Primary Empty Sella Syndrome in Childhood: Association with Precocious Puberty Robert
Rapaport, MD*; Roberto Logrono, MD**
Primary empty sella syndrome has been considered an infrequent finding in childhood. One hundred and twelve cases have been reported in children ages 0.7 to 18 years. The frequency of primary empty sella ranged from 1% to 58%. Endocrine abnormalities were described in nearly all , children while visual abnormalities were noted in only 6%. Growth hormone deficiency was the single most common hormonal dysfunction noted in children with primary empty sella. Precocious puberty has been rarely reported in association with primary empty sella. We report the case of a 7-1/2 year old girl with gonadotropin dependent central precocious puberty and a partially empty sella who had no other hypothalamic-pituitary dysfunction. We suggest that pubertal abnormalities be included among the endocrine disorders potentially associated with the primary empty sella syndrome.
Introduction
Empty sella refers to the radiographic appearance of an enlarged or deformed sella turcica that is partially or completely filled with cerebrospinal fluid. Most cases reported, especially in adults, are secondary to irradiation, pituitary tumors, infarction or surgery. When idiopathic, resulting from intrinsic developmental defects, the empty sella is termed primary. When this radiographic picture is accompanied by symptoms, such as headaches or visual or endocrine abnormalities it is referred to as primary empty sella syndrome (PESS).lI Primary empty sella has been considered a rare occurrence in childhood.2.3 It is usually associated with hormonal or visual disturbances. Central precocious puberty in most girls is considered idiopathic.w6 Few reports have described primary empty sella associated with precocious puberty.
We describe the case of a 7-year 6-month-old girl in whom central gonadotropin dependent precocious puberty was diagnosed in association with a partially empty sella. In addition, we review cases of primary empty sella syndrome reported in childhood.
Case
were
*Associate Professor Clinical Pediatrics, UMD-New Jersey Medical School and Children’s Hospital of New Jersey, Newark, NJ; **Department of Pathology, St. Barnabas Medical Center, Livingston, NJ. Correspondence to: Robert Rapaport, MD, Director, Division of Pediatric Endocrinology, Children’s Hospital of New Jersey, 15 So. 9th St., Newark, NJ 07107. Phone: Business 201-268-8337, Fax 201-4826647, Home 212-772-6492.
Report
The patient was a 7-year 6-month-old-girl evaluated because of early pubertal development. The patient had breast development for the previous four months and pubic hair growth for three months. She had evidence of acne as well as increased perspiration for one month. There was no history of vaginal discharge. There was no reported increase in growth velocity. There was no history of exposure to hormone containing medications. The patient was the product of a normal pregnancy and delivery. Her past medical history and review of systems
non-contributory.
Family history was also non-contributory. Her mother had menarche at the age of 13 and her father began shaving at the age of 15. There was no family history of early or
precocious puberty. On initial examination the patient was alert and active. Her height was 121.8 cm (between 25-50th percentile) and
466
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
weight 24.3 kg (between 50-75th percentile). Her pulse rate was 90 beats/min; blood pressure, 93/58 mmHg; and
respiratory
rate, 24/min.
There
was no
unusual skin
pigmentation. Her fundi and visual fields by confrontation were normal. The thyroid gland was not palpable. Examination of the chest, heart and abdomen were normal. Neurologic examination was normal. Sexual development revealed ample bilateral axillary sweating but no axillary hair; breast development was 4.5 cm in diameter bilaterally, Tanner stage II-III; perilabial public hair growth corresponded to Tanner stage II-III and there was no clitoral hypertrophy. Rectal examination was normal. Initial evaluation included normal complete blood count, chemical profile, thyroid function tests and urinalyses. Her bone age was 8 to 8.5 years at chronological age of 7 years 6 months. Ultrasound of the abdomen and pelvis was normal. Magnetic resonance imaging of the head revealed a partially empty sella (Fig.). A gonadotropin releasing hormone (GnRH) stimulation test revealed a pubertal response with a peak luteinizing hormone concentration of 28.9 mIU/ml at 30 minutes, and a peak follicle stimulation hormone concentration of 25.2 mIU/ml at 60 minutes. At the age of 8 year 1 month, her height was 125.7 cm, just below the 50th percentile (interval annual growth rate of 6.7 cm), and her weight was 26.5 kg (between the 5075th percentile). Her breast development had increased to Tanner stage IV and her pubic hair growth to Tanner stage III. Her bone age had advanced to 10 years. Thyroid stimulating hormone response to thyroid releasing horTable 1.
mone was
normal. Growth hormone and cortisol increased
normally following glucagon administration. A pelvic ultrasound this time revealed prominent ovaries for patient’ss age containing several follicles. The patient was started on luteinizing hormone releasing hormone agonist therapy. Following six months of therapy, her pubertal progression ceased and her annual growth rate decreased to 5.6 cm/year. Discussion While secondary empty sella, seen mostly in adults, has been described in association with pituitary tumors, infarction, surgery or radiation,’ primary empty sella in children has been postulated to be the consequence of congenital incompleteness or deficiency of the sellar diaphragm and intermittent pressure changes in the cerebrospinal fluid, resulting in herniation of the subarachnoid space into the sella, from above, with compression of the pituitary tissue. 1,1,7-10 Other possible etiologies for primary empty sella have included perinatal trauma,9,11 ischemia with subsequent interruption of the blood supply to the hypophysis 12 and rupture of an intrasellar cyst 13 Recently, Komatsu et al. 17 have reported finding antipituitary antibodies in 47% to 75% of adult patients with primary empty sella syndrome. Primary empty sella was thought to be rare in children.2,3 It’s reported frequency, as detected by computerized tomographic scanning, varies from 1.1% to 58% (Table 1). Costigan et al.3 only found 4 cases of primary empty sella
Frequency of PESS diagnosed by computerized tomography.
467
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
Table 2.
Reports of children with PESS#
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
syndrome among 360 investigated cases of radiographic sellar enlargement. However, Shulman et a1.2 found 9 cases of primary empty sella among 37 consecutive cases referred to a pediatric endocrinology unit. When evaluating children because of growth hormone deficiency, the frequency of primary empty sella varies from 10% to 58%.9,1 1,15,19 We are unaware of any data describing the frequency of primary empty sella among children evaluated by magnetic resonance imaging (MRI). Some of the visual and endocrine manifestations found in association with empty sella in childhood were previously reviewed in Clinical Pediatrics by Costigan et al. in 1983.3 3 We have found reports of 89 additional cases (Table
2). Visual disturbances reported include clouding of vision, photophobia, color vision defects, bitemporal quadrantanopsia and hemianopia.10,19 Endocrine manifestations include single or multiple anterior pituitary hormone deficiencies, 2,7,9,11,15-17,19-21 diabetes insipidus,8,15,24 hyperprolactinemia,19 hypothalamic dysfunction of growth hormone and gonadotropin secre-
tion,~ hypoparathyroidiSM,2 premature adrenarchel9 and scrotal hair.25 Primary empty sella syndrome has also been
reported in three children following thyroid hormone replacement for primary hypothyroidism,’ 8°22°23 two of whom developed psuedotumor cerebri (Tables 2 and 3). Including those cases previously summarized,3 we found a total of 112 cases of primary empty sella reported in children (Table 3). The mean age at diagnosis was 10 years Table 3.
Reported
cases
of PESS
with
a
range of 7 months to 18 years. When data
were
available, there was a male:female ratio of 1.4:1.0. Visual abnormalities were described in 6% of children. The most common endocrine abnormality reported in association with primary empty sella syndrome has been growth hormone deficiency, isolated or associated with other pituitary hormone deficiencies2,9,15, 16, 19-21 occurring in 70% to 76% of children. Whether growth hormone deficiency is due to pituitary or hypothalamic dysfunction is now known. We performed growth hormone releasing factor stimulation tests in three patients with primary empty sella and found a subnormal peak growth hormone response suggestive of a pituitary defect.z° Four patients with short stature and PESS have been reported to have normal pituitary function.8,15,19 In most children diagnosed as having central precocious puberty, no organic etiology can be found for the condition. However, with the advent of newer imaging techniques such as computerized tomography, Cacciari et a1.4 demonstrated hypothalamic masses, presumably hamartomas, in up to 33 % of girls with precocious puberty. The association of central precocious puberty with primary empty sella has been documented infrequently. Scire et al. 15 mentioned in a brief report primary empty sella in 3 out of 16 cases of central precocious puberty, without providing detailed data however. Two cases of precocious puberty, a 7-year-old girl and a 10-year-old boy, have been described in association with primary empty sella by Shulman et al.2 and Costigan et a1.,3 respectively.
(Total)
469
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
Figures A and B. Magnetic resonance imaging showing partitally empty sella B. Sagital view: 1) Partially empty sella; 2) Small pituitary gland 2) Partially empty sella;
A. Coronal view: 1 ) Pituitary stalk; 3) Small pituitary gland
Our patient had central precocious puberty documented by a pubertal response of gonadotropins to GnRH stimulation, associated with a primary empty sella. She had normal responses of thyroid stimulating hormone, growth
The advent of
magnetic
imaging techniques such as imaging may help define the true primary empty sella syndrome in newer
resonance
incidence of childhood.
hormone and cortisol to stimulation and normal baseline serum prolactin level suggesting no additional hypothalamic-anterior pituitary dysfunction. She had normal serum and urinary electrolyte concentration implying normal antidiuretic hormone function. We conclude that endocrine abnormalities, especially growth hormone deficiency, are common among children with the primary empty sella syndrome. Pubertal abnormalities, including precocious puberty, should now also be considered among the hormonal defects associated with primary empty sella syndrome. Conversely, the radiographic evaluation of children with hypothalamic-pituitary dysfunction, including precocious puberty, may reveal an underlying primary empty sella.
References 1. Stanhope R, Adlard P: Empty
sella
syndrome.
Devel Med Child
Neurol 1987; 29:397-99. 2. Shulman DI, Martinez CR, Bercu BB, et al.: Hypothalamic-pituitary dysfunction in primary empty sella in childhood. J Pediatr 1946; 18:540-44. 3.
Daneman D, Harwood-Nash D, et al.: The "empty sella" in childhood. Clin Pediatr 1983; 23:437-40. 4. Cacciari E, Frejaville E, Cicognati A, et al: How many cases of true precocious puberty in girls are idiopathic? J Pediatr 1983; 102:357-60. 5. Kulin HE: Precocious puberty. Clin Obstet Gynecol 1947; 30:714-34. 6. Loriaux DL: The pathophysiology of precocious puberty. Hosp Pract 1989; 24:55-61.
Costigan DC,
470
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
ST, Isseh NM, Kalantar SM,
et al: Primary empty sella with panhypopituitarism in a child. Helv Paediatr Acta 1984; 39:473-79. Querci F, Cattaneo F, Sileo I, et al: Empty sella syndrome and growth deficiency in childhood. Helv Paediatr Acta 1987; 42:49-53. Pocecco M, DeCampo C, Marinoni S, et al: High frequency of empty sella syndrome in children with growth hormone deficiency. Helv Paediatr Acta 1988; 43:295-301. Fusco R, Magli A, Guacci P, et al.: Empty sella syndrome. A case report. Opthalmol 1988; 196:92-97. Surtees R, Adams J, Price D, et al: Association of adverse perinatal events with an empty sella turcica in children with growth hormone deficiency. Hormone Res 1947; 28:5-12. Surtees R, Price DA: Empty sella syndrome and growth failure. Helv Paediatr Acta 1987; 42:335-36. Raiti S, Albrink MJ, Maclaren NK, et al: Empty sella syndrome secondary to intrasellar cyst in adolescence. AJDC 1976;130:100912. Komatsu M, Hondo T, Yamauchi K, et al: Antipituitary antibodies in patients with the primary empty sella syndrome. J Clin Endocrinol Metab 1988; 67:633-38. Scire G, Cianfarani S, Spadoni GC, et al: Primary empty sella and endocrinopathies in childhood: high prevalence among children with precocious puberty. Eur J Pediatr 1988; 147:665-66. Radfar N, Raji MR, Dastur KG, et al.: Hypopituitarism in children with "primary empty sella syndrome (PESS)." Pediatr Res 1985; 19:191A. Parks JS, Tenore A, Bongiovanni AM, et al.: Familial hypothyroidism with large sella turcica. N Engl J Med 1978; 298:698-702. McVie R: Abnormal TSH regulation, pseudotumor cerebri and empty sella after replacement therapy in juvenile hypothyroidism. J Pediatr 1984; 105:768-70. Nass R, Engel M, Stoner E, et al.: Empty sella syndrome in childhood. Pediatr Neurol 1986; 2:224-29. Rapaport R, Skuza K, Schenkman S, et al: Response to human growth hormone releasing hormone (GHRH) in familial and isolated primary empty sella (PESS). Pediatr Res 1986; 20:219A. White MC, Chahal P, Banks L, et al.: Familial hypothyroidism associated with an enlarged pituitary foss and an empty sella. Clin Endocrinol 1986; 24:63-70. LaFranchi SH, Hanna CE, Krainz PL: Primary hypothyroidism, empty sella and hypopituitarism. J Pediatr 1986; 18:571-73. Hoffman WH, England BG, Gomez LM, et al: Empty sella associated with inappropriate TSH secretion. Neuropediatr 1987; 18 :3739. Osella G, Terzolo M, Caraci P, et al: Diabetes insipidus associated with empty sella: report of two cases. J Endocrinol Invest 1990; 13:351-52. Rosenstain D, Smith D, Deeb ZL: Scrotal hair in an infant with empty sella turcica. J Pediatr 1990; 116:493-94.
7. Dawod
syndrome
8. 9.
10. 11.
12. 13.
14.
15.
16.
17. 18.
19.
20.
21.
22. 23.
24.
25.
471
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
Rapaport, MD*; Roberto Logrono, MD**
Primary empty sella syndrome has been considered an infrequent finding in childhood. One hundred and twelve cases have been reported in children ages 0.7 to 18 years. The frequency of primary empty sella ranged from 1% to 58%. Endocrine abnormalities were described in nearly all , children while visual abnormalities were noted in only 6%. Growth hormone deficiency was the single most common hormonal dysfunction noted in children with primary empty sella. Precocious puberty has been rarely reported in association with primary empty sella. We report the case of a 7-1/2 year old girl with gonadotropin dependent central precocious puberty and a partially empty sella who had no other hypothalamic-pituitary dysfunction. We suggest that pubertal abnormalities be included among the endocrine disorders potentially associated with the primary empty sella syndrome.
Introduction
Empty sella refers to the radiographic appearance of an enlarged or deformed sella turcica that is partially or completely filled with cerebrospinal fluid. Most cases reported, especially in adults, are secondary to irradiation, pituitary tumors, infarction or surgery. When idiopathic, resulting from intrinsic developmental defects, the empty sella is termed primary. When this radiographic picture is accompanied by symptoms, such as headaches or visual or endocrine abnormalities it is referred to as primary empty sella syndrome (PESS).lI Primary empty sella has been considered a rare occurrence in childhood.2.3 It is usually associated with hormonal or visual disturbances. Central precocious puberty in most girls is considered idiopathic.w6 Few reports have described primary empty sella associated with precocious puberty.
We describe the case of a 7-year 6-month-old girl in whom central gonadotropin dependent precocious puberty was diagnosed in association with a partially empty sella. In addition, we review cases of primary empty sella syndrome reported in childhood.
Case
were
*Associate Professor Clinical Pediatrics, UMD-New Jersey Medical School and Children’s Hospital of New Jersey, Newark, NJ; **Department of Pathology, St. Barnabas Medical Center, Livingston, NJ. Correspondence to: Robert Rapaport, MD, Director, Division of Pediatric Endocrinology, Children’s Hospital of New Jersey, 15 So. 9th St., Newark, NJ 07107. Phone: Business 201-268-8337, Fax 201-4826647, Home 212-772-6492.
Report
The patient was a 7-year 6-month-old-girl evaluated because of early pubertal development. The patient had breast development for the previous four months and pubic hair growth for three months. She had evidence of acne as well as increased perspiration for one month. There was no history of vaginal discharge. There was no reported increase in growth velocity. There was no history of exposure to hormone containing medications. The patient was the product of a normal pregnancy and delivery. Her past medical history and review of systems
non-contributory.
Family history was also non-contributory. Her mother had menarche at the age of 13 and her father began shaving at the age of 15. There was no family history of early or
precocious puberty. On initial examination the patient was alert and active. Her height was 121.8 cm (between 25-50th percentile) and
466
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
weight 24.3 kg (between 50-75th percentile). Her pulse rate was 90 beats/min; blood pressure, 93/58 mmHg; and
respiratory
rate, 24/min.
There
was no
unusual skin
pigmentation. Her fundi and visual fields by confrontation were normal. The thyroid gland was not palpable. Examination of the chest, heart and abdomen were normal. Neurologic examination was normal. Sexual development revealed ample bilateral axillary sweating but no axillary hair; breast development was 4.5 cm in diameter bilaterally, Tanner stage II-III; perilabial public hair growth corresponded to Tanner stage II-III and there was no clitoral hypertrophy. Rectal examination was normal. Initial evaluation included normal complete blood count, chemical profile, thyroid function tests and urinalyses. Her bone age was 8 to 8.5 years at chronological age of 7 years 6 months. Ultrasound of the abdomen and pelvis was normal. Magnetic resonance imaging of the head revealed a partially empty sella (Fig.). A gonadotropin releasing hormone (GnRH) stimulation test revealed a pubertal response with a peak luteinizing hormone concentration of 28.9 mIU/ml at 30 minutes, and a peak follicle stimulation hormone concentration of 25.2 mIU/ml at 60 minutes. At the age of 8 year 1 month, her height was 125.7 cm, just below the 50th percentile (interval annual growth rate of 6.7 cm), and her weight was 26.5 kg (between the 5075th percentile). Her breast development had increased to Tanner stage IV and her pubic hair growth to Tanner stage III. Her bone age had advanced to 10 years. Thyroid stimulating hormone response to thyroid releasing horTable 1.
mone was
normal. Growth hormone and cortisol increased
normally following glucagon administration. A pelvic ultrasound this time revealed prominent ovaries for patient’ss age containing several follicles. The patient was started on luteinizing hormone releasing hormone agonist therapy. Following six months of therapy, her pubertal progression ceased and her annual growth rate decreased to 5.6 cm/year. Discussion While secondary empty sella, seen mostly in adults, has been described in association with pituitary tumors, infarction, surgery or radiation,’ primary empty sella in children has been postulated to be the consequence of congenital incompleteness or deficiency of the sellar diaphragm and intermittent pressure changes in the cerebrospinal fluid, resulting in herniation of the subarachnoid space into the sella, from above, with compression of the pituitary tissue. 1,1,7-10 Other possible etiologies for primary empty sella have included perinatal trauma,9,11 ischemia with subsequent interruption of the blood supply to the hypophysis 12 and rupture of an intrasellar cyst 13 Recently, Komatsu et al. 17 have reported finding antipituitary antibodies in 47% to 75% of adult patients with primary empty sella syndrome. Primary empty sella was thought to be rare in children.2,3 It’s reported frequency, as detected by computerized tomographic scanning, varies from 1.1% to 58% (Table 1). Costigan et al.3 only found 4 cases of primary empty sella
Frequency of PESS diagnosed by computerized tomography.
467
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
Table 2.
Reports of children with PESS#
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
syndrome among 360 investigated cases of radiographic sellar enlargement. However, Shulman et a1.2 found 9 cases of primary empty sella among 37 consecutive cases referred to a pediatric endocrinology unit. When evaluating children because of growth hormone deficiency, the frequency of primary empty sella varies from 10% to 58%.9,1 1,15,19 We are unaware of any data describing the frequency of primary empty sella among children evaluated by magnetic resonance imaging (MRI). Some of the visual and endocrine manifestations found in association with empty sella in childhood were previously reviewed in Clinical Pediatrics by Costigan et al. in 1983.3 3 We have found reports of 89 additional cases (Table
2). Visual disturbances reported include clouding of vision, photophobia, color vision defects, bitemporal quadrantanopsia and hemianopia.10,19 Endocrine manifestations include single or multiple anterior pituitary hormone deficiencies, 2,7,9,11,15-17,19-21 diabetes insipidus,8,15,24 hyperprolactinemia,19 hypothalamic dysfunction of growth hormone and gonadotropin secre-
tion,~ hypoparathyroidiSM,2 premature adrenarchel9 and scrotal hair.25 Primary empty sella syndrome has also been
reported in three children following thyroid hormone replacement for primary hypothyroidism,’ 8°22°23 two of whom developed psuedotumor cerebri (Tables 2 and 3). Including those cases previously summarized,3 we found a total of 112 cases of primary empty sella reported in children (Table 3). The mean age at diagnosis was 10 years Table 3.
Reported
cases
of PESS
with
a
range of 7 months to 18 years. When data
were
available, there was a male:female ratio of 1.4:1.0. Visual abnormalities were described in 6% of children. The most common endocrine abnormality reported in association with primary empty sella syndrome has been growth hormone deficiency, isolated or associated with other pituitary hormone deficiencies2,9,15, 16, 19-21 occurring in 70% to 76% of children. Whether growth hormone deficiency is due to pituitary or hypothalamic dysfunction is now known. We performed growth hormone releasing factor stimulation tests in three patients with primary empty sella and found a subnormal peak growth hormone response suggestive of a pituitary defect.z° Four patients with short stature and PESS have been reported to have normal pituitary function.8,15,19 In most children diagnosed as having central precocious puberty, no organic etiology can be found for the condition. However, with the advent of newer imaging techniques such as computerized tomography, Cacciari et a1.4 demonstrated hypothalamic masses, presumably hamartomas, in up to 33 % of girls with precocious puberty. The association of central precocious puberty with primary empty sella has been documented infrequently. Scire et al. 15 mentioned in a brief report primary empty sella in 3 out of 16 cases of central precocious puberty, without providing detailed data however. Two cases of precocious puberty, a 7-year-old girl and a 10-year-old boy, have been described in association with primary empty sella by Shulman et al.2 and Costigan et a1.,3 respectively.
(Total)
469
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
Figures A and B. Magnetic resonance imaging showing partitally empty sella B. Sagital view: 1) Partially empty sella; 2) Small pituitary gland 2) Partially empty sella;
A. Coronal view: 1 ) Pituitary stalk; 3) Small pituitary gland
Our patient had central precocious puberty documented by a pubertal response of gonadotropins to GnRH stimulation, associated with a primary empty sella. She had normal responses of thyroid stimulating hormone, growth
The advent of
magnetic
imaging techniques such as imaging may help define the true primary empty sella syndrome in newer
resonance
incidence of childhood.
hormone and cortisol to stimulation and normal baseline serum prolactin level suggesting no additional hypothalamic-anterior pituitary dysfunction. She had normal serum and urinary electrolyte concentration implying normal antidiuretic hormone function. We conclude that endocrine abnormalities, especially growth hormone deficiency, are common among children with the primary empty sella syndrome. Pubertal abnormalities, including precocious puberty, should now also be considered among the hormonal defects associated with primary empty sella syndrome. Conversely, the radiographic evaluation of children with hypothalamic-pituitary dysfunction, including precocious puberty, may reveal an underlying primary empty sella.
References 1. Stanhope R, Adlard P: Empty
sella
syndrome.
Devel Med Child
Neurol 1987; 29:397-99. 2. Shulman DI, Martinez CR, Bercu BB, et al.: Hypothalamic-pituitary dysfunction in primary empty sella in childhood. J Pediatr 1946; 18:540-44. 3.
Daneman D, Harwood-Nash D, et al.: The "empty sella" in childhood. Clin Pediatr 1983; 23:437-40. 4. Cacciari E, Frejaville E, Cicognati A, et al: How many cases of true precocious puberty in girls are idiopathic? J Pediatr 1983; 102:357-60. 5. Kulin HE: Precocious puberty. Clin Obstet Gynecol 1947; 30:714-34. 6. Loriaux DL: The pathophysiology of precocious puberty. Hosp Pract 1989; 24:55-61.
Costigan DC,
470
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
ST, Isseh NM, Kalantar SM,
et al: Primary empty sella with panhypopituitarism in a child. Helv Paediatr Acta 1984; 39:473-79. Querci F, Cattaneo F, Sileo I, et al: Empty sella syndrome and growth deficiency in childhood. Helv Paediatr Acta 1987; 42:49-53. Pocecco M, DeCampo C, Marinoni S, et al: High frequency of empty sella syndrome in children with growth hormone deficiency. Helv Paediatr Acta 1988; 43:295-301. Fusco R, Magli A, Guacci P, et al.: Empty sella syndrome. A case report. Opthalmol 1988; 196:92-97. Surtees R, Adams J, Price D, et al: Association of adverse perinatal events with an empty sella turcica in children with growth hormone deficiency. Hormone Res 1947; 28:5-12. Surtees R, Price DA: Empty sella syndrome and growth failure. Helv Paediatr Acta 1987; 42:335-36. Raiti S, Albrink MJ, Maclaren NK, et al: Empty sella syndrome secondary to intrasellar cyst in adolescence. AJDC 1976;130:100912. Komatsu M, Hondo T, Yamauchi K, et al: Antipituitary antibodies in patients with the primary empty sella syndrome. J Clin Endocrinol Metab 1988; 67:633-38. Scire G, Cianfarani S, Spadoni GC, et al: Primary empty sella and endocrinopathies in childhood: high prevalence among children with precocious puberty. Eur J Pediatr 1988; 147:665-66. Radfar N, Raji MR, Dastur KG, et al.: Hypopituitarism in children with "primary empty sella syndrome (PESS)." Pediatr Res 1985; 19:191A. Parks JS, Tenore A, Bongiovanni AM, et al.: Familial hypothyroidism with large sella turcica. N Engl J Med 1978; 298:698-702. McVie R: Abnormal TSH regulation, pseudotumor cerebri and empty sella after replacement therapy in juvenile hypothyroidism. J Pediatr 1984; 105:768-70. Nass R, Engel M, Stoner E, et al.: Empty sella syndrome in childhood. Pediatr Neurol 1986; 2:224-29. Rapaport R, Skuza K, Schenkman S, et al: Response to human growth hormone releasing hormone (GHRH) in familial and isolated primary empty sella (PESS). Pediatr Res 1986; 20:219A. White MC, Chahal P, Banks L, et al.: Familial hypothyroidism associated with an enlarged pituitary foss and an empty sella. Clin Endocrinol 1986; 24:63-70. LaFranchi SH, Hanna CE, Krainz PL: Primary hypothyroidism, empty sella and hypopituitarism. J Pediatr 1986; 18:571-73. Hoffman WH, England BG, Gomez LM, et al: Empty sella associated with inappropriate TSH secretion. Neuropediatr 1987; 18 :3739. Osella G, Terzolo M, Caraci P, et al: Diabetes insipidus associated with empty sella: report of two cases. J Endocrinol Invest 1990; 13:351-52. Rosenstain D, Smith D, Deeb ZL: Scrotal hair in an infant with empty sella turcica. J Pediatr 1990; 116:493-94.
7. Dawod
syndrome
8. 9.
10. 11.
12. 13.
14.
15.
16.
17. 18.
19.
20.
21.
22. 23.
24.
25.
471
Downloaded from cpj.sagepub.com at Purdue University on March 13, 2015
