Hypopituitarism and stalk agenesis: a congenital syndrome worsened by breech delivery?
Thirty-seven patients with idiopathic hypopituitarism, of whom 12 had multiple pituitary hormone deficiencies (MPHD) and 25 isolated growth hormone deficiency (IGHD), were evaluated by magnetic resonance imaging (MRI). Twenty-two of the 37 showed congenital anterior pituitary hypoplasia, stalk agenesis and ectopic posterior pituitary gland at the infundibular recess (group A), while the remaining 15 presented isolated anterior pituitary hypoplasia (group B). Perinatal histories obtained from all patients demonstrated that 18/22 children of group A (81.81%) had histories of adverse perinatal events, with breech presentation in 15 (68.18%). Twelve of 12 children of group A born by breech delivery developed MPHD; 3 born by cesarean section for breech presentation had only IGHD. Patients of group B had also a high incidence of perinatal insults (12/15, 80%), but breech delivery was markedly less frequent (13.33 vs. 68.18% of group A) and responsible for only IGHD. Group B had also higher percentages of maternal spontaneous abortion and low birth weight. Our study suggests that several factors may play a role in the development of growth hormone deficiency. Some patients had severe perinatal insults apparently leading to hypopituitarism. We were able to define by MRI a group of patients with congenital abnormalities, such as anterior pituitary hypoplasia, stalk agenesis and posterior pituitary ectopia, among whom breech presentation was very common. In this group, breech delivery was always followed by MPHD while cesarean or normal delivery in such patients was followed by IGHD only.
Maghnie
M
M
Pediatric Department, University of Pavia, IRCCS Policlinico S. Matteo, Italy.
Larizza
D
D
Triulzi
F
F
Sampaolo
P
P
Scotti
G
G
Severi
F
F
D000328:Adult; D001946:Breech Presentation; D002585:Cesarean Section; D002648:Child; D005260:Female; D006130:Growth Disorders / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D013006:Growth Hormone / Q000172:deficiency / Q000627:therapeutic use*; D006801:Humans; D007018:Hypopituitarism / Q000473:pathology / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D010902:Pituitary Gland / Q000002:abnormalities* / Q000473:pathology; D010903:Pituitary Gland, Anterior / Q000002:abnormalities; D010904:Pituitary Gland, Posterior / Q000002:abnormalities; D010908:Pituitary Hormones, Anterior / Q000097:blood / Q000172:deficiency; D011247:Pregnancy
D010908:Pituitary Hormones, Anterior; D013006:Growth Hormone