Floppy Baby Syndrome

What Is Floppy Baby Syndrome?

Floppy baby syndrome is a term, in medical literature referred to as hypotonia of the newborns and infants, in which the muscles of the baby show lower tone and a child appears with less muscular strength. Muscular tone is registered as one of the 5 Apgar score components, graded from 0 to 2. Tone is a muscle resistance to stretching.

If a newborn shows lesser tonus with specific diagnostic assessments, it is declared to have a floppy baby syndrome. This condition may be a sign of other different illnesses and is not an isolated condition.

Symptoms

The suspicion that a baby has a hypotonic syndrome is given right at birth, as the baby’s extremities appear to hang from the obstetrician’s hand freely, and in a healthy baby tone, legs and arms are in flexion. A child may naturally take some unusual positions with hypermobility of the joints.

Baby’s head appears as the muscles of the neck can’t hold it in vertical position, and the head easily drops depending on the position. The tone may be lowered some time after the birth, and usually the mother comes with suspicion that a baby appears floppy and that something is wrong.

Movements of the limbs are limited and weak, and motoric activity is less in general, although the baby is alert and responsive. A baby usually, has problems with swallowing, which causes saliva to appear around the face. Crying is weak. Babies with weak muscular tone have often paradoxical movements of the chest while breathing, which may also lead to suspicion. Hypotonia may not be generalised but rather on only one arm.

Causes

Floppy baby syndrome may be caused by various innate illnesses orf can be a transient condition that would spontaneously or with exercises improve.

Depending on whether there is an obvious weakness of the muscles or not, the cause is either neuromuscular or of other etiology. Hypotonia is in 75% of children caused by some perinatal ischemia of the brain or spine or genetical syndromes. Hypotonia may appear as a sign in:

• Premature babies
• Neuropathy (illness of the spinal cord, syringomyelia, spinal dysraphism, cerebral palsy); problem may lie in peripheral or central motoric neurone.
• Myopathy (myasthenia gravis, muscular dystrophy, Marfan syndrome and Ehlers-Danlos syndrome)
• Innate syndromes (Prader Willi syndrome, Down syndrome, Riley-Day syndrome, trisomy 13, spinal muscular atrophy, metabolic diseases Tay-Sachs and Pompe)
• Botulism
• Drug-related hypotonia
• Kernicterus, congenital hypothyroidism or hypoglycemia
• Benign essential hypotonia (unclassified hypotonic disorders).

Diagnosis

Pediatrician will have to take full history from the mother about her health and genetical illnesses, pregnancy and whether a child has been conceived naturally or artificially; has she had any problems during pregnancy (polyhidramnios, fetal movements or any abnormality seen on ultrasound), has she had any troubles during labour (breech or any other difficult position) and what was the general condition of the baby after birth, what was baby’s Apgar score, birth weight, length, chances for hypoxia/anoxia during the delivery.

Then, it is important to assess where the problem and cause of the floppy syndrome lies. Genetical testing including karyotype and FISH studies are performed, but also EEG, CT or MRI or even lumbar puncture, if the problem appears to be in the brain.

If a baby shows signs of weakness, it is indicated that muscle diagnostic procedures are performed (EMG, muscle or nerve biopsy, creatine kinase test, DNA-based mutation testing).

Some genetical disorders show specific signs on face or other parts of the body, for example, specific signs of Down syndrome are small head and ears, eyes slant upward, protruding tongue, flat back of the head, palm crease; and for Prader Willi : strabismus, narrowing of the head in temporal region, poor responsiveness.

Other signs that may appear and that may resemble as a genetical condition are tongue fasciculation, fish mouth, ptosis of the eyelid, flat back of the head, macrocephaly, frog leg position.

Specific tests

• Traction response : traction of the baby’s arms upwards while laying down in the sitting position, the baby lifts its head slower than normal and shows inability for countratraction, which is a sign of low tone.
• Ventral suspension : baby holds in pronatory position on practitioners hand and its legs and arms are not flexed in floppy baby, also head is bent down without tonus of the muscles of the neck.

It is important to check for muscle reflexes. Examiner should measure the strength of each individual muscle. Other analyses such as blood tests are also recommended in order to find out about the level of electrolytes (low K⁺⁺ and phosphates), thyroxin level, albumins (malnutrition). ^{(5) (6)}

Treatment

Initial treatment that could be beneficial for any etiology is physiotherapy. Specific treatment depends on the age of the infant and general condition and associated diseases. This treatment will improve muscle strength. Besides the striate muscles of the arms, leg and neck are affected, muscles of the chest are also affected, which is why it is important to prevent respiratory infections.

Proper nutrition is important adapted to the child’s abilities and needs. Children with metabolic illness will need to maintain stable body weight, since they are prone to obesity. Overall specific therapy for each individual disease is used.

Life Expectancy

Life expectancy depends on the nature of the illness that manifested with floppy baby syndrome. Babies with the severest types usually live to the ages of one or two years old. Mild and moderate disorders develop differently, depending on the treatment and impact on the health, with more-less slower development from healthy children.

Prognosis

There is not much improvement, even though the cause is found and specific treatment began, and the prognosis is usually progressive depending on the type of illness.

The effects on the quality of life are different depending on the illness characteristics. Physical treatment improves hypotonia for most of the cases, but there is still some delay in development of speech, sitting, walking. Some children have severe type of illness and they are more limited in daily functioning. (6)

References:

1. http://pedsinreview.aappublications.org/content/30/9/e66.figures-only.
2. http://www.therapiesforkids.com.au/conditions/down-syndrome.
3. http://obgynkey.com/neurological-examination.
4. http://malaysianewsss.blogspot.rs/2016/12/anak-saya-ni-dah-lembik-tak-bermaya.html.
5. Clinical Approach To The Floppy Child. Van Toorn, R. August : s.n., 2004, The Floppy Child.CME.22(8), pp. 449-55.
6. Hypotonia. Encyclopedia of childrens health. [Online] [Cited: 2 5, 2017.] http://www.healthofchildren.com/G-H/Hypotonia.html.