{"paper_id":"5a018c8e-7733-42d5-9bdb-317020a2a6ff","body_text":"Kobe University Repository : Kernel\nPDF issue: 2026-06-09\nDienogest Use for Recurrent Pyosalpinx as a\nLong-Term Complication of Cloacal Exstrophy: A\nCase Report\n(Citation)\nJournal of Pediatric and Adolescent Gynecology,36(3):328-330\n(Issue Date)\n2023-06\n(Resource Type)\njournal article\n(Version)\nAccepted Manuscript\n(Rights)\n© 2023 North American Society for Pediatric and Adolescent Gynecology.\nThis manuscript version is made available under the Creative Commons Attribution-\nNonCommercial-NoDerivatives 4.0 International license.\n(URL)\nhttps://hdl.handle.net/20.500.14094/0100482082\nYoshimura, Shohei \n; Okata, Yuichi \n; Iwabuchi, Serena \n; Watanabe, Aya \n;\nUemura, Kotaro \n; Tomioka, Yuichiro \n; Samejima, Yoshitomo \n; Bitoh, Yuko\n\n\nDienogest use for recurrent pyosalpinx as a long- term complication of cloacal \nexstrophy: A case report \nShohei Yoshimura, Yuichi Okata, Serena Iwabuchi, Aya Watanabe, Kotaro Uemura, \nYuichiro Tomioka, Yoshitomo Samejima, Yuko Bitoh \nDivision of Pediatric Surgery, Department of Surgery, Kobe University Graduate School \nof Medicine, Kobe, Japan \n \nCorresponding Author \nShohei Yoshimura \nDivision of Pediatric Surgery, Department of Surgery, Kobe University Graduate School \nof Medicine \n7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan \nPhone: +81-78-382-5942 \nFax: +81-78-382-5959 \nE-mail: khrrou0245yoshimuu@gmail.com \n \nDisclosure \nAll authors declare no conflict of interest. \n\n \nFinancial support \nNone of the authors received any specific grants from funding agencies in the public, \ncommercial, or not-for-profit sectors. \n \nPatient consent \nPatient consent is not required because no personal information or details are included. \n\n 1 \nAbstract 1 \nBackground 2 \nCloacal exstrophy (CE) is a rare congenital disease that requires multiple surgeries  for 3 \ncomplex gastrointestinal and genitourinary anomalies. Long-term complications are not 4 \nuncommon; however, they are poorly reported. Pyosalpinx is sometimes encountered 5 \nduring CE management in adolescents and young adults. 6 \nCase 7 \nA 28-year-old woman with a history of CE presented with fever, lower abdominal pain, 8 \nand vomiting and was diagnosed with left pyosalpinx. Computed tomography- guided 9 \ndrainage and intravenous antibiotic administration w ere successful; however, she had 2 10 \nreadmissions for recurrent pyosalpinx 1 week after discharge and again 4 months later. 11 \nShe was administered Dienogest, a synthetic progestin, to prevent recurrent pyosalpinx 12 \nand had no recurrence for 8 months. 13 \nSummary and conclusion 14 \nDienogest is a conservative treatment choice for preventing the recurrence of pyosalpinx 15 \nfor CE patients. 16 \n 17 \nKey Words: Cloacal exstrophy; Dienogest; Long-term complication; Pelvic 18 \n\n 2 \ninflammatory disease; Pyosalpinx 19 \n  20 \n\n 3 \nIntroduction 21 \nCloacal exstrophy (CE) is a rare congenital condition observed in 1 of 200,000-400,000 22 \nlive births  (1, 2) . CE remains a challenging disease compromising multiple 23 \ngastrointestinal, genitourinary, and skeletal anomalies; however, its survival rate is now 24 \napproximately 100% resulting from improved neonatal care and surgical management (1). 25 \nConversely, CE patients experience many genitourinary and gastrointestinal problems 26 \nfrom childhood to young adulthood. In particular, most females with  CE are prone to 27 \ngynecological complications throughout life  due to congenital anomalies of the vagina 28 \nand uterus, and their long-term outcomes are poorly described in living adults (2, 3). 29 \nPyosalpinx is a severe sequela of pelvic inflammatory disease (PID) in which the fallopian 30 \ntubes are filled with pus.  PID is an ascending infection from the vagina or cervix to the 31 \nupper genital tract and  is attributed to Chlamydia trachomatis , Neisseria gonorrhoeae, 32 \nbacterial vaginosis, and enteric pathogens  (4). CE patients have been reported to 33 \nexperience recurrent PID, such as pyosalpinx or  tubo-ovarian abscess (TOA ) and are 34 \noften difficult to treat (3, 5). 35 \nHere, we present a case of a young woman with recurrent pyosalpinx following CE 36 \nmanagement in childhood and adolescence. Dienogest (DNG), a synthetic progestin, was 37 \neffective in controlling recurrent pyosalpinx; thus , the pathogenesis of recurrent 38 \n\n 4 \npyosalpinx in CE patients and the efficacy of DNG are discussed. 39 \n 40 \nCase 41 \nA 28-year-old woman with a history of CE presented with fever, lower abdominal pain, 42 \nand vomiting and was transferred to our hospital with a diagnosis of left pyosalpinx. She 43 \nwas born with a weight of 2622 g at 36 weeks of gestation by cesarean section and was 44 \ndiagnosed with CE. She underwent primary closure of CE and ileostomy on day 4 of life 45 \nand continent urinary diversion by right to left transureteroureterostomy with cutaneous 46 \nright ureterostomy, augmentation cystoplasty using an ileal patch, and intes tinal 47 \nvaginoplasty at the age of 5 years. Her first menstruation was at age 12, but due to 48 \ndysmenorrhea, she underwent a right hemi-hysterectomy for uterine didelphys and left 49 \nuterine to fallopian tube anastomosis at the age of 15 years. After that, her condition was 50 \nwell controlled in the outpatient clinic  without medication . She managed her bowel 51 \nmovements with colostomy and required clean intermittent catheterization four times a 52 \nday. She was sexually active and had sexual intercourse with her partner one week before 53 \nadmission. 54 \nLaboratory evaluation at admission revealed a C-reactive protein level of 17.52 mg/dl and 55 \na creatinine level of 1.07 mg/dl , suggesting acute kidney injury . Enhanced abdominal 56 \n\n 5 \ncomputed tomography (CT) showed a 79×52 mm left pyosalpinx and multiple small 57 \nabdominal abscesses, leading to bilateral hydronephrosis and vomiting due to intestinal 58 \nobstruction (Fig. 1) . CT-guided drainage  was performed for the left pyosalpinx. T he 59 \nbrown pus contained Streptococcus anginosus , Streptococcus agalactiae , and 60 \nBacteroides fragilis. Samples collected from the cervix tested negative for Chlamydia 61 \ntrachomatis and Neisseria gonorrhoeae. Renal function improved within normal limits , 62 \nand symptoms of fever, abdominal pain, and vomiting disappeared a few days after 63 \ndrainage. Conservative antibiotics (intravenous tazobactam/piperacillin and penicillin G 64 \nfor a total of 2 weeks ; thereafter, oral amoxicillin/c lavulanate) were administered  65 \nsuccessfully, the drain was removed on day 21, and she was discharged on day 25. 66 \nShe had 2 readmissions which were 1 week after discharge, and then again 4 months later 67 \nfor recurrent pyosalpinx. During these hospitalizations, only intravenous antibiotics were 68 \nadministered. Abdominal ultrasonography ( US) at the second hospitalization showed 69 \nhydrosalpinx and anastomotic stenosis between the left uterus and left fallopian tube, 70 \nwhich suggested a cause of recurrent pyosalpinx (Fig. 2A). A transvaginal approach using 71 \nhysteroscopy for releasing the anastomotic stenosis between the left uterus and fallopian 72 \ntube was considered; however, it was  difficult due to a complicated surgical history . 73 \nTherefore, the decrease in menstrual fluid in the left fallopian tube may have prevented 74 \n\n 6 \nrecurrent pyosalpinx, and in the third hospitalization, she started DNG at 2 mg daily orally. 75 \nAfter DNG administration, fluid collection in the left fallopian tube completely 76 \ndisappeared on abdominal US ( Fig. 2B), and s he had no recurrence for 8 months  after 77 \nDNG administration. 78 \n 79 \nSummary and conclusion 80 \nIn this case,  a young woman following CE management in childhood and adolescence 81 \nsuffered from recurrent pyosalpinx, and D NG, a synthetic progestin, was effective in 82 \npreventing recurrent pyosalpinx. Only one young adolescent girl with a history of CE has 83 \nbeen reported to experience pyosalpinx and TOA and undergo a radical hysterectomy (5). 84 \nThus, this is the first report to treat pyosalpinx as a long- term complication during CE 85 \nmanagement successfully. 86 \nCE is a challenging disease that requires multiple surgeries for complex gastrointestinal, 87 \ngenitourinary, and skeletal anomalies. Because of the improvements in neonatal care and 88 \nsurgical techniques, the survival rate has been approaching 100% in the last few decades 89 \n(2, 3). However, as long-term survivors of CE have become more common, CE patients 90 \nhave been found to suffer from many gynecological disorders. A recent systematic review 91 \nreported that 57.1% of female CE patients had vaginal -related issues and that 14.3% to 92 \n\n 7 \n71.0% had uterine anomalies, such as uterine didelphys and uterine bipartitus (2). Owing 93 \nto these anomalies , many females with CE experienced dysmenorrhea during puberty, 94 \nwith related gynecologic surgery performed in approximately two- thirds of cases (2). In 95 \nour case, right hemi -hysterectomy and left uterus -fallopian tube anastomosis were 96 \nperformed for the management of dysmenorrhea, and recurrent pyosalpinx occurred as a 97 \nlong-term complication of the anastomosis. 98 \nOn the other hand, regarding upper genital tract tissues, including PID such as pyosalpinx 99 \nand TOA in CE patients, no detailed observational studies and only limited case report s 100 \nhave been found (5). PID is one of the most common gynecological problems in young 101 \nwomen (4), and it  must be considered a major problem that reduces quality of life , 102 \nespecially in CE patients. 103 \nPyosalpinx is a severe form of PID in which the fallopian tube is filled with pus. 104 \nTreatment of pyosalpinx ranges from conservative intravenous antibiotics and image -105 \nguided drainage to laparoscopic aspiration, salpingostomy, and salpingectomy. 106 \nUrogenital anomalies, including CE, have been reported to be one of the risk factors for 107 \npyosalpinx, and surgical treatment is performed in most cases (5, 6). In our case, the 108 \nprimary treatment for pyosalpinx was successful by CT-guided drainage and intravenous 109 \nantibiotics administra tion; however, pyosalpinx re curred twice. On the abdominal US 110 \n\n 8 \nfindings at recurrence,  the cause of recurrent pyosalpinx was suspected to be  left 111 \nhydrosalpinx resulting from stenosis of the uterus-fallopian tube anastomosis, which is a 112 \nlong-term complication of CE management. 113 \nDNG is a unique fourth-generation synthetic progestogen mainly used for the long-term 114 \nmanagement of endometriosis worldwide. It is considered effective in decreasing the size 115 \nof endometriomas and reducing endometriosis-associated pain (7). In addition, it has less 116 \nsevere side effects, such as abnormal uterine bleeding and headache, with long-term use 117 \ncompared with other progestin products (8). In cloacal anomal ies, ovarian function is 118 \nnormal; thus, ovary-releasing estrogen stimulate s the endomet rium in the uterus  after 119 \npuberty, and some experience menstrual flow obstruction (3). Hormonal suppression of 120 \nendometrial stimulation and menses prevents the continued accumulation of obstructed 121 \nmenstrual products in CE patients (3). In this patient, hormonal suppression by DNG may 122 \nhave played an important role in reducing menstrual blood flow, which contributed to  123 \nreduced fluid accumulation in the left fallopian tube and controlled recurrent pyosalpinx 124 \nwithout severe side effects. 125 \nIn conclusion, patients  with CE often  experience gynecological problems as long-term 126 \ncomplications. Pyosalpinx in patients with CE is a major issue that can lead to multiple 127 \nhospitalizations and reduce the patient’s quality of life. DNG is a conservative treatment 128 \n\n 9 \noption for recurrent pyosalpinx as a long-term complication of CE. 129 \n  130 \n\n 10 \nReferences 131 \n1. Phillips TM. (ed.) Spectrum of cloacal exstrophy. Seminars in pediatric surger: 132 \nElsevier; 2011. 133 \n2. Musleh L, Privitera L, Paraboschi I, Polymeropoulos A, Mushtaq I, Giuliani S. 134 \nLong-term active problems in patients with cloacal exstrophy: a systematic review. J 135 \nPediatr Surg. 2022;57(3):339–347. 136 \n3. Breech L. Gynecologic concerns in patients with cloacal anomaly. Semin Pediatr 137 \nSurg. 2016;25(2):90–95. 138 \n4. Brunham RC, Gottlieb SL, Paavonen J. Pelvic inflammatory disease. N Engl J 139 \nMed. 2015;372(21):2039–2048. 140 \n5. Naiditch JA, Radhakrishnan J, Chin AC, Cheng E, Yerkes E, Reynolds M. Fate 141 \nof the uterus in 46XX cloacal exstrophy patients. J Pediatr Surg. 2013;48(10):2043–2046. 142 \n6. Fumino S, Iwai N, Tokiwa K, Hibi M, Iwabuchi T. Tubo- ovarian abscess after 143 \ncolonic vaginoplasty for high cloacal anomaly in a 13- year-old girl. Eur J Pediatr Surg. 144 \n2002;12(5):345–347. 145 \n7. Uludag SZ, Demirtas E, Sahin Y , Aygen EM. Dienogest reduces endometrioma 146 \nvolume and endometriosis-related pain symptoms. J Obstet Gynaecol. 2021;41(8):1246–147 \n1251. 148 \n\n 11 \n8. Vannuccini S, Clemenza S, Rossi M, Petraglia F. Hormonal treatments  for 149 \nendometriosis: the endocrine background. Rev Endocr Metab Disord. 2022;23(3):333–150 \n355 151 \n  152 \n\n 12 \nFigure 1 Enhanced abdominal CT findings at diagnosis 153 \nA: Axial imaging showing a 79×52 mm left pyosalpinx on the back of the bladder  and 154 \nmultiple small abdominal abscesses. 155 \nB: The coronal imaging revealed bilateral hydronephrosis associated with compression 156 \nby the pyosalpinx. 157 \nCT, computed tomography. 158 \n 159 \nFigure 2 Abdominal US findings before and after DNG administration 160 \nA: Before DNG administration, abdominal US showed left hydrosalpinx and suspected 161 \nanastomotic stenosis between the left uterus and left fallopian tube. 162 \nB: Under DNG administration, no fluid collection in the left fallopian tube was observed 163 \non abdominal US. 164 \nDNG, Dienogest; US, ultrasonography. 165","source_license":"CC0","license_restricted":false}