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CASE REPORT
Year : 2020  |  Volume : 13  |  Issue : 4  |  Page : 413-415  

Central precocious puberty: A case report


Department of Pediatrics, Bharati Vidyapeeth University Medical College, Pune, Maharashtra, India

Date of Submission25-Nov-2019
Date of Decision28-Jan-2020
Date of Acceptance11-Mar-2020
Date of Web Publication20-Jul-2020

Correspondence Address:
Zubin Ajay Mahajan
I-8, Shivanagari Society, Near Mahatma Society, Kothrud, Pune - 411 038, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_322_19

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  Abstract 


Precocious puberty is the onset of physical and hormonal signs of pubertal development before the age of 9 years in males and 8 years in females. A 2-year 9-month-old Indian male child was brought to the hospital with complaints of excessive growth of pubic hair and increase in penile length for 2 months. On local examination of the genitalia, pubic hair was ++, his penile length was 9 cm, and testicular volume was >4 mL. The Sexual Maturity Rating score was A1P2T4. Serum testosterone levels – 592.76 mmol/L; serum luteinizing hormone – 2.18 IU/L; and serum follicle-stimulating hormone – 4.69 IU/L. His X-ray of the left wrist showed bone age >6 years. Magnetic resonance imaging of the brain showed an ill-defined, T2-hyperintense mass involving the right half of the tuber cinereum. A diagnosis of central precocious puberty due hypothalamic hamartoma was made. During the course of his admission, he was given injection leuprodex 3.75 mg intramuscularly once in 28 days. He was discharged with a plan to follow-up on outpatient basis.

Keywords: Central precocious puberty, Sexual precocity, hypothalamic hamartoma


How to cite this article:
Mahajan ZA, Mehta SR. Central precocious puberty: A case report. Med J DY Patil Vidyapeeth 2020;13:413-5

How to cite this URL:
Mahajan ZA, Mehta SR. Central precocious puberty: A case report. Med J DY Patil Vidyapeeth [serial online] 2020 [cited 2020 Aug 8];13:413-5. Available from: http://www.mjdrdypv.org/text.asp?2020/13/4/413/290177




  Introduction Top


Puberty is a time interval, characterized by the acquisition of the secondary sexual characteristics, accelerated linear growth, increase in the secretion of sex hormones, maturation of gonads, and the potential for reproduction.[1] The age of onset of puberty is considered 14 and 12 years in males and females, respectively.[2]

Precocious puberty is the onset of physical and hormonal features of pubertal development at an earlier age than expected.[3] The onset of puberty before the age of 9 years in males and 8 years in females is considered sexual precocity.[4]

The reason for choosing this case is the atypical presentation of hypothalamic hamartoma with precocious puberty instead of refractory seizures. Also, it has a low incidence, especially in males and it exhibits excellent physical examination and investigative findings.


  Case Report Top


A 2-year 9-month-old Indian male child was brought to the hospital with complaints of excessive growth of pubic hair and increase in penile length for 2 months. The patient's family denies any convulsion, visual disturbance, altered sensorium, vomiting, focal neurological deficit, fever, fatigue, intolerance to cold, head injury, central nervous system (CNS) infection, or space-occupying lesion. Past history, perinatal history and family history of the patient are not significant. The developmental milestones were normal. The anthropometry showed that his height and weight were in the category of 50th–97th centile, and his head circumference is between the 3rd and 50th centile.

On examination, his vitals were stable, and systemic examination was normal. On local examination of the genitalia, pubic hair were ++, his stretched penile length was 9 cm [Figure 1] and [Figure 2],[5] and testicular volume >4 mL [Figure 3].[5] The Sexual Maturity Rating score was A1P2T4. The patient does not have acne, axillary body hair or and café-au-lait spots. After initial assessment, following differentials were taken into the consideration: organic brain lesions, hypothalamic hamartoma, hydrocephalus and congenital adrenal hyperplasia, adrenal gland tumors, and familial male precocious puberty.
Figure 1: Original photo showing increased penile length and pubic hair

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Figure 2: Percentile curves of stretched penile length in boys 0–18 years

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Figure 3: Percentile curves of testicular volume in boys 0–18 years

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The laboratory investigations were as follows: complete blood count and renal function tests were normal. Serum testosterone levels were 592.76 (9–38 mmol/L), serum luteinizing hormone (LH) – 2.18 (1.24–7.8 IU/L), and serum follicle-stimulating hormone (FSH) – 4.69 (1.5–12.5 IU/L). X-ray of the left wrist showed bone age >6 years [Figure 4]. Magnetic resonance imaging (MRI) of the brain showed an ill-defined, nonenhancing, T2-hyperintense mass involving the right half of the tuber cinereum suggestive of hypothalamic hamartoma. The final diagnosis is a 2-year 9-month-old male child diagnosed with central precocious puberty due to hypothalamic hamartoma of the tuber cinereum. He was started on injection leuprodex 3.75 mg single dose intramuscularly once in 28 days, with a plan to follow-up with the assessment of height, weight, and secondary sexual characteristics on outpatient basis.
Figure 4: Original photo of X-ray left wrist showing bone age >6 years

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  Discussion Top


Epidemiology and classification

The overall incidence of sexual precocity is 1:5000–1:10,000 children.[4] The female:male ratio is approximately 10:1.[6]

Pathophysiology

Precocious puberty can be broadly classified into central and peripheral precocious puberty [Table 1].[4] Central precocious puberty is gonadotropin dependent since it is a consequence of over activation of the hypothalamic-pituitary axis, which ultimately results in the enlargement of gonads and development of secondary sexual characteristics in addition to spermatogenesis or oogenesis.[7]
Table 1: Classification of precocious puberty

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The high-amplitude pulses of GnRH cause pulsatile increase in the pituitary gonadotropins – LH and FSH. Increase in the FSH level contributes to the enlargement of the gonads in both sexes and eventually promote follicular maturation in girls and spermatogenesis in boys. Increased LH levels stimulate the production of sex steroids by testicular Leydig cells or ovarian granulosa cells.[3] The excessive estrogen or androgen causes penile enlargement and body hair in boys and breast development in girls, respectively.[2]

In contrast, peripheral precocious puberty is gonadotropin independent and is characterised by increased levels of estrogen or androgens that cause premature growth without the stimulation of hypothalamic-pituitary axis.[3]

Clinical features

In males, the condition typically presents with increase in penile length and excessive development of pubic, axillary and facial hair with or without testicular enlargement. In females, there is premature breast development and increase in body hair.[1] Some features such as development of acne and axillary body odor overlap in both the sexes. There is pubertal growth spurt in these children, which leads to premature epiphyseal closure ultimately culminating to short stature.

Investigations

All the patients who show the signs of precocious puberty should be investigated further using laboratory tests and radiological investigations. Tests that should be performed include serum FSH serum LH and serum testosterone levels.[7] Thyroid profile should be conducted to rule out the cases of severe hypothyroidism in suspected cases. An X-ray of the wrist joint may be done to estimate the current bone age of the child.[7] An MRI of the brain to investigate central causes and computed tomography of the abdomen and pelvis for peripheral causes of sexual precocity should be done when they are strongly suspected.

Treatment

The treatment of precocious puberty if often directed toward the cause. For central precocious puberty, excision of the lesion is the preferred management modality.[3] Surgery is recommended for refractory seizures. Radiation therapy should be considered for inoperable cases.[3] However, the treatment of the cause will not reverse the effects of sexual precocity.[3] Continuous administration of GnRH agonists provides negative feedback and results in the decreased levels of LH and FSH 2–4 weeks after initiating treatment since pituitary responds only to pulsatile GnRH.

Outcome and follow-up

The patient should be followed up with the assessments of height, weight, and secondary sexual characteristics, initially monthly for the first few months and thereafter after every 3 months. Bone age should be assessed using the wrist X-ray every 6 months to observe slowing of bone growth. Continuous LH-releasing hormone analog therapy is recommended to suppress the cyclical surge of LH and FSH to arrest the increasing penile length and testicular growth and preventing short stature due to premature epiphyseal closure. The long-term consequences of precocious puberty are short stature, early sexual activity and behavioral disturbances.[7]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mehta A, Hindmarsh P. BMJ Best Practice. London, UK: BMJ Best Practice; 2019. Available from: https://bestpractice.bmj.com/topics/en-gb/1127. [Last updated on 2019 Oct 20; Last accessed on 2019 Nov 23].  Back to cited text no. 1
    
2.
Merriam-Webster's Collegiate Dictionary. Springfield, MA: Merriam-Webster Incorporated. Available from: https://www.merriam-webster.com/dictionary/puberty. [Last accessed on 2019 Nov 22].  Back to cited text no. 2
    
3.
Kaplowitz PB. Precocious Puberty. New York, USA: Medscape. Available from: https://emedicine.medscape.com/article/924002-overview. [Last updated on 2018 Feb 14; Last accessed on 2019 Nov 21].  Back to cited text no. 3
    
4.
Mayo Clinic Staff. Precocious Puberty. Mayo Clinic, USA: Mayo Clinic. Available from: https://www.mayoclinic.org/diseases-conditions/precocious-puberty/symptoms-causes/syc-20351811. [Last updated on 2019 Apr 5; Last accessed on 2019 Nov 23].  Back to cited text no. 4
    
5.
Jaiswal VK, Khadilkar V, Khadilkar A, Lohiya N. Stretched penile length and testicular size from birth to 18 years in boys from western Maharashtra. Indian J Endocrinol Metab 2019;23:3-8.  Back to cited text no. 5
    
6.
Partsch CJ, Sippell WG. Pathogenesis and epidemiology of precocious puberty. Effects of exogenous oestrogens. Apmis 2001;109:S144-55.  Back to cited text no. 6
    
7.
Andrew Calabria. Precocious Puberty. Kenilworth, NJ, USA: MSD Manual Professional Version. Available from: https://www.msdmanuals.com/professional/pediatrics/endocrine-disorders-in-children/precocious-puberty. [Last updated on 2018 Oct16; Last accessed on 2019 Nov 20].  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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