A 'Made in One Piece' Skeleton in a 22-Year-Old Man Suffering from Sickle Cell Anaemia

Dumarey, Nicolas; Martin, Paul; Jayankura, Marc; Putz, Philippe; Verhas, Michel; Peretz, Anne

doi:10.1007/s100670070047

Cited by 5 publications

(4 citation statements)

References 8 publications

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“…Once the infection was treated, the patient had a staged bilateral THR procedure. The patient regained his ability to walk with crutches after the surgeries 4 . Another case was reported in 2008: a 25-year-old female with severe ankylosis of her hips and knees secondary to rheumatoid arthritis, and her function was severely limited.…”

Section: Discussionmentioning

confidence: 90%

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Ankylosis of the hips and knees due to sickle cell disease

Elayan

Hamdan

2012

F1000Res

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This is a case report of a 29-year-old Saudi male with sickle cell disease (SCD) with severe stiffness of his joints, mainly both knees and hips, secondary to complications of SCD. He was severely crippled: unable to sit, stand or walk, and was bedridden for 8 years when he was presented to us. Radiographs showed fusion of both knees and hips. There was no evidence of active osteomyelitis by Gallium scan. The patient’s hemoglobin S decreased to levels below 30% by exchange transfusion. Bilateral total hip replacement, as well as unilateral total knee replacement, was carried out to improve his level of function. There is only one reported case of such severe and multiple joint complications in a single patient suffering from SCD.The increased life expectancy that medical advances have offered to the sickle-cell patients has led to the appearance of sickle-cell-related complications, which were previously only seen rarely. These complications were successfully managed and the patient was able to move and transfer using a wheel chair.

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Section: Discussionmentioning

confidence: 90%

“…Total bilateral hip and unilateral knee arthroplasty were performed to correct hip and knee ankylosis secondary to SCD. To our knowledge, total bilateral hip and unilateral knee arthroplasty has not been described previously in the literature except in one case of an African male with a similar presentation 4 .…”

Section: Introductionmentioning

confidence: 80%

Ankylosis of the hips and knees due to sickle cell disease

Elayan

Hamdan

2012

F1000Res

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“…In these patients many young adults will be seeking treatment for avascular necrosis of the femoral head. Review of the literature showed few reports on hip replacement arthroplasties in SCD, with the number of patients ranging from one [9] to 30 [1] and a high complication rate [7,12,16,22]. The incidence of sickle cell haemoglobinopathies presents major health care problems in Bahrain [18].…”

Section: Introductionmentioning

confidence: 99%

Total hip replacement in sickle cell disease

Al-Mousawi

Malki

Al-Aradi

et al. 2002

International Orthopaedics

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We report on 35 total hip replacement arthroplasties in 28 patients with avascular necrosis of the femoral head secondary to sickle cell disease (SCD). There were 15 men and 13 women with a mean age of 27.5 years. In all patients Harris hip scores improved from a mean of 36 pre-operative to 86 post-operative. However, at a mean follow-up of 9.5 (5-15) years six hips failed due to symptomatic aseptic loosening and one due to late deep infection. Our results support the decision to offer the procedure for patients with arthritic hips secondary to SCD. It is important that patients and surgeons should be aware of the wide varieties of complications.Résumè Nous rapportons sur 35 arthroplasties totales de la hanche dans 28 malades avec nécrose avasculaire de la tête fémorale secondaire à drépanocytose. Il y avait 15 hommes et 13 femmes avec un âge moyen de 27.5 années. Dans tout le score de la hanche de Harris amélioré d'une moyenne de 36 préopératoire à 86 postopératoire. Cependant, à une suite moyenne de 9.5 (5-15) années six hanches ont manqué dû à descellement aseptique symptomatique et un dû à infection profonde tardive. Nos résultats supportent la décision d'offrir la procédure pour les malades avec les hanches arthritiques secondaire à drépanocyt-ose. C'est important que les malades et chirurgiens doivent être informés des variétés larges de complications. IntroductionAvascular necrosis of the femoral head is one of the significant complications affecting the musculoskeletal system in patients with sickle cell haemoglobinopathy [6]. The reported incidence of femoral head necrosis varies from less than 10% to more than 30% [15,23]. In many patients both hips and other bones are affected [8].Improved medical care of children with sickle cell disease (SCD) has led to prolonged life span. In these patients many young adults will be seeking treatment for avascular necrosis of the femoral head. Review of the literature showed few reports on hip replacement arthroplasties in SCD, with the number of patients ranging from one [9] to 30 [1] and a high complication rate [7,12,16,22]. The incidence of sickle cell haemoglobinopathies presents major health care problems in Bahrain [18]. The aim of this report is to present our experience, and the problems encountered, with total hip replacement in avascular necrosis of the femoral head in SCD patients. Patient and methodsThirty-seven total hip replacements were performed between 1984 and 1995 for 30 SCD patients with avascular necrosis of the femoral head. One patient moved from the region and another died 3 years after hip replacement from acute pulmonary crisis. Thirtyfive hips in 28 patients were available for follow-up. There were 15 men and 13 women with a mean age of 27.5 (19-42) years. Seven patients, five of whom were women, had bilateral hip replacements. The second side was replaced within 2 years of the first operation. The mean number of previous admission to hospital for medical problems related to SCD was 6.5 (range 3-22 admissions). Blood group was A...

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“…The 99m Tc sulfur colloid scan has been used in a variety of clinical settings to demonstrate bone marrow elements, with the normal distribution of radiocolloid approximately 80% within the liver, 15% within the spleen, and 5% within the bone marrow. [12][13][14][15] In our patient, the radionuclide scan provided encouraging evidence that no further medical management was needed in the light of her appropriate, intramedullary hematopoiesis, and it revealed this information in a relatively painless way for the patient.…”

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confidence: 99%

FLAG chemotherapy followed by allogeneic stem cell transplant using nonmyeloablative conditioning induces regression of myelofibrosis with myeloid metaplasia

Tanner

Hoh

Bashey

et al. 2003

Bone Marrow Transplant

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Summary:A 38-year-old woman with agnogenic myeloid metaplasia complicated by the poor prognostic factors of severe osteosclerosis, prominent hepatosplenomegaly, and profound anemia was treated with FLAG chemotherapy to decrease her organomegaly before undergoing a nonmyeloablative allogeneic stem cell transplant from a matchedsibling donor. The patient's pre-and post transplant course were complicated by an autoimmune disorder and her post transplant course was complicated by severe hepatic and gastrointestinal GVHD. A technetium-99m sulfur colloid scan 4 months post transplant and bone marrow studies 8 months post transplant demonstrated intramedullary hematopoiesis, complete resolution of marrow fibrosis, and partial resolution of osteosclerosis.

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A 'Made in One Piece' Skeleton in a 22-Year-Old Man Suffering from Sickle Cell Anaemia

Cited by 5 publications

References 8 publications

Ankylosis of the hips and knees due to sickle cell disease

Ankylosis of the hips and knees due to sickle cell disease

Total hip replacement in sickle cell disease

FLAG chemotherapy followed by allogeneic stem cell transplant using nonmyeloablative conditioning induces regression of myelofibrosis with myeloid metaplasia

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