Bow leg deformity correction and lengthening with TSF system in an adolescent with hereditary hypophosphatemic rickets
Background: X-linked hereditary hypophosphatemic rickets (XHPR) is the dominant form of hereditary rickets characterized by alteration of phosphate metabolism and defective mineralization of the growth cartilage and the newly formed bone collagen. Case report: We report a case of a 12-year-old girl...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2022-06-01
|
| Series: | Journal of Orthopaedic Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2773157X22000418 |
| _version_ | 1797988176414900224 |
|---|---|
| author | Megremis Panos Megremis Orestis |
| author_facet | Megremis Panos Megremis Orestis |
| author_sort | Megremis Panos |
| collection | DOAJ |
| description | Background: X-linked hereditary hypophosphatemic rickets (XHPR) is the dominant form of hereditary rickets characterized by alteration of phosphate metabolism and defective mineralization of the growth cartilage and the newly formed bone collagen. Case report: We report a case of a 12-year-old girl with disproportionately short stature and bowed legs, diagnosed with X-linked hereditary hypophosphatemic rickets at the age of 2 years. Despite adequate medical treatment, her growth response was unsatisfactory. The Taylor spatial frame (TSF) was chosen as the most appropriate technique, which would ensure this dual objective of increasing the stature of the body and correcting the deformity of the lower limbs. This process was completed within a total period of 14 months. A total increase of 13 cm in the stature of the patient was achieved. In the final follow-up, five years postoperatively, a normal alignment of the lower extremities was maintained. Conclusion: This Hexapod frame system can lengthen the legs by distraction histogenesis, and at the same time, correct all the constituents of the deformity. Besides, TSF assisted by a web-relied software program, allows the execution, at any time throughout the correction process, of all appropriate modifications of the prescription needed to correct the deformity. |
| first_indexed | 2024-04-11T07:59:41Z |
| format | Article |
| id | doaj.art-ea519baa7690429293ee6e77eb7314f4 |
| institution | Directory Open Access Journal |
| issn | 2773-157X |
| language | English |
| last_indexed | 2024-04-11T07:59:41Z |
| publishDate | 2022-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of Orthopaedic Reports |
| spelling | doaj.art-ea519baa7690429293ee6e77eb7314f42022-12-22T04:35:48ZengElsevierJournal of Orthopaedic Reports2773-157X2022-06-0112100035Bow leg deformity correction and lengthening with TSF system in an adolescent with hereditary hypophosphatemic ricketsMegremis Panos0Megremis Orestis1A΄ Orthopaedic Department, Athens Children's Hospital «P. & A. Kyriakou», Athens, Greece; Corresponding author. Megalou Alexandrou 6, Mati Attiki, Greece, Tel.: + 306945713506, fax: + 302294033473.General Peripheral Hospital of Attiki: Geniko Nosokomeio Attikes KAT, 6th Orthopaedic Department, Athens, GreeceBackground: X-linked hereditary hypophosphatemic rickets (XHPR) is the dominant form of hereditary rickets characterized by alteration of phosphate metabolism and defective mineralization of the growth cartilage and the newly formed bone collagen. Case report: We report a case of a 12-year-old girl with disproportionately short stature and bowed legs, diagnosed with X-linked hereditary hypophosphatemic rickets at the age of 2 years. Despite adequate medical treatment, her growth response was unsatisfactory. The Taylor spatial frame (TSF) was chosen as the most appropriate technique, which would ensure this dual objective of increasing the stature of the body and correcting the deformity of the lower limbs. This process was completed within a total period of 14 months. A total increase of 13 cm in the stature of the patient was achieved. In the final follow-up, five years postoperatively, a normal alignment of the lower extremities was maintained. Conclusion: This Hexapod frame system can lengthen the legs by distraction histogenesis, and at the same time, correct all the constituents of the deformity. Besides, TSF assisted by a web-relied software program, allows the execution, at any time throughout the correction process, of all appropriate modifications of the prescription needed to correct the deformity.http://www.sciencedirect.com/science/article/pii/S2773157X22000418Bowed legsShort statureX-linked ricketsHexapod external fixator |
| spellingShingle | Megremis Panos Megremis Orestis Bow leg deformity correction and lengthening with TSF system in an adolescent with hereditary hypophosphatemic rickets Journal of Orthopaedic Reports Bowed legs Short stature X-linked rickets Hexapod external fixator |
| title | Bow leg deformity correction and lengthening with TSF system in an adolescent with hereditary hypophosphatemic rickets |
| title_full | Bow leg deformity correction and lengthening with TSF system in an adolescent with hereditary hypophosphatemic rickets |
| title_fullStr | Bow leg deformity correction and lengthening with TSF system in an adolescent with hereditary hypophosphatemic rickets |
| title_full_unstemmed | Bow leg deformity correction and lengthening with TSF system in an adolescent with hereditary hypophosphatemic rickets |
| title_short | Bow leg deformity correction and lengthening with TSF system in an adolescent with hereditary hypophosphatemic rickets |
| title_sort | bow leg deformity correction and lengthening with tsf system in an adolescent with hereditary hypophosphatemic rickets |
| topic | Bowed legs Short stature X-linked rickets Hexapod external fixator |
| url | http://www.sciencedirect.com/science/article/pii/S2773157X22000418 |
| work_keys_str_mv | AT megremispanos bowlegdeformitycorrectionandlengtheningwithtsfsysteminanadolescentwithhereditaryhypophosphatemicrickets AT megremisorestis bowlegdeformitycorrectionandlengtheningwithtsfsysteminanadolescentwithhereditaryhypophosphatemicrickets |