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MRI FINDINGS
Small pituitary fossa Aplastic anterior
pituitary Normal posterior
pituitary and infundibular stalk
No e/o ectopic location of anterior and posterior pituitary
DISCUSSION
Congenital pituitary gland absence (aplasia) is a rare anomaly that may involve absence of both the anterior and the posterior pituitary lobes and, in many cases, the pituitary stalk
Imaging findings Absence of an identifiable pituitary gland Small sella turcica
Differential diagnosis: Severe hypoplasia of the pituitary gland Empty sella
MRI FINDINGS
absence of an identifiable pituitary gland
Small and flat sella turcica, sometimes covered by a layer of dura
Absent / hypoplastic infundibular stalk
Ectopic posterior pituitary
DISCUSSION Congenital pituitary gland absence (aplasia) is a rare
anomaly that involves absence of both the anteriorand the posterior pituitary lobes and, in many cases, the pituitary stalk
In patients with neonatal panhypopituitarism,severe symptoms of hypoglycemia may appear during the 1st hours of life
Other symptoms include Seizures Apnea Cardiovascular collapse and arrest
Infants are of normal length and weight at term or postterm
Physical examination may be unrevealing; however, some male infants have a microphallus, a poorly developed scrotum, and/or small undescended testes
The normal size of the infant at birth has been explained by the presence of maternal growth hormone
Facial abnormalities—include, cleft lip and palate, poorly developed nasal septum, and hypotelorism
Although the size of infants is normal at birth, growth retardation and delayed bone age may be found at 6 – 8 weeks of age
Neonatal hypoglycemia associated with poorly functioning anterior pituitary gland may represent a series of separate syndromes with no structural brain anomalies or with defects such as Craniofacial defects Absence of septum pellucidum Septo-optic dysplasia Arrhinencephaly, Holoprosencephaly Anencephaly
IMAGING FINDINGS
Characteristic imaging finding of pituitary aplasia is absence of an identifiable pituitary gland
Small and flat sella , may be covered by a layer of dura
Absence of pituitary stalk, posterior pituitary Differential diagnosis
1. Severe hypoplasia of the pituitary gland Sella is empty but generally not flat Infundibulum and posterior pituitary are generally
present
2. Empty sellaPituitary gland is severely flattened.
REFRENCES
1. Kalhan SC, Parimi PS. Metabolic and endocrine disorders. In: Fanaroff AA, Martin RJ,eds. Neonatal-perinatal medicine: diseases of the fetus and infants. 7th ed. St Louis, Mo:Mosby, 2002; 1355–1367.
2. Barkovich AJ. Congenital malformations of the brain and skull. In: Barkovich AJ. Pediatric neuroimaging. 3rd ed. Philadelphia, Pa:Lippincott Williams & Wilkins, 2000; 351–352.
3. Sadeghi-Nejad A, Senior B. A familial syndrome of isolated “aplasia” of the anteriorpituitary: diagnostic studies and treatment inthe neonatal period. J Pediatr 1974;84:79–84.