Connatal Cyst in a 50-Year-Old Patient

In this letter to the editor, we would like to report a case of incidental connatal cyst in a 50-year-old male patient. The patient was on migraine medications; however, he had recurrent episodes of headaches with nausea and was asked to perform a magnetic resonance imaging (MRI) by a treating physician. There is no past history of seizures, paresis or plegia, sensorineural deficits, cranial nerve involvement, urinary incontinence, etc. in the patient. The patient was born of a normal delivery at term, with no history of neonatal hospitalization, prolonged jaundice, and respiratory distress. Neurological examination was normal. On MRI, T2-weighted (T2W) coronal images showed bilateral hyperintense cysts located superolaterally to frontal horns ([Fig. 1A] and [B]). Similarly axial T2W images showed the hyperintense cyst anterior to the frontal horns ([Fig. 1C]). On fluid-attenuated inversion recovery coronal images and T1W, they appeared hypointense ([Fig. 2A] and [B]) without any evidence of associated inflammation and periventricular gliosis. Given the location and no significant neonatal history in the patients, the findings on MRI are consistent with frontal horn cyst aka coarctation of lateral ventricles or connatal cysts.

Fig. 1 (A and B) Magnetic resonance imaging (MRI) T2-weighted coronal images; arrows depicting a well-defined, T2 hyperintense subcentimeter, cystic areas adjoining the superolateral margins of frontal horns and body of the lateral ventricles, bilaterally. (C) MRI T2-weighted axial image; arrow showing a T2 hyperintense cyst anteriorly adjoining the superolateral margins of frontal horns and body of the lateral ventricles.

Fig. 2 (A and B) Magnetic resonance imaging fluid-attenuated inversion recovery coronal images; arrows depicting periventricular hypointense cystic areas with no associated gliosis or volume loss, consistent with connatal cysts.

Connatal cysts are rare normal aberrations in ventricular structure. They are cystic areas found superolaterally to frontal horns of lateral ventricles and are considered to be formed due to adhesions between walls of lateral ventricle. Hence, they are also called coarctation of lateral ventricles.[1] [2] The lesions are most commonly found in neonates, and have been associated with preterm infants. They are usually seen to regress in pediatric age within 1 or 2 months. The incidence is reported to be 0.38%.[3] Majority of studies suggest that they are seen in less than 1% of low-birth-weight infants.[4] The aberration must be differentiated from other similar findings, especially subependymal cysts and cystic periventricular leukomalacia. The differentiation is based on the location of the cyst. Connatal cysts are usually seen near superolateral frontal horn, subependymal cysts at the level of or posterior to foramen of Monro and periventricular leukomalacia found predominantly in the white matter above the ventricles or occipital area.[5] [6]

Subependymal cysts usually present following grade 1 germinal matrix hemorrhage in preterms. And periventricular leukomalacia may develop into porencephalic cysts and is also seen with hemorrhage or infarction with poor prognosis.[4] [7] A very few case reports describe the presence of connatal cysts in adults[8] [9] significance of such findings in adults is not well studied; however, we believe them to be nonsymptomatic aberrant part of lateral ventricles. Treatment is not necessary even if the cyst is seen in adults.

^* K.M. is the co-first author.

Publication History

Article published online:
27 October 2023

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