Juvenile polyposis. A report on two cases (author's transl)]

The discovery of multiple polypi in a small child does not necessarily indicate that this is a case of familial adenomatosis. Two cases of juvenile polyposis were confirmed by histological examinations, and their clinical and, more particularly, their radiological characteristics were studied. The only way to explore the full extent of the lesions in a satisfactory manner is by double-contrast examination of the whole colon. The authors stress the value of radiological examinations, though the frequency with which they should be carried out is open to discussion. Review of the published literature reveals that the association of true familial polyposis or adenocarcinomatosis exists in the same family or even in the same patient. Diagnostic difficulties are usually due to the fortuitons co-existence of a lymphoid hyperplasia, the significance of which is still not known.     

Filiform polyposis

Filiform polyposis presents a characteristic radiographic appearance consisting of long, filamentous filling defects in an otherwise normal colon. It has previously been reported in patients with a prior history of ulcerative colitis and in one patient with granulomatous colitis. The authors document its development in patients with ulcerative and granulomatous colitis. The authors have also seen a solitary filiform polyp in a patient with previously undiagnosed inflammatory bowel disease, and filiform polyps in the stomach in a patient with documented Crohn disease involving the small bowel. It is believed that this represents a nonspecific sequela of diffuse mucosal inflammation; it should not be mistaken for a neoplastic form of polyposis.     

Juvenile osteochondritis dissecans

Juvenile osteochondritis dissecans (JOCD) has been a recognized entity for more than 100 years. Despite our long recognition of OCD, the natural history and most effective therapies are poorly understood. Although conclusive evidence of an exact cause is lacking, there is widespread agreement that JOCD is related to repetitive trauma. Patients with JOCD present with vague pain and occasionally, mechanical symptoms. The diagnosis of JOCD can be confirmed on plain radiographs. Magnetic resonance imaging has emerged as the study of choice to evaluate the stability of the lesion and integrity of the overlying articular cartilage. Treatment decisions are based on the stability of the lesion. Stable JOCD lesions should be treated initially with activity modification and possibly, immobilization. Unstable lesions and stable lesions not responding to an initial course of nonoperative therapy should be surgically treated. Surgical treatment is based on the radiographic and arthroscopic characteristics of the lesion. Multiple techniques from simple arthroscopic drilling and fixation to salvage techniques for cartilage restoration are discussed.     

Colonic polyp patterns in familial polyposis

The diagnosis of familial polyposis depends on there being more than 100 adenomatous polyps in the large bowel. The polyps are the result of intramucosal microadenomatous growth. The age at which this occurs varies, and in the early stages of polyp development relatively few larger polyps may be seen. The numbers and size of the polyps as seen on double-contrast barium enema were compared with the macroscopic findings on the resected specimens in 27 patients with proven polyposis. Of these patients, 23 (83%) were diagnosed when polyps were first found at sigmoidoscopy. Radiologically the predominant polyp size was more than 5 mm in only four cases, 2-5 mm in 22 (81%), and less than 2 mm in one. Of the 22 with predominately 2-5 mm polyps, eight had significant numbers of nodules smaller than 2 mm and three had considerable numbers of polyps larger than 5 mm. Eleven (41%) were thought to have fewer than 70 polyps. Pathologically the nodular pattern (less than 2 mm) predominated in 11 (41%) and 14 had polyps of 2-5 mm. More than 100 polyps were present in each case, with fewer than 500 polyps in eight. In the 11 patients thought radiologically to have fewer than 70 polyps, the nodular pattern predominated in nine. In the initial stages of polyp growth, the larger polyps are less numerous, and the background nodular pattern is a useful diagnostic feature of familial polyposis.     

Gastrointestinal polyposis in the Cowden syndrome

A case of Cowden disease (multiple hamartoma syndrome) with marked gastrointestinal polyposis is presented. The differential diagnosis of gastrointestinal polyposis syndromes is discussed.