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10b - Answer: 9-year-old female presents with intractable headaches

Pomeranz, Stephen
Stephen J Pomeranz, MD
Chief Medical Officer, ProScan Imaging. Founder, MRI Online
Includes DICOM files

HISTORY: 

This 9-year-old female presents with intractable headaches.

(QUIZ ANSWER) MOST LIKELY DIFFERENTIAL OF MASS: 

Rathke cleft cyst.

Using the diagnostic web viewer, we have provided images that assist in telling our clinical story. Areas of significance are indicated below.

FINDINGS:

Multiple series, posterior fossa and craniocervical junction within normal limits. Normal posterior pituitary bright spot. Note, this is displacing the posterior rightward aspect of the nominally enlarged sella turcica. Lesion occupies the anterior two-thirds of the sella and suprasellar region causing displacement of the pituitary stalk superiorly and draping to the right as evidenced on the coronal postcontrast T1 data set. Postcontrast, there is sort of nodular enhancement with absent enhancement centrally. This is reproduced on the sagittal FLAIR data set. Sagittal sequence demonstrates delayed intralesional central curvilinear regions of enhancement separate from the posterior neurohypophysis and anteriorly distorted, compressed adenohypophysis. No intraaxial region of abnormal enhancement on the sagittal, coronal, or short-axis review. Lesion measures 15mm craniocaudad with short-axis diameter of 9 x 9mm. Target contour to the suprasellar lesion at the level of the parasellar carotid arteries on T2 data set. No fluid-fluid level. No findings to suggest intralesional hemorrhage. 

Note mild prominence of the optic nerve sheaths bilaterally with equivocal flattening posterior globe to suggest soft findings of pseudotumor, idiopathic intracranial hypertension perhaps related to symptoms. 

In the coronal plane, the lesion abuts thecal sac decussation of the optic nerves without origin signal intensity to suggest primary optic nerve glioma. Following contrast administration, vigorous enhancement is shown in the sagittal plane along the margin of the lesion with central region of nonenhancement, somewhat angular architecturally. Rathke cleft cyst, craniopharyngioma favored based on these findings and location as above. 

Supratentorial brain demonstrates ventricles midline, normal in size. No region of abnormal enhancement and normal gray-white differentiation. No signal abnormality. 

Expected flow voids patent. No structural brain lesion. No focus of susceptibility signal alteration on the IRSSH data set. 

Extraaxial, sellar, suprasellar lesion demonstrates signal intensity isointense to brain on ADC and diffusion sequences. Best image resolution sagittal T2 clear data set with normal torquing along the inferior chiasmatic bifurcation. 

CONCLUSION: 

1. Sellar, suprasellar mass with claw sign. Draping of the pituitary infundibulum and preserved normally enhancing rightward adenohypophysis favoring Rathke cleft cyst. Craniopharyngioma is a differential consideration. Cystic or nontypical pituitary adenoma, macroadenoma is less likely. Optic chiasmatic glioma and EG are unlikely as the stalk is uninvolved. 

2. Recommend dedicated interrogation of the optic visual fields. Repeat imaging in 3 months.

3. Likely not related to the patient's intractable headaches; rather, note mild dilatation of the optic nerve sheaths such that pseudotumor could be considered.

4. Suggest endocrinology evaluation as Rathke cyst and craniopharyngioma should not have elevated serum proteins. 

LESSON 2, TOPIC 30

Case Challenge: Sella MRI Cases

Case Challenge

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Content reviewed: July 15, 2021

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