Spinothoracic disorders

Orthopedic problems of spine and thoracic cage often co­exist and may be congenital, developmental or acquired. Apart from following defects, spinothoracic defects are also integral components of many skeletal dysplasias (Chapter 23.4).

Torticollis (Wry neck) is a sign and not a disease, characterized by twisting of neck with head tipped on one side and chin rotated on other side. Presence of torticollis indicates a congenital or acquired deformity of cervical musculature or spine (Table 23.2). Important types of torticollis are as follows:

Congenital torticollis is commonly caused by sterno- mastoid tumor, i.e. birth trauma to sternocleidomastoid

TABLE 23.2: Causes of torticollis

Congenital

• Birth trauma (Sternocleidomastoid tumor)

• Vertebral defects: Klippel-Feil syndrome

• Atlanto-axial joint instability

Acquired:

• Transient torticollis

• Post-traumatic cervical injury

• Indammatory:

- Local: Osteomyelitis, Pott's spine

- Adjoining: Retropharyngeal abscess, adenitis

- Systemic: Rheumatoid arthritis

• Neurological:

- Central: Dystonia, posterior fossa tumors

- Spinal: Tumors, syringomyelia

• Others: Hysteria, phenothiazine dyskinesia

muscle and manifests in early infancy (Ch 12.9.2). However, structural vertebral defects must be excluded before advising neck exercises, which may injure cervical spinal cord.

Klippel-Feil syndrome, i.e. multiple coalitions of cervi­cal vertebrae, is a rare cause of recurrent torticollis, characterized by a triad of: (a) short and webbed neck, (b) low hairline and (c) restricted neck movements. Other congenital defects, e.g. genitourinary malformations, deafness, CHDs and Sprengel deformity (winged scapula) are common.

Transient torticollis is common during viral infections or minor cervical trauma/strain, which improves spon­taneously in 2-3 days.

Atlanto-axial joint instability is a rare but potentially life-threatening problem due to: (a) congenital absence of hypoplasia of odontoid process, or (b) ligamental laxity, e.g. in Down syndrome or axial myopathies, or (c) post- traumatic or post-inflammatory degeneration of cervical vertebrae. Normally, flexion/extension movements of neck occur at occipital-C₁ joint, while rotation occurs at C₁-C₂ joint. Neither of these joints are intrinsically stable and depend on surrounding joint capsules and ligaments for stability.

Recurrent torticollis may be first indication of atlanto­axial joint instability and these children are at-risk for sudden death due to spinal compression or chronic myelopathy. Detailed neuroimaging is indicated in all suspected cases, followed by preventive advice, e.g. avoidance of contact sports.

Surgical correction with cervical spine fusion is indi­cated in children with recurrent torticollis with severe instability and/or chronic myelopathy.

Spinal deformities are usually developmental or acquired (Table 23.3), tend to worsen with growth and may lead to significant cosmetic deformities, compressive myelopathy and respiratory insufficiency.

TABLE 23.3: Causes of spinal deformities

TABLE 23.4: Causes of back pain

• Idiopathic scoliosis or kyphosis

• Congenital vertebral anomalies

• Postural: Leg-length discrepancy

• Neuromuscular disorders

- Central : Cerebral palsy

- Spinal : Poliomyelitis

- Vertebral : Pott's spine, trauma, tumors

- Myopathies : Duchenne muscular dystrophy

• Miscellaneous:

- Postural, arthrogryroposis

Clinically, these deformities manifest either with abnormal spinal curvature, e.g. scoliosis, kyphosis or lordosis; or restricted spinal movements. A normal child has some degree of physiological lordosis in cervical and lumbar region and kyphosis in thoracic and sacral region.

Clinically, Adams test is frequently used to detect subclinical scoliosis, which reveals asymmetry of posterior back contour on forward bending, with ele­vation towards the convex side of scoliosis.

Diagnosis is confirmed on standing spinal X-rays and Kobb's method (angle between long axis of proximal and distal spine at the point of maximum curvature) is used to assess severity of scoliosis. As idiopathic sclerosis rarely leads to compressive myelopathy, neuroimaging is routinely not necessary.

Course: Idiopathic scoliosis is usually progressive during period of skeletal maturation, though the rate of progression depends on various factors, being higher in: (a) early-onset scoliosis, (b) females, specially with premenarchal onset, and (c) severe scoliosis (gt;3o°) at the time of diagnosis.

Management includes long-term brace support, specially till skeletal maturation is complete and/or surgical correction for severe scoliosis (gt;45°).

Congenital scoliosis is evident at birth or manifests later, caused by: (a) defective vertebral formation, e.g. hemivertebrae, or (b) defective vertebral segmentation, e.g. presence of unsegmented bars. Spinal dysraphism and urogenital disorders are common in these cases.

Progression of deformity and risk of compressive myelopathy depends on the severity of developmental defect, though ~25% cases are non-progressive. Orthotic treatment is of little value, which should be corrected surgically if severe or progressive.

Neuromuscular spinal deformities, due to static lesions (cerebral palsy, poliomyelitis) or progressive disorders (Duchenne muscular dystrophy) are common in Indian children. Even static pathology may lead to progressive deformities in children due to developing skeletal system.

• Developmental defects

- Congenital scoliosis/kyphosis (pre-adolescents)

- Idiopathic scoliosis/kyphosis (adolescents)

• Indammatory disorders

- Local: Pott's spine, osteomyelitis

- Adjoining: Pyelonephritis, epidural abscess

- Systemic: JRA, spondyloarthropathies

• Traumatic disorders

- Direct trauma: Sports injuries, accidents

- Mechanical stress: Gymnasts, heavy schoolbags

- Pathological #: Hemolytic anemia, osteoporosis

• Neoplastic disorders

- Primary/metastatic vertebral tumors

- Spinal cord tumors

- Bone marrow malignancies e.g. leukemia

• Psychological: Conversion reactions

JRA: Juvenile rheumatoid arthiritis

Management in these cases aims to prevent further deformities and provide maximum functional indepen­dence, by external supports (braces), physiotherapy and surgical intervention in selected cases.

Back pain in children is frequently organic, unlike psychol ogical or mechanical back pain in adults and needs detailed evaluation, if persistent.

Common causes of prolonged back pain in Indian children are spinal trauma, developmental defects and inflammatory disorders of spine, each accounting for ~30% of cases (Table 23.4).

Complete physical and neurological evaluation with neuroimaging studies is indicated in children with following danger signals: (a) progressively increasing or persistent back pain, (b) neurological signs, e.g. limb weakness, paresthesias or bladder/bowel dysfunction,

(c) constitutional features, e.g. fever and weight loss, and

(d) spinal deformities.

Thoracic cage deformities are usually cosmetic, but may lead to significant respiratory insufficiency/failure. Some important abnormalities are as follows:

Pectus carinatum (Pigeon chest), i.e. prominent sternum with increased antero-posterior diameter of chest, may be congenital or acquired following rickets, recurrent respiratory infections in early childhood, or obstructive airway disorders.

Pectus excavatum (Funnel chest), i.e. depressed sternum with decreased antero-posterior diameter of chest, may also be congenital or acquired due to causes as for pectus carinatum. Mitral valve prolapse is relatively common in children with congenital pectus excavatum.

Cervical ribs are accidentally diagnosed in children on routine X-rays and rarely symptomatic, though in adults, have been associated with Raynaud's phenomena. No treatment is needed.