181 © 2018 Annals – 181 – 14:178 178 21 November 2017 14: 2018, accepted Ann Pediatr Surg 17 August 2017 management. of Pediatric Surgery. Keywords: cervical agenesis, cervicouterineprimary transection, amenorrhea, hematometra, uterine injury Department of Obstetrics andUniversity, Gynecology, Jeddah, Faculty Saudi of Arabia Medicine, King Abdulaziz Correspondence to Ettedal A.Department Aljahdali, of MBBCh, Obstetrics SBOG, andPO Gynecology, CBG Box King OBGYN 80215, Abdulaziz AFSA, JeddahTel: University + 21589, 966 Hospital, Saudi 504 Arabia 637 282; e-mail: [email protected] Received Dilated uterus full of blood. Fig. 1 Annals of Pediatric Surgery The examination of thewith hysteroscopy showed patient a normal under vaginal shape generalwith and size, anesthesia a normalthus, cervical canal using threeattempted intraoperative centimeters with in US, length; aposterior cervical perforation Hegar was dilatation dilator. diagnosedlaparoscopy by was was During US performed, and the showing immediate abilateral procedure, bulky distended uterus with multiple tubes endometriotic and spots inminimal fused the pelvic uterovesial fimbrial pouch adhesions. and posteriorly ends The just Hegar and at dilatorwhich the was was removed level placed under of directbe vision. the No observed. bleeding uterosacral could During ligament, tube, manipulation some of of theirrigation the left old was collected fallopian performed,observed blood at the came and site out. of again theinserted Suction perforation. and A no suction the drain procedure was bleedinglaparotomy was and completed was uterocervical with reconstruction a later. plan for Three weeks later, shejunction was reconstruction. admitted An for cervicouterine abdominovaginal approach 1 cm in length. The rest of ∼ 2018 Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited. r Copyright 6].Inthispaper,themanagementofthefirstcaseof – 1687-4137 © 2018 Annals of Pediatric Surgery DOI: 10.1097/01.XPS.0000525974.71558 the study was normal innodes terms of (Fig. other organsanalysis and 2). were lymph normal Hormonal as well. profile and chromosomal Case report A 14-year-old Saudi girlUniversity was in referred to Jeddahamenorrhea King from Abdulaziz with Taifwith cyclic as the a abdominal transabdominallocal case hospital pain ultrasound showing of hematometra (US)Patient for primary and consent report hematosalpinx. 1 was from parents. acquired year, from On thesexual examination, patient and characteristics: she the axillary breast had and Tanner normal pubichymen, stage secondary hair; and vaginal 3; andhistory, opening. normal normal she According external had to3.5 genitalia, her a years. medical traumatic A car carpelvic accident drove fractures, over at hermechanical the leading pelvis, ventilation age causing of with to multiple bleeding hematuria admission and thatconservative per to treatment continued vaginal only for ICU for 2 months inTransabdominal and 1 the US hospital. and week; MRIenlarged of she uterus the pelvis with received indicatednormal hematometra an ovaries and (Fig.canal hematosalpinx, 1), with normalcervicouterine vagina, an junction and area a cervical of stenosis or agenesis at the Introduction Cervical developmental pathology is a verywith rare atresia condition, being soassociated far with vaginal reported atresia. in Clinical diagnosis is raredifficult usually cases before and often vaginal surgery. agenesis is Transverse alsoincomplete a vaginal fusion rare between septum condition the thatMüllerian vaginal or ducts components results and of from the the PhallicClinical part of presentation the depends urogenital on sinus. complete. whether Complete agenesis it leads to is themenstrual accumulation partial of blood or in the[1 uterus and thetraumatic structures cervicouterine transection above is reported. it Cervical agenesis is oneanomalies of that the can Müllerian occur developmental vaginal and atresia is rarely usually isolated. associated Herethat we with has are been reporting referred a as case a cervical cervicouterine agenesis transection, and so found far to notWe be reported report in a literature. caseteenager of patient traumatic who cervicouterine presented transectionhematometra. with in She amenorrhea was and primarilyto investigated have and intact found fulland length urinary cervical system. canal, Operative normalour management uterus, diagnosis confirmed of transectionher rather history than of agenesis trauma with our at case the with age medical of diagnostic 3 years. approach We and report Ettedal A. Aljahdali Unusual traumatic uterine injury:cervicouterine first transection reported 178 Case report

Downloaded from https://journals.lww.com/aps by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3l7ttZ9b/VuKxIwH3Dy/2pqEl0VxTbhh37J87j9nSKYU= on 08/07/2018 Downloaded from https://journals.lww.com/aps by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3l7ttZ9b/VuKxIwH3Dy/2pqEl0VxTbhh37J87j9nSKYU= on 08/07/2018 Cervicouterine transection Aljahdali 179 was planned. After laparotomy, both the uterus and the probe was inserted through the incision and directed fallopian tubes were distended with old menstrual blood, toward the cervix to dilate the fibrous area. At the same the peritoneum was dissected, and bladder was pushed time, a transvaginal small dilator was inserted through the down to expose the anterior uterine wall and well- cervical canal and forced through the fibrous transected developed lower uterine part and full cervix with normal end, and then gradual dilatation of both fibrous ends till a thickness and length with a small area of fibrosis at the size 16 F, folley catheter was passed through the cervix to junction between the cervix and the uterus about 1-cm in the uterine cavity (Fig. 4). The uterine incision was length. This finding indicated the transection of the closed and then the cervicouterine junction was sutured uterus at the level of the internal cervical ostium, which with multiple interrupted stitches (Fig. 5). Medium means that the uterus was transected at the time of the suction soft drain was inserted into the pelvic cavity. The car accident that she had at the age of 3 (Fig. 3). procedure was completed and the uterine catheter was kept inside for 1 month with antibiotics coverage. A longitudinal incision on the lower anterior uterine wall was performed, old blood was drained out, and a uterine Fig. 4

Fig. 2

Uterus and cervix with the catheter (arrows) passing and connecting both. MRI pelvis showing the dilated uterus with normal cervical length and a small area of fibrosis in between (arrow). Fig. 5

Fig. 3

Hegar dilator passing through the cervix at the level of the internal OS (arrow) to the uterine cavity. Anastomosis of the uterus with the cervix.

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Fig. 6 Fig. 7

Catheter passing through the cervix to the uterine cavity with the dye showing normal uterine cavity.

During her postoperative period, she was stable, afebrile with no pain, and there was visibility of menstrual blood The dye is passing out through the vagina. through the uterocervical stent. After 1 month, the patient was admitted for removal of the catheter, with an examination under anesthesia. The intraoperative catheter Although different surgical techniques are reported for the could be removed easily with minimal bloody discharge, management of cervical agenesis, the method of choice and washing with saline was performed, followed by remains controversial [9]. Hysterectomy was the management hysterosalpingogram, which showed a normal cervical canal for these cases before because of the complication of cervical in continuity with the uterine cavity (Figs 6 and 7). recanalization and the difficulty of having normal viable pregnancy [10]. Improved surgical techniques and preserva- Discussion tion of the uterus for future fertility are considered to be the first line of treatment at present [11]. In this case, we report a We report this case as an unusual presentation of primary traumatic cervicouterine transection that was repaired with amenorrhea with hematometra likely because of cervical anastomosis of the transected part over the Foley’s catheter, agenesis. On review of the literature, cervical agenesis or which kept in as a stent to promote epithelization of the aplasia is a rare congenital variation of Müllerian cervicouterine junction and prevent stenosis or obstruction. anomalies and commonly associated with partial or Follow-up of these patients is recommended as restenosis or complete vaginal atresia, agenesis, or septum [7]. These obstruction can occur again as reported by others [10–12]. patients usually present after puberty with cyclic abdominal pain with different-size pelviabdominal masses depending on the time of presentation after Conclusion puberty. US is a less reliable imaging tool, but still Cervical agenesis isolated or with different degrees of remains the first method for diagnosis. However, MRI is vaginal aplasia is rare and always reported as the known to be the best method for diagnosis in these cases; congenital cause of primary amenorrhea and hematome- still, it is difficult to differentiate between complete tra in adolescent patients. In our patient, traumatic agenesis and dysgenesis of the cervix, and the diagnosis misdiagnosed uterocervical transection during her child- needs to be confirmed intraoperatively [8]. In our hood was the diagnosis on the basis of her history and patient, the MRI (Fig. 2), with her clinical history of intraoperative findings. To our knowledge, this is the trauma with documented different pelvic organ injuries first reported case of cervicouterine obstruction because at the age of 3 years with bleeding per vagina for a period of traumatic cervicouterine transaction injury. of time, and considering that only conservative manage- ment had been followed, misdiagnosis of uterine Conflicts of interest transection was expected. This diagnosis was supported There are no conflicts of interest. by our intraoperative findings of adhesions between the base of the bladder and the cervicouterine area and References cervix, and also normal cervical length and patent canal 1 Lodi A. Contributo clinic statistic malformazionidella vagina osservatenella clinica with a normal uterus and other pelvic organs. Ginecologica di Milano dal 1906 al 1950. Ann Obstet Gine 1951; 73:1246.

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2 Opoku BK, Djokoto R, Owusu-Bempah A, Amo-Antwi K. Huge abdominal Oxford desk reference: obstetrics and gynaecology. Oxford: Oxford mass secondary to transverse vaginal septum and cervical dysgenesis. University Press; 2011. p. 533. Ghana Med J 2011; 45:174–176. 8 Kobayashi A, Fukui A, Funamizu A, Kobayashi A, Fukui A, Funamizu A, Ito A, 3 Ribeiro SC, Yamakami LY, Tormena RA, Pinheiro Wda S, Almeida JA Fukuhara R, Mizunuma H, et al. Laparoscopically assisted cervical Baracat EC, et al. Septate uterus with cervical duplication and canalization and neovaginoplasty in a woman with cervical atresia and vaginal longitudinal vaginal septum. Rev Assoc Med Bras 2010; 56: aplasia. Gynecology and minimally invasive therapy 2017; 6:31–33. 254–256. 9 Falcone T, Hurd WW. Assisted reproductive technology: clinical aspects. In: 4 Poll LW, Flake P. Images in clinical medicine. Imperforate hymen with Clinical reproductive medicine and surgery: a practical guide. Basel: hematocolpometra. N England J Med 2011; 365:157. Springer Science + Business Media; 2017. pp. 299–315. 5 Reed MH, Griscom NT. Hydrometrocolpos in infancy. Am J Roentgenol 10 Al-Jaroudi D, Saleh A, Al-Obaid S, Agdi M, Salih A, Khan F, et al. Pregnancy Radium Ther Nucl Med 1973; 118:1–13. with cervical dysgenesis. Fertil Steril 2011; 96:1355–1356. 6 Jain N, Gupta A, Kumar R, Minj A. Complete imperforate tranverse vaginal 11 Shah TN, Venkatesh S, Saxena RK, Pawar S. Uterovaginal anastomosis for septum with septate uterus: a rare anomaly. J Hum Reprod Sci 2013; complete cervical agenesis and partial vaginal agenesis: a case report. Eur J 6:74–76. Obstet Gynecol Reprod Biol 2014; 174:154–155. 7 Arulkumaran S, Regan L, Papageorghiou A, Monga A, Farquharson D. 12 Tan Y, Bennett MJ. Urinary catheter stent placement for treatment of cervical Editors. Common gynaecological procedures and surgery. Jump up to: a b n. stenosis. Aust N Z J Obstet Gynaecol 2007; 47:406–409.

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