The newborn girl is affected by circulating maternal estrogens; thus, it is common to see whitish, clear mucoid, or occasionally bloody discharge from the vagina. Transient discharge should subside after approximately 2 weeks as maternal estrogen levels in the neonate decline, so only reassurance is required. Initiate an evaluation if discharge or bleeding persists beyond this time period. (See Chapter 103, Vaginitis.) Disorders of infancy and childhood are listed below. In addition, Table 99-2 describes causes of prepubertal vaginal bleeding.
TABLE 99-2Differential Diagnosis of Prepubertal Vaginal Bleeding |Favorite Table|Download (.pdf) TABLE 99-2 Differential Diagnosis of Prepubertal Vaginal Bleeding
Visible lesion or mass present
Traumatic injury including sexual assault, straddle injury
Genital warts or ulcers
No visible lesion or mass
Vaginal foreign body
Traumatic injury including sexual assault, straddle injury
Exogenous hormone withdrawal
Congenital Vaginal Obstruction
The two most common types are due to an imperforate hymen or a transverse vaginal septum, also known as vaginal atresia. A transverse vaginal septum is thought to result from failure in canalization of the vaginal plate at various levels. An imperforate hymen is a remnant of the urogenital membrane. Other lesions, such as cloacal malformation and persistent urogenital sinus, result from an interruption of the normal differentiation of the hindgut. The vaginal plate and sinovaginal bulbs subsequently do not develop and the upper vagina and rectum enter the urogenital sinus.5,6 These lesions sometimes lead to functional obstruction of normal mucoid secretions and cause hydrocolpos, distension of the vagina (colpos), or hydrometrocolpos, distension of the uterus (metro) and vagina.7,8
Congenital vaginal obstruction can present at variable times during infancy and childhood. Hydrocolpos in the newborn period may present as an abdominal mass or a bulging mass at the introitus.2,7,8 On rare occasions, vaginal obstruction can lead to recurrent urinary tract infections, obstructive uropathy, and renal failure secondary to compression by the mass. Rarely, an abscess can occur and requires emergent drainage.7,8 An undiagnosed imperforate hymen may present at puberty as primary amenorrhea and intermittent lower abdominal pain that worsens every month. On physical examination, a bluish bulge, also known as hematocolpos, may be seen at the introitus.
Treatment of vaginal atresia and imperforate hymen is surgical correction. Surgical drainage of hydrocolpos or hydrometrocolpos is necessary to relieve the obstruction, but not imperative in the newborn period as it is best to delay definitive reconstruction until the adolescent years.7,8
Labial adhesions, also known as labial agglutination, usually begin posteriorly and extend superiorly toward the clitoris, often leaving a small opening anteriorly. There is usually a thin white line or demarcation that represents the fused portion.1 The prevalence is 1.8% to 3.3% in 1- to 6-year age group, with a peak incidence in the 13- to 23-month age group.9,10 A suggested etiology is an estrogen deficiency in the prepubertal period and inflammation that results in thinning of the superficial mucosal layers.1
The clinical presentation is often related to a parent noting that the vaginal area appears to be “closing” or found during routine physical examination by a clinician. Congenital absence of the vagina or imperforate hymen can be distinguished from adhesions by the thin raphe or vertical line connecting labial adhesions.9,10
Most cases of labial adhesions are self-resolving warranting reassurance, especially in asymptomatic cases. With symptoms such as pain, inflammation, or urinary tract infections, first-line therapy is topical estrogen cream applied to the fine thin raphe twice a day for 2 to 8 weeks. Assess for breast budding as some local cream application may result in peripheral stimulation. Alternatively, a topical steroid cream, such as betamethasone has led to equivalent results.9 Repeat treatment for a recurrence.1 Surgical separation in the operating room or office may be indicated if symptoms persist, recurrent urinary tract infections occur or an inability to void due to complete agglutination.9,10 Manual separation is not recommended because of the risk of recurrence and potential for psychological trauma with repetitive manual separation.9,10
Urethral prolapse is protrusion of urethral mucosa through the urethral meatus. Although uncommon, it tends to occur more often in prepubescent African Americans girls.11,12
On examination, the prepubertal vaginal orifice may be obscured and a ring of congested and edematous urethral tissue may be seen at the introitus. The urethral mucosa typically appears red or purple, and some areas may appear black and necrotic. Some children may present with dysuria or vaginal bleeding.2,11,12
Treatment is with topical estrogen cream applied to the area twice daily until resolution, while monitoring for signs of peripheral estrogen stimulation. Resolution may occur in one to several weeks. A sitz bath may also be helpful. If the child is constipated, recommend additional fiber in the diet and stool softeners to prevent straining with defecation. Other treatments include topical steroid or antibiotic cream. Oral antibiotics may be considered for evidence of superimposed infection. Medical treatment failures or suspicion for urethral incarceration warrants surgical excision of the redundant tissue.11,12
Precocious puberty is defined as the onset of secondary sexual characteristics that is greater than 2.5 standard deviations below the mean age of pubertal onset for the population. In a study of more than 17,000 girls aged 3 to 12 years by the Pediatric Researchers in Office Setting (PROS) investigators noted that the normal age for the onset of puberty is younger than previously believed and varies with regard to race (e.g., African American girls can have an onset of puberty as early as 6 years and Caucasian girls at the age of 7).3,4 Most physicians, however, still use an 8-year age range as the cut-off pending the outcome of other prospective studies.13
Precocious puberty is often described as either gonadotropin-dependent precocious puberty (GDPP) or gonadotropin-independent precocious puberty (GIPP) (Fig. 99-2). GDPP occurs secondary to activation of the hypothalamic–pituitary–gonadal axis, and GIPP occurs secondary to steroid production, regardless of gonadotropin secretion. Some young girls may present with isolated menarche, thelarche, or pubarche as a variant of normal puberty.13 Clinical symptoms will relate to the particular disease process. For example, girls with GDPP, also known as central precocious puberty, may present with pubertal changes such as vaginal bleeding, breast and pubic hair growth, acne as well as neurologic abnormalities such as headaches, seizures, focal deficits, polyuria, polydipsia, vision changes, or galactorrhea.
Algorithm of precocious puberty.
Girls with GIPP, or peripheral precocious puberty, may present with thyroid and abdominal or ovarian masses. There may be a history of estrogen cream use, which has propagated precocious development. Skin findings such as acne, or café au lait spots can be consistent with genetic conditions, such as McCune–Albright syndrome.13
In the ED setting, determine whether a disease process requires immediate attention warranting referral to a pediatrician or a pediatric endocrinologist. Initiate an evaluation if there is a suspicion for trauma, malignant neoplasm, or CNS abnormality. If history reveals access to or use of hormonal medications, stopping the medications should cause symptom resolution.13
Typically, ovarian cysts result from the mini-pubertal period that neonates experience in the first 6 months of life and most resolve spontaneously. Cysts of any size in the neonatal period should be closely observed due to risk of ovarian torsion.14 Beyond 6 months of age, cysts may also occur and should be followed to resolution.15 Although ultrasounds are very sensitive, occasionally they cannot distinguish between duplication cysts of the intestine, mega ureter, or mullerian anomaly. Cysts may have an appearance that is simple (typically functional and self-resolving) or complex (e.g., sign of an old hemorrhagic cyst or of an ovarian tumor). The most common tumors presenting in this age group are germ cell tumors.
In general, asymptomatic simple cysts, which are resolving, may be observed. Surgical management is indicated for any type of cyst, which persists or increases in size, and for complex cysts if tumor is suspected. Immediate surgical evaluation is necessary for symptoms of torsion as even small cysts may result in ovarian torsion. Hormone-producing cysts should be considered in girls with signs of precocious puberty.14–17