Langerhans cell histiocytosis (LCH) is a rare disease often involving the hypothalamo-pituitary axis. Extreme obesity is frequent, but effective treatment is not. Malabsorptive bariatric surgery techniques are effective for weight loss in hypothalamic dysfunction. Laparoscopic sleeve gastrectomy (LSG) has recently emerged as a restrictive bariatric procedure to be used before biliopancreatic diversion or gastric bypass, thus reducing surgery-associated morbidity in particularly obese patients.
Report: We report a 20-year-old man, current smoker, puberty at thirteen, diagnosed as having LCH involving lungs and maxilla treated with butazolidin. He related polyuria, polydipsia and hyperphagia. Normal libido, no erectile dysfunction. Weight 198 kg, height 190 cm, BMI 54.84 kg/m2. Scarce body hair, goiter, bilateral gynecomastia. Testis 34 ml. Obstructive sleep apnea.
Laboratory: pOsmolarity 289 mosm/kg (270290), Uosmolarity 79 mosm/l per kg. TSH 13 mcU/ml (0.35), T4L 0.9 ng/dl (0.81.7), positive anti-TG and anti-TPO AB. Undetectable GH, LH and FSH, f-testosterone 1.1 pg/ml (955), IGF-I 214 ng/ml, PRL 329 mcUI/ml (79208). Normal ACTH and ACTH-stimulated cortisol.
NMR: Infiltration of hypothalamus and pituitary stalk.
Diagnosis: Central diabetes insipidus, primary autoimmune hypothyroidism, secondary hypogonadism, morbid obesity.
Treatment: Desmopressin, levothyroxine, testosterone gel. Hypocaloric diet, exercise, behaviour therapy, and sibutramine were of no benefit. Weight reached 220 kg (BMI 60.9 kg/m2). A LSG was used as first-step intervention. Maximum weight loss was 37 kg (EWL% excess weight loss percentage 26.4%) four months following surgery. Due to voracious eating the patient reached 198.8 kg in the following 6 months.
Conclusion: Restrictive bariatric surgery, such as LSG, does not seem to be effective as a first step prior to a malabsorptive procedure in especially obese subjects with hypothalamic dysfunction.
03 - 07 May 2008
European Society of Endocrinology