1) Precocious puberty is defined as the onset of secondary sexual characteristics before 8 years of age in girls and 9 years in boys. 2) Precocious puberty can be classified as gonadotropin-dependent (GDPP) or gonadotropin-independent (GIPP). GDPP is caused by early activation of the hypothalamic-pituitary-gonadal axis while GIPP is caused by excess sex hormone secretion from other sources. 3) Evaluation and management depends on determining if the cause is central or peripheral. Brain imaging is important to identify potential lesions for GDPP. GnRH agonists are the primary treatment for GDPP while treatment for

1. PRECOCIOUS PUBERTY

2. Definition of precocious puberty
Precocious puberty is defined
as the onset of secondary
sexual characteristics before
8 yr of age in girls and 9 yr in
boys.

4. Classification:
Complete
Central
( gonadotropin-
dependent puberty,
GDPP)
Peripheral
(gonadotropin-
independent puberty,
GIPP)
Incomplete
Premature
thelarche
Premature
pubarche
Premature
menarche

5. Gonadotropin-dependent precocious
puberty ( GDPP)
also known as true precocious puberty
early activation of the entire hypothalamic-
pituitary-gonadal (HPG) axis
is caused by the secretion of high-amplitude pulses of
gonadotropin-releasing hormone (GnRH) by the
hypothalamus.
Although the onset is early, the pattern and timing of
pubertal events usually progresses in the normal
sequence.

6. • Non- CNS lesion:
- Idiopathic
- Genetics
-Prolonged, untreated severe hypothyroidism
• CNS lesion:
-CNS tumour
- CNS irradiation
- hydrocephalus, cysts, trauma, CNS inflammatory disease,
Gonadotropin-dependent
precocious puberty

7. condition occurs at least 5- to 10-fold more
frequently in girls than in boys
Approximately 90% of sexual precocity in girls is
idiopathic
75% of boys have a structural CNS
abnormality

8. Causes of CNS lesion:
Hypothalamic hamartomas are the most common
brain lesion causing true precocious puberty.
Hamartomas are non-malignant tumours of the tuber
cinereum that consists of disorganized collection of
neurons and glias.
ectopically located neural tissue containing GnRH-
secretory neurons and may function as an accessory
GnRH pulse generator
Other tumour: astrocytoma, optic and hypothalamic
glioma

9. Causes of CNS lesion: (cont.)
Radiation therapy for leukemia or intracranial
tumours  irradiation is directed to the
brain
hypothalamic
anatomically
area or to areas of the
distant from the hypothalamus 
increases the risk of precocious puberty

10. Clinical manifestations:
Begin at any age, follows the sequence observed in normal
puberty
In girls:
Breast enlargement comes first
Pubic hair may appear simultaneously but more often
laters
 Menarche is a late event ( irregular cycle and usually
anovulatory )
The pubertal growth spurt occurs early in female puberty

12. In boys:
Testicular enlargement
( unnoticed)
Enlargement of penis
Axillary hair, acne, voice
deepens
Erections are common
spermatogenesis
observed as early as 5-6
yr of age

13. In both gender:
weight, and osseous maturation are
Height,
advanced
Without treatment, 30% early closure of the
epiphyses > height less than the 5th percentile as
adults
Emotional and mood swings are common

14. In intracranial lesion ( eg: hamartoma ) :
Hypothalamic signs:
diabetes insipidus
hyperthemia
unnatural crying or laughing(gelastic seizures)
cachexia
In optic glioma : proptosis
In irradiation of brain : signs of growth hormone
deficiency may present

15. Gonadotropin-independent precocious
puberty ( GIPP)
Independent of gonadotropin secretion and no
activation of the HPG axis
aka precocious pseudopuberty
caused by excess secretion of sex hormones
(estrogens or androgens) derived either from the
gonads or adrenal glands or from exogenous
sources

16. Causes of GIPP :
Girls Boys
Ovarian cysts Leydig cell tumour
Ovarian tumours Human chorionic gonadotropin
(hCG) secreting germ cell tumors
Granulosa theca cell tmour Familial male-limited precocious
puberty
Both in boys and girl:
Exogenous estrogen
Adrenal pathology ( eg: androgen-
secreting tumour and CAH)
Teratoma
McCune-Albright syndrome

17. How to approach:
Onset of age?
Is the cause of precocity central or peripheral? Need to ask the
pattern of pubertal development in GDPP  normal pubertal
development but at an earlier age
How quickly is the puberty progressing?
rapid bone maturation suggest either GDPP or GIPP
Presence of headaches or seizures ? CNS lesion
Previous history of CNS disease or trauma?
Are the secondary sexual characteristics virilizing or feminizing?
 feminizing in Sertoli cell tumor
 Virilization in CAH
Any exposure to exogenous sex steroids?? (medicinal or cosmetic
sources)
Timing of pubertal onset in his or her parents and siblings? family
history of similar symptoms?

18. Physical examination:
Measurements of height, weight, and calculation of height velocity (cm/yr)
Pubertal staging:
 In girls:
- Breast staging, pubic hair,
 Inboys:
- Testicular volume? Penile size? Pubic hair?
Abdominal examination:
 Palpate for mass ( in ovarian cyst and tumour)
Neurological examination (neurological deficit?)
Eye examination :
 Fundoscopy :look for papilledema ( in CNSlesion)
 Visualfield
Look for signs of virilization in female? Ambigious genitalia? Hirsutism?
Dermatological exam to evaluate for cafe-au-lait spots( in McCune-Albright
syndrome).

19. Investigations:
Serum LH
concentration
If basal level of LH
are low or
intermediate
If LH and FSH levels
not increase with
GnRH stimulation
( GIPP)
If LH and FSH levels
increase with GnRH
stimulation
( GDPP )
If basal level of LH
are markedly elevated Confirm GDPP
Proceed with GnRH stimulation test

20. ** Patients with GDPP must proceed with brain imaging to exclude any CNS lesion
Contrast-enhanced MRI is use to detect any hypothalamic and infundibular lesion

21. Other investigations:
Sex hormone
 To establish degree of biochemical pubertal enhancement
 Serum estradiol are low or undetectable in the early phase of sexual
precocity
 Serum testosterone levels are detectable or clearly elevated
Thyroid function test
- To be done if there is any clinical evidence of hypothyroidism
Radiographic assessment of bone age:
- If the patient has a normal bone age, he or she is unlikely to have
GDPP

22. Several ix to identify the peripheral cause of
precocious puberty ( GIPP ):
- Serum testosterone and estradiol
- Serum LH and FSH
- Renal profile (check on dehydration or electrolytes
imbalance)  in aldosterone deficiency
- Serum cortisol  to screen for Cushing syndrome
- Abdominal and pelvic ultrasound  to identify
presence of ovarian cysts or tumour
- Ultrasound of testes  possibility of Leydig cell
tumour

23. Management of GDPP:
The treatment options depend upon the cause of the
precocious puberty
If (GDPP) is caused by an identifiable central nervous
system (CNS) lesion therapy is directed toward the
underlying pathology
For most patients with GDPP  primary treatment
option  gonadotropin-releasing hormone
(GnRH) agonist
slows accelerated
GnRH agonist administration 
puberty and improves final height

24. The decision of whether to treat GDPP with a GnRH
agonist depends on:
- child’s age
- the rate of pubertal progression
- height velocity
- rate of bone age advancement.

25. Management for GIPP
GIPP does not respond to GnRH agonist
therapy. Instead, treatment is directed at the
underlying pathology:
Children with tumors of the testis, adrenal gland,
and ovary treated by surgery.
Those with hCG-secreting tumors  require some
combination of surgery, radiation therapy, and
chemotherapy depending upon the site and histologic
type.

26. Management for GIPP (cont.)
A large functioning follicular cyst of the ovary 
Cysts develop and regress spontaneously
conservative management
Children whose sexual precocity is caused by
exposure to exogenous sex steroids  exposure
identified and removed
Children with identifiable defects in adrenal
steroidogenesis ( CAH )  glucocorticoid therapy

27. Incomplete precocious puberty
Definition: isolated manifestations of precocity
without development of other signs of puberty.
Incomplete
Premature
thelarche
Premature
pubarche
Premature
menarche

28. Premature thelarche
Transient condition of isolated breast development
that most often appears in the first 2 yr of life, often
persists for 3-5 yr, and is rarely progressive
mostly  idiopathic
either remit spontaneously or are very slowly
progressive.
no other signs of pubertal development and their
growth rate is normal.
Serum estradiol : usually normal
Mx: reassurance and monitoring regularly for any
other sign of pubertal advancement

29. Premature pubarche
Appearance of sexual hair before the age of 8 yr in girls or 9
yr in boys without other evidence of maturation
Slowly progressive condition that requires no therapy
Longitudinal observations suggest that ~50% of affected
girls are at high risk for
 Hyperandrogenism
 Polycystic ovary syndrome
 Metabolic syndrome

30. Premature menarche
Diagnosis of exclusion
Isolated vaginal bleeding in the absence of other
secondary sexual characteristics
Very rare
Carefully exclude:
 Vulvovaginitis
 Foreign body
 Sexual abuse