Deformity Correction in Children with Hereditary Hypophosphatemic Rickets

Abstract

Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Magnetic Resonance Imaging Features as Surrogate Markers of X-Linked Hypophosphatemic Rickets Activity

“…The cohort of patients described in the current report accurately reflects children with XLH managed in 2012-2013 in a reference center providing multidisciplinary care. Sex ratio, stature, and bone deformities are similar to those described in other reports [32][33][34].…”

Results of deformity correction in children with X-linked hereditary hypophosphatemic rickets by external fixation or combined technique

“…Standard conservative medical treatment consists of oral phosphate substitution and supplementation of active vitamin D [3,9,12]. Series of studies were published describing performed surgical treatment even when a patient's resistance to conservative treatment was stated [8,14,16]. Studies reveal that excess of fibroblast MAD mechanical axis deviation, mLDFA mechanical lateral distal femoral angle, mMPTA mechanical medial proximal tibial angle Fig.…”

“…Although, the conservative treatment is not always successful. In such cases, operative procedures for correction of multiplanar bone deformities can be indicated to prevent secondary orthopaedic complications such as pain, pathologic fractures, degenerative arthropathy or for cosmetic reasons [13][14][15].…”

“…Patients with deformities have been treated by various methods of corrective osteotomy and fixation devices, such as Kirschner wires, plates, temporary epiphysiodesis, Ilizarov devices and intramedullary nailing devices [14][15][16][17][18][19][20]. Different problems related to surgical correction were reported: increased rate of non-union after osteotomies, delayed union, recurrent deformity, joint stiffness, deep intramedullary infection, refracture, nerve palsy, leg length discrepancy, and pin tract infection [17][18][19][20].…”

“…In this case, application of a circular external device is crucial [14,17,18]. A combination of external fixation and intramedullary rigid osteosynthesis allows to reduce the period of external fixation [19].…”