Pituitary non-secreting macroadenoma apoplexy in an adolescent. patient report and review of the literature

Pituitary macroadenomas are rare in children and adolescents, and when encountered are usually hormone secreting. Symptomatic pituitary non-secreting macroadenoma apoplexy in an adolescent is rare and potentially life-threatening. A 15 year-old patient is described, hospitalized due to headache, fever and photophobia 4 days prior to admission. A meningeal syndrome was postulated, based on clinical examination and cerebrospinal fluid testing. However, clinical examination and hormone testing revealed partial failure of the anterior pituitary. Computed tomography of the brain demonstrated a space-occupying lesion of the pituitary. Magnetic nuclear resonance imaging suggested the presence of a pituitary macroadenoma. Hypophysectomy was performed. Histological examination revealed an extensive infarction of a pituitary adenoma. Hormonal substitution with thyroxine and corticosteroids was administered. This report emphasizes that pituitary non-secreting macroadenoma apoplexy may rarely be the cause of headache and fever in an adolescent, thus causing difficulties in differential diagnosis from acute meningitis.     

Hemorrhagic pituitary apoplexy in an 18 year-old male presenting as non-ketotic hyperglycemic coma (NKHC)

Pituitary apoplexy is an acute clinical event usually caused by hemorrhage or infarction in a pituitary adenoma. We report the unusual case of hemorrhagic pituitary apoplexy in an 18 year-old male with previously undiagnosed type 2 diabetes mellitus who presented with unexplained hyperglycemia (glucose 49.2 mmol/l [887 mg/dl]) and obtundation and in whom an initial diagnosis of non-ketotic hyperglycemic coma (NKHC) was made. MRI revealed a heterogeneous mass arising from an expanded sella turcica into the suprasellar cistern. Despite well-controlled glucose levels on continuous insulin infusion, dexamethasone, and initiation of bromoergocriptine (parlodel) therapy, the patient's vision and pupillary responses deteriorated acutely. Following emergency transphenoidal surgery, the patient's vision and mental status improved. Data confirmed preoperative panhypopituitarism; serum prolactin was 396 ng/ml (microg/l). Immunostudies demonstrated tumoral labeling for prolactin, but not for ACTH, GH, TSH, LH, FSH, or P53.     

Excessive growth

Tall stature and excessive growth syndrome are a relatively rare concern in pediatric practice. Nevertheless, it is important to identify abnormal accelerated growth patterns in children, which may be the clue in the diagnosis of an underlying disorder. We present a case of pituitary gigantism in a 2 1/2-year-old child and discuss the signs, symptoms, laboratory findings, and the treatment. Brief discussions on the differential diagnosis of excessive growth/tall stature have been outlined. Pituitary gigantism is very rare in the pediatrics age group; however, it is extremely rare in a child that is less than 3 years of age. The nature of pituitary adenoma and treatment options in children with this condition have also been discussed.     

Pituitary adenomas in childhood: development and diagnosis

Pituitary adenomas account for approximately 2.7% of all supratentorial tumors in the pediatric age range, and children are more likely than adults to develop a functioning adenoma. X chromosome inactivation studies indicate that pituitary adenomas arise from the clonal expression of a single mutated cell, and various intracellular mechanisms contribute to tumoral transformation. Functional pituitary tumors in childhood result in physical and biochemical effects of excess production of the oversecreted hormone, such as ACTH, prolactin, human growth hormone, TSH, LH, or FSH. In the clinical approach to pituitary adenomas, it is important to establish the presence of hormonal excess prior to undertaking imaging studies.     

儿童垂体腺瘤的治疗

目的　总结 13例儿童垂体腺瘤的治疗经验 ,探讨儿童垂体腺瘤的治疗方法及预后。方法　自 1997年 1月～ 2 0 0 3年 11月同济医院共收治 13例垂体腺瘤患儿 ,其中男 8例 ,女 5例 ,年龄 9～ 15岁 ,平均 12 .8岁。病程 3个月～ 5年 ,平均 2年。 12例经口鼻蝶入路显微手术。所有病例通过影像学及内分泌检查 ,随访 1～ 5年。结果　手术病例中 ,肿瘤全切 11例 ,次全切除 1例 ,无手术死亡 ,未出现严重并发症。结论　经蝶显微手术是儿童垂体腺瘤最佳的治疗方法 ,手术后放疗要谨慎。长期、正规的随访具有特别重要的意义     

High Resolution CT Scanning of the Pituitary Gland in Growth Disorders

ABSTRACT. We have performed 217 GEC 8800 CT scans of the hypothalamus and pituitary glands of 202 children with disorders of growth and development. Pituitary morphological abnormalities were common. Intrapituitary low density lesions were found in 17 % of the whole series and in 58% of children with tall stature. Seventy-seven children with idiopathic growth hormone deficiency could be divided on the basis of pituitary morphology seen on CT scan into pituitary aplasia (n = 11) and pituitary hypoplasia (n = 53). Patients with pituitary aplasia had an absent adenohypophysis which probably dated from early intrauterine life and therefore could not be related to birth trauma. We have found a high incidence of evolving endocrinopathy in children with pituitary insufficiency: thus, if a short child is investigated the initial endocrine findings need to be repeated as the pattern of pituitary insufficiency changes with time. An evolving endocrinopathy starting in later childhood is suggestive of the presence of a cerebral tumour. Children with subnormal growth velocities and a normal growth hormone response to pharmacological tests have a wide spectrum of pituitary morphological abnormalities which may be associated with growth hormone neurosecretory dysfunction.     

High resolution CT scanning of the pituitary gland in growth disorders

We have performed 217 GEC 8800 CT scans of the hypothalamus and pituitary glands of 202 children with disorders of growth and development. Pituitary morphological abnormalities were common. Intrapituitary low density lesions were found in 17% of the whole series and in 58% of children with tall stature. Seventy-seven children with idiopathic growth hormone deficiency could be divided on the basis of pituitary morphology seen on CT scan into pituitary aplasia (n = 11) and pituitary hypoplasia (n = 53). Patients with pituitary aplasia had an absent adenohypophysis which probably dated from early intrauterine life and therefore could not be related to birth trauma. We have found a high incidence of evolving endocrinopathy in children with pituitary insufficiency: thus, if a short child is investigated the initial endocrine findings need to be repeated as the pattern of pituitary insufficiency changes with time. An evolving endocrinopathy starting in later childhood is suggestive of the presence of a cerebral tumour. Children with subnormal growth velocities and a normal growth hormone response to pharmacological tests have a wide spectrum of pituitary morphological abnormalities which may be associated with growth hormone neurosecretory dysfunction.     

Pituitary adenomas in childhood

Pituitary adenomas are common tumors composed of adenohypophysial cells.Although they usually arise in the sella turcica, they may occasionally be ectopic. Pituitary adenomas are rarely diagnosed in. childhood and adolescence, but their mass effect and endocrine abnormalities can compromise both quality and length of life. Many signs or symptoms of pituitary adenoma, complained of in adulthood, not became evident during adolescence, suggesting true prevalence of this tumor in teenagers is higher than expected. Pititury adenoma occuring during adolescence are associated with features or therapeutic needs sometimes different from those occuring in adulthood. At the onset of disease, delay in growth was rarely observed in teenagers with pituitary adenomas. Many girls complain of oligoamenorrhoea and galactorrhoea, while heActache and delay in pubertal development are the most commons features in boys. Hypopituitarism is occasionally encountered in adolescence. Early diagnosis and appropriate choice of therapy are necessary to avoid permanent endocrine complications of disease and its treatment.     

Optic nerve size evaluated by magnetic resonance imaging in children with optic nerve hypoplasia, multiple pituitary hormone deficiency, isolated growth hormone deficiency, and idiopathic short stature

OBJECTIVE: To objectively define criteria for intracranial optic nerve (ON) size in ON hypoplasia (ONH) on magnetic resonance imaging (MRI) scans. STUDY DESIGN: Intracranial ON sizes from MRI were compared between 46 children with ONH diagnosed by ophthalmoscopy (group 1, isolated ONH, 8 children; and group 2, ONH associated with abnormalities of the hypothalamic-pituitary axis and septum pellucidum, 38 children) and children with multiple pituitary hormone deficiency (group 3, multiple pituitary hormone deficiency, 14 children), isolated growth hormone deficiency (group 4, isolated growth hormone deficiency, 15 children), and idiopathic short stature (group 5, idiopathic short stature, 10 children). Intracranial ON size was determined by the cross-sectional area, calculated as [pi x (1/2) height x (1/2) width]. RESULTS: Groups 1 and 2 had lower intracranial ON size than did groups 3, 4, and 5 (P < .001). No patients in groups 3 through 5 who had MRI after 12 months of age (when 95% adult size of ONs is attained) had ONs <2.9 mm 2 . Visual acuity correlated significantly with ON size (P < .01). CONCLUSIONS: Magnetic resonance imaging of the ONs with cross-sectional area <2.9 mm 2 in a short child more than 12 months of age, with or without hypothalamic-pituitary axis abnormalities, confirms the clinical diagnosis of ONH.     

Pituitary pseudotumor with unusual presentation reversed shortly after the introduction of thyroxine replacement therapy.

Pituitary pseudotumor (pituitary thyrotroph hyperplasia) caused by unrecognized and untreated hypothyroidism has been described as a rare condition, mostly in adults. There are only a few reports on this condition in children. Here we describe an unusual association of pituitary pseudotumor with hypothyroidism, vaginal bleeding, and growth retardation in a girl at an early stage of puberty. Hormonal testing showed low thyroxine and high TSH levels, hyperprolactinemia, low growth hormone levels and prepubertal levels of gonadotrophins. Ovarian cyst was detected by ultrasound. A large intrasellar mass expanding beyond the sella turcica was detected on MRI. Homogeneous contrast enhancement confirmed pituitary hyperplasia. Therapy with L-thyroxine resulted in rapid improvement of the clinical signs, normalization of the hormone levels, and resolution of the pituitary hyperplasia on MRI within 40 days. In children, prolonged unrecognized primary hypothyroidism might be accompanied by growth deficiency and pubertal disharmony. Pituitary hyperplasia should be sought in these cases.     

Primary empty sella: differences and similarities between children and adults

To identify possible differences between empty sella in children and adults we studied 43 subjects (age 13.6 ±5.4 years, range 4.1–27 years) with hypothalamic-pituitary disorders and empty sella at magnetic resonance imaging. Pituitary function, presence of non-endocrine symptoms, perinatal history, sellar volume, pituitary height, midline or intrasellar anatomical abnormalities were evaluated. Twenty subjects had isolated growth hormone deficiency, 17 multiple pituitary hormone deficiency and 6 puberty disorders (3 precocious puberty, 2 idiopathic delayed puberty, 1 Kallmann syndrome). The group with multiple pituitary hormone deficiency had a higher percentage of subjects with complete empty sella, i.e pituitary height < 2 mm (p = 0.016), or intrasellar anatomical abnormalities (p = 0.0002) than the other groups. The subjects with puberty disorders had a mean sellar volume higher than the other groups (p < 0.05). Apart from pituitary dysfunction, symptoms of the empty sella syndrome were infrequent (9.3% of cases) in our subjects. The age of our subjects, the frequent association between empty sella and pituitary dwarfism and the non-enlarged sellae suggest a different aetiology, perhaps congenital, for empty sella in our subjects. As in adults, empty sella may be associated with both pituitary hypo- and hyperfunction.     

MR and CT diagnosis of carotid pseudoaneurysm in children following surgical resection of craniopharyngioma

We report the cases of two children who underwent CT, MR, MRA and angiography in the diagnosis of postoperative aneurysmal dilatation of the supraclinoid carotid arteries following surgical resection of craniopharyngioma. Craniopharyngiomas are relatively common lesions, accounting for 6–7% of brain tumors in children. They are histologically benign, causing symptoms by their growth within the sella and suprasellar cistern with compression of adjacent structures, especially the pituitary gland, hypothalamus and optic nerves, chiasm, and tracts. Complete surgical resection, particularly of large tumors, is complicated by the fact that the lesions are usually found within the circle of Willis, with displacement and adherence to the adventitia of these vessels [1, 2]. Recent reports in the neurosurgical literature have described aneurysmal dilatation of the supraclinoid internal carotid arteries following aggressive surgical resection of craniopharyngioma [3, 4].     

Pituitary adenoma and unexpected sudden infant death: A case report

Pituitary adenomas can cause sudden death in adults, but they are quite rare during infancy. Herein we present an unusual case of sudden infant death associated with a pituitary adenoma. The clinical presentations were ?quietness”? complained of by the parents and probable gigantism with accelerated growth rate during the first 2 months of life. The female infants had been in good health until one day when, at the age of 3 months, she was found immobile in bed. Microscopic examination showed that the cells of the entire pituitary gland were replaced by bizarre undifferentiated tumor cells. There were no invasive lesions in the surrounding brain tissue. It was also found that the cortex of the adrenal glands was atrophic. No metastatic lesions were observed in any organ. While the mechanism leading our infant to death is both controversial and unknown, we speculated that insufficient secretion of steroid hormones might have caused cardiovascular collapse. © 1994 Wiley-Liss, Inc.     

Organ- and non-organ-specific auto-antibodies in children with hypopituitarism on growth hormone therapy

Serum non-organ-specific antibodies (NOSA) against nuclear, mitochondrial (AMA), smooth muscle, liver/kidney microsomal (LKM), reticulin, ribosomal, and organ-specific antibodies (OSA) against pituitary gland, gonads (testis, ovary) adrenal cortex, thyroid (thyroglobulin and microsomal), pancreas islet cells, gastric parietal cells and intestinal epithelial cells were evaluated in 45 patients with hypopituitarism (mean age 12.4±4.0 years). In 22 of them, 9 with isolated growth hormone (GH) deficiency (IGHD) and 13 with multiple pituitary hormone deficiency, MRI showed anterior pituitary hypoplasia with structural stalk abnormality and ectopic posterior pituitary. Twelve had isolated small anterior pituitary and IGHD and 11 had normal morphology of pituitary gland and IGHD. Controls were healthy age-sex-matched subjects. Thyroid antibodies were detected by a passive haemagglutination test while indirect immunofluorescence was used for the others. The auto-antibodies were found in 7/45 asymptomatic patients, a frequency not significantly different from that in controls; 5 were type NOSA and 2 type OSA. Pituitary antibodies were positive in 1 girl with IGHD and normal pituitary morphology. One girl developed hyperthyroidism during the follow up. Autoantibodies were equally distributed between the three groups and the frequency was not dissimilar from that in controls; this suggests that these patients are not at a higher risk of developing auto-immune disease, at least during the first two decades.Conclusion Pituitary insufficiency in children with different MRI features seems unlikely to be secondary to an auto-immune process.     

Comparison of in-hospital morbidity and mortality in HIV-infected and uninfected children after surgery

Purpose

Increasingly HIV-infected children can be expected to require surgery. The aim of this study was to compare the outcome of HIV-infected and HIV-unexposed children undergoing surgery.

Patients and methods

A prospective study of children less than or equal to 60?months admitted to a tertiary pediatric surgical service from July 2004 to July 2008. Children underwent age-definitive HIV testing and were followed up postoperatively for complications, length of stay and mortality.

Results

Three hundred and twenty-seven children were enrolled: 82 (23?%) HIV-infected and 245 (67?%) were HIV-unexposed. Eighty-four (26?%) children were malnourished, which was higher in the HIV-infected group [41 (50.0?%) vs. 43 (17.5?%), relative risk (RR) 2.9; 95?% confidence interval (CI) 2.0–4.1; p?<?0.0001]. Three hundred and twenty-eight surgical procedures were performed. A similar number of major [28 (34.2?%) vs. 64 (26.1?%); p?=?0.2] and emergency procedures [37 (45.1?%) vs. 95 (38.8?%); p?=?0.34] were performed in each group. HIV-infected children had a higher rate of contamination at surgery [40 (48.7?%) vs. 49 (20?%); RR 2.43 (CI 1.7–3.4); p?<?0.0001]. There were more complications in the HIV-infected group [34 (41.5?%) vs. 14 (5.7?%); RR 7.3 (CI 4.1–12.8); p?<?0.0001]. The most common complications were surgical site complications 30 (55?%), followed by postoperative infections, 19 (34?%). Infections with drug-resistant organisms occurred more commonly in HIV-infected children [11/19 (58?%) vs. 2/13 (15?%); RR 3.8 (CI 1.3–14.2); p?=?0.02]. The median length of hospital stay was longer in the HIV-infected group [4 (IQR 2–14) vs. 2 (IQR 1–4) days; p?=?0.0001]. There was a higher mortality amongst the HIV-infected group [6 (7.3?%) vs. 0 (0?%); p?<?0.0001].

Conclusion

HIV-infected children have a higher rate of postoperative complications and mortality compared with HIV-unexposed children.     

Cerebral infarction in pediatric acquired immunodeficiency syndrome

Cerebral infarction is an uncommon complication of AIDS in pediatric patients. We have seen three HIV-infected children who developed acute neurological deficits due to stroke. Cerebral infarction must be considered in the work-up of a child with AIDS who presents with focal neurological deficit, seizure or mental status change.Stroke is a complication of HIV infection that occurs in approximately 1% of affected children [1]. At autopsy, evidence of cerebral infarction was documented in 10–30% of children with HIV infection [2]. We have seen three children with focal infarction who are HIV positive.     

Pituitary stalk interruption syndrome in 59 children: the value of MRI in assessment of pituitary functions

Pituitary imaging abnormality is a specific indicator of hypopituitarism. This study involved a retrospective review of 59 children diagnosed with pituitary stalk interruption syndrome (PSIS). Of the 59 eligible patients, 54 were born by breech delivery, and there was a significant difference between numbers of patients with breech and head-presenting birth. In order to discuss the relationship between pituitary functions and delineation of pituitary structure in magnetic resonance imaging (MRI), a control analysis was carried out in children with PSIS. Fifty-nine children were subdivided into two groups: group I (partial PSIS, 20 cases) and group II (complete PSIS, 39 cases). There was a significantly small anterior pituitary in both groups of PSIS compared with controls (P?<?0.001). The incidence of ectopic posterior pituitary (EPP) was significantly higher in group II (P?<?0.001). Before and after hormone replacement therapy, pituitary functions were measured and compared with controls. The levels of growth hormone (GH), free thyroxine (FT4), thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), and cortisol (COR) were significantly lower in group II (P?<?0.05). The dosage of levothyroxine sodium in group II was significantly higher than in group I (P?<?0.01). Conclusion. On the basis of birth history, breech presentation may a forewarning for subsequent pituitary hormone deficiencies. Grades of MRI can predict occurrence and severity of PSIS, which are also correlated with the levels of the pituitary target hormone deficiencies. Interruption of pituitary stalk and ectopic posterior pituitary both represent important markers of pituitary structure and function.     

Diagnostic value of pituitary MRI in differentiation of children with normal growth hormone secretion, isolated growth hormone deficiency and multiple pituitary hormone deficiency

Pituitary height, volume and morphology were investigated by MRI in patients aged 3.5-24.9 years with growth hormone deficiency (GHD) in relation to birth history and hormonal findings. Three groups with comparable age, sex and pubertal stage were studied: group I (n=42)--patients with isolated growth hormone deficiency (IGHD); group II (n=22)-- patients with multiple pituitary hormone deficiency (MPHD); and group III (n=30)--healthy controls. Pituitary height and volume differed significantly between the three groups, with the smallest in group II and largest in group III (p <0.001 for both). Both variables correlated significantly with peak GH value in the patient groups (p <0.001). The specificity of pituitary dysmorphology in the determination of GHD was 100% and its sensitivity in differentiation of IGHD and MPHD was 95%. Ectopic neurohypophysis was present in 75% of breech births and 27% of head-presenting patients (p <0.01). This study emphasizes the differential diagnostic value of pituitary MRI and its contribution to the understanding of the pathogenesis and prognosis in GHD.     

Pituitary abnormalities detected by high resolution computed tomography with thin slices in primary hypothyroidism and Turner syndrome

Pituitary hyperplasia, microadenoma or an empty sella was detected in three children with primary hypothyroidism and three with Turner syndrome with the use of high resolution contrast-enhanced computed tomography (CT) with thin slices. Hyperplasia or microadenoma of the pituitary gland frequently occurs secondary to primary hypothyroidism and gonadal dysgenesis, and recognition of these results may eliminate unnecessary surgery in favor of hormone replacement therapy. High resolution contrast-enhanced CT, especially coronal CT, with thin slices is very helpful in demonstrating these pituitary abnormalities.     

Pituitary hyperplasia in children with short stature and primary hypothyroidism

We present eight cases with short stature, pituitary hyperplasia, and hypothyroidism. Pituitary hyperplasia due to primary hypothyroidism was diagnosed on the basis of clinical manifestations, endocrine examination and MRI. After 2 to 6 months of L-thyroxine replacement therapy, the signs of hypothyroidism disappeared; free triiodothyronine, free thyroxine, thyrotropin and prolactin became normal; and pituitary enlargement regressed. In two children, the growth rate remained low when treated with L-thyroxine, but with additional recombinant human growth hormone (rhGH), the height increased by 11 cm per year. No recurrence of lesions was found on follow-up.