The Management of the Haemophilic Arthropathy

Book Series: Frontiers in Arthritis

Volume 2

by

Carulli Christian

DOI: 10.2174/97816810835371160201
eISBN: 978-1-68108-353-7, 2017
ISBN: 978-1-68108-354-4
ISSN: 2468-6662 (Print)
ISSN: 2468-6670 (Online)



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Table of Contents

Foreword

- Pp. i

Massimo Innocenti

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Preface

- Pp. ii-iii (2)

Christian Carulli

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List of Contributors

- Pp. iv-v (2)

Christian Carulli

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Pathogenesis of the Haemophilic Arthropathy

- Pp. 1-13 (13)

Daniela Melchiorre, Silvia Linari, Fabrizio Matassi and Giancarlo Castaman

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Pharmacokinetic Approach to the Treatment of Haemophilia

- Pp. 14-24 (11)

Giancarlo Castaman and Maria Chiara Susini

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Haematological Care of the Haemophilic Patient

- Pp. 25-40 (16)

Giancarlo Castaman and Silvia Linari

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Laboratory Aspects

- Pp. 41-58 (18)

Enrichetta Paladino, Maria Chiara Susini, Giuseppe Mazza, Paolo Quaglierini and Giancarlo Castaman

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Nursing of Patients with Haemophilia

- Pp. 59-69 (11)

Maria Virginia Puliga

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Imaging of Haemophilic Arthropathy

- Pp. 70-96 (27)

Giuliana Roselli, Giovanni D`Elia and Filippo Parretti

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Sonographic Findings and Scoring Method of Target Joints

- Pp. 97-109 (13)

Daniela Melchiorre and Marco Matucci-Cerinic

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The Conservative Management of the Haemophilic Arthropathy

- Pp. 110-118 (9)

Christian Carulli, Caterina Martini and Irene Felici

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Lifestyle Strategies and Physical Therapy

- Pp. 119-137 (19)

Francesco Demartis and Massimiliano Tani

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Arthroscopy

- Pp. 138-146 (9)

Christian Carulli and Massimo Innocenti

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Total Knee Arthroplasty

- Pp. 147-161 (15)

Christian Carulli, Fabrizio Matassi and Massimo Innocenti

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Hip Arthroplasty

- Pp. 162-170 (9)

Christian Carulli, Roberto Civinini and Anna Rosa Rizzo

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The Management of Foot and Ankle Arthropathy

- Pp. 171-180 (10)

E. Carlos Rodriguez-Merchan

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Elbow Arthroplasty

- Pp. 181-195 (15)

Massimo Ceruso, Marco Biondi, Prospero Bigazzi and Sandra Pfanner

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Revision Surgery in the Lower Limb of Haemophilic Patients

- Pp. 196-212 (17)

Massimo Innocenti, Christian Carulli and Roberto Civinini

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Postoperative Rehabilitation

- Pp. 213-228 (16)

Pietro Pasquetti, Lorenzo Apicella, Elisa Pratelli and Giuseppe Mangone

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Complications of the Orthopaedic Surgery in Haemophilia

- Pp. 229-239 (11)

Luigi Piero Solimeno, Mattia Alessio Mazzola and Gianluigi Pasta

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Microsurgery and Plastic Surgery in Haemophilia

- Pp. 240-264 (25)

Giulio Menichini, Dario Melita, Federico Cipriani, Antonio Amenta and Marco Innocenti

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Haematological Care in Patients with Haemophilia and Inhibitors Candidate to Orthopaedic Surgery

- Pp. 265-275 (11)

Giuseppe Tagariello, Marco Basso, Alberto Ricciardi and Paolo Radossi

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Miscellaneous

- Pp. 276-290 (15)

Christian Carulli and Gianluigi Pasta

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Final Considerations

- Pp. 291-298 (8)

Giorgia Saccullo and Michael Makris

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Subject Index

- Pp. 299-306 (8)

Christian Carulli

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Preface

Haemophilia is one of the most common rare diseases, characterized by bleedings and haemorrhages related to an inherited deficiency of coagulative factors. For decades it has been associated with higher rates of mortality and morbidity, until clotting factor concentrates were diffused, significantly limiting most of the complications. A dramatic raise of morbidity and mortality after blood transfusions was reported when HIV and Hepatitis infections were discovered. The development of recombinant concentrates, the modern prophylactic treatment, and the multidisciplinary approach to this disease lead over the years to the reduction of such complications and improvements in the management of the related clinical settings.

Then, why another book on the management of the haemophilic arthropathy? Simply because arthropathy may be to date considered the most frequent complication of Haemophilia.

Since childhood, the first falls in the physiological development of gait ability and the high frequency of impacts during games and sports activity may induce bleedings in muscles and joints. While a haematoma in muscles usually shows a self-resolution, blood in some joints, named “target joints”, may induce early negative effects, producing the so-called “arthropathy”. Such degenerative and inflammatory condition finally results in a mild to severe irreversible damage, that nowadays represents not a cause of mortality but rather a source of severe disability.

Even the powerful efficacy of bleeding prophylaxis, musculoskeletal alterations are still yet highly represented. Thus, the management of the haemophilic arthropathy has gained importance being to date one of the most essential goals of the modern approach to Haemophilia. Lifestyle modifications, selected sports activity, periodic evaluations by the multidisciplinary team (haematologist, orthopaedic surgeon, skilled nurse, radiologist, physiotherapist, lab personnel, and several other figures), and tailored prophylactic treatments represent the best way to prevent articular degenerative changes or to delay the progression of the arthropathy. In cases of fair results with this approach, it is possible to adopt conservative therapies, as braces, physical therapy, and articular injections with several substances and different indications. This would mean to avoid the early recourse to surgical procedures that until a decade ago was the only choice to ensure an acceptable quality of life in young symptomatic patients. On the other hand, a significant number of patients still now found no improvements with these strategies. In such cases, surgery is mandatory. With respect to the past, knee arthroplasty, ankle fusions, and arthroscopy are not the only orthopaedic procedures useful to address a joint arthropathy. Elbow and ankle arthroscopy, hip, ankle, and elbow arthroplasty are gaining popularity given the good outcomes and high reproducibility, simultaneously with the development of modern implants and devices, less invasive techniques, and biomaterials with better tribology and performance. Nowadays, it is possible to delay a joint replacement by a minimally invasive surgery, and also to achieve a long-term survival of implant after an arthroplasty. Joint fusions are unfrequently indicated, mostly after failure of the above mentioned procedures. Amputations are to date very uncommon, and proposed only in difficult cases when no limb salvage procedures are feasible. As expected, joint replacements in young haemophilic patients will fail, and revision arthroplasty often associated with reconstructive and plastic surgery will progressively arise. Thanks to modern modular revision implants, also such challenging conditions have been well addressed. Finally, no orthopaedic procedures may produce a good result without a valid and tailored rehabilitative protocol: specific approaches under control of the multidisciplinary team now ensure an effective functional recovery, and a better feeling referred by the operated patients.

Our future target will be the prevention of arthropathy by a multimodal and multidisciplinar approach, in order to make Haemophilia an early diagnosis but no more a source of disability. In specific challenging cases, as patients with inhibitors, the goal will eventually be the limitation of the natural history of arthropathy by all conservative or minimally invasive means that are now available, more than surgical procedures.

This textbook represents an updated overview on all aspects related to Haemophilia and its orthopaedic complications; it may be considered the most multidisciplinary textbook on this topic, focusing on this disease from the bench to the surgical room.

Christian Carulli
Orthopaedic Clinic, University of Florence,
Florence,
Italy

List of Contributors

Editor(s):
Carulli Christian
University of Florence
Orthopaedic Clinic
Florence
Italy




Contributor(s):
Antonio Amenta
Plastic Surgery and Reconstructive Microsurgery Unit
University of Messina
Messina
Italy


Lorenzo Apicella
Physical Medicine and Rehabilitation, Aggregate Venue of Florence
University of Pisa
Florence
Italy


Marco Basso
Pharmacology and Hematology
Castelfranco Veneto
Treviso
Italy


Prospero Bigazzi
Hand Surgery and Reconstructive Microsurgery Unit
AOU Careggi
Florence
Italy


Marco Biondi
Orthopaedic Clinic
University of Florence
Florence
Italy


Christian Carulli
Orthopaedic Clinic
University of Florence
Florence
Italy


Giancarlo Castaman
Department of Heart and Vessels
AOU Careggi
Florence
Italy


Massimo Ceruso
Hand Surgery and Reconstructive Microsurgery Unit
AOU Careggi
Florence
Italy


Federico Cipriani
Plastic Surgery and Reconstructive Microsurgery Unit
University of Florence
Florence
Italy


Roberto Civinini
Orthopaedic Clinic
University of Florence
Florence
Italy


Francesco De Martis
Department of Heart and Vessels AOU Careggi
Center for Bleeding Disorder
Florence
Italy


Giovanni D’Elia
Department of Radiology
AOU Careggi
Florence
Italy


Irene Felici
Orthopaedic Clinic
University of Florence
Florence
Italy


Marco Innocenti
Plastic Surgery and Reconstructive Microsurgery Unit
University of Florence
Florence
Italy


Massimo Innocenti
Orthopaedic Clinic
University of Florence
Florence
Italy


Silvia Linari
Department of Heart and Vessels
Center for Bleeding Disorder, AOU Careggi
Florence
Italy


Michael Makris
Sheffield Haemophilia and Thrombosis Centre
Royal Hallamshire Hospital
Sheffield
UK
/
Department of Infection, Immunity and Cardiovascular Disease
University of Sheffield
Sheffield
UK


Giuseppe Mangone
AOU Careggi
Florence
Italy


Caterina Martini
Orthopaedic Clinic
University of Florence
Florence
Italy


Fabrizio Matassi
Orthopaedic Clinic
University of Florence
Florence
Italy


Marco Matucci-Cerinic
Rheumatology Unit
University of Florence
Florence
Italy


Giuseppe Mazza
Institute for Liver and Digestive Health
University College of London
London
UK


Daniela Melchiorre
Rheumatology Unit
University of Florence
Florence
Italy


Dario Melita
Plastic Surgery and Reconstructive Microsurgery Unit
University of Florence
Florence
Italy


Giulio Menichini
Plastic Surgery and Reconstructive Microsurgery Unit
University of Florence
Florence
Italy


Enrichetta Paladino
Atherotrombotic Disease Unit, Department of Heart and Vessels
AOU Careggi
Florence
Italy


Filippo Parretti
Department of Radiology
University of Florence
Florence
Italy


Pietro Pasquetti
Rehabilitation Unit
AOU Careggi
Florence
Italy


Gianluigi Pasta
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico
Milan
Italy


Sandra Pfanner
Hand Surgery and Reconstructive Microsurgery Unit
AOU Careggi
Florence
Italy


Elisa Pratelli
AOU Careggi
Florence
Italy


Virginia Puliga
Department of Heart and Vessels Azienda Ospedaliero-Universitaria Careggi
Center for Bleeding Disorders
Florence
Italy


Paolo Quaglierini
University of Florence
Florence
Italy


Alberto Ricciardi
Department of Orthopedics
Castelfranco Veneto General Hospital, Castelfranco Veneto
Treviso
Italy


Paolo Radossi
Transfusion Service
Haemophilia Centre and Hematology, Castelfranco Veneto
Treviso
Italy


Anna Rosa Rizzo
Orthopaedic Clinic
University of Florence
Florence
Italy


E. Carlos Rodriguez-Merchan
Department of Orthopaedic Surgery
“La Paz” University Hospital-IdiPaz
Madrid
Spain


Giuliana Roselli
Department of Radiology
AOU Careggi
Florence
Italy


Giorgia Saccullo
Policlinico Paolo Giaccone
Centro di Riferimento Regionale per le Coagulopatie Congenite
Palermo
Italy


Luigi Piero Solimeno
Emergency Trauma Department
Ca' Granda Foundation, IRCCS Policlinico Hospital
Milan
Italy


Maria Chiara Susini
Department of Heart and Vessels Azienda Ospedaliero
Center for Bleeding Disorders, Universitaria Careggi
Florence
Italy


Giuseppe Tagariello
Transfusion Service
Haemophilia Centre and Hematology
Castelfranco Veneto, Treviso
Italy


Massimiliano Tani
University of Florence
Florence
Italy




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