++
Partial destruction of hypothalamic nuclei resulting
in hormonal dysfunction with obesity, growth retardation, and hypogonadism.
++
Adiposogenital Syndrome; Dystrophia Adiposo-Genitalis;
Neuropituitary Dystrophy; Cerebral Adiposity; Pituitary Infantilism of
Adults; Fröhlich Syndrome; Fröhlich Obesity; Morbus
Fröhlich Syndrome; Launois-Cléret Syndrome.
++
Unknown, but male prevalence.
++
Several organic lesions of the hypothalamus may
cause this disorder, including tumors (craniopharyngioma), encephalitis,
Friedreich ataxia, demyelinating diseases, and microcephaly. Originally
described as delayed puberty, hypogonadism, and obesity associated with a
tumor impinging on the hypothalamus. Presence of obesity indicates damage to
the appetite-regulating regions of the hypothalamus. Hypothalamic
dysfunction and hypopituitarism mainly affect the somatotropic hormones and
the gonadotropins.
++
Obesity with prepubertal onset affecting
breasts, abdomen, femoral regions, and hips. Delayed development of
secondary sexual characteristics. Headache, visual impairment, mental
retardation. Polyuria and polydipsia as a result of diabetes insipidus.
++
Based on the clinical and biochemical features (low
serum gonadotropin levels; positive vasopressin test for polyuria, if
diabetes insipidus is present). Radiologic examinations may show delayed
ossification of skeletal structures, suprasellar calcification or
destruction, depending on the etiology.
++
Dehydration as a consequence of
diabetes insipidus requires preoperative fluid replacement and check of
serum electrolytes. The patient may be anxious and uncooperative because of
mental retardation and visual disturbances. Sedative premedication and/or the
presence of the primary care giver for induction of anesthesia may be helpful.
++
Venous access can be difficult.
Depending on the grade of obesity, a rapid sequence induction should be
considered. Obesity and upper airway obstruction may render face-mask
ventilation difficult.
++
Prader-Labhart-Willi Syndrome: Infantile hypotonia, early
childhood-onset obesity, hypogonadism, mental retardation.
Bruch H: The Froehlich's syndrome: Report of the original case. Am J Dis Child 58:1282,
1939.
Reichlin S: Neuroendocrinology, in Williams RH, Larsen RP (eds): Williams' Textbook of Endocrinology. 9th ed.
Philadelphia, WB Saunders, 1998, p 165.