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Hematologic Disorders

Hemophilia Hemophilia, a genetically determined disorder, is characterized by abnormal blood coagulation as a result of a functional deficiency of factor VIII or IX. Since biblical times, the crippling deformities of the musculoskeletal system and death resulting from uncontrolled hemorrhage have been well depicted in the pages of history. The term hemophilia, coined by Hopff in 1828, means “blood loving.” Wright is credited with being the…

Metabolic and Endocrine Disorders of Bone

Biology of Healthy Bone Bone is a dynamic living tissue composed of two major constituents, an organic and a mineral component. The organic components consist of (1) cells, including osteoblast, osteoclast, and osteocytes, and (2) proteins, mainly type 1 collagen (COL1) and other non-collagenous proteins. Maintenance of healthy bone is a complex process that involves the synchronization of multi-organ systems that controls calcium and phosphate metabolism,…

Orthopaedic-Related Syndromes

Marfan Syndrome Patients with Marfan syndrome are a clinically diverse group, but they classically exhibit tall stature, long thin limbs, long thin spider-like digits (arachnodactyly), dislocation of the ocular lens, and cardiac anomalies. In 1896, Marfan, a French pediatrician, described to his colleagues the clinical features of Gabrielle, a 5-year-old girl with long thin limbs (leading him to describe the girl’s condition by the term dolichostemelia…

Skeletal Dysplasias

Overview This chapter describes the major skeletal dysplasias seen in the pediatric population. In each of these conditions a structural abnormality in the bone itself leads to disturbances in growth of the trunk or extremities. More than 200 bone dysplasias have been described, most of which are extremely rare. Most of the bone dysplasias result in short stature, which is defined as a height that is…

Muscle Diseases

The author wishes to acknowledge the contribution of Lori A. Karol for her work in the previous edition version of this chapter. Muscular Dystrophies Overview The muscular dystrophies are a group of genetically determined, progressive diseases of skeletal muscle ( Table 35.1 ). Muscular dystrophies are not inflammatory and thus are classified as myopathies rather than as myositis. By definition, pathologic changes occur within the muscle…

Disorders of the Peripheral Nervous System

Hereditary Motor and Sensory Neuropathies Hereditary motor and sensory neuropathies (HMSNs) are a group of genetically acquired progressive peripheral neuropathies ( Boxes 34.1 and 34.2 ). The most common of this group is Charcot-Marie-Tooth disease, described by the team of Charcot and Marie and, independently, by Tooth, in 1886. Dyck and Lambert classified the HMSNs in 1968 ( Table 34.1 ). Box 34.1 Dyck-Lambert Features of…

Poliomyelitis

The original language of this chapter by Dr. Tachdjian has been left unaltered as much as possible because we currently have no direct experience with acute poliomyelitis. Poliomyelitis is an acute infectious disease caused by a group of neurotropic viruses that initially invade the gastrointestinal and respiratory tracts and subsequently spread to the central nervous system (CNS) through the hematogenous route. The poliomyelitis virus has a…

Disorders of the Spinal Cord

Myelomeningocele Management of a child with myelomeningocele is one of the most challenging tasks faced by pediatric orthopaedic surgeons. a a References , , , , , , , . Typically, patients with myelomeningocele are referred to as having spina bifida, but a more specific definition of terms is in order. Myelomeningocele is one of the more severe forms of what is termed spinal dysraphism, which…

Disorders of the Brain

Levels of Involvement The neuromuscular system may be affected at various levels, each of which is characterized by changes in motor function peculiar to the site and extent of involvement. The differential features of various levels of motor function are illustrated in Table 31.1 . TABLE 31.1 Differentiation of Motor Disorders at Various Levels of Neuromuscular Function. Adapted from DeJong RN. The Neurological Examination . 3rd…

Lower Extremity Injuries

Pelvis and Acetabulum Pelvis Pediatric pelvic ring fractures are less common in children than in adults and are most often the result of high-energy trauma, most commonly a motor vehicle versus a pedestrian. Although mortality is rare, a pelvic ring fracture in a child should prompt investigation of other organ systems, including the vascular, genitourinary, and neurologic systems, for potentially life-threatening injuries as concomitant musculoskeletal and…

Upper Extremity Injuries

The author wishes to acknowledge the contribution of <ce:bold>John </ce:bold>A. Herring for his work in the previous edition version of this chapter. Injuries to the Clavicle The clavicle is one of the most frequently broken bones in children, , which is not surprising given that it is the only connection between the arm and trunk and consequently is subjected to all the forces exerted on the…

Spinal Injuries

The author wishes to acknowledge the contribution of John A. Herring for his work in the previous edition version of this chapter. Traumatic Injuries of the Cervical Spine Cervical spine injuries are rare in children and are often difficult to diagnose because of an inability to obtain a clear history and the difficulty of imaging an immature spine. Therefore, a high index of suspicion is necessary…

General Principles of Managing Orthopaedic Injuries

Skeletal injuries are common in children, with an estimated 40% of boys and 25% of girls sustaining a fracture by 16 years of age. Because of the properties of the immature skeleton, these injuries have different characteristics, complications, and management than those of similar injuries in adults. A number of studies have examined the epidemiology of fractures in children. , , , , Most studies have…

Malignant Bone Tumors

The author wishes to acknowledge the contribution of John A. Herring for his work in the previous edition version of this chapter. Osteosarcoma Osteosarcoma (osteogenic sarcoma) is the most common malignant bone tumor in children and adolescents. The most prevalent neoplasm is composed of high-grade malignant osteoblasts that directly form tumor osteoid or bone, although fibrous and cartilaginous elements may coexist or even predominate and there…

Benign Musculoskeletal Tumors

Simple Bone Cysts (Solitary Bone Cyst, Unicameral Bone Cyst) Incidence Simple bone cysts are benign tumors of childhood and adolescence. They represent approximately 3% of all primary bone tumors sampled for biopsy and nearly always occur during the first 2 decades of life, most often between 4 and 10 years of age. These cysts have a male predominance, with a 2:1 male-to-female ratio. Most cysts occur…

General Principles of Tumor Management

Tumors of the Musculoskeletal System Tumors of the musculoskeletal system present a variety of challenges. The pediatric orthopaedist manages many benign tumors easily with a good outcome, but occasionally serious complications develop. Surgeons with specific expertise in oncology provide the best treatment for patients with malignant tumors. Inexperience may lead to fatal treatment errors even at the stage of primary biopsy. Modern survival rates far exceed…

Infections of the Musculoskeletal System

The author wishes to acknowledge the contribution of John A. Herring for his work in the previous edition version of this chapter. Overview The evaluation and treatment of children with musculoskeletal infection is a multidisciplinary process, frequently involving pediatrics, orthopaedic surgery, infectious disease, emergency medicine, intensive care, radiology, anesthesiology, laboratory, and pathology. Under most circumstances a conclusive diagnosis can be derived after history, physical examination, laboratory…

Arthritis

Joints General Considerations A joint is a connection between bones of the skeleton. Joints can be classified as fibrous, cartilaginous, or synovial. Fibrous joints are represented by the sutures of the skull, whereas an example of a cartilaginous joint is the symphysis pubis; neither of these joint types allows gross motion. Synovial joints, also termed diarthrodial joints, are the movement units of the skeleton and the…

Limb Deficiencies

Normal development of the limbs begins at the end of the fourth week after fertilization, with limb buds forming in the mesoderm along the flank of the embryo ( Fig. 21.1 ). The limb bud is divided into three major regions. The apical ectodermal ridge (AER), in which several fibroblast growth factors (FGFs) are expressed, keeps the adjacent mesenchymal cells in an undifferentiated, rapidly proliferating state.…

Limb Length Discrepancy

Leg Length Inequality Leg length inequality in children is a frequent parental concern or physical finding noted by the orthopaedist. It is commonly identified incidentally during screening examinations such as those performed on armed forces recruits, as well as during scoliosis screening and routine assessments of the asymptomatic adult population. , , The management of this deformity varies from no treatment to extensive multistage reconstruction to…