Skeletal structure and processes of bone growth, modeling and remodeling were studied in a supernumerary metatarsal surgically removed from a 3-year-old boy affected by Cutis Marmorata Telangiectatica Congenita (CMTC), associated with hypertrophy of the right upper and lower limbs and postaxial hexadactylism of the homolateral hand and foot. No other anomalies were observed. The excess of periosteal growth, due to congenital anomaly, induced an abnormal development of both modeling and remodeling processes. In bone modeling, osteoblast activity on the periosteal surface was not paralleled by osteoclast resorption along the wall of the medullary canal, and this enormously increased the cortical thickness. In bone remodeling, osteoclastic resorption cavities were not refilled by secondary Haversian systems, thus inducing a severe bone loss. While the alteration of bone growth and modeling can be ascribed to the congenital disease, the unbalanced bone remodeling appears mainly to depend on mechanical disuse of the supernumerary metatarsal.

Inhalation of a foreign body (FB) into the bronchial tree rarely occurs asymptomatically and, if leading to recurrent pneumonia, can be very difficult to diagnose. The present report deals with the case of a 10-year-old boy who had three episodes of pneumonia in the left lower lobe caused by the asymptomatic inhalation of a FB 12 months before. Standard thoracic CT, done during the third episode, revealed a slight reduction in the volume of the left lung with air bronchograms, multiple areas of bronchiectasis, and parenchymal consolidation of a segment of the lower lobe. Flexible fiberoptic bronchoscopy revealed a FB at the distal end of the left lower lobar bronchus, surrounded by granulation tissue and fully obstructing the anterior basal segmental bronchus. High-resolution CT (HRCT) images showed an inverted C-shaped image obstructing a bronchus. Removal of the FB was successful only with rigid bronchoscopy under total anesthesia. The FB was an air-pistol rubber bullet that the boy remembered playing with 12 months before. Two months after removal of the FB (ie, 14 months from its asymptomatic inhalation) and treatment with oral steroids, antibiotics, and respiratory physiotherapy, the patient recovered completely, and HRCT showed complete normalization of the lung. We conclude that, when the radiographic density of the FB is greater than the surrounding pulmonary parenchyma, HRCT can reveal the FB, and diagnostic flexible fiberoptic bronchoscopy can be avoided.

A 14-month-old girl developed chronic stridor and dyspnoea. Four months later she presented arthritis, anterior uveitis and positive ANA. Juvenile chronic arthritis (JCA) was diagnosed. Laryngoscopy demonstrated the presence of cricoarytenoid arthritis (CA). The left vocal cord was adducted and immobile, while the right vocal cord had decreased mobility. Erythema and swelling of the arytenoid cartilage on both sides was seen. Steroid treatment resulted in the resolution of these symptoms and made airway control unnecessary. This case demonstrates that CA may be the first sign of JCA, preceding peripheral arthritis. CA should be considered in every child with chronic stridor and laryngeal obstruction.

Radiographic signs of osseointegration with the use of fluted, tapered, modular, titanium stems in revision hip arthroplasty with bone loss have not been previously categorized. Serial radiographs of 64 consecutive hips with mean follow-up of 6.2 years were retrospectively reviewed. Bone loss was classified as per Paprosky classification, osseointegration was assessed according to a modified system of Engh et al, and Harris Hip Score was used to document pain and function. Seventy-four percent of the hips had type 3 or 4 bone loss. All stems were radiographically osseointegrated. Early minor subsidence was seen in 6.2% of the hips; definite bony regeneration, 73% of the hips; and stress shielding, 26% of the hips. These osseointegration patterns were different from those described for extensively porous-coated cobalt-chromium stems and had a bearing on the evaluation of fixation of these stems.

The authors describe a rare case of congenital, bilateral arteriovenous fistulas of the lung, without other symptoms of hemorrhagic telangiectasis. The disease was diagnosed in a ten-year-old child, because of a chronic hypossiemia state. The angiography with digitalized technique defined, at the best, the vascular anomalies; the examen cannot be substituted by other imaging methods. First a therapeutic embolisation in angiography was tried without success because of the dimension of the arteriovenous shunt. Then a local excisional therapy was carried out, on one lung. One year after the operation, the oxygenative and clinical conditions of the patient are excellent.

The Authors describe a case of Blackfan-Diamond anemia with ambiguous genitalia and other minor anomalies. They point out the elements of differential diagnosis with other precocious erythroblastopenic conditions and suppose a recessive inheritance of the disease because of family consanguinity in two generations.

Galactosyl-hydroxylysine, a specific bone collagen marker, has been prepared directly from human urine samples by high-performance liquid chromatography (HPLC) on a preparative column. The compound is the didansyl derivative, as proved by HPLC and mass spectrometry under fast atom bombardment conditions. Since this compound is not commercially available, the procedure reported appears to be the simplest way to prepare it, which is necessary to measure the urinary excretion of this collagen metabolite by HPLC.