Fibrochondrogenesis is a rare autosomal recessive form of osteochondrodysplasia, causing abnormal fibrous development of cartilage and related tissues.It is a lethal rhizomelic (malformations which result in short, underdeveloped limbs) form of dwarfism, exhibiting both skeletal dysplasia (malformations of bone) and fibroblastic dysplasia (abnormal development of fibroblasts, specialized cells that make up fibrous connective tissue, which plays a role in the formation of cellular structure and promotes healing of damaged tissues). Death caused by complications of fibrochondrogenesis occurs in infancy. Presentation
Fibrochondrogenesis is a congenital disorder presenting several features and radiological findings, some which distinguish it from other osteochondrodysplasias. These include: fibroblastic dysplasia and fibrosis of chondrocytes (cells which form cartilage); and flared, widened
long bone metaphyses (the portion of bone that grows during childhood).Other prominent features include dwarfism, shortened ribs that have a concave appearance, micrognathism (severely underdeveloped jaw), macrocephaly (enlarged head), thoracic hypoplasia (underdeveloped chest), enlarged stomach, platyspondyly (flattened spine), and the somewhat uncommon deformity of bifid tongue (in which the tongue appears split, resembling that of a reptile). Cause
The cause of platyspondyly in fibrochondrogenesis can be attributed in part to odd malformations and structural flaws found in the vertebral bodies of the spinal column in affected infants.Fibrochondrogenesis alters the normal function of chondrocytes, fibroblasts, metaphyseal cells and others associated with cartilage, bone and connective tissues. Overwhelming
disorganization of cellular processes involved in the formation of cartilage and bone (ossification), in combination with fibroblastic degeneration of these cells, developmental errors and systemic skeletal malformations describes the severity of this lethal osteochondrodysplasia.
Intrathoracic space competition from heart enlargement and pulmonary edema (complications of CHF) that compress airway construction and bronchioles. Bronchial obstruction secondary to intraluminal edema. Bronchial mucosa edema. Diagnosis
The diagnosis of cardiac asthma is accomplished through workup of congestive heart failure, complete with:

Evaluation of current symptoms with specific consideration of chronological progression or worsening. Past Medical History screen with consideration for conditions that predispose to heart failure, such as prior heart attack, history of coronary revascularization, high blood pressure, or diabetes (heart). Physical Exam with emphasis on listening for abnormalities of heart sound, abnormalities of lung sound, or presence of increased fluid retention in neck or in extremities. Laboratory Evaluation with specific interest in B-type natriuretic peptide levels. Electrocardiography to evaluate for irregularities in heart rhythm. Chest Radiograph to evaluate for presence of lung congestion or increased heart size. Echocardiography to evaluate heart function. Echocardiography is the preferred choice for diagnosis of heart failure in patients.As well as evaluation of lung function via:

Pulmonary function testing (PFT) complete with bronchoprovocation testing. PFTs represent the preferred method for evaluating for bronchial asthma. Management
Treatment of asthma symptoms in CHF patients is directed towards optimizing the patients cardiovascular status and correcting potential oxygen deficit. Current recommendations in acute asthma symptoms are utilization of diuretics such as furosemide, venodilators such as nitroglycerin, and morphine. The initial strategy should focus on decreasing patient fluid retention with diuretic therapy, thereby decreasing cardiac preload and overall fluid load in pulmonary circuit (pulmonary congestion).