Case Report

A case of partial empty sella syndrome A. P. Krithika, R. Somasekar, Pooja Pradeep*

ABSTRACT

An 8-year-old male child presented to our pediatric outpatient department with complaint of his height being short compared to peers of his age group. Systemic examination was normal. On further investigation, he was found to have a rare cause of pathological short stature. We want to present this case because of its rarity and to throw light on availability of treatment for the same.

KEY WORDS: Conjunctival xerosis, Empty sella syndrome, Hormones, Pallor

INTRODUCTION sella is due to injury to the pituitary gland itself as a result of surgery or radiation treatment.[15] Visual Empty sella syndrome (ESS) is the herniation of abnormalities can be seen due to arachnoid adhesions subarachnoid space into the sella turcica through the and traction on optic apparatus. They may have sellar diaphragm.[1] and commonly associated with initial improvement in visual symptom with surgery, non-visualization and some degree of flattening of followed by the recurrence of symptoms due to the the pituitary gland in a magnetic resonance imaging development of empty sella, whereas some patients (MRI) scan. Partial empty sella – <50% of sella is who do not have visual symptom initially may present filled with cerebrospinal fluid (CSF). Total empty with fresh onset of these symptoms. sella – more than 50% of sella is filled with CSF with gland thickness being <2 mm. Infrequent finding in Here, we describe an 8-year-old male child who childhood described by Busch in 1951.[2] Radiological presented to pediatric outpatient department (OPD) incidence in children was – 1–48%; male:female – with an inability to gain height according to the peers 1.4:1.[3] of his age group and was found to have partial ESS and multiple pituitary hormone deficiency on imaging Based on etiology, ESS can be classified into and investigation. primary and secondary. Primary ESS is due to congenital incomplete[4-9] or deficiency of sellar CASE REPORT diaphragm. Other causes are Perinatal trauma[9,10] and ischemia of blood vessels to hypophysis[11] It An 8-year-old male child presented to pediatric OPD is commonly associated with endocrinological and with complaints of inability to gain height compared visual disturbances in children. Endocrinological to peers of his age group. There was no history disturbances include multiple anterior pituitary suggestive of systemic involvement. There was no hormone deficiency[4,7,9,10] in which most common is history of previous trauma or operation. He had no growth hormone deficiency, diabetes insipidus,[8,12,13] past history of suggestive of any chronic illnesses. No hyperprolactinemia,[14] hypoparathyroidism,[2] and family history of short stature. premature adrenarche.[14] Visual disturbances occur [9,14] On examination, he was having pallor and conjunctival in 6% of children, which include clouding of xerosis. vision, photophobia, color vision defect, bitemporal quadrantanopia, and hemianopia. Secondary empty Anthropometry Systemic examination was normal. Liver function Access this article online test, renal function test, Mantoux and serology tests were normal. X-ray hand was done, and bone age Website: jprsolutions.info ISSN: 0975-7619 was corresponding to 3 years. Since bone age

Department of Paediatrics, Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu, India

*Corresponding author: Dr. Pooja Pradeep, Department of Paediatrics, Sree Balaji Medical College and Hospital, #7, Works Road, Chromepet, Chennai - 600 044, Tamil Nadu, India

Received on: 07-04-2018; Revised on: 09-05-2019; Accepted on: 14-06-2019

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pituitary hormone deficiency. Our patient had Anthropometry Measurement SD hypothyroidism, hypocortisolism, and low growth Height 96.5 cm ≤3 SD Weight 14.2 kg ≤3 SD hormone. Weight/height Btw−1 SD and −2 SD Master of Public 162.4 cm Btw−1 SD and −2 SD MRI is the gold standard modality which can readily Health confirm the diagnosis of an empty sella. On T1 2 Body mass index 15.24 kg/m sagittal MRIs, extension of CSF into the sella is easily (normal) US:LS 1:1 identified, and remaining gland is compressed along SD: Standard deviation the floor.

Bone age=3 years There is no definite treatment policy for ESS in Height age=4 years children. It is generally asymptomatic and incidentally Chronological age=8 years detected. It requires no specific treatment. When it is accompanied by endocrine dysfunction, replacement age (4 years) and hypopituitarism Serum Hormonal Assay is managed with hormonal supplementation. Hormone Serum levels Impression Thyroid FT3–3 pg/ml Central REFERENCES FT4–0.78 pg/ml hypothyroidism 1. Bergland RM, Ray BS, Torack RM. Anatomical variations TSH–5.53 uIU/ml in the pituitary gland and adjacent structures in 225 human Cortisol 6.24 µg/dl Decreased autopsy cases. J Neurosurg 1968;28:93-9. Growth 0 h–0.492 ng/ml Decreased 2. Busch W. Morphology of sella turcica and its relation to the hormone 30 min–0.578 ng/ml pituitary gland. Virchows Arch Pathol Anat Physiol Klin Med (glucagon 60 min–0.868 ng/ml 1951;320:437-58. stimulation 90 min–1.46 ng/ml 3. Rappaport T, Oleske J, Solman S, Deflaus C. Suppression test) 120 min–0.944 ng/ml of immune function in GHD children during treatment with human GH. J Pediatr 1990;109:434. Finally, the child was found to have partial empty sella 4. Shulman DI, Martinez CR, Bercu BB, Root AW. Hypothalamic- with multiple pituitary hormone deficiency. In view of pituitary dysfunction in primary empty sella syndrome in childhood. J Pediatr 1986;108:540-4. low hormonal levels, the child was initially started on 5. Costigan DC, Daneman D, Harwood-Nash D. The “emptysella” hydrocortisone, and thyroxine level was incremented in childhood. Clin Pediatr 1983;23:437-40. until it was normalized. Once it normalized, the child 6. Dawod ST, Isseh NM, Kalantar SM, Jorulf HK, Ajlouni KM. was started on growth hormone therapy. At present, Primary empty sella syndrome with panhypopituitarism in a child. Helv Paediatr Acta 1984;39:473-9. the child is on growth hormone therapy and is being 7. Querci F, Cattaneo O, Sileo F, Nosari I, Lepore G, Corbellini A, followed upon. et al. Empty sella syndrome and growth deficiency in childhood. Helv Paediatr Acta 1987;42:49-53. 8. Pocecco M, de Campo C, Marinoni S, Tommasini G, Basso T, DISCUSSION Muzzolini C, et al. High frequency of empty sella syndrome in children with growth hormone deficiency. Helv Paediatr Acta Partial empty sella refers to the radiographic 1989;43:295-301. appearance of an enlarged or deformed sella turcica 9. Fusco R, Magli A, Guacci P. Empty sella syndrome. A case that is partially filled with CSF. When idiopathic, report. Opthalmologica 1988;196:92-7. 10. Surtees R, Adams J, Price D, Clayton P, Shalet S. Association of resulting from intrinsic developmental defect, the adverse perinatal events with an empty sella turcica in children [16] empty sella is termed as primary. When this with growth hormone deficiency. Horm Res 1987;28:5-12. radiographic picture is accompanied by symptoms 11. Surtees R, Price DA. Empty sella syndrome and growth failure. such as endocrine or visual abnormalities, it is referred Helv Paediatr Acta 1987;42:335-6. [17] 12. Scirè G, Cianfarani S, Spadoni GL, Manca Bitti ML, Fonte MT, to as primary ESS. Boscherini B, et al. Primary empty sella and endocrinopathies in childhood: High prevalence among children with precocious Primary empty sella has been considered as a puberty. Eur J Pediatr 1988;147:665-6. rare occurrence in childhood.[4,5] In our case, the 13. Osella G, Terzolo M, Caraci P, Orlandi F, Angeli A. Diabetes child was diagnosed to have primary ESS. In insipidus associated with Empty sella: Report of two cases. J Endocrinol Invest 1990;13:351-2. children, it is commonly associated with growth 14. Nass R, Engel M, Stoner E, Pang S, New MI. Empty sella hormone deficiency, hypogonadism, or multiple syndrome in childhood. Pediatr Neurol 1986;2:224-9.

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15. Lenz AM, Root AW. Empty sella syndrome. Pediatr Endocrinol 17. Stanhope R, Adlard P. Empty sella syndrome. Dev Med Child Rev 2012;9:710-5. Neurol 1987;29:397-9. 16. Rapaport R, Logrono R. Primary empty sella syndrome in childhood: Association with precocious puberty. Clin Pediatr Source of support: Nil; Conflict of interest: None Declared (Phila) 1991;30:466-71.

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