What are the other Names for this Condition? (Also known as/Synonyms)

• Curved Spine due to Infantile Scoliosis
• Early-Onset Scoliosis
• Infantile Idiopathic Scoliosis

What is Infantile Scoliosis? (Definition/Background Information)

• Scoliosis is a common medical disorder characterized by abnormal curvature of the spine; a side to side spinal curve is observed. This disorder is caused by a defect that causes the spine (or the backbone) to bend or curve sideways, to look like an “S” or “C” instead of an “I”
• Infantile Scoliosis is a form of idiopathic scoliosis that affects children (especially males), who are younger than 3 years of age. The musculoskeletal disorder usually develops within the first 6 months of life
• The most common curve type is a left curve; right curves (especially if observed in girls), generally lead to a poor prognosis
• Infantile Scoliosis may sometimes automatically resolve itself. But in some cases, it may progress with severe complications. A treatment of the condition will include observation, physical therapy, use of braces, and surgery (in rare cases)

The exact cause of this disorder is not known, though two theories have been put forward. These are:

• The first theory is termed “the intra-uterine molding theory“, according to which, the spine is bent at the time of birth and gets worse with growth
• The second theory, termed as “post-delivery theory”, states that placing the infant on their backs, leads to a flattening of their skulls and causes abnormal spinal curvature (scoliosis)

The prevalence of scoliosis is observed more in Europe; hence, a genetic causal factor is suggested. But, the truth of this statement is yet to be proven

Who gets Infantile Scoliosis? (Age and Sex Distribution)

• Infantile Scoliosis is the term given to scoliosis occurring between birth and the first 3 years. Generally, boy babies are more affected than girl babies
• No ethnic or racial predominance is observed; however, the incidence is more prevalent in Europe
• Males account for 60% of the incidence of early-onset scoliosis and females account for 90% of the incidence of late-onset scoliosis
• Infantile Scoliosis is relatively rare and comprises only 1% of all idiopathic scoliosis cases in children

What are the Risk Factors for Infantile Scoliosis? (Predisposing Factors)

Risk factors for Infantile Scoliosis include:

• Age: Within 3 years after birth
• Gender: Boys are at high risk for contracting this disorder
• Geographical area: European children are more susceptible to Infantile Scoliosis
• Genetic factors may also increase the risk for this condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Infantile Scoliosis? (Etiology)

In 80% of the cases of Infantile Scoliosis, the exact cause cannot be pinpointed or fixed. It is thought of as an idiopathic condition. Idiopathic is a term which means that the underlying causes of a disease or disorder are unknown.

In the balance 20% of the cases, the causes can be fixed which may include other well recognized syndromes and the disorder has been termed syndromic scoliosis.

• Congenital spinal column abnormalities called as congenital scoliosis
• Neurological disorders and genetic conditions that are referred to as neuromuscular scoliosis

The exact cause for the incidence of Infantile Scoliosis is not known, but the following theories have been proposed relating to its cause:

• The first theory states that mechanical factors during intrauterine life are mainly responsible for the increased occurrence of plagiocephaly and developmental dysplasia of the hip and scoliosis
• A second theory proposes that multifactorial causes, such as predisposing genetic factors in combination with external factors, such as defective motor development, collagen disorders, joint laxity, and nursing posture of the infant can lead to scoliosis
• The third theory revolves around genetic inheritance, but research is still under process to find out the exact gene(s) involved

Other causative factors may include:

• Older mothers from poorer families
• Breech presentation
• Premature babies
• Low birth weight male babies

Most of the curves occur in the first year of life and this has correlation with the nursing posture of the infant. The incidence is more in Europe because Europeans follow the practice of nursing in the supine position.

What are the Signs and Symptoms of Infantile Scoliosis?

Infantile Scoliosis is usually detected during the first year of the baby’s life and signs and symptoms may be noted by the pediatrician during routine examination or by the parents.

• Usually Infantile Scoliosis presents itself through a long, thoracic curve to the left
• In rare cases, a thoracic and lumbar double curve may also be seen
• The shoulders appear to be of different sizes
• The position of the head may not be over the pelvis
• A raised and prominent hip, uneven waist is observed
• Different heights of the rib cages are common in patients with scoliosis
• Dimples, hairy patches, or color changes are seen on the skin overlying the spine
• The entire body leans on one side

How is Infantile Scoliosis Diagnosed?

Infantile Scoliosis is generally detected during the first 6 months of life. The diagnosis depends upon:

• Age of onset of the condition
• Absence of other spinal cord abnormalities
• Location of the curve

The diagnosis and confirmation of Infantile Scoliosis is based upon:

• Findings of the physical examination: A routine physical examination may be conducted by the pediatrician, nurse, or a caretaker The physical examination will reveal whether the curve is typically between the shoulder blades or if it is present in the thoracic region of the spine, and whether the spine curves to the left
• Neurologic examination and MRI scan is done to check that scoliosis is not caused by any neurological conditions and also to verify that the spinal cord is not affected by any other disorder
• X-rays give clear images of the bones and will allow for a more precise measurement of the spinal curvature
• A thorough clinical and radiologic examination can help rule out congenital, muscular, or neurological causes
• Anteroposterior radiographs may be done to evaluate the severity of the curve

Other tests that may be performed may include:

• CT scan to get a full picture of the scoliosis curve
• MRI scanning is necessary to check on the neural axis abnormalities associated with infantile Scoliosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Infantile Scoliosis?

Complications due to Infantile Scoliosis can include:

• Risks involved while administering anesthesia while opting for a surgical therapy and it will include:
  • Bleeding
  • Pneumonia
  • Wound complications such as poor healing and secondary infections
• Device related problems such as:
  • Allergic to metal
  • Bending, breaking, or loosening of the device
  • Neurologic defect due to stretching of the spinal cord from expansion
  • Crankshaft complication generally arises when the isolated posterior fusion method of surgery is used
• Paralysis: It is a very dangerous complication of scoliosis. It is reported that the incidence of paralysis as a complication of scoliosis is 0.72%. The occurrence of this complication seems to be at high rate in Infantile Scoliosis, because of the significant involvement of the neural axis
• Infection: Infections are likely to occur in any surgical procedures
• Pseudoarthrosis: This is a medical condition wherein the spine fails to fuse and is more common in adults
• Decompensation: A medical condition wherein the spine loses its flexibility causing the patient to lean on one side. This occurs due to an overcorrection of the spinal curve
• Flat-back syndrome: A medical abnormality wherein the patients need to hyper-extend their hips to stand or has to adopt a flexed-hip and flexed-knee gait, which will increase fatigue at the back
• Lower back pain: Pain in the lower part of the spine is a common complication which may be caused due to unfused levels of the spine or degeneration of the fused spine
• Rod fractures: Risk of rod fracture may occur in 15% of the cases suffering from scoliosis

How is Infantile Scoliosis Treated?

In most cases Infantile Scoliosis can resolve itself without any treatment. Also, children with mild curves do not require any treatment.

• When the curve is larger, it tends to be more progressive
• Curves between 10 and 20 degrees can resolve without any treatment
• When a curve is greater than 20 degrees, treatment needs to be given

The management methods for Infantile Scoliosis will include the following components:

• Observation methods
• Orthosis methods
• Operative methods

The final decision as to which management methods needs to be applied will depend on the rib-vertebral angle difference (RVAD, or also called the Mehta angle) which is a useful guide in distinguishing between resolving and progressive idiopathic Infantile Scoliosis. Scoliosis curves with RVAD of less than 20 degrees may be curable, but scoliosis with RVAD more than 20 degrees is considered to be progressive

• Orthosis methods are employed when the curve is progressive in nature
• Various types of orthosis available in the treatment of the condition include:
  • Hinged Rissar jacket
  • The plaster spinal jacket
  • Milwaukee  brace
  • Boston brace
• Braces should be used during the day and it should be removed only while exercising and swimming
• Braces should be used until skeletal maturity is attained, because curves do not progress after attainment of skeletal maturity
• Operation methods are used only in children who are over 10 years old
• Segmental posterior wiring to 2L-rods without fusion is preferred until combined posterior and anterior fusion can be done. Pedicle screw instrumentation can be done on children who have further growth potential
• An expert opinion of a specialist needs to be obtained regarding the progressiveness of the condition using the methods of physical examination and x-rays

Surgical therapy: The need for surgery to rectify scoliosis will depend on the following factors:

• Time of onset of the scoliosis
• Degree of the curve
• The exact location where scoliosis has occurred
• Response to treatment using brace
• Rate at which the curve is progressing
• Acceptability of the cosmetic appearance to the parent and patient

Once the child reaches skeletal maturity, the need for definitive fusion is recognized that will mean the removal of all implants and instrumentation.

• If any congenital problems or thoracic insufficiency is observed, then another type of growing rod system may be used (termed as vertical expandable prosthetic titanium rib or VEPTR)
• Another method of treatment available in the management of scoliosis in young children is the application of a localizer cast that will help in holding the curve and prevent further progression of the curve
• Pediatric isola spine system is another technique used to correct the condition, wherein screws and washers are applied posteriorly to anteriorly

How can Infantile Scoliosis be Prevented?

Prevention of Infantile Scoliosis is not possible (mainly if it is caused due to some unknown reasons); the treatment is aimed at mainly reducing the progression of the curve.

What is the Prognosis of Infantile Scoliosis? (Outcomes/Resolutions)

• A patient with non-progressive Infantile Scoliosis can enjoy a normal quality life and the prognosis is very good
• Prognosis will also depend on the RVAD. A difference of 20 degrees is non-progressive in nature in most cases and can be easily resolved
• Infantile curves of about 30 degrees can deteriorate without treatment
• Girls with right-sided curves tend to be more progressive
• 90% of the cases with early onset scoliosis resolve on its own; the balance 10% may progress to severe and disabling complications

An excellent prognosis is seen in the following cases:

• Male sex
• A left-sided curve
• A low initial curve measurement
• RVAD less than 20 degrees
• When onset of scoliosis occurs in the first year of life

Additional and Relevant Useful Information for Infantile Scoliosis:

The term Scoliosis is derived from the Greek word ‘skol’, meaning “twists and turns” and it refers to a sideways curve in the spine, which is either to the right or to the left.

• In the period between 460-377 BC, Hippocrates wrote about scoliosis and remedies for the condition
• Infantile Scoliosis is very rare and accounts for only 1% of the total cases of idiopathic scoliosis in North America; the same rate stands are 4% in Europe