Pediatric and Adolescent Gynecology Flashcards
A 7-year-old premenarchal female presents with left lower quadrant pain. On ultrasound, she is found to have a 7-cm complex cyst arising from her left ovary. What is the appropriate management for this cyst?
A. Close observation, with ultrasound every 6 months
B. Fenestration
C. Surgical excision of the cyst, leaving the left ovary in situ
D. Ten days of antibiotics
E. Reassurance since this is a normal finding
ANSWER: C
COMMENTS: The clinical presentations of ovarian masses are highly variable. Symptoms may include acute pain often due to torsion, intermittent pain, an abdominal mass, or “acute appendici- tis.” It is important to assess the menstrual status and sexual history.
Initial evaluation generally includes ultrasound, which may show a simple cyst, a complex cyst, a solid mass, and blood flow to the ovary.
Lab tests for tumor markers such as CA-125, alpha-fetoprotein (AFP), hCG, and inhibin should be performed.
The most common benign lesions are unilateral follicular cysts (50%).
The most simple cysts are likely to regress. However, if they are larger than 5 cm in diameter, the risk of torsion is much higher, and cyst resection should be considered.
Complex cysts could represent cysts that have already torsed or an ovarian neoplasm. Complex cysts should be resected in all premenarchal females.
Excision of the cyst with preservation of the ovary should be performed when- ever possible.
Fenestration would be inappropriate in this scenario. There is an increased incidence of neoplasms in complex cysts, so fenestration could seed the intraabdominal cavity.
In an adolescent, a complex cyst could be the result of hemorrhage into a functional cyst. Therefore surgical excision is not necessary at the time of diagnosis of an isolated complex cyst.
Careful clinical and ultrasound follow-up for symptom resolution as well as involution of the cyst should be performed. If the cyst persists, it should be resected.
What is the incidence of adnexal torsion in the pediatric population?
Adnexal torsion accounts for 2.7% of cases of children with acute abdominal pain.
Within children ages 1 to 20 years, the incidence of adnexal torsion has been estimated at 4.9:100,000.
What are the typical clinical findings in a child with ovarian torsion?
Sudden onset of severe unilateral pain in the lower abdomen or pelvic area. Pain is commonly associated with nausea and/or vomiting. If the torsion remains untreated, the child may develop leukocytosis and a low-grade fever [1].
What ultrasound findings suggest ovarian torsion?
The hallmarks of ovarian torsion on Doppler ultrasound are ovarian enlargement and absent Doppler arterial flow [3, 4]. However, as venous flow is affected first, the presence of arterial flow does not exclude torsion [5].
What is the recommended treatment of ovarian torsion?
Ovarian torsion is a surgical emergency. Laparoscopic detorsion with ovarian tis- sue conservation is favored over oophorectomy to preserve ovarian function even if the ovary appears necrotic (blue-black) [1]. The risk of malignancy is low (0.4 to 5%) and oophorectomy should only be performed if there is clear evidence of malignancy [2, 6]. More than half of torsions in post-pubertal patients occur in the setting of an adnexal mass. Concurrent cystectomy can be performed in these patients to decrease the need for repeated surgery [1].
Should oophoropexy be routinely performed?
Routine oophoropexy is controversial. The risk of recurrence of ovarian torsion is 2 to 12%, with higher risk in cases of spontaneously torsed normal ovaries [7]. Oophoropexy does not eliminate the risk of recurrence of ovarian torsion with a reported rate of approximately 9% after ovarian fixation [8]. Oophoropexy can be offered in the setting of repeated ipsilateral torsion, absent contralateral ovary, elongated ovarian ligament and torsion of a normal ovary [1]. Laparoscopic oophoropexy can be performed by shortening the ovarian ligament with permanent suture or fixing the ovary to the round ligament, uterosacral ligament or the poste- rior uterus [9].
What is the incidence of fetal ovarian cysts?
Ovarian cysts occur in approximately 1 in 2500 fetuses and are the most common abdominal anomalies diagnosed in female fetuses [10]. They are categorized by their sonographic appearance as simple cysts (thin-walled, round, anechoic, uni- locular cysts measuring>2cm) or complex cysts (thick-walled, heterogeneous, containing hyperechoic components) [11].
What are the risks associated with fetal ovarian cysts?
Fetal ovarian cysts may develop intracystic hemorrhage, rupture and ovarian torsion [11].
What is the management of a fetal ovarian cyst greater than 5 cm?
Consideration should be given to antenatal percutaneous aspiration to decrease the risk of ovarian torsion. In utero aspiration of anechoic ovarian cysts has been shown to increase the incidence of in utero cyst involution and to decrease the rate of oophorectomy [12].
What ultrasound findings of ovarian cysts in children and adolescents are concerning for malignancy?
Ovarian mass size >8 cm with complexity [13].
What is the most common ovarian tumor in children?
The most common ovarian tumor in children, a mature cystic teratoma (dermoid cyst), is also the most common germ cell tumor in children. Germ cell tumors are the most common ovarian tumors in children and adolescents [13]. The majority of ovarian tumors in children and adolescents are benign. Only from 4% to 11% of adnexal masses are malignant [14].
What is the prevalence of ectopic pregnancy?
Ectopic pregnancy has been reported in upwards of 18% of women presenting to an emergency department for first trimester vaginal bleeding, abdominal pain or both [15]. Ectopic pregnancy occurs in 1.5 to 2% of all pregnancies and ruptured ectopic pregnancy accounts for 6% of maternal deaths [16].
What risk factors increases an adolescent’s risk of ectopic pregnancy?
Previous ectopic pregnancy, prior history of pelvic inflammatory disease, early initiation of oral contraceptives (at age < 16 years), use of intrauterine device for contraception, smoking (current or previous use), consumption of > 10 Grams per day of alcohol, previous tubal surgery, in utero exposure to DES (diethylstilbes- trol), history of infertility, use of assisted reproductive technology.
How is ectopic pregnancy diagnosed?
By confirming pregnancy (with serum hCG level) and transvaginal ultrasound. Sonographic visualization of a gestational sac with a yolk sac, an embryo, or both in the adnexa definitively diagnoses an ectopic pregnancy.
Where are ectopic pregnancies most frequently located?
More than 95% of ectopic pregnancies are located in the fallopian tube. The remaining are interstitial (2 to 4%), ovarian (<1%), cervical (<1%), abdominal (<1%), and along a previous cesarean section uterine scar (<1%). Of ectopic preg- nancies that occur in the fallopian tube, 70 to 80% occur in the ampulla, 12% are isthmic and 5% are located on the fimbria.
What are the contraindications to treating ectopic pregnancy with methotrexate and when is surgery indicated?
Surgery is indicated if the patient is hemodynamically unstable, has signs of intra- peritoneal bleeding or symptoms of ongoing ruptured ectopic pregnancy such as pelvic pain. In addition the following absolute contraindications to the use of
methotrexate require the surgical treatment of ectopic pregnancy: evidence of immunodeficiency; moderate to severe anemia, leukopenia or thrombocytopenia; sensitivity to methotrexate; active pulmonary or peptic ulcer disease; clinically important hepatic dysfunction or renal dysfunction; breastfeeding; inability to par- ticipate in follow up.
Surgical management can also be considered in the setting of the following relative contraindications to methotrexate therapy: embryonic cardiac activity detected by transvaginal ultrasonography; high initial hCG concentra- tion (>5000 mIU/mL); ectopic pregnancy >4 cm in size as imaged by transvaginal ultrasonography; refusal to accept blood transfusion.
What are possible etiologies of hydrometrocolpos?
Hydrometrocolpos may result from imperforate hymen, transverse vaginal septum, vaginal atresia in the setting of a functional uterus, OHVIRA (obstructed hemiva- gina with ipsilateral renal anomaly) or cloacal anomaly.
What are the typical presenting symptoms of hydrometrocolpos?
Progressively worsening, episodic lower abdominal cramping and pelvic pain after onset of puberty. Less common symptoms include back pain, urinary retention, urinary incontinence and constipation. A partial or microperfo- rate transverse vaginal septum allows for some egress of menstrual blood. The patient usually reports dysmenorrhea. In the case of OHVIRA, regular periods from the unobstructed hemivagina are associated with progressively worsening dysmenorrhea.
What complications can occur as a result of hydrometrocolpos?
The distended vagina can cause mass effect on other pelvic structures leading to
hydronephrosis and urinary retention.
How is an imperforate hymen diagnosed?
On physical examination, an abdominal/pelvic mass may be palpable and the hymen bulges and may appear bluish. Ultrasound imaging shows a cystic structure in the lower abdomen and pelvis (representing the distended vagina) with the uterus at the cranial end of the structure. An MRI is typically not necessary for diagnosis.
How is imperforate hymen treated?
Surgically with cruciate of annular hymenal incisions. The evacuation of blood from the vagina leads to the resolution of symptoms. Less commonly, imperforate hymen may be diagnosed in infancy. In infants, surgical intervention is reserved for symptomatic patients (as with hydronephrosis) due to the risk of reclosure and infection.
How is a transverse vaginal septum diagnosed?
In contrast to an imperforate hymen, examination reveals no bulging noted at the introitus and the external genitalia is normal in appearance. The vagina appears to end in a blind pouch with no visualization of the cervix. As with imperforate hymen, pelvic ultrasound shows a cystic pelvic mass with a uterus at the cranial end. Ultrasound or MRI may help delineate the thickness and location of the vag- inal septum. Approximately 46% of transverse vaginal septa occur in the upper vagina, 40% in the mid vagina and 14% in the lower vagina.
How is a transverse vaginal septum treated?
The septum is surgically resected and the upper and lower vagina are anastomosed in an end-to end fashion. An indwelling stent or vaginal mold is typically left in place in the immediate postoperative period. Thereafter, the use of vaginal dilators are recommended to prevent stricture.
How is vaginal atresia diagnosed?
Physical examination of the external genitalia reveals lack of a vaginal orifice or a vaginal dimple. On rectal examination, the distended upper vagina may be palpa- ble.
Transperineal ultrasound or MRI are useful to assess the distance between the hymenal tissue and the proximal aspect of the obstructed vagina.
The following image represents the findings in a 5-year-old girl who presents to the emergency department with a history of genital trauma. In taking a history, which of the following mechanisms of injury would not be consistent with the pattern of injuries seen?
A fall on an upturned chair leg
B fall straddling a cross bar on a bicycle
C penetrating water injury from a jet of water
D fall on a toy with a sharp narrow edge
E a motor vehicle accident with pelvic fracture
B
The most common mechanism of genital trauma is a straddle injury.
A straddle injury causes perineal trauma as a result of falling or striking an object or surface with the force of one’s body, crushing the soft tissue between the object and the bony margins of the pelvic outlet.
In a straddle or blunt injury the object is not capable of penetrating above the pelvic floor.
Straddle injuries are the most common mechanism of injury in the 5- to 9-year-old age group of girls.
Penetrating injuries can be accidental or intentional (i.e. related to sexual abuse). Penetrating injuries comprise only 3%–20% of accidental genital injuries.
The pattern of injuries seen with a straddle injury and a penetrating injury of the vagina vary.
Either type of injury can involve the posterior fourchette.
Straddle injuries tend to demonstrate ecchymosis, linear abrasions or lacerations and haematomas from extravasation of blood into the loose areolar tissues of the mons, clitoris and labia.
Hymenal and vaginal injuries are far more common with penetrating injuries, with hymenal involvement in 40%–80% of penetrating injuries.
In the image above, a laceration extending into and involving the hymen is evident.
Hence the mechanism of injury is likely penetrating.
The mechanism of genital injury taken on history from either a verbal child or their caretaker should be plausible, timely and most importantly consistent with the findings evident on physical examination.
As a fall straddling a crossbar of a bicycle would be of a straddle-injury type without a mechanism for penetration, and as the examination reveals a laceration of the hymen extending into the vaginal mucosa, the history and examination would be inconsistent.
A lack of correlation between the history and physical findings is an indication to consider a sexual abuse evaluation.
Each of the other options listed above could result in hymenal and vaginal lacerations.
SPSE 1
A 5-year-old is referred for assessment of vaginal bleeding with the following finding evident on examination – see Figure 67.2. Which statement is true regarding the treatment of this diagnosis?
A 90% of patients will require surgical management.
B Medical therapy includes topical testosterone twice daily.
C Prepubertal patients are more likely to fail medical therapy.
D Referral to child protective services is mandatory.
E If >15–30 mm in size, surgery is more likely.
E
urethral mucosal prolapse has an incidence of 1 : 2880 children; it is more common between the ages of 4 and 8 years, and it varies with ethnicity.
The presenting symptom is usually painless bleeding without interference of voiding patterns.
Described as an annular, congested or oedematous, anteriorly placed introital mass, it is the protrusion of the urethral mucosa beyond the urethral meatus.
once prolapsed, the muscular tone of the urethral meatus constricts the tissue circumferentially resulting in the oedema, congestion and in some circumstances necrosis of the mucosal tissue.
It is thought that several factors contribute to the prolapse.
First, there may be anatomical defects with poor adhesion of the urethral mucosa to the underlying smooth muscles of the urethra. often there is a precipitating event such as an increase in intra-abdominal pressure from conditions including constipation and straining for bowel movements, or an upper respiratory tract infection and accompanying cough or crying. This precipitating event, when occurring in a susceptible child with prepubertal/unoestrogenised tissue, results in prolapse of the tissue. While the physical findings are often confused for abuse, it is not related to sexual abuse.
medical therapy with topical oestrogen therapy and sitz baths with or without antibiotic treatment will resolve the prolapse in 33%–70% of cases.
Surgery, which involves excision of the prolapsed mucosa, is indicated when medical therapy fails, in patients with profuse bleeding, when the mucosa is necrosed, when the prolapse is particularly large (15–30 mm diameter), and in postpubertal patients.
In regard to patients presenting with vaginal bleeding, the differential diagnosis in a prepubertal girl includes vaginitis, foreign body (as discussed in detail for question (3), premature puberty or premature menarche, genital trauma or abuse (see questions 4 and 5), skin conditions such as lichen sclerosis with excoriations, urethral mucosal prolapse and vaginal/cervical rhabdomyosarcoma.
These diagnoses can be distinguished on history, physical exam and investigations.
SPSE 1
Which of the following is not a correct pairing of histological ovarian tumour type with its corresponding tumour marker?
A β-hCG : choriocarcinoma
B alpha-fetoprotein : endodermal sinus tumour
C inhibin : granulosa cell tumour
D LDH : immature cystic teratoma
E CA125 : serous cystadenocarcinoma
D
All histological cell types are evident in ovarian neoplasms in children and adolescents; however, the proportion of each type varies compared with adult populations.
Germ cell tumours predominate (73%), followed by epithelial tumours (13%). Stromal tumours comprise a small proportion of ovarian masses at all ages.
The benign germ cell tumour, mature cystic teratoma, is the most common childhood and adolescent ovarian neoplasm.
The benign stromal tumour is the fibrothecoma.
malignant germ cell tumours include: dysgerminoma, immature teratoma, endodermal sinus tumour, mixed germ cell tumour, embryonal carcinoma and choriocarcinoma.
malignant sex-cord-stromal tumours are the granulosa cell and Sertoli–leydig tumours.
Epithelial lesions may be benign (cystadenomas), borderline (low malignant potential) or malignant (cystadenocarcinomas).
The ovarian malignancies in children and adolescents and their related tumour markers are indicated in Table 67.1.
SPSE 1
How are benign ovarian masses differentiated from malignant ones on ultrasound?
As US is the imaging modality of choice for evaluation of ovarian masses, it is prudent for the surgeon to recognize ultrasonographic patterns they may encounter.
While benign cysts typically appear with clear cystic areas on US with thin septa, the presence of thick septations >2–3 mm and nonhyperechoic solid nodular or papillary components favors a diagnosis of malignancy.
The use of an ultrasonographic morphology index (MI) has been described as a tool to relate US findings to tumor histology, with a score of 7–10 being concerning for malignancy.
Another new ultrasonographic tool used to evaluate ovarian neoplasms is the ovarian crescent sign (OCS). When seen, the presence of this sign can indicate normal ovarian tissue surrounding a cyst or tumor that may assist in excluding an invasive ovarian malignancy, although the OCS can be present in cases of borderline malignancy and in metastatic disease to the ovaries.
Use of the OCS and MI in conjunction with emergent or nonemergent presentations in 19 year olds with ovarian tumors/ torsion was noted to be a very accurate management tool in a recent study.
No malignancies were noted in asymptomatic patients with an ovarian mass that had an MI of 4 or less in a group of 1236 cases.
The OCS was noted to be present in 96% of benign masses and in 3% with microscopic malignancy.
It would appear that using MI and OCS may reduce the number of surgical procedures performed on functional cysts, and may be useful in choosing the optimal treatment of adnexal masses.
H&A
How is endometriosis diagnosed?
Endometriosis refers to the presence of endometrial glands and functioning stroma outside the uterine lining and is the most common pathologic condition of the pelvis in adolescents with chronic pelvic pain.
As many as 75% of adolescent girls with medically refractory pelvic pain are found to have endometriosis at the time of diagnostic laparoscopy.
Endometriosis is a progressive disease, and serial studies in adults with endometriosis reveal that 60% of affected women report the onset of pelvic symptoms earlier than age 20, with the prevalence and severity of the disease increasing with age.
Endometriosis has been found in patients as early as prior to menarche and within 1–5 months of menarche.
Endometriosis has been associated with Müllerian duct anomalies that create obstructed outflow tracts of the genitalia such as imperforate hymen, vaginal septum, complete transverse vaginal septa, and uterine anomalies such as noncommunicating uterine horns. This type of endometriosis typically resolves after operative correction of the obstruction.
There is also a genetic predisposition with firstdegree relatives of affected patients having an increased risk of endometriosis.
Preoperative complaints noted in 90% of patients with endometriosis include both cyclic pain with menses and acyclic pain with gastrointestinal dysfunction.
Primary warning signs of endometriosis in adolescents include
(1) prolonged intake of both nonsteroidal anti-inflammatory drugs (NSAIDS) and oral contraceptive pills to treat dysmenorrhea,
(2) family history of endometriosis,
(3) more frequent absenteeism from school during menses, and
(4) prescription of an oral contraceptive pill before age 18 years because of severe primary dysmenorrhea.
Although symptoms can suggest the diagnosis of endometriosis, the definitive diagnosis is made by laparoscopic visualization or biopsy (or both).
Findings at laparoscopy include thin pelvic adhesions between the ovaries and the peritoneum along with superficial implants on the peritoneum of the cul-de-sac, the uterosacral ligaments, and the rectovaginal septum. 94 Contrary to endometriosis in adults, the appearance of endometriosis differs in adolescents, as there is a predominance of peritoneal defects and pockets of scarred, broad ligament defects referred to as Allen–Masters windows.
These Allen–Masters lesions are pathognomonic for endometriosis in adolescents.
In addition, white, vesicular and hemorrhagic appearing lesions are often noted as well.
Filling the pelvis with fluid and placing the laparoscope under the fluid may make some of these lesions easier to visualize.
If endometriosis is suspected, but not seen, random cul-de-sac peritoneal biopsies have been recommended.
Ovarian endometriomas are uncommon in the adolescent female and are best managed with surgical excision of the endometrioma. Not excising the endometrioma’s cyst wall results in a higher rate of recurrence and pain.
Short-term recurrence after the initial procedure is low, but continuous, long-term follow-up is important for adolescent patients who have undergone surgical treatment of endometriosis in the adolescent period.
There is no cure for endometriosis, and recurrence is common with the optimal treatment still debated.
The treatment in adolescents is based on alleviation of symptoms and slowing the progression of the disease.
For patients undergoing an operation, when possible, removal of all visible lesions by resection or destruction with ablative techniques such as electrocoagulation, endocoagulation, or laser should be performed.
Surgery alone is not adequate and should be considered cytoreductive in nature.
Postoperative menstrual suppression is also needed to treat microscopic residual disease using hormonal therapy in the form of oral contraceptives, progesterone-only medication (or both), or via placement of levonorgestrel containing intrauterine systems along with NSAIDS.
Noninterventional pain therapies may have a place in managing the chronic pain associated with endometriosis.
Long-term outcomes, such as infertility and chronic pain, in those diagnosed with endometriosis as adolescents are not well known.
H&A
