Mild trigonocephaly associated with microcephaly: surgical outcomes for 15 cases

Surgical treatment for mild trigonocephaly associated with microcephaly is often attempted if neuroradiological studies show evidence of increased intracranial pressure. However, it is difficult to decide how to approach surgically these patients. Herein, we report the surgical outcomes of the patients we have treated surgically in our center.

We performed surgery on 15 patients (ten girls and five boys) who were all diagnosed as microcephalic during infancy. All patients presented clinical symptoms and evidence of short stature. Symptoms included mental retardation, language delay, hyperactivity, motor dysfunction, and self-mutilation (head banging). Head circumferences were > 2 standard deviations below the normal range for their sex and age at the time of surgery. All patients were diagnosed with mild trigonocephaly based on three-dimensional computed tomography (3D-CT). No abnormal findings could be observed in the brain of 14 patients, as assessed by magnetic resonance imaging (MRI). One patient showed brain atrophy. 3D-CT showed marked digital markings in all. Intracranial pressure (ICP) was measured under normocapnia. Increased ICP could be observed in 13 patients. Decompressive cranioplasty was performed in all patients. After surgery, evidence of enlargement of the head circumference could be observed in six patients. Some degree of intelligence problems remained. However, every patient made some improvement in at least one of the other symptoms.

We suggest that decompressive cranioplasty may be indicated in patients with mild trigonocephaly associated with microcephaly, if pre-surgical evaluation shows high ICP and no abnormal brain findings can be identified on MRI.