Uterus Didelphys With an Obstructed Hemivagina and Ipsilateral Renal Agenesis: A Rare Cause of Acute Abdomen in Pubertal Girls By K.G. Skondras, C.C. Moutsouris,
G.C. Vaos, G.C. Barouchas, and L.D. Demetriou
Athens, Greece l The unique clinical syndrome of uterus didelhphys with an obstructed hemivagina and ipsilateral renal agenesis, presenting as acute abdomen in three pubertal girls, is reported. Accurate preoperative diagnosis and appropriate treatment will offer relief of symptoms and decrease morbidity and unnecessary procedures. Copyright o 1991 by W.6. Saunders Company
obstructed hemivagina so that both uterine cavities opened into a larger single vagina. The vaginal septum was also excised. Postoperative excretory urography (IVP) showed absence of the left kidney, which was confirmed by renal scan. The patient had an uneventful recovery and she was free of symptoms 2 years after operation. Vaginoscopy showed free communication between the two hemivaginas.
INDEX WORDS: Uterus, abnormalities; vagina, abnormalities; kidney, abnormalities; abdomen, acute.
Case 2
U
TERUS DIDELPHYS with an obstructed hemivagina is always associated with renal agenesis, mostly ipsilateral to the blind vaginal pouch.“2 A review of the English-language literature shows only 119 previously reported cases of this complex malformation. An early diagnosis is possible in young girls who complain of steadily worsening dysmenorrhea and intermittent lower abdominal pain between menses and present with a pelvic mass.2 However, no case of acute abdomen as the initial clinical presentation of uterus didelphys with an obstructed hemivagina in pubertal girls has been reported so far. In the present report, three such cases are discussed and the importance of early diagnosis and treatment of this uncommon disease complex is stressed. CASE
REPORTS
Case 1 A 13-year-old girl was admitted to the hospital because of severe left lower quadrant abdominal pain. Clinical presentation had started 6 days earlier during menstruation with lower abdominal pain, pronounced dysmenorrhea, and increasing fever. Clinical examination showed a tender and firm large mass extending from the symphysis pubis to the left upper quadrant of the abdomen. Pelvic ultrasonography showed a cystic mass in the left hemipelvis. Laparotomy showed a uterus didelphys and a hemorrhagic and necrotic left ovarian mass. After removal of this mass, hematometrocolpos and hematosalpinx of the left half of the uterus didelphys were identified (Fig 1A). Diagrammatic representation of underlying pathology is demonstrated in Fig 1B. The left salpinx was removed. An opening was made at the lower blind end of the
From the Department of Paediatric Surgery, Aglaia Kyriakou Children’s Hospital, Athens, Greece. Date accepted: May 22, 1990. Address reprint requests to Kostas G. Skondras, MD, Second Depatiment of Paediatric Surgery, Aglaia Kyriakou Children’s Hospital, Athens I15 27, Greece. Copyright o 1991 by W.B. Saunders Company 0022-3468/9112615-0015$03.oOiO
1200
A 12-year-old girl was admitted to the hospital complaining of severe lower abdominal pain, high fever (39S”C) and vomiting. She had normal and regular menstruai period, with the exception of some discomfort at onset of menstruation. Rectal examination showed a hard mass that filled the pelvis. At laparotomy the right ovary and tube were found to be necrotic. A right salpingooophorectomy was performed. A uterus didelphys and a cystic mass between bladder and vagina, which was considered to be a hematometrocolpos, were also identified. An opening was made at the lower blind end of the imperforate hemivagina and the medial vaginal septum was excised. Postoperatively, IVP showed agenesis of the right kidney. The patient had an uneventful recovery and she left the hospital on the 15th postoperative day. Dilatation of the newly created intravaginal opening was performed at weekly intervals for a period of 3 months. Two years later she was menstruating normally.
Case 3 A 12-year-old girl was admitted to the hospital complaining of persisting severe lower abdominal cramps and high fever (38S”C). For the last 2 months she had an intermittent lower abdominal ache that worsened at onset of menstruation. On rectal examination, a tender, firm, right-sided pelvic mass was detected. Pelvic and abdominal ultrasonography demonstrated a cystic and solid mass displacing the uterus to the left. At laparotomy a hemorrhagic and necrotic cystic mass of the right ovary and tube were identified. A right salpingooophorectomy was carried out. A hematometrocolpos of the right half of a uterus didelphys was drained throught its vaginal wall and a thick imperforate medial vaginal septum was excised. Postoperatively the right kidney was not visualized on IVP. The patient had an uneventful recovery and left the hospital on the 12th postoperative day. She was menstruating normal within 6 months following operation. DISCUSSION
Uterus didelphys with an obstructed hemivagina has been recorded in the gynecological literature.‘.’ The obstructed hemivagina will result in symptomatic ipsilateral hematometrocolpos and hematosalpinx. Occasionally, patients present with symptoms from the urinary tract.4 Acute urinary retention as the initial symptom of uterus didelphys with an obstructed hemivagina has also been reported.5 In
JournalofPediafricSurgery,
Vol26,No lO(October),1991:pp1200-1201
UTERUS DIDELPHYS
WITH HEMATOCOLPOS
1201
Fig 1. (A) Uterus didelphys (ud) with left hematosalpinx (hs). (6) Diagram of uterus didelphys with left hematometrocolpos hematosalpinx. Note obstructed hemivagina (oh) and hemorrhagic left ovary (ov).
extremely rare cases, necrosis and rupture of the hematosalpinx may result in a typical peritonitis, as in the cases reported herein. All of the patients presented with acute abdomen and underwent emergency operations. The presence of a paravaginal cystic mass containing old blood and associated with ipsilateral renal agenesis in a patient who complains of worsening dysmenorrhea since menarche is almost pathognomonic for this disease complex.6 Excision of the vaginal septum is recommended by most investigators as the procedure of choice.7-9 A high rate of successful full-term pregnancies, ranging
(hmc) and
from 33% to 70%, has been reported with this minor surgery, whereas a low rate of 13% follows major operations.4,6 However, hemihysterectomy is recommended in patients with a high, thick-walled obstruction, massive ovarian involvement, endometriosis, and adenomyosis.’ Careful follow-up is essential. The solitary kidney is often diseased.” Pyocolpos due to closure of the septal incision’ and dysmenorrhea due to cervical stenosis” have been reported. These patients should also be watched for complications of endometriosis’ and vaginal adenosis.13
REFERENCES 1. Woolf RB. Allen WM: Concominant malformations. The frequent simultaneous occurrence of congenital malformations of the reproductive and urinary tracts. Obstet Gynecol 2:236-265, 1953 2. Rock JA, Jones HW Jr: The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol 138:339-342,198O 3. Tridenti G, Armanetti M, Flisi M, et al: Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis in teenagers: Report of three cases. Am J Obstet Gynecol 159:882-883,1988 4. Allan N, Cowan LE: Uterus didelphys with unilateral imperforate vagina. Obstet Gynecol22:422-426, 1963 5. Adams WG, Wilson EC, Holloway HJ, et al: Uterus didelphys with unilateral imperforate vagina: A rare cause of acute urinary retention. J Urol 121:131-132, 1979 6. Gilliland B, Dyck F: Uterus didelphys associated with unilateral imperforate vagina. Obstet Gynecol48:%-8s, 1976 (suppl)
7. Anderson KA, McAninch JW: Uterus didelphia with left hematocolpos and ipsilateral renal agenesis. J Ural 127:550-553, 1982 8. Burbige KA, Hensle TW: Uterus didelphys and vaginal duplication with unilateral obstruction presenting as a newborn abdominal mass. J Urol 132:1195-1198, 1984 9. Radhakrishnan J, Reyes HM: Unilateral renal agenesis with hematometrocolpos: Report of two cases. J Pediatr Surg 18:749750,1982 10. Ashley DJB, Mostofi FK: Renal agenesis and dysgenesis. J Urol83:211-230, 1960 11. Westerhout FC Jr, Hodgman JE, Anderson GV, et al: Congenital hydrocolpos. Am J Obstet Gynecol89:957-959,1964 12. Venter PF, Theron MS: Double uterus with a unilateral blind vagina: A report of three cases. S Afr Med J 59:838-840, 1981 13. Hansen K, Egholm M: Ditfirse vaginal adenosis. Acta Obstet Gynecol Stand 54:287-292.1975
G.C. Vaos, G.C. Barouchas, and L.D. Demetriou
Athens, Greece l The unique clinical syndrome of uterus didelhphys with an obstructed hemivagina and ipsilateral renal agenesis, presenting as acute abdomen in three pubertal girls, is reported. Accurate preoperative diagnosis and appropriate treatment will offer relief of symptoms and decrease morbidity and unnecessary procedures. Copyright o 1991 by W.6. Saunders Company
obstructed hemivagina so that both uterine cavities opened into a larger single vagina. The vaginal septum was also excised. Postoperative excretory urography (IVP) showed absence of the left kidney, which was confirmed by renal scan. The patient had an uneventful recovery and she was free of symptoms 2 years after operation. Vaginoscopy showed free communication between the two hemivaginas.
INDEX WORDS: Uterus, abnormalities; vagina, abnormalities; kidney, abnormalities; abdomen, acute.
Case 2
U
TERUS DIDELPHYS with an obstructed hemivagina is always associated with renal agenesis, mostly ipsilateral to the blind vaginal pouch.“2 A review of the English-language literature shows only 119 previously reported cases of this complex malformation. An early diagnosis is possible in young girls who complain of steadily worsening dysmenorrhea and intermittent lower abdominal pain between menses and present with a pelvic mass.2 However, no case of acute abdomen as the initial clinical presentation of uterus didelphys with an obstructed hemivagina in pubertal girls has been reported so far. In the present report, three such cases are discussed and the importance of early diagnosis and treatment of this uncommon disease complex is stressed. CASE
REPORTS
Case 1 A 13-year-old girl was admitted to the hospital because of severe left lower quadrant abdominal pain. Clinical presentation had started 6 days earlier during menstruation with lower abdominal pain, pronounced dysmenorrhea, and increasing fever. Clinical examination showed a tender and firm large mass extending from the symphysis pubis to the left upper quadrant of the abdomen. Pelvic ultrasonography showed a cystic mass in the left hemipelvis. Laparotomy showed a uterus didelphys and a hemorrhagic and necrotic left ovarian mass. After removal of this mass, hematometrocolpos and hematosalpinx of the left half of the uterus didelphys were identified (Fig 1A). Diagrammatic representation of underlying pathology is demonstrated in Fig 1B. The left salpinx was removed. An opening was made at the lower blind end of the
From the Department of Paediatric Surgery, Aglaia Kyriakou Children’s Hospital, Athens, Greece. Date accepted: May 22, 1990. Address reprint requests to Kostas G. Skondras, MD, Second Depatiment of Paediatric Surgery, Aglaia Kyriakou Children’s Hospital, Athens I15 27, Greece. Copyright o 1991 by W.B. Saunders Company 0022-3468/9112615-0015$03.oOiO
1200
A 12-year-old girl was admitted to the hospital complaining of severe lower abdominal pain, high fever (39S”C) and vomiting. She had normal and regular menstruai period, with the exception of some discomfort at onset of menstruation. Rectal examination showed a hard mass that filled the pelvis. At laparotomy the right ovary and tube were found to be necrotic. A right salpingooophorectomy was performed. A uterus didelphys and a cystic mass between bladder and vagina, which was considered to be a hematometrocolpos, were also identified. An opening was made at the lower blind end of the imperforate hemivagina and the medial vaginal septum was excised. Postoperatively, IVP showed agenesis of the right kidney. The patient had an uneventful recovery and she left the hospital on the 15th postoperative day. Dilatation of the newly created intravaginal opening was performed at weekly intervals for a period of 3 months. Two years later she was menstruating normally.
Case 3 A 12-year-old girl was admitted to the hospital complaining of persisting severe lower abdominal cramps and high fever (38S”C). For the last 2 months she had an intermittent lower abdominal ache that worsened at onset of menstruation. On rectal examination, a tender, firm, right-sided pelvic mass was detected. Pelvic and abdominal ultrasonography demonstrated a cystic and solid mass displacing the uterus to the left. At laparotomy a hemorrhagic and necrotic cystic mass of the right ovary and tube were identified. A right salpingooophorectomy was carried out. A hematometrocolpos of the right half of a uterus didelphys was drained throught its vaginal wall and a thick imperforate medial vaginal septum was excised. Postoperatively the right kidney was not visualized on IVP. The patient had an uneventful recovery and left the hospital on the 12th postoperative day. She was menstruating normal within 6 months following operation. DISCUSSION
Uterus didelphys with an obstructed hemivagina has been recorded in the gynecological literature.‘.’ The obstructed hemivagina will result in symptomatic ipsilateral hematometrocolpos and hematosalpinx. Occasionally, patients present with symptoms from the urinary tract.4 Acute urinary retention as the initial symptom of uterus didelphys with an obstructed hemivagina has also been reported.5 In
JournalofPediafricSurgery,
Vol26,No lO(October),1991:pp1200-1201
UTERUS DIDELPHYS
WITH HEMATOCOLPOS
1201
Fig 1. (A) Uterus didelphys (ud) with left hematosalpinx (hs). (6) Diagram of uterus didelphys with left hematometrocolpos hematosalpinx. Note obstructed hemivagina (oh) and hemorrhagic left ovary (ov).
extremely rare cases, necrosis and rupture of the hematosalpinx may result in a typical peritonitis, as in the cases reported herein. All of the patients presented with acute abdomen and underwent emergency operations. The presence of a paravaginal cystic mass containing old blood and associated with ipsilateral renal agenesis in a patient who complains of worsening dysmenorrhea since menarche is almost pathognomonic for this disease complex.6 Excision of the vaginal septum is recommended by most investigators as the procedure of choice.7-9 A high rate of successful full-term pregnancies, ranging
(hmc) and
from 33% to 70%, has been reported with this minor surgery, whereas a low rate of 13% follows major operations.4,6 However, hemihysterectomy is recommended in patients with a high, thick-walled obstruction, massive ovarian involvement, endometriosis, and adenomyosis.’ Careful follow-up is essential. The solitary kidney is often diseased.” Pyocolpos due to closure of the septal incision’ and dysmenorrhea due to cervical stenosis” have been reported. These patients should also be watched for complications of endometriosis’ and vaginal adenosis.13
REFERENCES 1. Woolf RB. Allen WM: Concominant malformations. The frequent simultaneous occurrence of congenital malformations of the reproductive and urinary tracts. Obstet Gynecol 2:236-265, 1953 2. Rock JA, Jones HW Jr: The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol 138:339-342,198O 3. Tridenti G, Armanetti M, Flisi M, et al: Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis in teenagers: Report of three cases. Am J Obstet Gynecol 159:882-883,1988 4. Allan N, Cowan LE: Uterus didelphys with unilateral imperforate vagina. Obstet Gynecol22:422-426, 1963 5. Adams WG, Wilson EC, Holloway HJ, et al: Uterus didelphys with unilateral imperforate vagina: A rare cause of acute urinary retention. J Urol 121:131-132, 1979 6. Gilliland B, Dyck F: Uterus didelphys associated with unilateral imperforate vagina. Obstet Gynecol48:%-8s, 1976 (suppl)
7. Anderson KA, McAninch JW: Uterus didelphia with left hematocolpos and ipsilateral renal agenesis. J Ural 127:550-553, 1982 8. Burbige KA, Hensle TW: Uterus didelphys and vaginal duplication with unilateral obstruction presenting as a newborn abdominal mass. J Urol 132:1195-1198, 1984 9. Radhakrishnan J, Reyes HM: Unilateral renal agenesis with hematometrocolpos: Report of two cases. J Pediatr Surg 18:749750,1982 10. Ashley DJB, Mostofi FK: Renal agenesis and dysgenesis. J Urol83:211-230, 1960 11. Westerhout FC Jr, Hodgman JE, Anderson GV, et al: Congenital hydrocolpos. Am J Obstet Gynecol89:957-959,1964 12. Venter PF, Theron MS: Double uterus with a unilateral blind vagina: A report of three cases. S Afr Med J 59:838-840, 1981 13. Hansen K, Egholm M: Ditfirse vaginal adenosis. Acta Obstet Gynecol Stand 54:287-292.1975
