A case report treatment of a medial condylar humeral fracture in an adult with osteopetrosis (author calvin chien)


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authors: Dr Calvin CHIEN, MBBS, Dr Rajesh BEDI, DNB (Ortho), Dr Richard D. LAWSON, FRACS (Ortho). Case study of an adult with osteoporosis suffering a fracture.

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A case report treatment of a medial condylar humeral fracture in an adult with osteopetrosis (author calvin chien)

  1. 1. Sunday, February 12, 2012A Case Report: Treatment of a medial condylar humeral fracture inan adult with osteopetrosis - (author: Calvin Chien) Abstract: Patients with osteopetrosis often present with orthopaedic problems such as frequent fractures. Management of fractures with open reduction and internal fixation is difficult but possible. We report on a 22 year old patient with a medial humeral condyle fracture treated successfully with internal fixation using a pre-contoured plate. Introduction:In 1904 Albers-Schoenberg described a condition characterised by marked radiographic density of the bones(1). Despite the sclerotic radiographic appearance of the thickened cortices and its material hardness,osteopetrotic bone is weak, brittle and prone to fracture after minor trauma (1). Most literature regardingtreatment of osteopetrotic patients with fractures concentrates on paediatric patients or on the difficulty ofoperative intervention in adults (2). We report the case of an adult patient with osteopetrosis and a lowmedial column fracture (Milch Type I (1)) of the distal humerus after minor trauma. The fracture was treatedoperatively utilising internal fixation with a pre-contoured peri-articular plate.Case: A 22 year old female with known osteopetrosis presented with an elbow injury after bracing herself with the right arm after a fall. The mechanism described suggested a valgus injury to the right elbow resulting in a Milch Type I (3) low medial column fracture of the distal humerus (Fig. 1). There were no neurological deficits. As an adolescent she had previous injuries including one to the radius of the same side limiting elbow extension by twenty degrees. She was also partially blind and was receiving psychiatric treatment for depression.Two days later, open reduction of the right distal humerus was performed with internal fixation using a pre-contoured medial condylar locking plate (Fig 2). This was done through a posterior approach afteridentifying the ulnar nerve. Anterior transposition of the ulnar nerve was done before closure. The patientwas discharged two days later in a plaster-of-paris back slab with outpatient follow-up. After two weeks thearm was placed in a range of movement elbow brace with unrestricted range of motion. Serial radiographswere performed at four-weekly intervals and complete bony union with disappearance of the fracture linewas evident on the radiographs taken at fourteen weeks (Fig 3). Outpatient as well as a home-basedphysiotherapy program was arranged and full pre-injury range of motion was achieved by ten weeks.Discussion:Osteopetrosis is a rare hereditary disease of the osteoclasts first described by Albers-Schönberg, a Germanradiologist, in 1904. Defective osteoclastic activity or a reduced number of osteoclasts results in a failure of
  2. 2. bone remodelling (4). This is manifested on radiographs as an increase in bone mass and osteoscleroticchanges (4).Osteopetrosis can be classified into three main forms: a malignant autosomal recessive, intermediateautosomal recessive and benign autosomal dominant form; the vast majority of these cases are the benignautosomal dominant form. The malignant autosomal recessive type, also known as infantile, is characterisedby growth retardation, failure to thrive and cranial nerve palsies manifesting as proptosis, deafness andblindness. In addition, pancytopenia and thrombocytopenia may result from bone marrow failure. Manyfeatures of the intermediate form of osteopetrosis are similar to those of the malignant form but theintermediate form is less severe and later in onset. It is often diagnosed after a fracture, usually occurring inthe first decade. Benign osteopetrosis has been further subdivided into types I and II. However, recent geneticstudies have shown that autosomal-dominant osteopetrosis type I is caused by an increase in osteoblasticactivity rather than osteoclastic dysfunction. In this case osteoblasts deposit excessive amounts of bonematrix (4). Type II autosomal dominant osteopetrosis is the form Albers-Schönberg first described and so isoften named after him. The onset is in later childhood and is usually diagnosed incidentally during aradiographic examination (4). It is also associated with increased fracture frequency. Other manifestationsinclude coxa vara, osteoarthritis, spondylolysis, back pain, osteomyelitis and cranial nerve palsies.Radiographic features include skull-base thickening, vertebral end-plate thickening and endobone appearance(4).Isolated medial condylar fractures of the humerus in adults are uncommon and we have not discovered areport of this fracture in an osteopetrotic patient. Medial condylar fractures are intra-articular and like lateralcondylar fractures are prone to non-union (1). Usually, the mechanism for this fracture is through a valgusforce on an extended elbow where the force is transmitted via the olecranon or coronoid process into themedial condyle (3). The fracture can also arise from an avulsion injury of the condyle through forcefulcontraction of the forearm flexors. With minimally displaced fractures of the medial humeral condyle, goodfracture healing and functional outcomes can be expected with non-surgical treatment consisting ofimmobilisation in a splint and a gradually increasing permissible range of motion (7). On the other hand,studies specifically examining displaced medial humeral condylar fractures treated by open reduction internalfixation reported good or excellent outcome in 86% of patients (2). As mentioned earlier, patients withosteopetrosis are prone to infections and the reported incidence of post-operative infection is 12% (2).Furthermore, some authors have reported delayed and non-union following fractures in osteopetrotic patients(2). A study has shown fracture healing time in osteopetrotic mice to be more than twice as long (2).Despite the difficulties of surgery, the risk of infection, and the higher incidence of delayed and non-union,the patient achieved an excellent functional outcome with no surgical complications. Open reduction andinternal fixation to a fractured medial humeral condyle in a young osteopetrotic patient is certainly an option.REFERENCES1. Albers-Schönberg H. Roentgenbilder einer seltenen Knochennerkrankung. Munch Med Wochenschr1904;51:365.2. Armstrong DG, Newfield JT, Gillespie R. Orthopedic management of osteopetrosis: results of a surveyand review of the literature. J Pediatr Orthop 1999;19:122–132.3. Milch H. Fractures and fracture dislocations of the humeral condyles. J Trauma 1964;15:592-607.4. Tolar J, Teitelbaum SL, Orchard PJ. Osteopetrosis. N Engl J Med 2004; 351:2839-2849.5. Abe S, Watanabe H, Hirayama A, Shibuya E, Hashimoto M, Ide Y. Morphological study of the femur in
  3. 3. osteopetrotic (op/op) mice using microcomputed tomography. Br J Radiol 2000;73:1078-82. 6. Bollerslev J, Mosekilde L. Autosomal dominant osteopetrosis. Clin Orthop Relat Res. 1993;294:45-51. 7. El Ghawabi MH. Fracture of the medial condyle of the humerus. J Bone Joint Surg Am 1975;57:677-80. 8. Jupiter JB, Neff U, Regazzoni P, Allgower M. Unicondylar fractures of the distal humerus: an operative approach. J Orthop Trauma 1988;2:102-109.9. Shapiro F. Osteopetrosis: Current clinical considerations. Clin Orthop Relat Res 1993;294:34-44.10. Marks SC Jr, Schmidt CJ. Bone Remodeling as an Expression of Altered Phenotype: Studies of FractureHealing in Untreated and Cured Osteopetrotic Rats. Clin Orthop Relat Res 1970;137:259-264.Author(s) take full responsibility for the content of their article, including originality, copyrights, andcompliance with all relevant Internet laws and guidelines. Articles are not edited for content by the InternetMedical Journal.Broken Bones: The X-Ray Atlas of Fractures