Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report

Albers- Schonberg disease is an extremely rare genetically inherited disorder. Also known as Autosomal dominant Type II osteopetrosis, it is usually diagnosed incidentally. Due to the rarity of the disease, the condition is often missed and under- diagnosed. This is a case report of an eight- year...

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Main Authors: Ang Poh Thean, Charles, Awang, Mohd Shukrimi, Mor Japar Khan, Ed Simor Khan, Sabir Husin Athar, Satriya, Hao, Faun
Format: Article
Language:English
Published: Juniper Publishers 2019
Subjects:
Online Access:http://irep.iium.edu.my/71480/
http://irep.iium.edu.my/71480/
http://irep.iium.edu.my/71480/1/71480_Is%20Conservative%20Approach%20a%20Better.pdf
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spelling iium-714802019-04-05T03:03:03Z http://irep.iium.edu.my/71480/ Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report Ang Poh Thean, Charles Awang, Mohd Shukrimi Mor Japar Khan, Ed Simor Khan Sabir Husin Athar, Satriya Hao, Faun RD701 Orthopedics Albers- Schonberg disease is an extremely rare genetically inherited disorder. Also known as Autosomal dominant Type II osteopetrosis, it is usually diagnosed incidentally. Due to the rarity of the disease, the condition is often missed and under- diagnosed. This is a case report of an eight- year old Malay girl that was diagnosed retrospectively after developing complications to the initial treatment given. Due to the underlying undiagnosed osteopetrosis rendering her prone for infections, a simple minimally invasive percutaneous K-wiring of her left tibia for a closed fracture was complicated with osteomyelitis causing significant morbidity. On retrospective review, her blood and radiographic investigations reveals low hemoglobin, raised alkaline phosphatase, rugger- jersey appearance of her spine radiograph, sclerosis over the base of skull and endo bones seen in her pelvic radiograph. She undergone wound debridement, and sequestrectomy a total of three times and was treated with intravenous antibiotics for six weeks and oral antibiotics for another six weeks. Despite the adequacy of surgical and antibiotic therapy, at one year follow up she still complains of on and off left proximal tibia pain significant enough to affect her daily activity. Therefore, it is important to make known the existence of this condition and double consider if surgical intervention is really needed or conservative approach will be a better option for primary treatment. Juniper Publishers 2019-01-18 Article PeerReviewed application/pdf en http://irep.iium.edu.my/71480/1/71480_Is%20Conservative%20Approach%20a%20Better.pdf Ang Poh Thean, Charles and Awang, Mohd Shukrimi and Mor Japar Khan, Ed Simor Khan and Sabir Husin Athar, Satriya and Hao, Faun (2019) Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report. Juniper Online Journal of Orthopedic & Orthoplastic Surgery, 2 (2). pp. 1-4. E-ISSN 2461-8266 https://juniperpublishers.com/jojoos/pdf/JOJOOS.MS.ID.555583.pdf
repository_type Digital Repository
institution_category Local University
institution International Islamic University Malaysia
building IIUM Repository
collection Online Access
language English
topic RD701 Orthopedics
spellingShingle RD701 Orthopedics
Ang Poh Thean, Charles
Awang, Mohd Shukrimi
Mor Japar Khan, Ed Simor Khan
Sabir Husin Athar, Satriya
Hao, Faun
Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report
description Albers- Schonberg disease is an extremely rare genetically inherited disorder. Also known as Autosomal dominant Type II osteopetrosis, it is usually diagnosed incidentally. Due to the rarity of the disease, the condition is often missed and under- diagnosed. This is a case report of an eight- year old Malay girl that was diagnosed retrospectively after developing complications to the initial treatment given. Due to the underlying undiagnosed osteopetrosis rendering her prone for infections, a simple minimally invasive percutaneous K-wiring of her left tibia for a closed fracture was complicated with osteomyelitis causing significant morbidity. On retrospective review, her blood and radiographic investigations reveals low hemoglobin, raised alkaline phosphatase, rugger- jersey appearance of her spine radiograph, sclerosis over the base of skull and endo bones seen in her pelvic radiograph. She undergone wound debridement, and sequestrectomy a total of three times and was treated with intravenous antibiotics for six weeks and oral antibiotics for another six weeks. Despite the adequacy of surgical and antibiotic therapy, at one year follow up she still complains of on and off left proximal tibia pain significant enough to affect her daily activity. Therefore, it is important to make known the existence of this condition and double consider if surgical intervention is really needed or conservative approach will be a better option for primary treatment.
format Article
author Ang Poh Thean, Charles
Awang, Mohd Shukrimi
Mor Japar Khan, Ed Simor Khan
Sabir Husin Athar, Satriya
Hao, Faun
author_facet Ang Poh Thean, Charles
Awang, Mohd Shukrimi
Mor Japar Khan, Ed Simor Khan
Sabir Husin Athar, Satriya
Hao, Faun
author_sort Ang Poh Thean, Charles
title Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report
title_short Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report
title_full Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report
title_fullStr Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report
title_full_unstemmed Is conservative approach a better option for primary treatment of closed tibia fractures in Albers- Schonberg disease? a case report
title_sort is conservative approach a better option for primary treatment of closed tibia fractures in albers- schonberg disease? a case report
publisher Juniper Publishers
publishDate 2019
url http://irep.iium.edu.my/71480/
http://irep.iium.edu.my/71480/
http://irep.iium.edu.my/71480/1/71480_Is%20Conservative%20Approach%20a%20Better.pdf
first_indexed 2023-09-18T21:41:21Z
last_indexed 2023-09-18T21:41:21Z
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