Archives ofDisease in Childhood 1990; 65: 1199-1202 1199 ORIGINAL ARTICLES Arch Dis Child: first published as 10.1136/adc.65.11.1199 on 1 November 1990. Downloaded from Endocrine function and morphological findings in patients with disorders of the hypothalamo-pituitary area: a study with magnetic resonance Emanuele Cacciari, Stefano Zucchini, Gabriella Carla', Piero Pirazzoli, Alessandro Cicognani, Massimo Mandini, Maurizio Busacca, Claudio Trevisan Abstract alteration on the other has not yet been clari- Evaluation of the seliar area was performed fied. In order to investigate this matter further, with magnetic resonance imaging in 101 the sellar area of 101 patients with various endo- patients (age range 0-8-27 years) with hypo- crine disorders was studied with magnetic pituitarism, isolated diabetes insipidus, hypo- resonance. gonadotrophic hypogonadism, and central precocious puberty. The hypopituitary Patients and methods patients (n=70) included multiple pituitary We examined 101 patients (56 males and 45 deficiency (n=23), pituitary deficiency with females; age range 0i8-27 years) with the diabetes insipidus (n=5), and isolated growth following endocrine disorders: hypopituitarism, hormone deficiency (n=42). The patients with isolated diabetes insipidus, hypogonadotrophic multiple pituitary deficiency showed patho- hypogonadism, and central precocious puberty. logical morphological findings in all cases, with stalk and posterior lobe always involved. (1) HYPOPITUITARISM The group with associated diabetes insipidus All patients (n= 70) had short stature (below 3rd had abnormal stalk in four of five cases and centile) at time of diagnosis and showed growth posterior lobe not visible in all cases. Only hormone deficiency (growth hormone concen- five of 42 (12%) patients with isolated growth tration peak <8 ,ug/l after pharmacological tests hormone deficiency had abnormalities of the and mean growth hormone concentration seilar area. In two out of four patients with during night profile <3 [ig/l). No patients had a isolated diabetes insipidus posterior lobe was positive history for postnatal head trauma, not seen. All patients with hypogonadotrophic intracranial tumour, or brain infection. All pati- hypogonadism (three with Kallmann's syn- ents received replacement treatment with pit- drome, one Prader-Willi syndrome, and two uitary or recombinant human growth hormone. idiopathic hypogonadism) appeared normal. Six patients had stopped taking growth hor- In precocious puberty (n=21) the three mone at the time of the examination. patients with onset of symptoms before age 2 A perinatal history was obtained from the http://adc.bmj.com/ years exhibited a hypothalamic hamartoma, parents or from case records. whereas in the others with onset of puberty These patients were subdivided into the between age 2 and 7 the magnetic resonance following three groups: image was normal in 17 of 18 patients. The probability of finding a pathological (a) Multiple pituitary deficiency magnetic resonance image was considerably There were 23 cases (20 males and three high in our patients with multiple pituitary females; mean (SD) age 18-3 (4-6) years, range on October 1, 2021 by guest. Protected copyright. deficiency, isolated diabetes insipidus, and 11 8-27) of whom 17 had adrenocorticotrophic precocious puberty with very early onset of hormone deficiency, 21 thyrotrophin releasing symptoms. On the contrary, purely functional hormone deficiency (protracted or delayed thy- Second Paediatric abnormality is suggested in most patients with roid stimulating hormone response to thyrotro- Cainic, isolated growth hormone deficiency, hypo- phin releasing hormone with peripheral thyroid University of Bologna, Italy gonadotrophic hypogonadism, and preco- deficiency during follow up), and 19 certain Emanuele Cacciari cious puberty with later onset of symptoms. gonadotrophin deficiency. All subjects were Stefana Zucchini having appropriate replacement treatment: Gabriella Carla Piero Pirazzoli hydrocortisone acetate for adrenocorticotrophic Alessandro Cicognani When a diagnosis of endocrine disorder involv- hormone deficiency, thyroxine for thyroid sti- Massimo Mandini ing the hypothalamo-pituitary axis is made mulating hormone deficiency, and human Centro Diagnostico through functional testing, it is impossible to chorionic gonadotrophin and/or testosterone Citta' di Bologna, known whether the defect has an anatomical enanthate for gonadotrophin Bologna, deficiency. Italy basis. Magnetic resonance imaging has proved Maurizio Busacca to be the best technique to visualise the sellar (b) Pituitary deficiency with diabetes insipidus Claudio Trevisan and juxtasellar area and it has been used There were five cases (four males and one Correspondence and requests successfully to evaluate children with hypo- female, range 6-15 years). All patients had idio- for reprints to: Professor Emanuele Cacciari, pituitarism, diabetes insipidus, or other dis- pathic diabetes insipidus and one patient also Clinica Pediatrica 2, orders in this area. 1-3 In particular the pituitary had thyroid stimulating hormone deficiency. via Massarenti 11, 40138, Bologna, gland and stalk are clearly seen, even if the asso- Diabetes insipidus was treated with synthetic Italy. ciation between neuroradiological findings on antidiuretic hormone, desmopressin (DDAVP, Accepted 8 June 1990 one hand and type or severity of endocrine Ferring). 1200 Cacciari, Zucchini, Carla', Pirazzoli, Cicognani, Mandini, Busacca, Trevisan (c) Isolated growth hormone deficiency weighted sequences (TR 364 msec, TE 30 msec) There were 42 cases (25 males and 17 females, and T2 weighted sequences (TR 1500 msec, TE mean (SD) age 11-6 (3) years, range 5-4-17-9). 50/100 msec). The height of the anterior lobe Arch Dis Child: first published as 10.1136/adc.65.11.1199 on 1 November 1990. Downloaded from was measured on the sagittal Ti weighted image, using a cursor on the display. A height of (2) ISOLATED DIABETES INSIPIDUS less than 2 mm was considered as an indication There were four cases (one male and three of small pituitary gland.4 5 Sedation was females, range 0 8-19 years). All cases suffered necessary in eight patients. from idiopathic diabetes insipidus and were having replacement treatment. Results (1) HYPOPITUITARISM (3) HYPOGONADOTROPHIC HYPOGONADISM All patients with multiple pituitary deficiencies, There were six cases (five males and one female, with or without diabetes insipidus, presented age range 14-16 years) including three patients abnormalities of the pituitary or of the stalk. with Kallmann's syndrome (age 14-8, 15-8, and 16 years), one patient with Prader-Willi syn- drome (15-4 years), and two with idiopathic (a) Multiple pituitary deficiency (table 1) hypogonadotrophic hypogonadism (14 and 15 The stalk was mainly involved: clearly tran- years). They had not received any previous sected in 10 cases (fig 1) and not seen in the treatment. remaining 13 cases. The posterior lobe was ectopic at the proximal stump in the 10 patients with transected stalk and in seven other pat- (4) PRECOCIOUS PUBERTY ients. Six patients did not show the characteris- There were 21 cases (20 females and one male, tic high signal intensity of the posterior lobe. mean (SD) age 7 5 (3 1) years; age range The anterior lobe was abnormal in 16 patients: 0 8-11-2). All cases started puberty before age 7 small in three cases (height of 1 9, 1 5, 1-8 mm) years and all but two were on treatment with and not seen in the remaining 13 cases; in five of synthetic luteinising hormone releasing hor- the latter the sellar area was partially or com- mone analogue (buserelin). pletely filled with cerebrospinal fluid (empty For magnetic resonance a 0-5 T magnet was sella). Mean (SD) anterior lobe height in the used in all patients (Philips Gyroscan) with seven cases with pituitary >2 mm was 3-6 (2 4) sagittal and coronal images of 2 5 mm slice mm. There was no correlation between height thickness. Images were obtained with TI of the anterior lobe and duration of replacement treatment. Two patients had agenesia of the corpus callosum and one hypogenesia of cere- Table 1 Magnetic resonance findings in the 23 patients with multiple pituitary deficiency without diabetes insipidus bellar vermis. In 12 patients (52%) a clear history of adverse No of Anterir lobe Stalk Posterior events was patients lobe perinatal found (breech delivery in seven cases, asphyxia in five cases); these sub- 5 <2 mm Transected Ectopic not http://adc.bmj.com/ 5 Normal Transected Ectopic jects did differ from the others concerning 1 Normal Not seen Ectopic magnetic resonance findings and type of hor- 3 <2 mm Not seen Ectopic 1 Normal Not seen Not seen monal deficiency. Similarly, the seven subjects 3 <2 mm Not seen Not seen with anterior lobe >2 mm (table) did not differ 3 Sella partially filled with Ectopic cerebrospinal fluid from the others for either the type of hormonal 2 Sella filled with cerebrospinal fluid Not seen defect or the presence of adverse perinatal events (four out of seven). on October 1, 2021 by guest. Protected copyright. (b) Pituitaty deficiency with diabetes insipidus (table 2) In all cases the posterior lobe was not seen. The stalk was not visible in four cases. The patient with growth hormone and thyroid stimulating hormone deficiency had the sella fully filled with cerebrospinal fluid (empty sella). One patient had an anterior lobe of 1-3 mm. No patients had a positive history for adverse peri- natal events. _F ~~~~~~~~~~~~/ I1 Table 2 Magnetic resonance findings in the five patients with pituitary deficiency and diabetes insipidus No of Anterior lobe Stalk Posterior Figure 1 Sagittal magnetic resonance image (A) and line diagram (B) illustrating a patients lobe hypopituitary patient with multiple deficiency: the stalk is transected (arrow), the bright signal 3 Normal Not seen Not seen ofthe posterior lobe (P) is absent in the sella and appears to be present in the median eminence 1 <2 mm Normal Not seen ofthe hypothalamus. The anterior lobe (arrowhead) is small in height (1 9 mm). Nornal I Sella filled with cerebrospinal fluid Not seen appearance ofthe hypothalamus (H); o: optic chiasm, ST: sella turcica).