Precocious puberty is defined as the onset of secondary sexual characteristics before age 8 in girls and 9 in boys. It can be classified as central (gonadotropin-dependent) or peripheral (gonadotropin-independent) puberty. Central puberty is treated with GnRH agonists to slow progression, while peripheral causes like tumors require treatment of the underlying condition. Evaluation involves assessing pubertal development, growth, bone age, and hormone levels to distinguish central from peripheral puberty and identify any lesions.

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

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
hypothalamic area or to areas of the brain
anatomically 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:
Height, weight, and osseous maturation are
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,
 In boys:
- 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 CNS lesion)
 Visual field
 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
Proceed with GnRH stimulation test
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

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
 GnRH agonist administration  slows accelerated
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