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Recurrent Urinary Retention Due to Imperforate Hymen After Hymenotomy Failure: A Rare Case Report and Review of the Literature
Pediatric Case Report Recurrent Urinary Retention Due to Imperforate Hymen After Hymenotomy Failure: A Rare Case Report and Review of the Literature Sara Abu-Ghanem, Rosa Novoa, Jacob Kaneti, and Eran Rosenberg Acute urinary retention (AUR) is unusual in children. We report for the first time a case of recurrent urinary retention due to massive hematocolpos resulting from an imperforate hymen in a 14-year-old girl. In case of AUR in adolescent girls, clinicians should keep in mind that imperforate hymen may be a causative factor and this condition may easily be treated surgically, but follow-up is still necessary to ensure that there is no recurrence. UROLOGY 78: 180 –182, 2011. © 2011 Elsevier Inc.
A
cute urinary retention (AUR) in children is a relatively rare entity but constitutes a genitourinary emergency. The etiology of AUR is agedependent. AUR in childhood can result from congenital anomalies, neurological disorders, lower urinary tract stones, urinary tract infection, constipation, adverse drug effect, inflammatory disorders, obstructing lesions, and iatrogenic or psychogenic disorders.1,2 Hematocolpos is defined as the accumulation of menstrual blood in the vagina, instead of its expulsion, and usually presents with intermittent lower abdominal pain. It is usually to the result of an imperforate hymen, which is a rare congenital obstructive abnormality of the female genital tract, with a reported occurrence of 1 in 2000 girls.3 To our knowledge, we report the first case of an adolescent girl with recurrent acute urinary retention as a result of hematocolpos associated with imperforate hymen.
CASE In September 2009, a 14-year-old girl was admitted to the pediatric emergency department with a 12-hour history of acute urinary retention, accompanied by dysuria and severe lower abdominal pain. There was no history of nausea, vomiting, altered bowel habits, or fever. She had not been taking any medications recently, including an-
From the Department of Urology, Soroka University Medical Center, Ben Gurion University, Faculty of Health Sciences, Beer-Sheva, Israel; and Department of Diagnostic Imaging, Soroka University Medical Center, Ben Gurion University, Faculty of Health Sciences, Beer-Sheva, Israel Reprint requests: Eran Rosenberg, M.D., Department of Urology, Soroka University Medical Center, Beer-Sheva 84105, Israel. E-mail: [email protected]; [email protected] Received: July 28, 2010, accepted (with revisions): October 14, 2010
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© 2011 Elsevier Inc. All Rights Reserved
tihistamines and anticholinergic drugs. The patient had not yet started her menses. On physical examination, her vital signs were normal. Secondary sexual characteristics were present. An abdominal mass extending from the pelvis to the umbilicus was noted. A pelvic examination was not performed in the emergency department. Urethral catheterization was administered for relief of the symptoms and a total of 700 mL of clear urine was drained. Urinalysis was normal. Transabdominal ultrasound showed significant echogenic fluid accumulation in the vagina, mild dilation of the uterus (hematometra), a full bladder with clear fluid (Figs. 1 and 2), and mild bilateral hydronephrosis. Because the patient was in acute distress, she was taken urgently to the operating room. Because of the patient’s cultural background and the importance of keeping the integrity of the hymen, simple vertical incision of the hymen was performed under general anesthesia. Approximately 600 mL of dark red menstrual blood was drained from the vagina. The symptoms resolved after treatment and she was discharged 2 days later. There is no record of a follow-up. Six months later, in April 2010, the patient was admitted again to the pediatric emergency department with similar complaints of acute urinary retention, dysuria, and lower abdominal pain for 3 days. Again, no other symptoms were reported and her vital signs were normal. On palpitation, a large mass corresponding to the size of a 12-14 weeks gravid uterus was found. On inspection done by the gynecologist, the hymen was found to be scarred and was bulging forwards. Transabdominal ultrasonography showed similar sonographic findings suggestive of hematocolpos. The patient was again taken urgently to the operating room and hymenotomy was performed under general 0090-4295/11/$36.00 doi:10.1016/j.urology.2010.10.022
Figure 1. Longitudinal view of the pelvis, demonstrating uterus of normal size and configuration, mild enlargement of uterine cavity. Large, anechogenic mass with involvement of the entire vagina (hematocolpos).
Figure 2. Transverse view showing dense fluid in the enlarged vagina compressing the bladder.
anesthesia by a cruciate incision. The edges were everted. Approximately 500 mL of dark red blood was drained. Approximately 600 cm2 of urine was also drained upon catheterization. The postoperative period was uneventful and the patient was discharged 2 days later. At 6-week postoperative follow-up, the patient still had not had a menstrual period but did not have any difficulty in urination. Pelvic examination revealed a normal sized hymenal orifice.
COMMENT The hymen is a balance sheet of mesoderm that is perforated normally during the later stages of embryonic development.3 Imperforate hymen is usually an isolated anomaly, although associated malformations, especially those of the genitourinary tract, have been reported. Most cases reported in the literature are sporadic; however, some familial cases have been described as presumptive of probable genetic predisposition.3 UROLOGY 78 (1), 2011
During the neonatal period, imperforate hymen may present with fetal ascites or acute renal failure.4,5 The hematocolpos or hydrocolpos may lead to variable degrees of hydroureter, and hydronephrosis.6 Diagnosis should ideally be done at birth by careful examination of the external genitalia of all newborn females. If the diagnosis is not made in the newborn period and the hymen remains imperforate, the mucus will be reabsorbed and the child usually remains asymptomatic until menarche. The clinical symptoms of teenagers include cyclic lower abdominal pain, primary amenorrhea, chronic constipation, low back pain, dysuria, and acute urinary retention.7 Urinary retention may occur when the retained menstrual products in the vagina compress the urethra and there is angulation of the urethra caused by pressure on the posterior wall of the bladder, again by retained menstrual products. Chuang and Kan demonstrated the compressive effect of hematocolpometra to the urinary outlet by computerized tomography scans.8 The incidence of patients with imperforate hymen presenting with AUR varies, ranging from 3-46%.9,10 In a series of 26 cases of imperforate hymen reported by Calvin and Nichamin, 12 cases of the 26 (46%) presented with AUR,9 and in an another series reported by Ben Temime et al, 4 of 13 patients (30%) with imperforate hymen presented with a sudden onset of complete bladder retention.10 To date, at least 20 cases of imperforate hymen with AUR have been reported. Most of these reported cases presented in adolescence. The youngest patient was a 3-month-old girl who had suffered from repeated urinary tract infections because of urinary retention related to pyocolpos.11 Most cases had abnormal external genitalia in additional to a chief complaint of urinary retention. Two cases had concomitant urological abnormality, such as contralateral renal agenesis,12 or genetic disorder, such as ectrodactyly ectodermal dysplasia-clefting syndrome.13 All cases had an uneventful recovery after surgical treatment and none reported a failure of surgical treatment resulting in recurrence of urinary retention caused by hematocolpos, as described in our case. Urinary retention is always treated by catheterization. It is convenient and easy to observe the hymen at the time of catheterization. A bulge along the posterior aspect of the introitus is typical. A high index of suspicion makes early diagnosis easier and prevents inappropriate laboratory work or imaging studies. Ultrasound is the preferred radiological method in the diagnosis of hematocolpos. Studies show that transrectal ultrasound is more effective than transabdominal ultrasound. Magnetic resonance imaging may be required in cases in which complicated obstructive abnormalities are suspected and ultrasound is insufficient. Standard treatment is surgical hymenectomy with T, X, plus, or cruciform incisions and removal of excess hymenal tissue.14 Because the hymen is a symbol of virginity in some communities, its destruction can be a source of social problems for some girls. There was a 181
recent report of 2 cases with imperforate hymen treated with a simple vertical incision,14 and in both cases, postoperative follow-up was uneventful. In our case, the girl underwent a similar “hymen-sparing procedure” but reperfusion of the hymen had occurred. Therefore, although the outcome after adequate hymenotomy for imperforate hymen is usually excellent, follow-up is still necessary to ensure that there is no recurrence. In addition, clinicians must keep in mind that with adequate surgery, abnormal menstruation or persistent problems of micturition/defecation seldom recur.15
CONCLUSION Although hematocolpos associated with imperforate hymen and urinary retention is uncommon, it should be included in the differential diagnosis in adolescent females with acute abdominal pain and urinary retention. Inspection of the external genitalia, along with catheterization and detailed history-taking, with emphasis on menstruation, can aid in preventing misdiagnosis and commencing with additional radiological diagnostic examinations and appropriate surgical treatment. Moreover, conservative surgery with a simple incision and suturing might be an alternative option to standard treatment for imperforate hymen, but follow-up is necessary to ensure that there is no re-closure of the hymen that might result in recurrence of urinary retention. References 1. Gatti JM, Perez-Brayfield M, Kirsch AJ, et al. Acute urinary retention in children. J Urol. 2001;165:918-921.
182
2. Asgari SA, Mansour Ghanaie M, et al. Acute urinary retention in children. Urol J. 2005;2(1):23-27. 3. Sakalkale R, Samarakkody U. Familial occurrence of imperforate hymen. J Pediatr Adolesc Gynecol. 2005;18:427-429. 4. Jacquemyn Y, De Catte L, Vaerenberg M. Fetal ascites associated with an imperforate hymen: sonographic observation. Ultrasound Obstet Gynecol. 1998;12:67-69. 5. Aygun C, Ozkaya O, Ayyyldyz S, et al. An unusual cause of acute renal failure in a newborn: hydrometrocolpos. Pediatr Nephrol. 2006;21:572-573. 6. Shen MC, Yang LY. Imperforate hymen complicated with pyocolpos and lobar nephronia. J Chin Med Assoc. 2006;69:224-227. 7. Posner JC, Spandorfer PR. Early detection of imperforate hymen prevents morbidity from delays in diagnosis. Pediatrics. 2005;115: 1008-1012. 8. Chuang YC, Kan YY. A girl with episodic abdominal pain. Lancet. 2007;369:1890. 9. Calvin JK, Nichamin SJ. Hematocolpos due to imperforate hymen. Am J Dis Child. 1936;51:832-846. 10. Ben Temime R, Najar I, Chachia A, et al. Imperforate hymen: a series of 13 cases. Tunis Med. 2010;88(3):168-171. [article in French]. 11. Brevetti LS, Kimura K, Brevetti GR, et al. Pyocolpos: diagnosis and treatment. J Pediatr Surg. 1997;32:110-111. 12. Benitez Navio J, Casanueva LT, Laguna Urraca G, et al. Right hematocolpos and hematometra with left renal agenesis. Arch Esp Urol. 1993;46:824-827. [article in Spanish]. 13. Kumar A, Mittal M, Prasad S, et al. Haematocolpos: an uncommon cause of lower abdominal pain in adolescent girls. J Indian Med Assoc. 2002;100:240-241. 14. Basaran M, Usal D, Aydemir C. Hymen sparing surgery for imperforate hymen: case reports and review of literature. J Pediatr Adolesc Gynecol. 2009;22(4):61-64. 15. Liang CC, Chang SD, Soong YK. Long-term follow-up of women who underwent surgical correction for imperforate hymen. Arch Gynecol Obstet. 2003;269:5-8.
UROLOGY 78 (1), 2011
A
cute urinary retention (AUR) in children is a relatively rare entity but constitutes a genitourinary emergency. The etiology of AUR is agedependent. AUR in childhood can result from congenital anomalies, neurological disorders, lower urinary tract stones, urinary tract infection, constipation, adverse drug effect, inflammatory disorders, obstructing lesions, and iatrogenic or psychogenic disorders.1,2 Hematocolpos is defined as the accumulation of menstrual blood in the vagina, instead of its expulsion, and usually presents with intermittent lower abdominal pain. It is usually to the result of an imperforate hymen, which is a rare congenital obstructive abnormality of the female genital tract, with a reported occurrence of 1 in 2000 girls.3 To our knowledge, we report the first case of an adolescent girl with recurrent acute urinary retention as a result of hematocolpos associated with imperforate hymen.
CASE In September 2009, a 14-year-old girl was admitted to the pediatric emergency department with a 12-hour history of acute urinary retention, accompanied by dysuria and severe lower abdominal pain. There was no history of nausea, vomiting, altered bowel habits, or fever. She had not been taking any medications recently, including an-
From the Department of Urology, Soroka University Medical Center, Ben Gurion University, Faculty of Health Sciences, Beer-Sheva, Israel; and Department of Diagnostic Imaging, Soroka University Medical Center, Ben Gurion University, Faculty of Health Sciences, Beer-Sheva, Israel Reprint requests: Eran Rosenberg, M.D., Department of Urology, Soroka University Medical Center, Beer-Sheva 84105, Israel. E-mail: [email protected]; [email protected] Received: July 28, 2010, accepted (with revisions): October 14, 2010
180
© 2011 Elsevier Inc. All Rights Reserved
tihistamines and anticholinergic drugs. The patient had not yet started her menses. On physical examination, her vital signs were normal. Secondary sexual characteristics were present. An abdominal mass extending from the pelvis to the umbilicus was noted. A pelvic examination was not performed in the emergency department. Urethral catheterization was administered for relief of the symptoms and a total of 700 mL of clear urine was drained. Urinalysis was normal. Transabdominal ultrasound showed significant echogenic fluid accumulation in the vagina, mild dilation of the uterus (hematometra), a full bladder with clear fluid (Figs. 1 and 2), and mild bilateral hydronephrosis. Because the patient was in acute distress, she was taken urgently to the operating room. Because of the patient’s cultural background and the importance of keeping the integrity of the hymen, simple vertical incision of the hymen was performed under general anesthesia. Approximately 600 mL of dark red menstrual blood was drained from the vagina. The symptoms resolved after treatment and she was discharged 2 days later. There is no record of a follow-up. Six months later, in April 2010, the patient was admitted again to the pediatric emergency department with similar complaints of acute urinary retention, dysuria, and lower abdominal pain for 3 days. Again, no other symptoms were reported and her vital signs were normal. On palpitation, a large mass corresponding to the size of a 12-14 weeks gravid uterus was found. On inspection done by the gynecologist, the hymen was found to be scarred and was bulging forwards. Transabdominal ultrasonography showed similar sonographic findings suggestive of hematocolpos. The patient was again taken urgently to the operating room and hymenotomy was performed under general 0090-4295/11/$36.00 doi:10.1016/j.urology.2010.10.022
Figure 1. Longitudinal view of the pelvis, demonstrating uterus of normal size and configuration, mild enlargement of uterine cavity. Large, anechogenic mass with involvement of the entire vagina (hematocolpos).
Figure 2. Transverse view showing dense fluid in the enlarged vagina compressing the bladder.
anesthesia by a cruciate incision. The edges were everted. Approximately 500 mL of dark red blood was drained. Approximately 600 cm2 of urine was also drained upon catheterization. The postoperative period was uneventful and the patient was discharged 2 days later. At 6-week postoperative follow-up, the patient still had not had a menstrual period but did not have any difficulty in urination. Pelvic examination revealed a normal sized hymenal orifice.
COMMENT The hymen is a balance sheet of mesoderm that is perforated normally during the later stages of embryonic development.3 Imperforate hymen is usually an isolated anomaly, although associated malformations, especially those of the genitourinary tract, have been reported. Most cases reported in the literature are sporadic; however, some familial cases have been described as presumptive of probable genetic predisposition.3 UROLOGY 78 (1), 2011
During the neonatal period, imperforate hymen may present with fetal ascites or acute renal failure.4,5 The hematocolpos or hydrocolpos may lead to variable degrees of hydroureter, and hydronephrosis.6 Diagnosis should ideally be done at birth by careful examination of the external genitalia of all newborn females. If the diagnosis is not made in the newborn period and the hymen remains imperforate, the mucus will be reabsorbed and the child usually remains asymptomatic until menarche. The clinical symptoms of teenagers include cyclic lower abdominal pain, primary amenorrhea, chronic constipation, low back pain, dysuria, and acute urinary retention.7 Urinary retention may occur when the retained menstrual products in the vagina compress the urethra and there is angulation of the urethra caused by pressure on the posterior wall of the bladder, again by retained menstrual products. Chuang and Kan demonstrated the compressive effect of hematocolpometra to the urinary outlet by computerized tomography scans.8 The incidence of patients with imperforate hymen presenting with AUR varies, ranging from 3-46%.9,10 In a series of 26 cases of imperforate hymen reported by Calvin and Nichamin, 12 cases of the 26 (46%) presented with AUR,9 and in an another series reported by Ben Temime et al, 4 of 13 patients (30%) with imperforate hymen presented with a sudden onset of complete bladder retention.10 To date, at least 20 cases of imperforate hymen with AUR have been reported. Most of these reported cases presented in adolescence. The youngest patient was a 3-month-old girl who had suffered from repeated urinary tract infections because of urinary retention related to pyocolpos.11 Most cases had abnormal external genitalia in additional to a chief complaint of urinary retention. Two cases had concomitant urological abnormality, such as contralateral renal agenesis,12 or genetic disorder, such as ectrodactyly ectodermal dysplasia-clefting syndrome.13 All cases had an uneventful recovery after surgical treatment and none reported a failure of surgical treatment resulting in recurrence of urinary retention caused by hematocolpos, as described in our case. Urinary retention is always treated by catheterization. It is convenient and easy to observe the hymen at the time of catheterization. A bulge along the posterior aspect of the introitus is typical. A high index of suspicion makes early diagnosis easier and prevents inappropriate laboratory work or imaging studies. Ultrasound is the preferred radiological method in the diagnosis of hematocolpos. Studies show that transrectal ultrasound is more effective than transabdominal ultrasound. Magnetic resonance imaging may be required in cases in which complicated obstructive abnormalities are suspected and ultrasound is insufficient. Standard treatment is surgical hymenectomy with T, X, plus, or cruciform incisions and removal of excess hymenal tissue.14 Because the hymen is a symbol of virginity in some communities, its destruction can be a source of social problems for some girls. There was a 181
recent report of 2 cases with imperforate hymen treated with a simple vertical incision,14 and in both cases, postoperative follow-up was uneventful. In our case, the girl underwent a similar “hymen-sparing procedure” but reperfusion of the hymen had occurred. Therefore, although the outcome after adequate hymenotomy for imperforate hymen is usually excellent, follow-up is still necessary to ensure that there is no recurrence. In addition, clinicians must keep in mind that with adequate surgery, abnormal menstruation or persistent problems of micturition/defecation seldom recur.15
CONCLUSION Although hematocolpos associated with imperforate hymen and urinary retention is uncommon, it should be included in the differential diagnosis in adolescent females with acute abdominal pain and urinary retention. Inspection of the external genitalia, along with catheterization and detailed history-taking, with emphasis on menstruation, can aid in preventing misdiagnosis and commencing with additional radiological diagnostic examinations and appropriate surgical treatment. Moreover, conservative surgery with a simple incision and suturing might be an alternative option to standard treatment for imperforate hymen, but follow-up is necessary to ensure that there is no re-closure of the hymen that might result in recurrence of urinary retention. References 1. Gatti JM, Perez-Brayfield M, Kirsch AJ, et al. Acute urinary retention in children. J Urol. 2001;165:918-921.
182
2. Asgari SA, Mansour Ghanaie M, et al. Acute urinary retention in children. Urol J. 2005;2(1):23-27. 3. Sakalkale R, Samarakkody U. Familial occurrence of imperforate hymen. J Pediatr Adolesc Gynecol. 2005;18:427-429. 4. Jacquemyn Y, De Catte L, Vaerenberg M. Fetal ascites associated with an imperforate hymen: sonographic observation. Ultrasound Obstet Gynecol. 1998;12:67-69. 5. Aygun C, Ozkaya O, Ayyyldyz S, et al. An unusual cause of acute renal failure in a newborn: hydrometrocolpos. Pediatr Nephrol. 2006;21:572-573. 6. Shen MC, Yang LY. Imperforate hymen complicated with pyocolpos and lobar nephronia. J Chin Med Assoc. 2006;69:224-227. 7. Posner JC, Spandorfer PR. Early detection of imperforate hymen prevents morbidity from delays in diagnosis. Pediatrics. 2005;115: 1008-1012. 8. Chuang YC, Kan YY. A girl with episodic abdominal pain. Lancet. 2007;369:1890. 9. Calvin JK, Nichamin SJ. Hematocolpos due to imperforate hymen. Am J Dis Child. 1936;51:832-846. 10. Ben Temime R, Najar I, Chachia A, et al. Imperforate hymen: a series of 13 cases. Tunis Med. 2010;88(3):168-171. [article in French]. 11. Brevetti LS, Kimura K, Brevetti GR, et al. Pyocolpos: diagnosis and treatment. J Pediatr Surg. 1997;32:110-111. 12. Benitez Navio J, Casanueva LT, Laguna Urraca G, et al. Right hematocolpos and hematometra with left renal agenesis. Arch Esp Urol. 1993;46:824-827. [article in Spanish]. 13. Kumar A, Mittal M, Prasad S, et al. Haematocolpos: an uncommon cause of lower abdominal pain in adolescent girls. J Indian Med Assoc. 2002;100:240-241. 14. Basaran M, Usal D, Aydemir C. Hymen sparing surgery for imperforate hymen: case reports and review of literature. J Pediatr Adolesc Gynecol. 2009;22(4):61-64. 15. Liang CC, Chang SD, Soong YK. Long-term follow-up of women who underwent surgical correction for imperforate hymen. Arch Gynecol Obstet. 2003;269:5-8.
UROLOGY 78 (1), 2011