Precocious puberty

Precocious puberty

1. PRECOCIOUS PUBERTY Dr.MANOJ PRABHAKAR Resident , Dept. of Paediatrics

2. 3 yrs old girl with complaints of enlargement of breast from 1 yr and per vaginal discharge for one yr.Child alright 1 yr back. Normal develeopmental milestones. White discharge per vagina + No H/O birth injury,head injury,encephalitis,headache or seizures. O/E b/l breast enlargement and firm in consistency.Nipple and areola developed , Axillary and pubic hair sparse. LH & FSH elevated. USG abdomen shows enlarged uterus and ovaries of adult size.

3. OBJECTIVES Physiology of Puberty Tanner staging Def. of Precocious Puberty Causes Treatment

4. PUBERTY Puberty is the developmental stage during which a child becomes a young adult, characterized by the maturation of gametogenesis , secretion of gonadal hormones, and development of secondary sexual characteristics and reproductive functions.

5. Hypothalamic-pituitary- gonadal axis

6. Tanner Staging

7. STAGES OF BREAST DEVELOPMENT B-1: pre-pubertal B-2: breast bud B-3: enlargement of breast and areola with no separation of the contours B-4: projection of areola and papilla to form a secondary mound above the level of the breast B-5: recession of the areola to the general contour of the breast with projection of the papilla only.

8. STAGES OF PUBIC HAIR DEVELOPMENT IN FEMALES Ph-1: pre-pubertal Ph-2: sparse growth of long slightly pigmented hair usually slightly curly mainly along the labia Ph-3: the hair is darker, coarser and curlier and spreads over the junction of the pubes Ph-4: the hair spreads covering the pubes Ph-5 the hair extends to the medial surface of the thighs and is distributed as an inverse triangle.

9. Tanner Staging – Males

10. STAGES OF PUBIC HAIR AND GENITAL DEVELOPMENT IN THE MALE. G-1: pre-pubertal G-2: the testis and scrotum enlarge, and the skin of the scrotum shows some reddening and change in the texture. Sparse growth of pigmented hair usually slightly curly mainly at the base of the penis (Ph-2) G-3: Testis and scrotum enlarge further, the penis grows mainly in length but also in breadth. The hair is darker, coarser and curlier and spreads over the junction of the pubes (Ph-3) G-4: Scrotum, testis and penis grow further with development of the glans and further darkening of the scrotal skin. The hair spreads covering the pubes G-5: adult stage with spreading of the hair to the medial surface of the thighs.

11. Thelarche denotes the onset of breast development, an estrogen effect. Pubarche denotes the onset of sexual hair growth, an androgen effect. Menarche indicates the onset of menses and spermarche the appearance of spermatozoa in seminal fluid

12. DEF: Onset of secondary sexual characteristics before the age of 8yrs in girls and 9 yrs in boys. Depending on the primary source of the hormonal production classified as : Central Peripheral

13. Classification : Central Precocious Puberty aka GDPP is caused by early maturation of the hypothalamic-pituitary- gonadal axis. It is characterized in girls by both breast development and pubic hair sexual maturation. In boys by both pubic hair and testicular enlargement.

14. Cntd .. Peripheral Precocious Puberty aka GIPP is caused by excess secretions of sex hormones derived either from the gonads or adrenal glands.

15.   Isosexual precocious puberty , feminizing signs appear in girls, masculinization in boys . Heterosexual precocious puberty  causes signs of masculine characteristics in girls and feminization in boys.

16. Conditions Causing Precocious Puberty CENTRAL PRECOCIOUS PUBERTY : Idiopathic Organic brain lesions Brain tumors , head trauma etc Hypothyroidism.

17. Peripheral Precocius puberty : Isosexual ( Feminizing) conditions in females: McCune-Albright Syndrome. Ovarian Tumors Heterosexual ( Masculinizing ) conditions in females: CAH Adrenal tumors Ovarian tumors Conditions Causing Precocious Puberty

18. Contd.. Isosexual ( Masculinizing )conditions in boys: CAH Leydig cell tumors hCG secreting tumors Heterosexual (Feminizing ) conditions in boys: Faminizing adrenocortical tumor.

19. EVALUATION MEDICAL HISTORY : - Age at onset Sex Pubertal Progression Symptoms suggestive of hypothyroidism H/o past CNS infection, headache, visual disturbances & seizures.

20. Natural course of idiopathic central precocious puberty.

21. EVALUATION Physical Examination : Measurements of height, weight, height velocity Pubertal staging according to Tanner’s staging. Evaluate androgen & estrogen effects. Inspection of skin. ( Café au lait macules in McCune –Albright Syndrome. Examination for signs of hypothyroidism.

22. EVALUATION Basic Radiology : Bone Age Pelvic & Abdominal Sonography

23. Hormone Evaluation: Intravenous administration of gonadotropin releasing hormone ( GnRH stimulation test) or a GnRH agonist ( leuprolide stimulation test) is a helpful diagnostic tool for boys. In girls, the central nature of sexual precocity can be proven by detecting pubertal levels of estradiol (>50 pg/ mL ), 20-24 hr after stimulation with leuprolide .

24. TREATMENT MEDICAL : Indications of treatment: Predicted adult height is less. Psychologically distressing to child. Rapid progression. GnRH Agonists : Inj . Leuprolide ( 0.5-0.3 mg/kg/dose) monthly.

25. TREATMENT Surgery : Tumors of the ovary,testis & adrenals require surgical removal. Hypothalamic Hamartomas is hazardous and is not recommended because they never grow or become malignant. Germ cell,pineal tumors & hCG producing suprasellar tumors can be treated by radiotherapy.