Imperforate Hymen: A Case of Pediatric Acute Urinary Retention

Imperforate Hymen: A Case of Pediatric Acute Urinary Retention

Ricardo González in Handbook Of Urological Diseases In Children defines Acute Retention as “… the inability to void occurring in a patient who was until then voiding normally. Chronic retention is the chronic inability to empty the bladder completely. The patient may simple void incompletely in an apparently normal fashion or simply dribble urine constantly (overflow incontinence).” (1) Urinary retention in children is evident through signs of dribbling, weak stream, the inability to initiate bladder emptying, abdominal pain and palpable abdominal mass.   

According to the study by Asgari et al the causes of urinary retention in boys found lower urinary tract stones in 38%, neurological disorders in 12%, and local inflammation in 10%; while in girls, urinary retention were caused by ureterocele in 21.4%, trauma in 17.8% and imperforate hymen in 10.7%.(2) They also observe that the incidence of lower urinary tract stones was 5.5-fold in boys (38.5% versus 7%). The rates of injury and stoppage were about equivalent in young men and young ladies. Kind obstructive injuries, prostatic utricle, urethral outside body, and prune midsection disorder were seen exclusively in young men, and ureterocele, imperforate hymen, and Hinman disorder all were seen only in young ladies. In their investigation of 86 patients, 24 (22 male and 2 female) had lower urinary tract stone, in which the most widely recognized area was urethral meatus (75%). Frequently, urethral stone in guys are begun from bladder. Essential urethral stones can likewise be shaped in the setting of urethral stricture, urethral diverticula, or urethral pocket. (2)

In a study by Gatti et al of 53 children, 37 boys 6 months to 17 years old and 16 girls 1 to 17 years old. In this study, Urinary tract infections were 6 times more common in females than males (31% versus 5%). Constipation and local inflammatory processes were twice as common in females than males (19% versus 11% and 12% versus 5%, respectively)(3)

According to Okafor and fellow researchers, Imperforate hymen “occurs when the sinovaginal bulb fails to canalize with the rest of the vagina” (4) and note that this fact can be corroborated by the absence of the track of mucus at the posterior commissure of the labia majora in newborns or by visualization of the bulging hymen after puberty. According to Messina et al, Imperforate hymen (IH) is the most frequent congenital malformation of the female genital tract. (5) Yildirim et al mention that “The estimated incidence of imperforate hymen (IH) is 0.1%–0.014%.” (6)

Incidence in girls

Urinary retention is a more common phenomena in male rather than female. Etiology for urinary retention can be widely classified as neurological, voiding dysfunction, constipation, drug complications, urinary tract infections, inflammation surrounding the area, neoplasm invading locally or any benign pathology obstructing the area.

Imperforate hymen is usually asymptomatic until puberty, as that is when the hormones start kicking in and the uterus is functional. Thus the symptoms are related pubertal phenomena such as menstrual cycle. The most common clinical complaints by the patient is of pelvic pain. A manifestation of this condition in infants due to the influence of maternal estrogen was noted by Farzaneh et al. In this condition, the utero vaginal secretions result in the formation of hydrocolpos. However, this is a very rare condition which is found only in 0.006% of all the patients with imperforate hymen. (7)

In a research by Mustafa Basaran and fellow researchers, the incidence of imperforate hymen at birth is 0.1 to 0.05%, which points to the most common cause of genital flow obstruction. (8) However, other than these syndromes, imperforate hymen is not associated with any urogenital anomalies and the treatment of choice is a simple surgery in which the excess hymen tissue is excised.

Writing from a context like Turkey, they note the meaning of surgical removal of the hymenal tissue. They locate the meaning of this surgical removal having diverse meanings within the sociocultural and connotations. In this context, it is to be noted that the psychological and sociocultural consequences for persons who are located in diverse cultures and religious communities cannot be the same and has repercussions for physicians who advice the same for their patients. The contexts of diagnosis and occurrences of the cases in America, Turkey, India, Bangladesh and Britain can be so different and would demand diverse healthcare responses. In order to authentically understand the nuances of diagnosis, treatment and healing, in diverse sociocultural contexts this is a significant observation to deal with for a range of healthcare professionals and researchers. For instance, such scenarios can be seen as merely healthcare-driven in one context while in other places, it can be also about morality, cultural purity.

Two case studies will be presented here, first one is of a girl around 20 days old. She was premature and born via vaginal delivery and was immediately admitted at the hospital because of abdominal distention and urinary retention. There was no history of trauma, however on physical examination, a large midline mass was palpated above the umbilicus along with obvious mucosal bulging under urethral meatus. Abdominal ultrasonography confirmed bilateral hydronephrosis. Even though the serum creatinine level and the electrolytes were normal, but the catheter insertion seemed impossible. As a first step, 300 ml of cloudy urine was drained through suprapubic access to the bladder. Thus, Magnetic resonance urography was used to rule out its differential diagnosis.

Imperforate hymen can also present with syndromes such as McKusick-Kaufman and Bardet-Biedle syndrome, however, isolated imperforate hymen are more common as compared to these and their prognosis is also good.

There is an ongoing debate on the time when the surgery should be performed. Kahn et al did a research on this phenomenon by assessing conservatively 2 girls who had uncomplicated imperforate hymen who had early (infant) diagnosis. They concluded that imperforate hymen which is asymptomatic can be treated expectantly in which there will be no spontaneous opening. However in symptomatic patients, incision of the hymen is necessary in which the excessive tissue is removed. Going towards delayed treatment when the symptoms arise can be good in the sense that it prevents risk of local anesthesia in children, though it increases the chances of endometriosis. In a study by Posner and Spandorfer, it was found that the earliest age to develop symptoms can be as low as 10.9 years, hence it is important to treat it before menarche. (9)

Underlying causes

In a study regarding associated abnormalities, results concluded that out of the total number 13 girls had an imperforate hymen, 6 had vaginal atresia and 4 had double genital systems, consisting of 2 with unilateral vaginal atresia, and 2 with unilateral imperforate hymen. One girl had-a urogenital sinus, however, there was no genital distension in 3 children, but 6 developed hydrocolpos, one had hydrometrocolpos, one unilateral hydrometrocolpos with hydrosalpinx in a double system, and 7 had haematocolpos, 3 haematometrocolpos, 2 unilateral haematocolpos, and one had unilateral haematometra with haematosalpinx. Further investigation showed that Anorectal abnormalities were present in 9 girls, out of which had a minor, low lesion amenable to anoplasty, and the other girl had high rectal atresia had the common variety with a colovestibular fistula. One girl reported with an anorectal atresia without a fistula.

Four had hydrocolpos with the distal end of the large bowel draining into a closed vagina and one had cloacal exstrophy with a double uterus and a vaginal lesion of which the investigation is still under process. Lastly, One girl was found to have true rectal stenosis (10)

Description of female reproductive structures

  Up until about week 7 to week 8 of pregnancy, both sexes have what’s known as a “genital ridge” — i.e. an identical preliminary set of genitalia that will eventually differentiate to become either male or female sex organs. That means that all our sex organs come from the same foundations: The testes in men are equivalent to labia and ovaries in women, and the penis is the equivalent of the clitoris.

All babies would develop female sex organs if it weren’t for the male hormone testosterone. At around week 7, the Y chromosome signals for the start of testosterone production, and male genitalia begin to develop. Peak concentrations of testosterone in your baby boy’s body are comparable to the amounts found in adult men at around week 16 of pregnancy. And then, between 16 and 20 weeks, testosterone levels fall until they reach the range found in early puberty by about 24 weeks.

Renal impairment

Renal impairment occurs in neonates receiving indomethacin for treatment of patent ductus arteriosus. Inhibition of cyclooxygenase within the neonatal kidney results in decreased prostaglandin synthesis and consequent reduction in renal perfusion. Indomethacin has been reported to cause short-term reduction in glomerular filtration that resolves after cessation of the drug. There is little information on the long-term effects of postnatal exposure to indomethacin. The aim of this study was to determine the incidence of renal impairment in infants treated with indomethacin in a single center, to determine whether there is evidence of renal impairment on day 30 or at discharge, and to identify risk factors for renal impairment. In a retrospective study, infants of less than 30 weeks completed gestation who received indomethacin to close the ductus arteriosus were matched with infants of the same gestation, birth weight, and severity of illness. Serum creatinine and glomerular filtration rates (GFR) were obtained prior to commencing indomethacin and on days 2, 7, and 30 following indomethacin administration. Acute renal failure was defined as an increase in creatinine of greater than 25%. Of those infants who were less than 30 weeks completed gestation, 24% had acute renal failure following indomethacin administration. There was a significant elevation in serum creatinine on day 2 and day 7 ( P<0.0001, P=0.002) and a decrease in GFR on day 2 and day 7 ( P<0.0001, P=0.01) following administration of indomethacin. Renal function had normalized by day 30 or discharge. The incidence of acute renal failure in neonates treated with indomethacin is clinically significant. Renal function returns to normal by day 30. Linear regression found no statistical significance for gestational age, day of indomethacin dosing, Clinical Risk Index for Babies (CRIB) score, and presence of an umbilical artery catheter to confound the effect of indomethacin on renal function. (11)

Infertility

Imperforate hymen, if not diagnosed and treated early can lead to some very serious complications like vaginal infection, kidney issues and even endometriosis in severe conditions. Endometriosis is basically a fertility reduction condition which can also lead to infertility. There is a great variation in its signs and symptoms as opposed to the severity. (12) However, the severity of your symptoms in no way indicate the stage of this condition.

Talking about the signs and symptoms, pelvic pain is proven to be the most common followed by many other, such as:

-          Pain during menstrual cycles

-          Pain preceding and proceeding menstrual cycle

-          Severe to moderate cramps when the dates are near

-          Heavy bleeding

-          May bleed on normal days (other than the ones for menstrual cycle)

-          Pain after sexual intercourse

-          Discomfort or pain during bowel movements

-          Sudden lower back pain

Contrary to the mass belief, imperforate hymen is also possible to exist without any sigs and symptoms. Thus, it is very important to get regular gynecology exams, especially during peak ages when the hormonal balance is changing (puberty). Also, it is advised to get regular checkups especially when you have 2 or more of the above listed signs and symptoms.

How is it investigated

Imperforate hymen is a congenital disorder where the hymen completely obstructs the vagina. The main etiology is when the hymen fails to perforate during fetal development. Thus, it is found in young girls as well as women with a varying degree of signs and symptoms. However, depending on factors such as uterus activity and sexual intercourse, the signs and symptoms have peak timings. For example, menstrual blood tends to accumulate in the vagina due to the obstruction, which can only happen after a girl reaches puberty.

It is imperative to understand these peak times in order to make the correct diagnosis and use precise investigative tools. Diagnosis of imperforate hymen is usually done in adolescent girls when they reach the age of menarche. Some of the most common clinical symptoms presented are as follows:

-          Amenorrhea

-          Cyclic pelvic pain

-          Vaginal infections

To further confirm the diagnosis, clinical examinations are down. Clinically it appears as a blue bulging membrane. However, if hematocolpos is present, the mass is pronounced and palpable on abdominal and rectal examination. (13)

Investigations for imperforate hymen mainly relies on clinical inspection, however, if necessary, following procedures can be performed to further confirm its presence;

-          Transabdominal ultrasound

-          Transperineally ultrasound

-          Transrectal ultrasound

Ultrasounds can also detect imperforate hymen during the fetal stage and right after the baby is born. The key finding in babies with imperforate hymen is a bulging mass at the abdominal region or at the pelvic, and at times, a bulging hymen may also be visible. This can be very effective yet while examining a neonatal vagina, there is a slight mucus discharge at the posterior commissure of the labia major. If this secretion is absent, it indicates blockage of the vagina which may be due to imperforate hymen or other vaginal obstructions.  

Treatment (hymen incision)

The best way to fix an imperforate hymen is a minor surgical procedure. It is also possible to safely perform this procedure on infants given that an early diagnosis is made. However, most parents opt to wait for pubertal age of the girls as adolescent girls heal much faster and with less complication chances than the infants. (14)

The area is first kept under local anesthesia. After profound anesthesia dose, the surgeon makes a small incision in a way to remove the excess tissue, leaving behind a normal vaginal opening. If any blood is accumulated in the vaginal prior to the procedure, it is carefully removed and cleaned to avoid any infections or delayed healing.

To prevent any relapse or regrowth, the surgeon may also place a ring for a period of time to maintain the normal vaginal opening. Another alternative for rings is dilators that are quite often. Dilators are devices resembling a tampon which are to be placed in the vaginal every day for 15 minutes during the healing phase.

Some people have reservations with surgeries due to certain myths that come along vaginal surgeries. Though contrary to the belief, there are no complications and the vaginal functions normally after surgery. The girl has regular periods without any signs of pain and can also have normal sexual intercourse followed by vaginal delivery during childbirth. (15)

Frequently missed neonatal diagnosis

Diagnosis vary in terms of clinical findings and signs and symptoms. Some patients may present with pronounced masses however some might present with small bulges. The same case is with signs and symptoms. As we discussed above, it is possible to have this condition with literally no signs at all.

Neonatal diagnosis is difficult due to the fragility factor and also that they are under the influence of their mother’s estrogen. ⁽⁴⁾ Also, the neonate is still growing and maturing, this what might seem as a probable pathology, can also be pseudo which will be self-resolved in the coming years. Neonates are also not very precise about their signs and symptoms. Since they are still in their learning stage, they might not be able to pinpoint what they exactly feel, which leads to having a list of differential diagnosis.

Requires careful examination

Imperforate hymen presents differently in different ages, depending on the hormone levels. We will classify the ages into two broad domains, infancy and adolescence.

Infants usually present with a hymenal bulge ranging from hydrocolopos or mucocolpos. The main etiology for this is the influence of maternal estrogen which comes in contact with the neonatal hymen. This type is usually self-limiting and asymptomatic. As we discussed earlier, parents opt out of surgical procedures in this age with eh hope of the deformity correcting itself by the time of puberty. (15)

In adolescence, this condition is symptomatically accompanied by a lot of pain and discomfort. It presents with cyclic or persistent pelvic pain and primary amenorrhea. F this condition co exists with pronounced hematometra, an abdominal mass may also be palpated. However, if the condition becomes chronic or severe, there are various other symptoms that start showing up such as constipation, nausea, back pain and pain on defecation. One major complication of this condition is hematosalpinx. In this condition, retrograde passage of blood flows into the fallopian tubes. This further results in endometriosis and adhesion formation. Another variation of this blood sinus is hemoperitoneum, in which the blood flows freely into the peritoneal cavity. 

Morbidity associated with missed diagnosis

Morbidity increase as age increases as there are greater chances or infections and other diseases to coexist.Even though imperforate hymen is one of the most common female gynecology problems, but it is also the most commonly missed. Diagnosis for this condition mainly depends on the clinical examinations which should be performed thoroughly. Any missed step may lead to missed diagnosis that might cause problems in the future.

As patients with imperforate hymen are at high risk of endometriosis, they have a much higher chance of secondary infections that might worsen the prognosis. Thus it is advisable to rule out the risks of anesthesia in children and perform the surgery as early as possible (specially before puberty as chances of blood accumulation increase that provide a perfect culture for bacterial infections). It is necessary to have regular gynecological examinations to rule out such potentially morbid conditions and it is imperative for the clinician to perform thorough check each time.

References

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