Case presentation
Patient, male, 11 years old
Main complaint: Growth retardation was found for more than 1 year
Current medical history: Parents complained that the child was found to be shorter than boys of the same age 1 year ago, the annual growth rate was slow (last year's height growth was unknown), the amount of food was average, with age, the growth backwardness was more obvious, the intelligence was normal, there was no breast, external genitalia development, no fatigue, no polyuria, no use of heightening products, no special treatment. During the course of the disease, good spirits, good appetite, normal stool, normal urination.
Past history: the patient is in good health. Both parents are over 170 cm tall.
Personal history: full term, no suffocation. Good condition during pregnancy.
Laboratory tests:
pituitary MR sweep:
Diagnosis: pituitary stalk block syndrome
Pituitary stalk blocking syndrome
Pituitary stalk blocking syndrome (PSIS), also known as pituitary stalk transection syndrome, refers to a series of clinical syndromes that occur when the pituitary stalk is abnormally thinned or absent due to various reasons, so that the connection between the hypothalamus and the anterior and posterior lobes of the pituitary gland is interrupted, and hormones secreted by the hypothalamus cannot be transported to the posterior pituitary lobe through the pituitary stalk, and cannot act on the anterior pituitary lobe through the pituitary portal system.
The main manifestations of the disease are a series of clinical symptoms caused by the lack of multiple hormones in the body, such as growth hormone deficiency caused by growth retardation, dwarfism, thyroid-stimulating hormone deficiency due to hypothyroidism, gonadotropin deficiency of the glandular gland and secondary sexual characteristics dysplasia.
pathogen
The pathogenesis of PSIS is unclear, but common causes are currently thought to be relevant:
1. Injuries to the hypothalamic pituitary gland: such as birth injuries, head trauma, skull base surgery, it is currently believed that abnormal childbirth caused by abnormal childbirth caused by injury to the hypothalamic pituitary gland area caused by the separation of the neuropituitary gland and the rupture of the pituitary stalk as the main causes.
2. Congenital dysplasia or inability of the pituitary gland and pituitary stalk, dysfunction of the downward development of the neuropituitary gland, failure of the pituitary gland and glandular pituitary gland to fuse in the saddle.
3. Hypoxemia or hypoperfusion at birth causes damage to the pituitary stalk and pituitary gland.
4. Congenital genetic factors. It is also considered to be part of midline craniofacial dysplasia. Although a variety of causes of PISP have been reported, none are clearly established.
Typical MRI
Typical manifestations of PSIS in MRI:
(1) The pituitary stalk is absent or small;
(2) T1WI posterior pituitary has no hyperintensity, and ectopic posterior pituitary hyperinflora nodules can be seen in the third ventricular funnel crypt or mid-center bulge, and posterior pituitary ectopicity is a characteristic sign of PSIS;
(3) Anterior pituitary dysplasia.
Typical MRI
01
Case 1
Male, 16 years old. Figures 1 and 2 show that the sagittal and coronal images of the flat sweep T1WI show the absence of the pituitary stalk; the posterior pituitary fossa is normal, and the posterior pituitary hyperinflorum signal disappears. The posterior lobe of the ectopic pituitary is located in the middle of the base of the third ventricular funnel crypt (arrow); Figure 3 shows absent pituitary stalk and anterior pituitary dysplasia for enhanced scan of TWI1 sagittal and coronal position images
02
Case 2
Male, 10 years old. Figures 1 to 2 are flat sweep T1WI sagittal and coronal images: significant thinning of the pituitary stalk The posterior pituitary lobe is located in the middle of the bottom of the third ventricular funnel crypt (white arrow); Figure 3 is an enhanced sagittal image: the pituitary stalk is significantly strengthened, showing clearer anterior pituitary dysplasia.
Debut: Lilac Garden Video Time
Author | xiao79bing
Content planning | Little Snowball, Peng Long
Title image source | Stand cool Heero
Illustration source | Courtesy of the author
References | (Swipe down)
Wang Weiwei, Tian Shiyun, Wu Jianlin, et al. MR examination method and its manifestations of pituitary stalk blocking syndrome[J]. China Medical Imaging Technology, 2020, 36(12):3.
Cheng Hua, Zeng Jinjin, Li Hang, et al. MRI manifestations of pituitary stalk blocking syndrome and their correlation with clinical significance[J]. Journal of Clinical Radiology, 2008.
Xiang Jun, Xiao Ping, Zhu NYong, et al. MRI characteristics and diagnosis of pituitary stalk block syndrome[J]. World of Medicine, 2007(S1):16-17.