Fibromatosis Colli

Fibromatosis Colli

Article
Bone, Muscle, & Joint
Diseases & Conditions
+2
Contributed byMaulik P. Purohit MD MPHNov 06, 2019

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

  • Pseudotumor of Infancy (Fibromatosis Colli)
  • SCM Tumor of Infancy
  • Sternocleidomastoid Tumour of Infancy

What is Fibromatosis Colli? (Definition/Background Information)

  • Fibromatosis Colli is a benign tumor of infancy occurring in the sternocleidomastoid muscle (SCM) located in the neck. This affects the muscle and it shrinks or shortens leading to sideways tilting of the head, a condition known as torticollis
  • The tumor is believed to develop from an underlying neck muscle injury, which may take place due to improper positioning of the fetus while still in the uterus, or from birth-related trauma
  • Most cases of Fibromatosis Colli are identified early and treated to complete resolution using conservative measures such as physiotherapy and muscle strengthening techniques. 10% of the cases may require a surgery to correct the abnormality
  • The prognosis of Fibromatosis Colli with prompt recognition and adequate therapy and exercises is generally excellent. In some cases, a delay in diagnosis may result in increased deformity necessitating intensive treatment

Who gets Fibromatosis Colli? (Age and Sex Distribution)

  • Fibromatosis Colli a rare condition affecting the newborn child. It is generally present at birth, but diagnosed a short while later (usually before 6 months)
  • The general incidence of this condition is around 1 in 250-300 live births
  • Both males and females are equally affected, though a slight male predominance is seen
  • No racial preference is seen, and no worldwide geographical restriction is noted

What are the Risk Factors for Fibromatosis Colli? (Predisposing Factors)

Risk factors for Fibromatosis Colli include:

  • Injury to the sternocleidomastoid (shoulder and neck) muscle while the fetus is in a development stage
  • Presence of developmental disorders involving the muscular and skeletal system
  • Abnormal position of baby while in the uterus
  • Any undue mechanical or physiological stresses during fetal development
  • Difficult or complicated delivery that might risk injury to the newborn
  • Defects of the forefoot and congenital hip joint malformation/dislocation at birth: Fibromatosis Colli can be associated with defects of the forefoot and hip joint. If a healthcare provider detects these defects, then the additional possibility of Fibromatosis Colli should be considered

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 Fibromatosis Colli? (Etiology)

  • Fibromatosis Colli is a benign tumor that is believed to be a scar remnant of an injury sustained to the sternocleidomastoid muscle (SCM) during the 3rd trimester or at childbirth
  • The scar is normally seen on the neck, nearer to the shoulder region. The scar muscles thicken to present a tumor-like appearance at the site; the fibrous tumor is only composed of muscles and is limited to SCM
  • Fibromatosis Colli causes a contraction or shortening of the muscle, leading to slanting of the head towards one side and limiting muscular motion range
  • The formation of this fibrous tissue tumor occur due to a host of factors that include:
    • Intrauterine crowding (less fluid in the womb during pregnancy, also known as oligohydramnios)
    • Neck muscle injury during delivery
    • Inherited/acquired developmental abnormalities affecting the musculoskeletal system

What are the Signs and Symptoms of Fibromatosis Colli?

Fibromatosis Colli may present the following signs and symptoms:

  • Presence of a spindle-shaped mass on the sternocleidomastoid muscle that connects the shoulder, neck, and lower jaw
  • A soft tumorous swelling may be noticed in the neck region, on the side towards which the head slant is observed
  • The tumor is usually less than 5 cm in length and less than 2 cm in thickness
  • The baby’s head is tilted to one side (mostly to the right side), with limited movement of the neck. The abnormal head position becomes apparent after a few months
  • Due to the baby frequently sleeping on one side, the head or skull base may flatten and acquire an asymmetrical shape
  • Improper head postures may create breastfeeding issues; feeding from one of the breasts may be difficult in some cases

How is Fibromatosis Colli Diagnosed?

A simple physical examination of the neck by the pediatrician, with study of birth history may be conclusive enough to diagnose the neck muscle abnormality in newborns and children. Often, such a diagnosis occurs during the first 6 months following birth.

The diagnostic tests performed for Fibromatosis Colli may include:

  • Physical exam with evaluation of medical history: Physical examination includes an assessment of the infant’s ability in performing basic age-appropriate physical activities
  • X-ray of the neck (cervical spine assessment)
  • Ultrasound scan of the neck and hip (if hip dysplasia is suspected); the study can show the tumor or muscle thickening at the neck region
  • MRI scan for a better and more detailed analysis and tumor characterization
  • Tests to determine other underlying conditions, such as neurological exam, MRI scan for skeletal survey, etc.
  • Tumor biopsy: Many experts say that a biopsy may not be necessary. But when necessary, a fine needle aspiration cytology (FNAC) is considered adequate

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 Fibromatosis Colli?

  • Complications of Fibromatosis Colli are mainly related to the extent of the deformity (severe sideways tilt of the head) and the presence of other abnormalities
  • In some infants, the neck appearance may be normal to mild and almost invisible. However, it may progressively worsen if left untreated, causing facial asymmetry

How is Fibromatosis Colli Treated?

The treatment measures for Fibromatosis Colli are basically meant to prevent worsening of the condition and correct the defect. The interventions could be surgical or non-surgical.

Surgical methods are mainly used if the condition is classified as severe by the physician and do not improve after non-surgical procedures, which form the usual line of treatment. The management measures include:

  • Physical therapy:
    • It is used to gently help increase the range of motion and provide proper shape to the head
    • It is also used to prevent further muscle contracture and loss of muscle function
    • This is achieved by using age-suitable stretching and musculoskeletal strengthening exercise program, which helps in decreasing muscle fatigue and stress
  • Use of special child neck collar (called TOT collar), after the age of 4 months
  • Application of warm compress or heat
  • Surgical techniques:
    • These are employed if symptoms persist after one year of conservative management of the condition
    • Tenotomy (or the cutting of a tendon) is the type of invasive procedure used; only 1 in 10 cases may merit a surgery
    • This is then followed by post-surgical physiotherapy
  • If required, Botox injections may be used to improve and strengthen the muscle condition
  • Correction of any underlying muscular or skeletal system defects, if present

Occasionally, a spontaneous resolution of the condition is seen and the child gets better without any major treatment measure being employed.

How can Fibromatosis Colli be Prevented?

  • In most cases, Fibromatosis Colli is a condition that may not be preventable
  • Adequate prenatal care and close monitor of child delivery process is emphasized to reduce risk of complications

What is the Prognosis of Fibromatosis Colli? (Outcomes/Resolutions)

  • Majority of the times, Fibromatosis Colli can be treated to complete recovery within a year (a high rate of success is often seen). The best results are when the baby is very young, the condition is diagnosed quickly, and therapy commenced as early as possible
  • Physiotherapy and stretching exercises to strengthen the muscle can bring about healing in most children and restore complete head and neck movement and resolve the abnormal tilt/posture
  • If prolonged therapy fail to achieve results, then surgical intervention may be required to correct the muscular defect; the prognosis in such cases is also good
  • A delay in diagnosis and/or a late start of treatment increases the chance of a greater deformity and severity of the condition, particularly if the child is over 1 year old

Additional and Relevant Useful Information for Fibromatosis Colli:

  • Torticollis is a muscular disorder that results in a twisted neck or a sideways tilted head, due to muscles of neck undergoing abnormal muscle contractions. It can be congenital or acquired

The following link will help you understand congenital muscular torticollis:

https://www.dovemed.com/diseases-conditions/congenital-muscular-torticollis-cmt/

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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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