Surgical consultation may be necessary for management of prepubertal genital bleeding. The differential diagnosis is broad, and includes more common vulvar dermatoses and bacterial infection, which typically respond to medical management, and less common etiologies such as trauma and malignancies, which require prompt surgical evaluation and management ( Fig. 70.3 ). Sexual abuse should also be considered, with a recognition of the complexity of the patient disclosure process, and that physical evidence of abuse is not often found even when children are examined acutely (reported sexual abuse occurred within the previous 72 hours). ^,

Potential etiologies of prepubertal bleeding with broad differential diagnosis ranging from common and benign to uncommon and worrisome diagnoses.

From Bloomfield V, Iseyemi A, Kives S. Clinical review: prepubertal bleeding. J Pediatr Adolesc Gynecol . 2023.

Uterine bleeding occurs in 5% of neonates in the immediate few weeks after birth, as a result of withdrawal of placental progesterone after in utero exposure to maternal estrogens. While surgical intervention is not required, debate remains about the clinical significance of neonatal uterine bleeding—it has been reported as a potential indicator of fetal distress and a potential biomarker of reproductive disorders (such as endometriosis) during adolescence. ^,

Pediatric anogenital lichen sclerosus is a chronic inflammatory skin condition with typical symptoms of vulvar itching or pain, painful defecation, and holding urine due to fear of dysuria. Physical findings usually include hypopigmentation of the anogenital area in a figure-of-eight pattern involving the clitoral hood, perineal and perianal skin. Repeated excoriations can lead to fissures and ecchymosis and associated genital bleeding ( Figs. 70.4 and 70.5 ). Initiation and maintenance medical treatment with topical ultrahigh potency corticosteroid ointment, or alternatively, immune modulators, generally leads to resolution of symptoms. Emerging evidence suggests that compliance with treatment is associated with a decreased risk of developing vulvar structural abnormalities and clitoral phimosis. ^,

Lichen sclerosus diagnosed in a Black prepubertal child. A sharp demarcation of hypopigmented in a figure-of-eight pattern is seen, with involvement of the clitoral hood. Perianal fissure may be associated with bleeding.

Courtesy of Dr. Kaiane Habeshian.

Lichen sclerosus diagnosed in a White prepubertal child. The ecchymosis noted along the medial aspect of the left labia was associated with bleeding.

Courtesy of Dr. Kaiane Habeshian.

Urethral prolapse occurs when the distal urethral mucosa protrudes through the urethral meatus. It typically presents as painless spotting or bleeding. Risk factors include obesity, Black race, and constipation. ^, On examination, a donut-shaped intralabial mass is noted, and it may appear friable or necrotic as a result of vascular congestion and strangulation ( Fig. 70.6 ). Urethral prolapse often resolves with application of topical estrogen. Surgical resection can be considered for severe symptoms, persistence despite medical management, or evidence of urethral necrosis. ^,

Urethral prolapse was found in this 6-year-old child with a history of recurrent, painless genital bleeding. The urethral mucosa has prolapsed and appears ischemic. The prolapse resolved following treatment with an estrogen cream.

Genital trauma can cause significant distress to children and their families due to concern for long-term sexual and reproductive sequelae. If nonaccidental genital trauma and/or sexual abuse are suspected, prompt collaboration with clinicians specializing in child protection is important in diagnosis and interpretation of exam findings. Sexual abuse and accidental falls onto sharp or pointed objects such as in-lawn sprinkler systems, fence posts, or bedposts can result in penetrating injuries and associated internal hemorrhage and hematoma formation. Targeted imaging, vaginoscopy, laparoscopy and/or laparotomy, and urological and gynecological consultation should be considered to determine the full extent of injuries and initiate repair if injury to the urinary tract, vagina, rectum, or peritoneal cavity are suspected, or if the child is not hemodynamically stable. ^{, ,}

Blunt genital trauma can also occur accidentally during childhood falls when the external genitalia strike blunt objects such as bicycle crossbars, pool ledges, or bathtubs. Urogenital tissues are compressed against the bony pelvis, resulting in injuries involving the mons, clitoris, labia, and/or perineum and typically sparing the hymen and vagina. Bleeding is the most common presenting symptom and prompts a thorough evaluation of the location and severity of the injury, including linear lacerations, abrasions, ecchymoses, and hematoma. In some pediatric emergency departments, the availability of mild and moderate sedation with nitrous oxide via mask and intranasal midazolam and fentanyl is useful for visualization of relatively minor injuries, especially in older children. At other institutions, intravenous anesthetics such as ketamine administered by pediatric emergency clinicians facilitates a thorough genital examination, vaginoscopy and repair of genital lacerations in the emergency department. Examination and repair under anesthesia in the operating room is necessary when adequate procedural sedation is unavailable in the emergency department and with larger lacerations including those extending deep into the vaginal canal. ^, Vaginoscopy is necessary when the source of bleeding is not readily apparent or the injury extends into the vagina and/or cannot be completely visualized.

Injury to the labia can cause significant vulvar hematomas. In the absence of acute ongoing hemorrhage or urinary retention, small or moderate hematomas can be managed conservatively with compression, ice, and pain control. Surgical exploration under general anesthesia is necessary for large hematomas with acute expansion, severe pain, and overlying pressure necrosis of the skin. ^, An incision is made over the area of maximum bulging at the mucocutaneous junction or vaginal epithelium, if visible. Clot is evacuated to facilitate visualization of the depth of the hematoma and ligation of bleeding vessels, followed by primary closure with reduction of dead space and placement of a drain, which is removed 24 hours later ( Figs. 70.7 and 70.8 ). ^, The presence of large hematomas in the face of minor trauma should alert surgeons to the possibility of a coagulation disorder ( Fig. 70.9 ).

Ruptured right vulvar hematoma in a 14-year-old female.

After evacuation of blood clots, primary closure with placement of a drain in a dependent portion of the hematoma.

An extensive vulvar hematoma is seen after a straddle injury. The patient was subsequently diagnosed with Factor VII deficiency.

Prepubertal vaginal bleeding accompanied by foul-smelling discharge is likely caused by a vaginal foreign body. Toilet paper is the most common foreign body, although small toys and batteries have been described. ^, Vaginal irrigation with the assistance of a CLS can result in immediate removal of toilet paper. After application of a topical anesthetic to the hymen and vaginal introitus, a small pediatric Foley catheter or nasogastric tube can be inserted into the vagina and the catheter then flushed with saline. Alternatively, a handheld endoscopy device can be used that allows for visualization of the entire vaginal canal. ^, Sedation or anesthesia may be necessary for young children or those who cannot tolerate the procedure with a topical anesthetic alone. Urgent vaginoscopy should be performed if there is concern for a caustic object, such as a button battery, which can lead to mucosal burning, stricture, or fistula formation. ^,

Several benign, albeit rare, neoplasms have been described as causing prepubertal vaginal spotting and bleeding. Cervicovaginal Mullerian papillomas appear as frond-like, polypoid lesions of the cervix and vagina during vaginoscopy and have been described in children (mean age of 5 years) with intermittent vaginal bleeding. These tumors are diagnosed via biopsy, followed by resection to control symptoms. Careful surveillance is recommended due to the possibility of malignant transformation.

Vaginal malignancies include rhabdomyosarcoma (RMS), endodermal sinus tumor, and malignant germ-cell tumor (GCT). These are rare and can present as prepubertal vaginal bleeding and/or a protruding vaginal mass ( Fig. 70.10 ). Most are diagnosed in children less than 5 years old and tissue biopsy is necessary for diagnosis. The treatment of vaginal RMS is a multimodal approach that preserves organ function, and includes intensive chemotherapy and local tumor reduction, reserving radiotherapy for selected children. ^, Management of malignant GCT after initial biopsy with multidrug cisplatin-based chemotherapy is associated with improved outcomes. ^{, ,}

This infant was found to have a mass protruding from her vagina that was an embryonic rhabdomyosarcoma (sarcoma botryoides). The mass was attached to the wall of the vagina and was able to be completely excised.

Imperforate hymen occurs in 0.05%–1% of the female population due to failure of canalization of the urogenital sinus. Newborns may present with a hydrocolpos caused by accumulation of cervical and vaginal secretions ( Fig. 70.11 ). Given the risk of reclosure and subsequent ascending infection, in the absence of symptoms or hydronephrosis, repair is generally delayed until after the onset of puberty and estrogenization of the genital tissue. More commonly, adolescents present with amenorrhea and progressively worsening episodes of abdominal pain as menstrual blood collects in the vagina and uterus leading to the development of a hematometrocolpos. A palpable abdominal mass and associated urinary retention, constipation, and in severe cases, renal failure have also been reported as presenting symptoms. ^{, ,} Transabdominal pelvic ultrasound is notable for a distended vagina and uterus. External genital examination reveals a bulging imperforate hymen with classical blue coloration ( Fig. 70.12 ). Needle aspiration should not be performed due to the potential development of a pyocolpos and ascending infection. Surgical excision of the hymen results in decompression of the uterus and vagina and resolution of associated symptoms. An incision is made within the hymenal tissue inferior to the urethra, excess hymenal tissue is resected, and the vaginal mucosa is sutured to the introital edge with interrupted absorbable sutures to avoid stenosis ( Fig. 70.13 ). A Foley catheter is sometimes placed. ^,

Mucocolpos, leading to urinary obstruction in a 2-day-old infant.

(A) Imperforate hymen in a 14-year-old girl with a 6-month history of recurrent abdominal pain. (B) Evacuation of the menstrual obstruction at hymenotomy.

(A) Imperforate hymen. (B) A cruciate incision is made in the apex of the hymen after identifying the outer hymenal borders. (C) The hymenal remnants are trimmed. (D) Interrupted sutures are utilized for hemostasis.

Failure of canalization of the junction between the urogenital sinus and the Mullerian tubercle results in a transverse vaginal septum (TVS) ( Fig. 70.14 ). TVSs are located at various levels above the hymen: upper third in about 45%, middle third in 30%–45%, and lower third in 15%–20%. ^, Most TVS are less than 1-cm thick and are imperforate or microperforate. As with an imperforate hymen, symptoms of an imperforate TVS after puberty include primary amenorrhea with associated abdominal pain, abdominal tenderness, or mass. However, examination of the external genitalia reveals a normal hymen with no intralabial bulge. The diagnosis may be more difficult to make with a microperforate TVS, which typically allows for egress of some menstrual blood and presents with severe dysmenorrhea. Examination under anesthesia and/or vaginoscopy under anesthesia may be required for visualization of the septum. While ultrasound reveals hematocolpos and/or hematometra, MRI remains the gold standard for imaging TVS and aids in preoperatively defining the level and thickness of the septum. Surgery includes excision of the septum with anastomosis of the proximal and distal vaginal mucosa. Various surgical techniques have been described including vaginal, laparoscopic, and abdominoperineal approaches. Preoperative vaginal dilatation can be used to lengthen the lower vaginal segment and facilitate resection and reanastomosis. For resection of a TVS more than 2-cm thick, an intestinal segment or skin graft may be required to bridge the gap between the upper and lower vagina. The placement of a vaginal stent following vaginal mucosal anastomosis may decrease the risk of circumferential stenosis (common at the anastomotic site), particularly with high or thick septae. A vaginal stent can be created by gauze placed in a sterile condom and coated with topical estradiol cream. It is left in place for 3–7 days along with an indwelling urinary catheter, antibiotics, deep vein thrombosis prophylaxis, and pain control. Postoperative dilations might be necessary, as the risk of vaginal stenosis remains high. Menstrual suppression and delay of surgical repair is an option for adolescents who are not sufficiently mature or prepared to perform postoperative dilation.

The transverse vaginal septum is the result of failed unification or canalization of the urogenital sinus and the Müllerian duct. It can arise anywhere in the vagina but is most common in the upper vagina.

Failure of the Mullerian ducts to make contact with the urogenital sinus results in vaginal agenesis. The uterus and vagina are intact in both vaginal agenesis and TVS, but the lower vagina is absent in the former. If not apparent with a hydrometra at birth, presentation occurs after puberty with primary amenorrhea and hematometrocolpos. MRI will depict the distance from the normal-appearing hymen to the distal end of the upper vagina by placement of Vitamin E capsule or another marker at the introitus. Repair requires the surgical creation of a functional neovagina with anastomosis to the upper vagina. In general, a vaginal pull-through procedure is possible with a distance of less than 3 cm from the perineum to the distal end of the upper vagina. Dissection in the fibrous tissue between the vesicovaginal and rectovaginal spaces facilitates localization of the bulging proximal vagina. The vagina is then entered to drain the collected menstrual blood, mobilized, and sutured to the introitus. ^, A graft is usually necessary to prevent stricture if the distance from the proximal vagina to the perineum exceeds 3 cm. Skin grafts, colon segments, and buccal mucosa are most commonly used. The use of amnion, peritoneum, absorbable adhesion barriers, and tissue-engineered biomaterial mesh has also been reported. ^, As with TVS, postoperative dilatations are typically necessary and menstrual suppression is an option, with delay of surgical repair until the patient is ready to undergo surgical management. Image-guided drainage by interventional radiologists for symptomatic hematometrocolpos can also be considered for continuing pain as a result of failure and/or lack of tolerance of hormonal menstrual suppression. ^,

Although extremely rare, cervicovaginal agenesis has been reported in individuals who present with primary amenorrhea, abdominal pain, and an abdominal mass ( Fig. 70.15 ). Hysterectomy with vaginoplasty is typically recommended because attempts at uterovaginal anastomosis have resulted in sepsis and death in the past. However, recently there have been a few reports of successful cervical canalization with vaginal neovaginoplasty. ^,

A sonographic sagittal view of the pelvis of a 16-year-old girl with cervicovaginal agenesis and a resultant hematometra. Note the dilated vagina and uterus ( UT ).

In menstruating individuals who develop severe dysmenorrhea, the diagnosis of a partially obstructed Müllerian tract should be considered. This most commonly occurs in individuals with an obstructed hemivagina due to a longitudinal vaginal septum with a complete duplication of the uterus and cervix (uterine didelphys) as well as upper vagina. Associated anomalies of the urinary system are common including ipsilateral renal agenesis, renal dysgenesis, pelvic kidney, or ectopic ureter. These individuals are usually older at diagnosis and present with progressively worsening severe dysmenorrhea with normal menses (from the nonobstructed side). Digital vaginal exam reveals a lateral bulge in the vagina. An MRI including visualization of the kidney(s) leads to the correct diagnosis ( Fig. 70.16 ). ^, Excision of the vaginal septum via a vaginal approach is performed at diagnosis.

MRI image of a 16-year-old girl with a complete uterine duplication and a blind right hemivagina. Note the obstructed and dilated right hemivagina ( asterisk ) and the resultant right-sided hematocolpos and hematometra.

Uterine duplication can also be associated with unilateral cervical agenesis and an obstructive functional uterine horn. MRI assists with delineation of anatomy and surgical planning of a laparoscopic hemihysterectomy of the obstructed side. ^,

Complete agenesis of the uterus, cervix, and upper vagina occurs in approximately 1 in 4500–5000 females and is also referred to as Mullerian agenesis or Mayer–Rokitansky–Küster–Hauser syndrome (MRKH). Up to 53% of patients have associated urologic and skeletal anomalies. Ovarian function is normal. The establishment of a pubertal timeline is important and MRKH is suspected when the normal onset of pubertal development (thelarche) is not followed by menarche within 3–4 years. Other individuals present with dyspareunia or the inability to participate in coitus. On physical exam, normal breast development, external genitalia, and hymen with no vaginal opening are found. MRI of the internal genitalia is the optimal diagnostic tool. It reveals normal ovaries and an absent or rudimentary uterus. Imaging of the kidneys with ultrasound or MRI should be performed, due to the prevalence of renal anomalies (in approximately 30%). ^, Chromosomal analysis is necessary to confirm a normal 46,XX karyotype as the differential diagnosis includes females with androgen insensitivity syndrome who have a 46,XY karyotype. Though not necessary for the diagnosis, chromosomal microarray may reveal copy number variants in individuals with isolated or syndromic MRKH. ^, Management in adolescents includes adequate psychosocial support and the creation of a functional neovagina when the patient is sufficiently mature and desirous of vaginal intercourse. The most commonly used nonsurgical method is self-dilation with the use of vaginal dilators ( Fig. 70.17 ). Dilation provides a vagina with normal mucosa, which may be advantageous should the patient pursue uterine transplantation (currently experimental) for future fertility. Sexual satisfaction after vaginal dilation is comparable with the normal population.

Sequential vaginal dilators are often used to create a neovagina in individuals with vaginal agenesis.

Primary vaginal dilation is successful in 90%–96% of individuals. Vaginoplasty is reserved for select individuals who are unsuccessful with vaginal dilation. Individuals who prefer surgical reconstruction should have comprehensive counseling to explain that postsurgical vaginal dilatation is still necessary for prevention of vaginal stenosis. Several vaginoplasty techniques have been reported, all of which are associated with unique immediate and long-term complications. The McIndoe procedure involves using split-thickness skin grafts from the buttock, thigh, or groin ( Fig. 70.18 ). Skin grafts result in external scars and, depending on the site of skin harvest, symptomatic hair growth, which may require laser treatment. Skin grafts do not provide lubrication and require ongoing dilation or regular coitus to prevent stricture. Segments of intestine, most commonly the sigmoid colon, have been used to create a neovagina. While this method decreases the need for dilation to prevent stenosis at sites of anastomosis, the development of inflammatory bowel disease has been described, and up to 38% report bothersome excessive vaginal discharge. Buccal mucosal grafts have also been used. Though not associated with external graft scars, ongoing vaginal dilation or intercourse are necessary for prevention of stricture. Other surgical procedures including the Davydov (mobilization of the peritoneum) and the laparoscopic Vecchietti procedure (creation of a neovagina with external traction temporarily anchored to the abdominal wall) have also been described. Currently, there is no consensus on the surgical approach that results in optimal sexual function and satisfaction.

Creation of a neovagina by using a split-thickness skin graft is shown in a teenager with vaginal agenesis. A urinary catheter has been inserted into the urethra. In (A), the perineal dissection has begun. The vaginal mold has been created and has been covered by the split-thickness skin graft (B). The mold has been inserted into the newly created vaginal space (C).

The operative approach to ovarian tumors in the pediatric population must balance preservation of reproductive potential with the risk of malignancy. The malignancy rate of ovarian masses in children is about 10% but significant overlap exists in the clinical presentation of benign and malignant masses. ^, Preoperative risk stratification is useful in determining surgical approach (e.g., ovarian-sparing surgery [cystectomy] versus oophorectomy). Following unilateral oophorectomy, gonadal function, as estimated by menstrual regularity, is not impaired but primary ovarian insufficiency and an earlier age of menopause has been reported. Ovarian-sparing surgery is generally the first choice, with the option of a second procedure in the event of unexpected malignancy on permanent histopathology. Several risk stratification models have been proposed to classify ovarian masses as low risk for malignancy (oophorectomy is unwarranted), high risk for malignancy (oophorectomy is warranted), and intermediate risk, based primarily on tumor markers, radiographic appearance, and size of the mass. ^,

The use of tumor markers to preoperatively stratify the risk of malignancy in adnexal masses remains controversial ( Table 70.2 ). Tumor markers are elevated in only around half (54%–56%) of malignant ovarian tumors in children, and are also elevated in more than 20% of benign ovarian neoplasms. ^, In addition, tumor markers are nonspecific indicators of ovarian malignancy. Elevation of alpha-fetoprotein (AFP) is seen in malignant tumors such as endodermal sinus tumors and immature and even benign teratomas. Lactate dehydrogenase (LDH) has a high sensitivity (elevated in 98% of malignant ovarian neoplasms). AFP, inhibin A, and inhibin B have a high specificity (>90%). β-HCG is a specific marker for dysgerminoma, the most common malignant GCT in children. If elevated, tumor markers are useful postoperatively to monitor for complete resection of tumor and to detect recurrence.

Various size thresholds, from 5–9 cm, have been used to help differentiate benign from malignant tumors. ^{, ,} Although masses ≥8 cm in size are more likely to be malignant, using this dimension as an absolute cutoff can result in missed malignancies. The nature of the mass is another risk determinant, with solid or heterogeneous/complex masses more likely to be malignant. ^, However, benign lesions, such as mature teratomas, may also have a complex radiographic appearance ( Figs. 70.19 and 70.20 ). Other elements of complexity that favor a diagnosis of malignancy are the presence of thick septations (>2–3 mm) and nonhyperechoic solid nodular or papillary components ( Table 70.3 ). ^, A relatively recent risk stratification strategy used a combination of the transabdominal ultrasound ovarian morphologic index (Ueland’s Morphology Index, MI) calculated by assessment of the complexity and volume of the mass ( Table 70.4 ) and the ovarian crescent sign (OCS), which suggests a benign pathology ( Fig. 70.20 ). An MI score of ≥7 with no edema of the OCS and/or an MI score of ≥5 with an absent OCS were predictive of malignancy, with a 93% sensitivity (95% CI 82–98) and a specificity of 97% (95% CI 95–99). No malignancies were associated with asymptomatic masses with MI ≤ 4 and with the OCS present on imaging.

An ultrasound study in this teenager with a benign teratoma shows a hyperechogenic ovarian mass with classic Rokitansky nodules ( arrow ). These nodules are indicative of calcific, dental, adipose, hair, and/or other sebaceous components, and are pathognomonic of an ovarian teratoma.

Ultrasound findings of the ovarian crescent sign (OCS). (A) Arrows show the presence of normal ovarian tissue with follicles (OCS) in a functional cyst. (B) Arrows show the OCS in an ovary with a mature teratoma. (C) Arrows show the OCS, which is enlarged due to edema from a twisted ovary with teratoma.

From Stankovic ZB, et al. Ovarian preservation in girls: prospective diagnostic study. J Pediatr Adolesc Gynecol . 2017;30:405–412.

Fertility-sparing surgery is a priority and is accompanied by postoperative surveillance based on histopathology. A conservative approach including observation for spontaneous resolution can be applied for the management of asymptomatic, 5–8 cm physiologic cysts, with no solid components beyond debris from hemorrhage. In the absence of findings suggestive of malignancy, a minimally invasive approach with laparoscopic cystectomy is employed even for very large cysts. For tumors where the risk of malignancy is low but uncertain, a hybrid minimally invasive approach may decrease the risk intraperitoneal spillage of cyst contents. Various methods have been described to decrease the risk of intraperitoneal spillage of cyst contents via extracorporeal cystectomy ( Fig. 70.21 ). Ovarian cystectomy is performed by incising the ovary on the antimesenteric surface to allow visualization of the cyst wall. Using blunt dissection or hydrodissection, the cyst wall is separated from the ovarian cortex, allowing cyst removal ( Fig. 70.22 ). Cystectomy is preferable to cyst drainage due to lower recurrence rates. Electrocautery or sutures may be needed to obtain hemostasis. Bipolar electrocoagulation may result in increased follicular destruction and decreased ovarian reserve compared to sutures, but pregnancy rates do not differ. Although marked distortion can occur, even a thin ovarian cortex should be preserved as histological examination demonstrates normal ovarian tissue. Approximating the ovarian cortex is not necessary.

16-year-old girl with a 30 cm left adnexal mass. Following a paraumbilical incision, the cyst was drained intraabdominally using a Verees needle attached to suction, the decompressed ovary was delivered through the incision, and cystectomy performed.

This 16-year-old girl was taken to the operating room for torsion of her right ovary (A). Following detorsion, it was apparent that she had a large ovarian cyst as the etiology of the torsion. The ovarian cortex was incised, and the cyst aspirated to decompress it (B). The lining of the cyst was then stripped from the ovary (C) and was completely excised (D). The ovarian cortex was then approximated, and she recovered uneventfully.

In the past, the preferred surgical approach for an ovarian mature teratoma was laparotomy, due to the concern for spillage of cyst contents and the development of a chemical peritonitis ( Fig. 70.23 ). More recent evidence suggests that despite an increased risk of cyst rupture during laparoscopy, intraperitoneal spillage of dermoid cyst contents is rarely associated with complications. ^, Ovarian teratomas can also be managed in a hybrid, minimally invasive fashion, via a 5-cm Pfannenstiel incision, and delivery of the ovary through the mini-laparotomy incision, followed by cystectomy ( Fig. 70.24 ).

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