PubMed · 8487817
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R Greene. 1993-06-03. Case 46-1992: correction.. https://pubmed.ncbi.nlm.nih.gov/8487817/
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Dyggve-Melchior-Clausen syndrome without mental retardation (Smith-McCort dysplasia) (SM) has clinical and radiographic findings similar to those of Dyggve-Melchior-Clausen syndrome (DMC) except for mental retardation. Iliac crest biopsies from two patients with SM were examined. The lace-like appearance of the iliac crests, which is a characteristic radiological sign of SM and DMC, was caused by bone tissue deposited in a wavy pattern at the osteochondral junction. The growth plate showed abnormal enchondral ossification with no columnarization of chondrocytes. Electron microscopy demonstrated chondrocytes with dilated cisternae of rough endoplasmic reticulum containing fine granular or amorphous material, similar to those reported in cases of DMC. Thus, SM has pathologic changes in common with DMC as a rough endoplasmic reticulum storage disorder, even though the mental condition is different.
We report on a patient whose clinical, radiologic, and histopathologic findings are compatible with the Piepkorn type of lethal short-limb osteochondrodysplasia, but who also showed multinucleated giant chondrocytes in cartilage. Multinucleated giant cells are an unusual finding in osteochondrodysplasias, having been reported in atelosteogenesis type I and boomerang dysplasia. This uncommon histopathologic finding and the clinical and radiographic findings strongly support the diagnosis of boomerang dysplasia in the present patient. Our patient supports the previously suggested existence of an entity including atelosteogenesis and boomerang dysplasia. If this is so, the current patient and that described by Piepkorn et al. [1977: Teratology 16:345-350] could represent the most severe clinical expression of that condition.
OBJECTIVE: To determine the efficacy of alar batten grafts for the correction of internal and external nasal valve collapse. DESIGN: In this retrospective study, a questionnaire was used to ask patients to rate their nasal breathing before and after application of alar batten grafts. SETTING: Private practice and academic tertiary referral medical center. PATIENTS: The questionnaire was given to 63 patients who underwent application of alar batten grafts between 1980 and 1995. Forty-six patients (73%) responded and were included in the study. INTERVENTION: Alar batten grafts were applied into a precise pocket via a limited endonasal incision or via the external rhinoplasty approach. The grafts consisted of curved septal cartilage or auricular cartilage and were applied to the site of maximal lateral nasal wall collapse. The convex surface of the cartilage was oriented laterally to allow maximal lateralization of the collapsed portion of the lateral nasal wall. In most cases, alar batten grafts were applied caudal to the existing lateral crura and extended from the lateral one third of the lateral crura to the piriform aperture. OUTCOME MEASURES: The degree of nasal airway obstruction was determined by subjective scoring on a scale from 1 (no obstruction) to 5 (complete obstruction) before and after surgery. The patency of the internal airway was also assessed on physical examination. RESULTS: The results of the study revealed that all but 1 of the 46 patients experienced an improvement in their nasal airway obstruction. The mean improvement in nasal airway obstruction was 2.5 on a scale of 5. Patients that had the least improvement had intranasal scarring in the region of the internal nasal valve, loss of vestibular skin, or excessive narrowing at the piriform aperture. Physical examination revealed a significant increase in the size of the aperture at the internal or external nasal valve after the application of the alar batten grafts. There was minimal postoperative fullness in the supraalar region, where the alar batten grafts were applied. With time, this fullness decreased, leaving little evidence of the graft and an overall improvement in the aesthetic result. CONCLUSIONS: Alar batten grafts are effective for long-term correction of internal and external nasal valve collapse that is not complicated by intranasal scarring in the region of the nasal valve, loss of vestibular skin, or excessive narrowing at the piriform aperture.