Brain malformation in single median maxillary central incisor

Area of Science:

• Pediatric Endocrinology
• Developmental Biology
• Neuroradiology

Background:

• Midline defects can affect craniofacial and brain development.
• Growth hormone deficiency can impair somatic growth and sexual maturation.
• Single median maxillary central incisor (SMMCI) is a rare dental anomaly.

Observation:

• A 12-year-old girl presented with SMMCI, impaired growth, and a midline defect affecting the cranium, brain falx cerebri, and pituitary gland.
• Clinical, radiographic, and MR imaging revealed the extent of the midline developmental anomaly.
• Severe growth hormone deficiency was diagnosed, with no other pituitary hormone deficiencies identified.

Findings:

• Treatment with growth hormone over a four-year period resulted in significant gains in body height.
• Successful sexual maturation was achieved following growth hormone therapy.
• The study highlights the developmental association between SMMCI, midline brain structures, and pituitary function.

Implications:

• Early diagnosis of midline developmental defects is crucial for timely intervention.
• Understanding the association between SMMCI and pituitary dysfunction can guide diagnostic pathways.
• This case provides insights into the management of growth disturbances in children with complex midline anomalies.