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
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.
Precautions before Anesthesia
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
Other Condition to Be Considered
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,
Reichlin S: Neuroendocrinology, in Williams RH, Larsen RP (eds): Williams' Textbook of Endocrinology. 9th ed.
Philadelphia, WB Saunders, 1998, p 165.
Presence of congenital tumors in the esophagus or
Odontoma Dysphagia Syndrome.
Incidence and Genetic Inheritance
Less than 10 cases have been described.
Autosormal dominant inheritance was suspected in 5 cases, while at least one case seemed
to be sporadic.
Odontoma is an undifferentiated mass (hamartoma)
arising from tooth germ or surrounding tissue. The three reported cases had
multiple odontomas (odontomatosis) associated with severe dysphagia. The
child reported by Bader died at the age of 6 years after esophageal surgery.