Alexander K. C. Leung, MD

The mother of this 11-month-old girl was concerned that her infant’s vagina was almost obscured by a membrane. This was noted incidentally a few days earlier. The mother was sure that the membrane was not there in the immediate neonatal period. Urination was unimpaired, and the infant was asymptomatic. She had no history of trauma to the genital area. Her past health was unremarkable. In particular, she had no history of unexplained fever, foul-smelling urine, or an overt urinary tract infection.

On examination, the vulva was flat. The edges of the labia minora were sealed in the midline by a thin, translucent membrane that extended from the posterior fourchette to just below the clitoris.

Labial fusion refers to partial or complete adherence of the labia minora. The condition is rare at birth and occurs most commonly in girls between 3 months and 4 years of age. The peak incidence is between 13 and 23 months, during which about 3.3% of girls are affected.

Labial fusion probably develops after denudation of the superficial squamous epithelial layer of the labial mucosa. Denudation occurs with inflammatory conditions of the labia minora, such as vulvitis or vulvovaginitis, most often secondary to poor perineal hygiene. Estrogen deficiency is associated with a reduction in the thickness of the labial epithelial cells and is a possible pathogenic factor.

Most girls with labial fusion are asymptomatic. The condition is usually discovered incidentally by a physician during a routine examination or by the mother while she is bathing the child. Labial fusion may predispose the child to asymptomatic bacteriuria or to urinary tract infection. Occasionally, the labial fusion causes urinary outflow obstruction with resultant bladder distention and hydronephrosis.

Treatment consists of precise application of a topical estrogen cream to the fused area twice a day until lysis of the adhesion is complete. Most cases resolve within 2 to 3 months. As the labia minora separate, petroleum jelly should be applied to the edges to prevent readhesion.


 • Leung AK, Robson WL. Labial fusion. 

. 2004;3:31-35.
 • Leung AK, Robson WL, Kao CP, et al. Treatment of labial fusion with topical estrogen therapy. 

 (Phila). 2005;44:245-247.
 • Leung AK, Robson WL, Tay-Uyboco J. The incidence of labial fusion in children. 

Articles

The mother of this 11-month-old girl was concerned that her infant’s vagina was almost obscured by a membrane. This was noted incidentally a few days earlier.

Labial Fusion

A 4-year-old boy was brought for evaluation of a tiny cystic mass on the penis. The lesion was first noted a year earlier when the foreskin became retractable. At that time, the child was asymptomatic and the family did not seek medical attention. There was no history of urethral discharge or meatal ulceration. Urination was normal. Past health was unremarkable.

The cystic lesion was located at the 3 o'clock position on the glans penis near the urethral meatus and measured 2 mm in diameter. It was nontender. The cyst was excised. Histological examination showed that the cyst was lined by columnar epithelium. The postoperative course was uneventful. The patient was followed up for 2 years with no recurrence of the lesion.

The cause of parameatal urethral cysts is unclear. Congenital lesions are believed to be caused by faulty separation of the foreskin from the glans or occlusion of the parameatal duct. Acquired cysts may result from an infection.

The cysts are usually asymptomatic, although they can be itchy or painful. Large cysts and those in close proximity to the urethral meatus may cause deflection and spraying of the urinary stream.

Treatment consists of complete excision of the cyst. The lesion often recurs after spontaneous rupture or aspiration.


 • Koga S, Arakaki Y, Matsuoka M, Ohyama C. Parameatal urethral cysts of the glans penis. 

. 1990;65:101-103.
 • Onaran M, Tan MO, Camtosun A, et al. Parameatal cyst of urethra: a rare congenital anomaly. 

. 2006;38:273-274.
 • Yoshida K, Nakame Y, Negishi T. Parameatal urethral cysts. 

A 4-year-old boy was brought for evaluation of a tiny cystic mass on the penis. The lesion was first noted a year earlier when the foreskin became retractable.

Parameatal Urethral Cyst

The mass protruding from the introitus of this 1-month-old girl was first noted at birth. She was born to a 26-year-old primigravida at term after an uncomplicated pregnancy and normal spontaneous delivery. The mother was not taking any medications during the pregnancy. There was no history of birth trauma.

The infant was not in distress. The flesh-colored smooth-surfaced lesion protruding from the vagina was characteristic of a hymenal tag. The rest of the examination findings were unremarkable.

Hymenal tags are present in 3% to 13% of normal female newborns. The tags are commonly located in the superior and inferior positions and tend to resolve spontaneously. New hymenal tags may appear postnatally as a result of extension of an intravaginal or external hymenal ridge.

The parents were reassured of the benign nature of the lesion and that it will resolve with time.


 • Berenson AB, Grady JJ. A longitudinal study of hymenal development from 3 to 9 years of age. 

. 2002;140:600-607.
 • Heger AH, Ticson L, Guerra L, et al. Appearance of the genitalia in girls selected for nonabuse: review of hymenal morphology and nonspecific findings.

The mass protruding from the introitus of this 1-month-old girl was first noted at birth. She was born to a 26-year-old primigravida at term after an uncomplicated pregnancy and normal spontaneous delivery.

Hymenal Tag

A 6-year-old boy presented with a swollen penis and lip within an hour after ingesting some peanuts. The swelling was nonpruritic. The child described a sense of tightness in the lips. He had no associated rash, abdominal pain, dysphagia, stridor, dyspnea, dysuria, or abnormality in the urinary stream. He was not taking any medications. He had no history of a recent viral infection, insect bite or sting, oral or genital trauma, or blood transfusion. There was no family history of asthma, atopic dermatitis, allergic rhinitis, or angioedema.

Examination revealed no signs of an allergic reaction other than the angioedema of the lips and penis. The foreskin could be retracted, and the urethral meatus was normal. The child was treated with hydroxyzine, 10 mg orally 4 times a day. The angioedema subsided within 72 hours.

Angioedema is most commonly caused by a type I-anaphylactic or IgE-mediated-immediate hypersensitivity reaction to food (notably, peanuts, cow’s milk, eggs, chocolate, or seafood), drugs (notably, antibiotics, opiates, or sedatives), stinging insect venom, preservative, latex product, or aeroallergen. Angio edema may also be a consequence of a type II cytotoxic reaction (transfusion reaction) or a type III antigen-antibody complex reaction (serum sickness). Vibratory angioedema and exercise-induced angioedema result from hypersensitivity to a mechanical or physical factor. Hereditary angioedema is an autosomal dominant disorder associated with the absence or dysfunction of C1 inhibitor.


 • Estrada JL, Gozalo F, Cecchini C, Casquete E. Contact urticaria from hops (

) in a patient with previous urticaria-angioedema from peanut, chestnut and banana. 

. 2002;46:127. • Leung AK, Robson WL. Penile and oral angioedema associated with peanut ingestion. 

. 2006;98:2011-2012.
 • Smith GA, Sharma V, Knapp JF, Shields BJ. The summer penile syndrome: seasonal acute hypersensitivity reaction caused by chigger bites on the penis. 

A 6-year-old boy presented with a swollen penis and lip within an hour after ingesting some peanuts.

Penile Angioedema After Peanut Ingestion

A 7-year-old boy was brought for evaluation of a rash on the chin that had appeared 2 weeks earlier. It was slightly itchy. The mother had applied a topical corticosteroid for a few days but then stopped because of worsening of the lesion.

The erythematous, scaly lesion with a well-defined border on this boy's face is characteristic of tinea faciei. As the lesion spreads peripherally, the center often clears and produces the classic annular lesion that is responsible for the designation of ringworm. Microscopic examination of a potassium hydroxide preparation of scrapings from the lesion showed numerous septate hyphae. This confirmed a dermatophyte infection; culture can also establish the diagnosis. Scrapings from scales at the edge of the lesion produce the best results.

Trichophyton rubrum, Trichophyton mentagrophytes, Trichophyton tonsurans

 are the most prevalent causative organisms.

 Occasionally, tinea faciei may simulate polymorphous light eruption, seborrheic dermatitis, contact dermatitis, impetigo, discoid lupus erythematosus, and acne rosacea.

Tinea faciei usually responds to application of a topical antifungal agent, such as micronazole, ketoconazole, econazole, naftifine, or terbinafine. In difficult-to-treat cases, oral itraconazole or fluconazole can be considered. This child was treated with topical terbinafine. The lesion resolved within 10 days.

 Criado PR, Costa AR, Vasconcellos C, et al. Tinea faciei in an infant caused by 

 Gorani A, Oriani A, Cambiaghi S. Seborrheic dermatitis-like tinea faciei. 

Tinea Faciei

Premature Adrenarche
A 7-year-old girl had growth of pubic hair for the past 6 months. The hair was initially limited to the labia majora and then extended gradually into the pubic area. She grew 8 cm (3.1 in) in the past year. Her health history was unremarkable; she was not taking any medications. On examination, her weight was 35 kg (77.2 lb); height, 125 cm (49.2 in). She did not have breast development, axillary hair, acne, or clitoral enlargement.

Premature or precocious adrenarche refers to an   earlier than normal secretion of adrenal androgens that   results in isolated development of pubic hair (pubarche)   before age 8 years in girls and 9 years in boys, without   the appearance of other signs of sexual maturation. It affects   about 0.8% of white girls aged 7 to 7.9 years. The   condition is more common in African American children.   The female to male ratio is approximately 10:1.

Premature adrenarche is secondary to an early isolated   maturation of the zona reticularis, with increased adrenal   androgen secretion for chronological age but with   normal glucocorticoid levels. Another suggested mechanism   is increased sensitivity of the sexual hair follicles   to androgens, because in some patients, premature pubarche   is associated with normal androgen levels.

Precocious puberty can be differentiated from premature   adrenarche by the concomitant appearance of   pubic hair with breast development in girls and with testicular   enlargement in boys. Other differential diagnoses   include virilization caused by congenital adrenal hyperplasia   and an adrenocortical or gonadal tumor. In premature   adrenarche, serum concentrations of dehydroepiandrosterone,   dehydroepiandrosterone sulfate (DHEAS),   androstenedione, and testosterone and urinary 17-ketosteroids   are usually increased for chronological age and   in the range of those found in early puberty. The bone   age is usually within 2 standard deviations of the chronological   age. Moderately elevated levels of serum androgen   other than DHEAS, bone age advancement, or signs   of atypical premature pubarche (such as cystic acne or   symptoms of systemic virilization) indicate the need for   a corticotropin test to rule out late-onset congenital adrenal   hyperplasia. Marked elevation of serum androgen   levels and advanced bone age suggest the possibility of   an adrenocortical or gonadal tumor.

Education and reassurance of the patient and family   along with psychological/emotional support are essential   to clinical management of premature adrenarche. Continued   observation and periodic reevaluation are necessary   because premature adrenarche might be the first sign   of precocious puberty. Premature adrenarche in girls is   associated with a higher incidence of polycystic ovary   syndrome later in life. In some girls, premature adrenarche   may be a forerunner of polycystic ovary syndrome   or syndrome X (obesity, hypertension, insulin resistance,   type 2 diabetes mellitus, and dyslipidemia). Affected   patients are at increased risk for early atherosclerotic   cardiovascular disease. In contrast, premature adrenarche   in boys is not associated with an increased incidence   of endocrine or metabolic abnormalities.

Girls with higher body mass index warrant particularly   close follow-up. Early identification of these patients   can allow for earlier treatment of the associated   conditions with a reduction in risk of early cardiovascular   disease.


• Andiran N, Yordam N, Kirazli S. Global fibrinolytic capacity is decreased in girls with premature adrenarche: a new pathological finding? 

 2005;18:1373-1381.
• Leung AK, Robson WL. Premature adrenarche.

 2008; 22:230-233.
• Saenger P, DiMartino-Nardi J. Premature adrenarche.

Isolated Scrotal Hair of Infancy
This infant was born at term to a 32-year-old gravida 2 para 3 after a normal vaginal delivery. It was a twin pregnancy that was otherwise uncomplicated. The mother had not taken any medications during the pregnancy and had no virilization or hirsutism. The parents were nonconsanguineous. There was no family history of congenital adrenal hyperplasia. The baby’s birth weight and length were 2784 g (6.1 lb) and 55 cm (21.7 in), respectively. Scrotal hair developed at age 1 month. The hair growth gradually progressed over the next 2 to 3 months. Physical examination at 4 months revealed curly, black terminal hair on the scrotum. The testes and penis were normal size. Levels of serum electrolytes and 17-hydroxyprogesterone were normal. The serum testosterone level was 1.8 nmol/L. Findings from an abdominal ultrasonographic examination were normal. The other identical twin also had scrotal hair first noted at 1 month of age and had a similar unremarkable clinical course.

Isolated genital hair of infancy   presumably results from transiently   elevated androgen levels in the first   few months of life and an increased   sensitivity of sexual hair follicles   to androgens. Shortly after birth, a   transient surge of gonadotropins   occurs and leads to a sharp increase   in testosterone levels, which   peaks at 1 to 3 months of age (2.8   to 13.9 nmol/L). Thereafter, the   gonadotropin levels fall; by age 6   months, serum testosterone levels   decrease to the low prepubertal levels   (0.3 to 0.7 nmol/L) that persist   until the beginning of puberty. In   isolated genital hair of infancy, sexual   hair usually occurs in an atypical   location, such as the scrotum in   boys and the mons pubis in girls. The growth of hair is slowly progressive in the first few   months of life, remains stationary for a few more months, and then might regress.

The development of sexual hair in infancy can be the first sign of virilization, which may   result from congenital adrenal hyperplasia, an adrenal tumor, or a gonadal tumor. In such cases,   evidence of excessive androgen production (eg, acne, phallic enlargement, hirsutism, and   markedly accelerated growth) usually appears at the same time as or shortly after the onset of   sexual hair development. In boys with congenital adrenal hyperplasia, the testes remain infantile   but other secondary sexual characteristics are advanced. In boys with an adrenal tumor, the   testes are usually small, whereas the reverse is true for boys with a testicular tumor.


• Leung AK, Hegde HR, Stephure DK. Scrotal hair in identical twin infants. 

 2005;44:1042-1044.
• Papadimitriou A, Beri D, Nicolaidou P. Isolated scrotal hair in infancy. 

Premature Adrenarche: A 7-year-old girl had growth of pubic hair for the past 6months. The hair was initially limited to the labia majoraand then extended gradually into the pubic area. Isolated Scrotal Hair of Infancy: Infant was born at term to a 32-year-old gravida 2 para 3 after a normal vaginal delivery. Scrotal hair developedat age 1 month.