[Caffey disease. A case report]. / Enfermedad de caffey. Reporte de un caso.

Infantile cortical hyperostosis or Caffey-Silverman syndrome is a disorder of unknown cause that affects the skeleton and some of the contiguous fascias and muscles. It occurs under all circumstances, in cities, rural communities, in all types of climates, seasons, races, social strata, and its incidence is the same among males and females. We report herein a very rare disease, little known in world literature, in order to disseminate within the orthopedic setting the musculoskeletal alterations we found in Caffey-Silverman disease. We report a seven year-old female patient diagnosed with Caffey-Silverman disease, with presence of its different manifestations that include swelling of the right forearm (indurated edema without phlogosis), pain, irritability with a chronicity involving a course of years. She is undergoing primary treatment consisting of observation and symptom relief. We know that it is a disorder with an unknown etiology. We also know there is hypoxia, local necrosis and periosteal reaction; however, the triggers of these changes are still a mystery. There are several hypotheses, but none of them has been proven. Some reports of familial occurrence suggest a possible hereditary factor. The natural self-limitation of this disease has made it difficult to establish the type of heredity; it is likely a trait with an autosomal dominant transmission with variable penetrance. A hereditary defect of periosteal arterioles has been propose as well as factors such as diet, an allergic base and an immune origin. Attempts have been made to isolate viruses and bacteria, but they have failed. Serologic infection tests have been negative. Caffey syndrome is little known in world literature, as there are only a few articles on it in the PUBMED, EMBASE, MEDIGRAPHIC, LILACS and ARTEMISA data bases. Being aware of this rare disease allows for early suspicion and a better workup and contributes to orthopedic knowledge as its musculoskeletal alterations are reported. Despite its low prevalence, it should be part of the differential diagnoses in children with soft tissue swelling and bone abnormalities with signs of irritability and fever.

[Patient with PHACE syndrome. Clinical case and literature review]. / Paciente con síndrome de PHACE. Caso clínico y revisión de la literatura.

BACKGROUND: PHACE syndrome is a neurocutaneous disease of unknown etiology. It consists of the association of a large hemangioma in the face or neck combined with one or more abnormalities in other parts of the body. A syndrome that is little known in world literature is reported and the musculoskeletal alterations that it involves are described. CLINICAL CASE: Female patient, 8 years and 9 months old, with diagnosis of PHACE syndrome and the following alterations: sunken chest, hemangiomas in the right paraciliary and left mandibular regions, and in the anterior and posterior right thigh with leg length discrepancy. Treatment consisted of braces and the patient was scheduled for lengthening surgery by means of monofocal callotaxis. DISCUSSION: The reported findings include the combined vascular malformations located in the extremities, which may be complicated by thrombophlebitis, regional osteolysis and even thromboembolism. Face and body capillaries are prone to darkening; there is propensity to skin hyperplasia and gradual hypertrophy of the underlying soft tissues and the skeleton. The percentage of reported cases of hemangiomas of the bones, spleen or kidneys is 6% or less. PHACE syndrome is little known in world literature, as we only found 71 papers in the following databases: Pubmed, Embase, Medigraphic, Lilacs, Artemisa. CONCLUSIONS: Reporting the musculoskeletal alterations that occurred with this syndrome in the case presented herein represents a contribution to orthopedic knowledge.

[Infected pseudoarthrosis of the tibia, a pediatric case. Case report and literature review]. / Seudoartrosis infectada en tibia, un caso infantil. Caso clínico y revisión de la literatura.

Chronic posttraumatic osteomyelitis and infected pseudoarthrosis of the tibia are complex problems associated with considerable morbidity which may compromise the viability of the involved limb. The most frequent age of presentation is 2 to 6 years, with predominance in males at a 2:1 ratio. We present the case of a male 4 year-old patient with diagnosis of infected pseudoarthrosis. During the physical exam a fistula was detected in the anterior aspect of the right leg, with discharge of purulent material, and inability for weight bearing and gait. X-rays, culture of the exudate and biopsy were performed. Treatment consisted of antimicrobial therapy, surgical debridement's, and application of an autologous bone graft. The infection was controlled, the tibia healed, and function was restored.

[Elongation with callotaxis for congenital brachymetatarsia]. / Elongación por callotaxis en braquimetatarsia congénita.

OBJECTIVE: To present 10 cases of congenital brachymetatarsia treated with elongation by means of callotaxis with external minifixators. INTRODUCTION: Brachymetatarsia of one or more of the matatarsals involving one or both extremities is a congenital condition, more frequent in the right foot, with the 4th metatarsal most commonly affected. MATERIAL AND METHODS: From May 2007 to September 2008, 7 patients with congenital brachymetatarsia, ages 8 to 15 years, were operated on. Two metatarsals were involved in 3 of them and thus the series included 10 procedures. All of them underwent lengthening through callotaxis with a monoaxial external mini-fixator. RESULTS: Mean lengthening was 21.1 mm, with a range of 17 to 25 mm. The complications included two cases of lengthening regression of 5 mm each. Delayed healing was reported in one of these two cases, and healing occurred spontaneously at 6 months. Nine metatarsals healed without any problems; no infections or material intolerance occurred. CONCLUSION: Lengthening of metatarsals through callotaxis with external mini-fixators is an appropriate procedure for pediatric and adolescent patients with congenital brachymetatarsia.