Persistence of the craniopharyngeal canal

Abstract

Craniopharyngeal canal persistence is a congenital abnormality characterized by a bone canal extending from the nasopharynx to the pituitary fossa. Ascension of microorganisms through the channel can occur, triggering meningitis.

Craniopharyngeal canal persistence is a congenital abnormality characterized by a bone canal extending from the nasopharynx to the pituitary fossa. Ascension of microorganisms through the channel can occur, triggering meningitis.

Cranial malformations such as craniopharyngeal canal persistence may appear in association with multiple pituitary deficiencies. This canal can serve as a mean for microorganisms to ascend into the intracranial cavity, triggering meningitis, and its persistence is usually associated with other anatomical defects.

A 12‐year‐old boy diagnosed prenatally with hydrocephalus and born with associated cranial malformations, including cleft palate and ocular hypertelorism, presented to our department. He had a history of headache and recurrent meningitis. Diagnostic investigation revealed short stature associated with body disproportion (height >span), a Tanner scale stage of 1, a bone age of 6 years and central hypothyroidism. A glucagon test revealed growth hormone of 3 ng/ml (normal above 5.0 ng/ml) and cortisol of 10 µg/dl (normal above 18 µg/dl), reflecting growth hormone and cortisol deficiencies.

Magnetic resonance imaging showed a huge cystic formation representing cerebrospinal fluid content, extending from the third ventricle to the oropharynx (Figure 1). The final diagnosis was craniopharyngeal canal persistence associated with meningoencephalocele and corpus callosum agenesis. The child is undergoing periodic hormonal control and being evaluated for reconstructive surgery.

FIGURE 1.

Magnetic resonance imaging of the brain with sagittal T1‐ (A) and T2‐(B) weighted sequences and a coronal T1‐weighted sequence (C) demonstrated a large cystic formation extending from the third ventricle to the oropharynx (arrows), reflecting the persistence of the craniopharyngeal canal

Craniopharyngeal canal persistence is a rare congenital abnormality.¹, ² This canal can serve as a mean for microorganisms to reach into the intracranial cavity, triggering meningitis, and its persistence is usually associated with other anatomical defects. The herniation of brain tissue and meninges through this channel constitutes meningoencephalocele.² Most clinical symptoms in patients with this condition are related to hypopituitarism, including pubertal delay, short stature, and amenorrhea.² The most recommended approach for these patients is transcranial or transfacial surgery.¹, ²

CONFLICT OF INTEREST

The authors declare that they have no conflict of interests.

AUTHOR CONTRIBUTIONS

All the authors made substantial contribution to the preparation of this manuscript and approved the final version for submission. GMP: contributed to write the case and identify the images. NVW: performed literature search and helped in identifying appropriate images. EM: reviewed and edited the case report and helped in identifying appropriate images.

ETHICAL APPROVAL

Informed consent for publication and related images has been obtained from the patient's parents.

CONSENT

Appropriate written informed consent was obtained from the patient's parents for publication of this case report and accompanying images.

Peron GM, Wilner NV, Marchiori E. Persistence of the craniopharyngeal canal. Clin Case Rep. 2021;9:e04855. 10.1002/ccr3.4855

DATA AVAILABILITY STATEMENT

All data generated or analyzed during this study are included in this published article.