EARLY DIAGNOSIS OF CRANIOSYNOSTOSIS IN INFANTS AT PRIMARY HEALTH CARE

Craniosynostosis or premature fusion of one or more cranial sutures in infants disturbs normal brain growth. This condition causes abnormal skull configuration, increased intracranial pressure, headache, strabismus, blurred vision, blindness, psychomotor retardation. The diagnosis of craniosynostosis is very simple. Pediatricians should routinely assess neurological status and measure head circumference and anterior fontanelle. When necessary, ultrasound of CNS, X-ray and cranial CT scan can be done. When it comes to this condition, early diagnosis and surgical intervention are of utmost importance. In this paper, we have presented a case on craniosynostosis in a female infant, discovered in the third month of life during systematic review that included measurement of head circumference, palpation of anterior fontanelle and cranial sutures. The child was referred to a neurosurgeon who performed the CT scan of endocranium and confirmed the initial diagnosis of craniosynostosis. With head circumference of 40 cm and fused anterior fontanelle, the surgery was timely performed at the sixth month of life due to early diagnosis.


INTRODUCTION
Craniosynostosis or premature fusion of cranial sutures in the infant period is a serious condition that disrupts brain growth and development due to limited endocranium (1).Clinical features depend on the number of fused sutures and duration of the disorder in question.Symptoms occur as a result of pressure on the brain tissue.The most common type of craniosynostosis represents the fusion of one suture, that is, the sagittal one (2).Craniosynostosis may be primary, i.e., present at birth or in the first months of life, and secondary which is more frequent, whereby it arises as a part of numerous other syndromes (3).
Clinical findings include an abnormal skull configuration, reduced head circumference with fused anterior fontanelle or fontanelle with reduced dimensions, increased intracranial pressure, impaired vision or blindness due to pressure on the optic nerve, psychomotor development delay, whereas epileptic seizures and other disorders are rarely identified (4).

CASE PRESENTATION
The patient is a full term female infant, born on March the 6 th , 2013, to mother who had not been pregnant before.The pregnancy was normal and supervised.Normal childbirth, BM of 2950 g, BL of 49 cm, head circumference of 33 cm (above third length percentile for her age class and sex) Apgar score 9. Family anamnesis is normal.
At the first systematic review on the ninth day of life, a spontaneously resolving mild degree of jaundice was noted, whereas the other findings were normal.At the second systematic review (2 months and 12 days of age), protrusion of the forehead in the midline, overlapping parietal bones, dimensions of anterior fontanelle 1 x 0.5 cm, folds in the occipital region of the scalp, head circumference of 36.5 cm (third length percentile) were identified.Neurological status was normal.Child was lively and attentive with no signs of psychomotor development delay.Other findings were normal.
The child was referred to a neurologist and neurosurgeon under the suspicion of craniosynostosis.CT revealed fusion of both lambdoid sutures with overlapping parietal and occipital bones.Brain parenchyma was of the proper volume and density with no focal changes.Subarachnoid space was very constricted in the occipital region.The head had occipital flattening.On the basis of clinical findings and CT scan results, the child was diagnosed with Craniosynostosis and referred to surgical treatment.

UDK: 2014; 9(3): 229-231 ID: 212447756 ISSN-1452-662X
Prikaz slu~aja With the upcoming surgery, at the age of 4 months and 16 days, the attending pediatrician performed an examination.Neurological status was normal and the head circumference was 37.5 cm, which is below the third length percentile for her age class and sex.
After the sixth month of life, the following surgery was performed: Suturectomia suturae lamdoideae billateralis.The child was admitted to the hospital with fused anterior fontanelle and head circumference of 40 cm which is below the third length percentile.The postoperative course was uneventful and the child was discharged home in good general condition.A pediatrician performed four more check-ups at the Health Care Center.At the age of seven months, the head circumference was 40.5 cm, at the age of eight months was 41 cm, at the age of 10 months was 42 cm, at the age of 11 months was 42 cm, still below the third length percentile for her age class and sex -see percentile growth curve of the head (Figure 1).Neurological status and psychomotor development of the child were within normal limits for the whole time.Child was being taken to regular check-ups with the neurosurgeon.

DISCUSSION
Clinical features of craniosynostosis with the fusion of one suture, and especially with the fusion of multiple cranial sutures, are very serious and therefore pediatricians should strive for early diagnosis.Treatment depends on the timing of the diagnosis and which sutures are involved.While the timing of surgery is controversial, most doctors prefer to do surgery when the child is 3-6 months old.Older children (12-18 months) with a very mild deformity will probably not need surgery, while younger children with an obvious deformity that is moderate to severe will probably benefit from early surgery (5,6).In my opinion, the diag-nosis can be easily set at the level of primary health care during regular medical examinations within the first months of life, or up to one year at the latest.Ultrasound of CNS as a part of prenatal diagnostics enables us to examine the condition of cranial sutures, fontanelles and brain structures at the stage of intrauterine life (7,8).As for postnatal diagnostics, regular measurement of head circumference and dimensions of anterior fontanelle, as well as palpation of the cranial sutures are simple diagnostic procedures.If the anterior fontanelle is still open, it would be advisable to perform an ultrasound of CNS due to increased intracranial pressure (9).Since changes in the retina might occur, it is desirable to do ophthalmologic examination, and possibly ENT screening (10).And finally, skull X-ray can be performed if necessary, as well as the CT scan of endocranium, as it was done in the case presented.
Although the head circumference is still below the third percentile for her age class and sex, there is an increase in head circumference by 3 cm, with the expectation of reaching its full size in time (Figure 2).

Figure 1 .
Figure 1.Infant's Head Circumference for AgePercentile Growth Chart

Figure 2 .
Figure 2. Head circumference growth per months