Edition 64 - 2017, July / Images

Images in Pediatric Endocrinology: CRANIAL COMPUTED AXIAL TOMOGRAPHY IN A CHILD WITH UNSTABLE WALKING – Ed. 64

Paula Plomer, Mariana Costanzo and Maria Isabel Di Palma

Servicio de Endocrinología. Hospital de Pediatría Juan. P Garrahan, Buenos Aires, Argentina

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  1. Multi-slice computed tomography with intravenous contrast. An expansive solid-cystic lesion, partially calcified is observed at the suprasellar region with extension to the third ventricle and the interpeduncular and prepontine cisterns.
  2. The solid portion showed heterogeneous enhancement with gadolinium. 3D reconstruction with bone window showed a calcium image at the tumor region.

 

 

Clinical case

A 9.4 year-old boy, born in La Paz (Bolivia), was referred to our hospital to confirm the diagnosis of craniopharyngioma.

He was born at term, with adequate birth weight, and without remarkable clinical history before the onset of the present complains.

His father referred progressive weakness of the right leg with functional impairment, associated with right arm weakness and dysarthria.

CT and angio CT performed in Bolivia, revealed a heterogeneous multilobulated and calcified tumor (5.4 cm) centered at the supraselar region, compatible with craniopharyngioma.  Angio CT ruled out associated vascular anomalies.

At first evaluation in our hospital he was in a regular general condition with drowsiness and 15/15 Glasgow score. Difficulties to sit, trunk instability, right brachiocrural hemiparesis with hemiparetic and broad based gait, ataxia, swallowing impairment; isochoric and photoreactive pupils were observed. No history of headache, nausea or vomiting was referred.

Anthropometry:

-Weight: 28,200 kg (-0.35 SDS)

-Height: 121.5 cm (-1.88 SDS)

– Pubertal status: G1, VP1, 2 ml scrotal testicles were observed.

Biochemical studies were requested with normal findings (Table 1)

Ophthalmologic evaluation: normal eye fundus, visual acuity test: right eye 3/10 and left eye 7/10 (previously known).  Normal confrontation visual field test.

Brain MRI confirmed a heterogeneous, solid-cystic and expansive lesion at the suprasellar region, with the cystic component extending towards the interpeduncular and prepontine cisterns, which generates mass effect with compression and distortion of the brain stem. The solid component presents a predominant hypointense signal that may correspond to a calcium deposit.

Surgery was performed with evacuation of the intra-cystic content, consisting on a “motor oil” type material, and a communication of the temporal cyst, by a subtemporal approach, with an intracystic Ommaya catheter was placed.

The patient recovered after surgery with significant clinical improvement of the right brachiocrural hemiparesis in the first 48 hours and the swallowing disorder in less than ten days. No evidence of hormonal deficits in the immediate or remote postsurgical period was detected.

Table 1

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Comments

Craniopharyngioma are rare embryonic malformations of the sellar and parasellar area derived from vestiges of the craniopharyngeal canal. This tumor accounts for 1 to 3% of all childhood and adolescent primary intracranial tumors, with an incidence of 0.5 to 2 cases per million persons per year. (1). Craniopharyngiomas can be detected at any age, however there is a bimodal distribution of the age at diagnosis, with the first peak incidence between the ages of 5 to 15 years and a second peak between 50 to 74 years of age. Despite a low histological grade (WHO I°)(2), craniopharyngiomas are locally aggressive, can infiltrate adjacent organs and structures such as the hypothalamus, pituitary, chiasm, and optic nerves and cause per se considerable morbidity and mortality. Clinical picture at the time of diagnosis is often dominated by its expansive nature or by nonspecific manifestations of intracranial pressure (eg, headache and nausea), visual impairment and endocrine deficits (3).

Other manifestations, although less frequent, are: chemical meningitis due to rupture of cysts to the subarachnoid space, short-term memory and attention disorders, seizures and, as in this patient, neurological impairment including hemiparesis, and  cranial nerves deficiency. (1,3)

The diagnosis is based on the clinical and radiologic findings, and is confirmed by the histological study (3). The skull radiograph is a simple and accessible study, and a useful tool to detect pituitary abnormalities. Different alterations have been reported, such as: dorsum erosion, selar widened and enlargement, sclerosis of the selar floor and calcifications (4). The latter is the most frequent finding that can be detected in about 60% of the cases (4) In this patient this study was not performed, however, a CT scan clearly demonstrate calcifications (90%) and gives a better assessment of the tumor size and the local involvement , especially with high resolution techniques. The study carried out on this occasion, a CT with 3D reconstruction, is not requested as a routine study. However it is interesting to observe how the calcification present in the tumor is visualized. MRI is the study of choice when planning the surgical approach (1), since it allows to evaluate, not only the tumor size, but also its extension and the infiltration of the adjacent tissues, mainly the hypothalamus.

 

References:

  1. Muller HL. Craneopharyngioma. Endocrine Reviews, June 2014,35(3):513-543.
  2.  Tatter S. The new WHO classification of tumors affecting de Central Nervous System http://neurosurgery.mgh.harvard.edu/newwhobt.htm Acceso
  3. Venegas E., Blanco C., Martin T y Soto A. Guía práctica del manejo y tratamiento de los craneofaringiomas y otras lesiones paraselares. Endocrinología y Nutrición 2015;62(1):e1-e13
  4. Sorva R, Jaaskinen J, Heiskanen O. Craniopharyngioma in Children and Adults. Correlation between radiological

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