested for the prenatal form of cortical hyperostosis. However, the available evidence suggests that both dominant and recessive inheritance is possible. Moreover, dominant inheritance seems to be more common in the mild prenatal form of cortical hyperostosis.
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6/55. Infantile cortical hyperostosis.

    Infantile cortical hyperostosis (Caffey disease) is characterized by radiological evidence of cortical hyperostosis, soft tissue swellings, fever and irritability. We report a case of Caffey disease highlighting its presentation as pyrexia of unknown origin, appearance on radionuclide bone scintigraphy and our unsatisfactory experience of treating it with ibuprofen, a prostaglandin inhibitor.
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7/55. 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.
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8/55. Lethal infantile cortical hyperostosis.

    We present an unusual case of the rare occurrence of lethal infantile cortical hyperostosis (Caffey disease). Clinical and Pathological aspects are discussed.
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9/55. 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.
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10/55. Generalized cortical hyperostosis (Van Buchem disease): nosologic considerations.

    Six of 41 presumed cases of Van Buchem disease described in the literature fit uniform diagnostic criteria. Segregation analysis of these 6 cases, in addition to another the authors report, supports a recessive mode of inheritance. Genetic heterogeneity is confirmed by the demonstration of a dominantly-inherited phenotype resembling Van Buchem disease. The probable etiology is a defect in the endochrondral modulatory step regulating transformation of osteoclast to osteoblast.
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