Vesicouterine Fistula – a Rare Delayed Complication of Cesarean Section S Akter Et Al Complications, Likeanaemia

On the basis of history, clinical examinations and it was soon found that labile factor and factor V were laboratory investigations- a diagnosis of Parahaemophilia one and same factor.1,3 was made. Patient was treated with FFP and The coagulation factors that function as enzymatically haematinics. Two weeks later, follow up physical active serine protease activators, factor VIII and examination and investigations showed disappearance factor V, are cofactors in the coagulation cascade. of ecchymosis, stoppage of per rectal bleeding and epistaxis with factor v level increasing up to 20% of Activated factor V is a cofactor in the conversion of normal range. She completely recovered after 04 prothrombin to thrombin by factor Xa in an action weeks having factor V level 30% and has been that are phospholipid and calcium dependent. The followed up at the out patient department. Family activator complex formed by factor Xa, activated screening of other family membersrevealed no factor V, phospholipid and calcium was called the coagulation abnormality. Due to non-availability, prothrombinase complex. Activation of factor V by mutation study was not performed. thrombin is achieved through a series of proteolytic cleavages. Activated factor V was inactivated by Discussion: activated protein C in a surface dependent reaction In 1943, Owren described a female patient who had a that requires calcium ion and protein S.9 bleeding diathesis attributable to the lack of single blood clotting factor, namely factor V or labile factor. Our patient presented with history and features which Owren designated the disease “parahemophilia,” to overlapped with the features of Haemophilia B and emphasize the clinical similarity to hemophilia. At Von Willebrand Disease. These overlapping features that time, only four coagulation factors were posed a diagnostic dilemma. Parahaemophilia is a considered: fibrinogen, calcium, prothrombin, milder disorder than other congenital haemorrhagic thrombokinase. After owren reported parahemophilia, disorder. Even though patient may develop

Journal of Bangladesh College of Physicians and Surgeons Vol. 38, No. 4, October 2020 complications, likeanaemia. If not timely diagnosed; 2. Charles WB: Parahaemophilia (Congenital Factor V did not complain of dysuria or lower abdominal pain. bladder either during uterotomy or uterine closure. b) In our case the supratrigonal vesicouterine fistula was 10. jόzwik M jόzwik M. clinical classification of 20. Moon SG , kim SH, Lee HJ, Moon MH, Myung JS. difficulty communicating with her classmates. Failed Written consent was taken from patient’s guardian to Deficiency). Can Med Assoc J. 1965; 92:979-981. it would have been fatal. She also did not give any history of urinary Inadequate downward mobilization of bladder or repaired through intraperitonial approach. In bladder, vesicouterine fistula. Int j gynecol Obst. 2000;70:353-7. Pelvic fistulas complicating pelvic surgery or diseases: eruption of her permanent teeth was the reason that publish her disease features and photographs. https://doi.org/10.1016/S0020-7292(00)00247-2 Factor V is labile coagulation factor which appears to 3. Gilbert C, White II. Coagulation factors V and VIII incontinence. Then she was given progesterone to direct injury to that portion of the bladder which spectrum of imaging findings. Korean J radiol the patient sought treatment. Her parents reported an Normal function and clinical disorder. In: Robert IH, fistula opening was closed in two layers after be necessary both for the production of fully potent Vesicouterine fistula – a rare delayed complication of stop menstruation for 2 months. At that time there sometimes adherent to the anterior vaginal wall or 11. Porcaro AB, Zicari M, Zecchini Antniolli S, Pianon R, (2001)2:97-104. https://doi.org/10.3348/kjr.2001.2.2.97, unremarkable medical history. She was in good Samuel EL, Thomas PS, eds. Blood, Principles & mobilization of uterine wall from bladder by intrinsic thromboplastin and for the rapid conversion lower segment wall5 , c) aberrant sutures placed so Monaco C, Migliorini F, et al. Vesicouterine fistulas PMid:11752977 PMCid:PMC2718108 health with no medications and no known allergies. practice of Hematology. 1995;1151-1179. cesarean section was no per vaginal bleeding or passage of bloody dissection and uterine opening was repaired in single following cesarean section: report on a case, review and of prothrombin to thrombin in the presence of tissue that a knuckle of bladder is caught in the sutures5. 21. Ali-El-Dein, El- Tabey N, El- Hefnawy A, Osman Y, She had no history of trauma to the mouth, teeth, or 4. Ruth AS: Parahemophilia. Medical Clinics of North urine. USG showed normal uterine and ovarian layer suture. There was no menouria after repair and update of the literature. Int urol Nephrol (2002) thromboplastin. Thus, the patients with Factor V S AKTERa, K BEGUMb, J SAHAc Repeated Cesarean sections may result in progressive Soliman S, Shaaban AA, Diagnosis , treatment and need jaws. America 1972;56:119-125. https://doi.org/10.1016/ S0025- structure, no stone in urinary bladder. Urine routine menses was commenced normally. 34:335-44. https://doi.org/10.1023/A:1024443822378, deficiency manifest impaired thromboplastin PMid:12899224 for hysterectomy in management of post cesarean section 7125(16)32427-0 microscopic examination revealed normal. devitalization and scarring of the uterus and bladder A general physical examination revealed a short- generation and prolongation of prothrombin time.10 base by damaging their vascular network, thus After correction of VUF, successful pregnancy vesico uterine fistula. Scand J Urol (2014) 48:460-5., 5. Miletich JP, Majerus DW, Majerus PW. Patients with Abstract: section. Diagnosis was confirmed by cystoscopy and 12. B.K. Park, S. H. Kim, J.Y. Cho, J.S.Sim and C.K. Seong, statured, well-oriented young girl with narrow, AFB was not found in endometrial tissue. Then predisposing to delayed fistula formation11. But to "Vesicouterine fistula after cesarean section: https://doi.org/10.3109/21681805.2014.903511, Diagnosis is based on prolonged prothrombin (PT) congenital factor V deficiency have decreased factor Xa A vesicouterine fistula is an abnormal pathway outcome was also reported. drooped shoulders (Figure-1). Further examination fistula was repaired through trans abdominal ultrasonographic findings in two cases," Journal of PMid:24694181 and activated partial thromboplastin times (APTT) binding sites on their platelets. J Clinic invest cystoscopic evaluation was done . Trigone was develop vesicouterine fistula after 8 years of LUCS showed an abnormal facility in opposing her 1978;62:824-831. https://doi.org/10.1172/JCI109194, between the urinary bladder and the uterus. It is a surgery. She regained normal vaginal menses 4 Conclusion: Ultrasound in Medicine, vol.18, no. 6, pp. 441443,1999. normal . There was a small opening about 1 mm in is most unlikely and rare. Her endometrial tissue and 22. Tancer ML. Observation on prevention and management shoulders (Figure 2) due to malformed or absent and on low plasma Factor V levels.Bleeding time PMid:701480; PMCid:PMC371834 rare urogynaecological fistula. In vesicouterine months after surgery. Vesicouterine fistula is a rare variety of genitourinary https://doi.org/10.7863/jum.1999.18.6.441, 11 fistula margin was send for histopathology but no of vesicovaginal fistula after total hysterectomy. Surg (BT) can be prolonged in severe cases. fistula, clinical presentations are cyclic haematuria supratrigone region of bladder. No feature of PMid:10361852 clavicles, which was later confirmed with a small 6. Moret A: Next generation sequencing in bleeding Keywords: vesicouterine fistula, delayed complication, granulomatous lesion was found. AFB stain was also fistula. Previously it was occurred after complicated Gynecol Obstet (1992) 175:501-6. Figure 3: X-ray Chest P/A view shows malformed Differential diagnoses include vWD, Haemophilia disorders: two novel variants in the F5 gene (Valencia-1 (menouria), amenorrhoea, urinary continence which granuloma was found. 13. Hadzi- Djokic JB, Pejcic TP, Colovic VC. Vesico-uterine malformed clavicle in chest radiograph (Figure 3). Youssef’s syndrome, cystoscopy. negative. vaginal delivery. Due to increasing trend of lower small clavicle. (homozygous type), inhibitors to coagulation factor, and Valencia-2) associated with mild factor V deficiency. mimics Youssef’s syndrome. This case presents, a fistula: Report of 14 cases. BJU int 2007;100:1361-3. 23. P. Narayanan, M. Nobbenhuis, K. M. Reynolds, A. Facial examination showed a brachycephalic head Methylene blue dye was pushed through cervix and it segment cesarean section, incidence of VUF also Sahdeb, R. H. Reznek, and A.G. Rockall, " fistulous in and very rarely combined deficiency of factor V and J Thromb Thrombolysis 2019 Nov;48(4). https://doi.org/ 37 year old multiparous woman with vesicouterine (J Bangladesh Coll Phys Surg 2020; 38: 209-212) If the diagnosis of VUF is early and the fistula https://doi.org/10.1111/j.1464-410X.2007.07067.x, with frontal and parietal bossing, hypoplasticzygomatic Figure 5: CBCT frontal view shows multiple 10.1007/s11239-019-01911-z, PMid:31267299 passed through the bladder opening. Then decision of rise. So surgeons should be more careful during PMid:17590179 malignant gynecologic disease; etiology, imaging and factor VIII. fistula which she developed 8 years after cesarean DOI: https://doi.org/10.3329/jbcps.v38i4.48984 presumed to be small, conservative measures with bones, bulging calvarium, and depressed nasal bridge laparotomy was taken. After opening the abdomen management," Radiographics, (2009) vol. 29;4:107383. impacted teeth. 7. Ding H:Phenotypic and genetic analysis of a pedigree prolonged catheterization with antibiotics and cesarean section to avoid complication. Patient must 14. El- Lamie I. urogenital fistulae: changing trends and with a broad alar base (Figure 2). Intraoral The level of factor V activity in thepatients’ plasma is bladder was found adherent with anterior uterine https://doi.org/10.1148/rg.294085223 PMid:19605657 Figure 8 X-ray Lateral Cephalogram shows normal affected with hereditary FV deficiency due to a novel anticholinergic for about 4 weeks have been reported be councelled about the consequence of repeat personal experience of 46 cases. Int urogynecol j Pelvic examination revealed persistence of the primary usually < 5% of that in normal plasma. Most bleeding deletional variant of F5 gene. Zhonghua Yi Xue Yi Introduction: has been known as Youssef’s syndrome; Type ii is floor Dysfunct (2008) 19:26772. https://doi.org/ maxilla with prognathic mandible. wall. About 1 mm of opening was found in body of to be effective and provides a cure rate in about 5% of cesarean section. 24. G. L. Brodhead . Spontaneous closure of large vesico-uterine dentition, delayed eruption of the permanent teeth, an episodes in patients with factor V deficiency can be Chuan Za Zhi 2019 Nov; 36(11). Vesicouterine fistula is a rare form of urogynaecological associated with dual menstrual flow via both the 10.1007/s00192-007-0426-5, PMid:17639345 uterus which communicated with base of bladder. 12,18,19 fistula, Medication reconciliation. (1920);98:437. Angle class III malocclusion, negative overjet, treated with sufficient fresh frozen plasma to raise the cases . Our patient had developed VUF , 8 References: 8. Tabibian S: A Comprehensive Overview of Coagulation fistula when compared with vesicovaginal fistula. It bladder and vagina and type iii is associated with Fistula was repaired. Catheter was kept in bladder for 15. Józwik M, Józwik M, LotockiW. Vesicouterine fistula-an bilateral posterior crossbite (Figure 4). 12 10 years after lower uterine cesarean section. Diagnosis 1. Iloabachie GC, Njoku O. Vesicouterine fistula. BRITISH 25. Rubino SM. Medical treatment of uterovesical fistula. plasma factor V level to 30%. Factor V and Congenital Factor V Deficiency. Semin represents about 1-4% of all cases of normal menses and lack of menouria . Usually, analysis of 24 cases from Poland. Int J Gynaecol Obstet Introduction: fontanelle and metopic sutures results in frontal Thromb Hemost 2019 July; 45(5). https://doi.org/ 1 21 days. Progesterone was given for 1 month to was made from history, clinical feature of Type 1 J Urol 1985;57:438-9. https://doi.org/10.1111/ j.1464-410X. Lancet 1977;309:900-0. https://doi.org/10.1016/ S0140-6736 urogynaecological fistula . About 83-93% of cases, it 1997;57(02): 169-172. https://doi.org/10.1016/ S0020-7292 1-3 Fresh frozen plasma (FFP) is the only treatment as immediate presentation occurs when there is direct 9 Cleidocranial dysplasia (CCD) is very uncommon bossing . 10.1055/s-0039-1687906, PMid:31121608 2,3,4 induce amenorrhoea. Urine colour was normal. Her Youssef’s syndrome and confirmed by cystoscopy 1985.tb06305.x, PMid:4027516 (97)02837-3 (77)91218-1 Factor V concentrates are not available. In acute is usually iatrogenic following cesarean section . injury to the bladder during surgery. Patients can autosomal inheritant congenital skeletal defect that 9. Desage S: The potential value of thrombin generation menstruation started 4 months after surgery. She also Figure 2- after excision of vesicouterine fistula which showed about 1 mm of fistula in supratrigone 2. Eisenkop SM, Richman R, Platt LD, Paul RH. Urinary The different clinical manifestations reflect the basic cases of severe bleeding, the addition of platelet Other causes of fistula may be due to following have early hematuria and/or urinary leakage, voiding 16. Yip SK, Leung TY. Vesicouterine fistula: an updated 26. Birge O, Ozbey EG, Erkam MM, Arslan D, Kayar i. primarily affects the bones and teeth. CCD is assay in the diagnosis of FV inhibitors. Int J lab hematol (VUF) Discussion : region of urinary bladder and dye test was positive. tract injury during cesarean section. Obst Gynecol mechanisms of skeletal development, patterning, induced abortion, obstructed labour, instrumental difficulty, low-grade pyrexia, urinary sepsis or be relieved of haematuria after surgery. review. Int urogynecol j pelvic Floor Dysfunct 1998;9:252-6. Youssef's syndrome following cesarean section. Case concentrates may be helpful.Prognosis is good with 2019 Oct; 41(5). https://doi.org/10.1111/ijlh.13027 1982;60:591-6 https://doi.org/10.1007/BF01901500, PMid:9849756 characterized by delayed closure of fontanelles, 8, 13 7 Other imaging procedure can be useful to fully reports. Obstet Gynecol 2015;2015:1- https://doi.org/ bone and cartilage formation, growth and early diagnosis and adequate treatment. delivery, vaginal birth after cesarean section, completely asymptomatic . Delayed presentation can The world health organization estimates 130,000 new presence of open skull sutures, hypoplastic or 4 10. Billoir P: Thrombin generation assay is a useful cases of urogenital fistula occuring per year. characterize the fistulous tract. But there is no clear 3. Buchholz NP, Daly- Grandeau E, Huber - buccholz MM. 17. Park OR, kim TS, Kim HJ. Sonographic diagnosis of 10.1155/2015/605325, PMid:26457214, PMCid:PMC4589585 homeostasis . The oral manifestations of CCD pre-operative tool to predict non-bleeding risk in a patient placenta percreta, brachytherapy, migrated intra occur when there is an infection or a progressive 20 aplastic clavicles, supernumerary teeth, delayed Figure 4: Intra-oral photograph shows bilateral Conclusion: 5 consensus on the best modality . Cystography, Urological complications associated with caesarean vesicouterine fistula . Ultrasound Obstet Gynecol (2003) include an underdeveloped maxilla with a high, with mild factor V deficiency.Transfus med 2020 Apr 6. uterine contraceptive devices . Less frequent causes devitalization of the posterior wall of the bladder11. Vesicouterine fistula represents only 2-9% of all 27. Bettez M, Breault G, Carr L, Tu Lm. Early versus crossbite, multiple retained deciduous teeth with hysterography, excretory urography, also ultrasonography section. Eur J obstet gynecol Biol 1994:56:161-3. 22:82-4. https://doi.org/10.1002/uog.161, PMid:12858310 eruption of permanent dentition, wide pubic narrow arched palate, prolonged retention of This is the first reported case on Parahaemophilia in https://doi.org/10.1111/tme.12680, PMid:32250000 are inflammatory bowel disease, endometriosis and urogenital delayed of vesicouterine fistula. J Can urol Assoc 2011; Patients with delayed presentation often have 21,22 https://doi.org/10.1016/0028-2243(94)90163-5 symphysis, short stature and a variety of other caries. 6,7 with or without intra uterine saline infusion can 18. Kilinc F , Bagis T, Guvel S, Egilmez T, ozkardes H. 5(4):457-9. https://doi.org/10.5489/cuaj.10065, PMid:21806894 deciduous teeth, failure of the secondary dentition to Bangladesh. Parahaemophilia is a very rare inherited bladder tuberculosis . Vesicouterine fistula was first 13, 14 11. Walker FJ, Sexton PW, Esmon CT. The inhibition of symptoms of urinary leakage from the vagina if the fistula . The frequency of vesicouterine fistulae in Unusual case of post- cesarean vesicputerine fistula skeletal changes. Delayed closure of the anterior erupt, delayed maturation among the permanent teeth Figure 6: CBCT occluso-palatal view shows coagulation disorder. If not investigated thoroughly, 8 complete the diagnostic workup. MRI has now 4. Yossepwitch O, Baniel J, Livne PM. Urological injuries PMCid:PMC3148396 A CBCT & panoramic X-ray show the presence of blood coagulation by activated protein C through the reported in the literature in 1908 by knipe . It was cervix is incompetent, cyclic hematuria (menouria), (Youssef's syndrome) int J Urol 2003; 10:236-8., 1, 5-7 relation to other urogenital fistula, as previously become the first choice in the investigation of during cesarean section: Intraoperative diagnosis and and multiple impacted supernumerary teeth. multiple supernumerary teeth in both jaw which are numerous supernumerary teeth which causes patients can be misdiagnosed. Screening test and selective inactivation of activated factor V. Biochi described by Youssef in 1957 as a clinical syndrome 11 15, 16 https://doi.org/10.1046/j.0919-8172.2003.00599.x, 28. K. Parveen, R. Gupta, Al=Badr and A.K. Hemal. Robot amenorrhea, infertility or first trimester abortion . reported, varies from 1% to 16.4% . It is very 23,24 management. J Urol 2004;172:196-9. https://doi.org/ impaction of multiple permanent tooth. associated coagulation factor assay should be done Biophys Acta 1979;71:333-342. https://doi.org/10.1016/ fistulas . PMid:12657106 assisted laparoscopic repair of rare post-cesarean section The incidence of CCD is one per million births. It 16 in number; 3 in both side of upper jaw, 6 in lower 0005-2744(79)90103-7 consisting of cyclic haematuria (menouria), amenorrhoea, Most of the cases present in delayed fashion, from likely that there is an increase in the relative 10.1097/01.ju.0000128632.29421.87, PMid:15201771 Figure 1: Frontal view shows narrow & dropped for proper diagnosis. Although factor V deficiency is 12 vesicocervical and vesicouterine fistula: a case series of a affects men and women with equal frequency. CCD left side & 4 in lower right side (Figure 5-7), and complete urinary incontinence in a patient who weeks to years after the inciting event . In such frequency of this type of fistula, because of the The definitive treatment for Youssef’s syndrome is 19. Bastakoti R, Saha R. Congervative management of shoulders, smile shows retained carious upper a milder disorder than Haemophilia A/B, patients 12. William HK, Earl WD. Blood coagulation Factor V and 9 5. Tancer ML. Vesico uterine fistula- a review. Obstet novel technique. Urology (2012) 80:477-82. https://doi.org/ appears spontaneously with no apparent genetic impaction of the most maxillary & mandibular had lower segment cesarean section . A suggested cases diagnosis can be done by detection of urine increase in cesarean section rates. Typically patient surgical. Conservative measure such as prolonged uterovesical fistula following primary cesarean section. anterior teeth resulting unpleasant smile. with factor V deficiency may sometimes require VIII: Structural and Functional Similarities and Their 18, 19 gynaecol Surv 1986;41:743-53. https://doi.org/10.1097/ 10.1016/j.urology.2012.04.027, PMid:22705111 nd classification of VUF, based on the routes of catheterization and medical management to JKMC 2014: 2:211-3. https://doi.org/10.3126/jkmc.v2i4.11799 cause in approximately 40% of affected patients, and permanent teeth, ectopic position of upper left 2 Relationship to Hemorrhagic and Thrombotic Disorders. colour after dye passing through cervical external os presents with cyclic haematuria or menouria, 00006254-198612000-00001, PMid:3540759 6,8 replacement therapy. With proper treatment; 10 one in three patients has unaffected parents . We nd Blood 1988;71:539-555. https://doi.org/10.1182/ blood.V71.3.539. menstrual flow proposed by Jόzwik and Jόzwik or by HSG or cystoscopy. Conservative treatment amenorrhoea and urinary continence, as described in cause induced amenorrhoea to help fistula healing premolar, dilacerations of lower left 2 molar and prognosis is good. If untreated, it can be fatal. 6. DiMarco CS, DiMarco DS, Klingel CL, Gebhart JB. herein report a case of CCD of a 13-year-old Figure 9: X-ray Frontal cephalogram shows bulging bloodjournal713539 in2000, divides vesicouterine fistula (VUF) into three may be approachable but surgery is the best option. 9 with oral contraceptive, progesterone and GnRH caries in several deciduous teeth. The Yossef’s syndrome . Our patient presented with Vesicouterine fistula: A review of eight cases. Int References: 13. Takemoto K:Challenges in management of unusual types. Type 1 is characterized by the triad of amenorrhoea analog have been reported in the literature with Bangladeshi girl& probably the first case from calvarium, narrow maxilla. We report a case of successful surgical repair of VUF these classical clinical features. Other presenting Urogynecol j Pelvic Floor Dysfunct 2006; 17:395-9., anterior-posterior (Figure 8) and lateral cephalogram 1. Owren PA: Parahemophilia, Hemorrhagic Diathesis Due acquired factor V deficiency: A case report. Medicine successful outcomes 25, 26 . Bangladesh to be documented in perspective to and menouria and the complete continence of urine which was developed after many years of cesarean complaints can include perineal irritation, vaginal https://doi.org/10.1007/s00192-005-0025-2, (Figure 9) show open sutures of the skull, large Discussion: to absence of previously unknown clotting factor. Lancet (Baltimore) 2019 Apr; 98(7). https://doi.org/10.1097/ dental surgery and orthodontics. section. fungal infection, and recurrent urinary tract Surgery can be performed transabdominally, endoscopically PMid:16523247 fontanelles, small maxillary sinuses, and a prognathic CCD is diagnosed by the clinical and radiological 1947;5:446-448.https://doi.org/10.1016/S0140-6736(47) MD.0000000000015259.PMid:31027075 a. Dr. Sumaya Akter, Assistant Professor. OBGYN 17 Case Presentation: findings. The clinical findings of CCD, although 91941-7 PMCid:PMC6831250 Case report: infections . or robotically. Transvaginal approach is less favored 7. Alkatib M , Franco AV, Fynes MM. Vesiciuterine fistula mandible. The cephalometric analysis confirmed the b. Prof. Kohinoor Begum, Professor, OBGYN A 13-year-old Bangladeshi girl was referred to the present at birth, could be easily missed because of Mrs “X”, 37 year old multipara came to our clinic Previously she had two lower segment cesarean because of higher location of the fistula and its following cesarean delivery-ultrasound diagnosis and class III skeletal Malocclusion. The anterior and c. Dr. Joysree Saha, Associate Professor, OBGYN 16 department of orthodontic, Sapporo dental College, their extremely low frequency, rare manifestation of with complaints of passage of bloody urine during section which is one of the important iatrogenic cause complexity . surgical management. Ultrasound Obstet Gynecol 2005; posterior cranial base are significantly reduce. 26:183-5. https://doi.org/10.1002/uog.1925, PMid:15997459 Dhaka, Bangladesh by a local dental surgeon. She the typical extraoral symptoms in early childhood, Adddress of Correspondence: Dr. Sumaya Akter, Affiliation : menstruation for 2 episodes following 3 months of of vesicouterine fistula9 . Probable factors which may Hysterectomy is not indicated for this treatment27. Increased horizontal mandibular growth is found presented with her mother with the chief complaint of and the clinical variety of the disorder 3, as was true Assistant Professor, OBGYN, Popular Medical College, Postal amenorrhoea. She had H/O repeat cesarean section 8 lead to formation of vesicouterine fistula after LUCS Disadvantage of the trans abdominal route are 8. Knipe WHW. Vesico-uterine fistula Am J Obstet Gynecol. along with anterior rotation of the mandible in delayed eruption of thepermanent anterior teeth. in the present case. Address: House #25, road#2, Dhanmondi-1205, Phone No: years back. There was no post operative complication are as follows – a) an inadequate reflection of the increased morbidity, long hospital stay and increased 1908;57:211-7. relation to the cranial base. The chest radiograph Figure 7: CBCT occluso-baccal view shows multiple 01711231534, Email : [email protected] Retained anterior deciduous teeth were carious & after surgery. She had no history of induced abortion bladder from the lower uterine segment during blood loss. These can be overcome by using 9. Youssef AF. Menouria following lower segment cesarean looked ugly that's why she was often teased at school. Figure 2: Close approximation of her shoulders in shows a very narrow thorax with oblique ribs and supernumerary teeth which causes impaction of A characteristic feature of CCD is aplastic or 28 Received: 20 January, 2020 Accepted: 13 July, 2020 or any other surgery in uterus after last LUCS. She Figure 1 - fistula between bladder base and uterus cesarean section is the main cause of injury of the endoscopic and robotic approach . section: a syndrome. Am J Obstet Gynecol. 1957;73:759-67 Her smile was very unpleasant, which resulted in front of the chest hypoplastic clavicles (Figure 3). permanent teeth. hypoplastic clavicleswhich is responsible for the 209

appearance of narrow, drooped shoulders and for the molecular genetic analysis was proposed. The 6. McNamara CM, O' Riordan BC, Blake M, Sandy JR. 12. Kreiborg S, Jensen BL, Larsen P, Schleidt DT, Darvann wide range of shoulder movement, resulting in the patient’s parents declined molecular genetic analysis Cleidocranial dysplasia: radiological appearances on T: Anomalies of craniofacial skeleton and teeth in dental panoramic radiology. DentomaxillofacRadiol. cleidocranial dysplasia. J Craniofac Genet Dev Biol. ability of the patient to approximate his or her for personal reasons; therefore, identification of the 2, 9,10 1999 Mar;28(2):89-97. https://doi.org/10.1038/sj.dmfr. 1999, 19: 75-79. shoulders in front of the chest . This ability is not responsible gene was not possible. 4600417.PMid:10522197 always recognized by the patient, as is true in the 13. Jensen BL, Kreiborg S: Development of the dentition in 7. Jensen BL, Kreiborg S. Craniofacial abnormalities in 52 cleidocranial dysplasia. J Oral Pathol Med. 1990, 19: present case. The successful treatment of patients with CCD requires a team approach and compliance of patient. schoolage and adult patients with cleidocranial dysplasia. 89-93.https://doi.org/10.1111/j.1600-0714.1990.tb00803. J Craniofac Genet Dev Biol. 1993 Apr-Jun;13(2):98-108. xPMid:2341976 The skull base is dysplastic with reduced growth, An interdisciplinary treatment approach involving resulting in increased skull width and leading to 8. Tanaka JLO, Ono E, Filho EM, Castilho JCM, Moraes 14. Modgil R, Arora KS, Sharma A, Mohapatra S, Pareek orthodontics, maxillofacial surgery, prosthodontics, brachycephaly and hypertelorism that are usually LC, Moraes MEL:Cleidocranial dysplasia: importance of S.Cleidocranial Dysplasia: Presentation of Clinical and pediatrics, neuropediatrics, ophthalmology, and associated with frontal and parietal bone bossing2. radographic images in diagnosis of the condition. J Oral Radiological Features of a Rare Syndromic Entity. orthopedics is obligatory17. Genetic counseling for Sci. 2006, 48: 161-166. https://doi.org/10.2334/josnusd. Mymensingh Medical Journal : MMJ, 2018, 27(2):424-428. The present case had the similar feature. 48.161.PMid:17023750 family planning should certainly be advised. Good 15. Garg RK, Agrawal P: Clinical spectrum of cleidocranial Midface deficiency; underdeveloped paranasal collaboration among the specialists, the patient, and 9. Suba Z, Balaton G, Gyulai-Gaal S, Balaton P, Barabas J, dysplasia: a case report. Cases J. 2008, 1: 377. sinuses; hypoplastic maxillary, nasal, and zygomatic the patient’s family is essential for an organized Tarjan I: Cleidocranialdyspasia: diagnostic criteria and https://doi.org/10.1186/1757-1626-1-377.PMid:1906371 bones; recession of the nasal bridge; a wide alar base; combined treatment. J Craniofac Surg. 2005, 16: 1122-1126. 7 PMCid:PMC2614945 treatment approach in which each member can https://doi.org/10.1097/01.scs.0000179747.75918.58. and prominent frontal, parietal, and occipital bones 16. Mohan RP, Suma GN, Vashishth S, Goel S: Cleidocranial contribute his or her expertise for the best treatment PMid:16327567 are common findings in patients with CCD. The dysplasia: clinicoradiological illustration of a rare case. J outcome 1, 10. The overall treatment goal is to underdeveloped maxilla with overall deficient 10. Chelvan HT, Malathi N, Kailasam V, Ponnudurai A: Oral Sci. 2010, 52: 161-166. doi: 10.2334/josnusd.52.161. establish functional occlusion and an aesthetic facial Cleidocranial dysplasia: A Family report. J Indian https://doi.org/10.2334/josnusd.52.161.PMid:20339249 growth of the midface combined with the direction of SocPedodPrev Dent. 2009, 27: 249-252. https://doi.org/ and dental appearance. Sometimes this disorder 17. Almeida LCA, Lima FBF, Matushita H, Valença mandibular condylar growth and its anterior rotation 10.4103/0970-4388.57661.PMid:19915277 causes psychological problems for the patients; MM,Castro TLF, Mendonça RN. Cleidocranial dysplasia, give the impression of relative or actual mandibular 11. Jensen BL, Kreiborg S: Craniofacial growth in a rare skeletal disorder with failure of the cranial closure: 2, 11, 12 therefore, proper motivation and support are prognathism . In the present case, the cleidocranialdysplaia- a roentgencephalometric study. J case-based update. Child's Nervous System(2020). doi: cephalometric analysis shows normal growth of the important. Craniofac Genet Dev Biol. 1995, 15: 35-43. org/10.1007/s00381-020-04831-z. midface but mandible is prognathic, resulting in a Conclusion: skeletal class III malocclusion. Clinician would be highly benefited by the Dental abnormalities are typical main features of knowledge of the clinical characteristics, family CCD, and they occur in 93.5% of affected patients 6. history, and diagnostic tools for CCD, that enable The primary dentition usually develops relatively them to achieve an early diagnosis and implement normally [13], while the permanent dentition is appropriate treatment to improve function and severely disturbed. Presence of supernumerary teeth, aesthetics. prolonged retention of the primary dentition, failed Reference: eruption of the permanent teeth, multiple crown and root abnormalities, crypt formation around impacted 1. Kolokitha OE, Papadopoulou AK: Cleidocranial dysplasia: Etiology, clinical characteristics, diagnostic teeth, and ectopic locations of teeth are the more information and treatment approach. Hell Orthod Rev. 9, 14 common dentition disturbances . A high arched 2008, 11: 21-33. palate is a less common oral manifestation of CCD. 2. Golan I, Baumert U, Hrala BP, Müssig D: Dentomaxillofacial In the present case, failed eruption of the permanent variability of cleidocranial dysplasia: Clinicoradiological teeth was the reason that the patient sought treatment. presentation of systemic review. J Dentomaxillofac She presented with multiple supernumerary teeth that Radiol. 2003, 32: 347-354. https://doi.org/10.1259/dmfr/ impeded normal eruption of the permanent teeth. 63490079. PMid:15070835 Other features seen in CCD are deformities of the 3. Golan I, Baumert U, Hrala BP, MubigD: Early craniofacial thoracic region, pelvic and pubic bones, and fingers. signs of cleidocranial dysplasia. Int J Pediatr Dent. 2004, More specifically, finger abnormalities include short, 14: 49-53.https://doi.org/10.1111/j.1365-263X. 2004. 00501. xPMid:14706028 tapered fingers and anomalies of the phalangeal, tarsal, metatarsal, carpal, and metacarpal bones 7, 9, 15, 16. 4. Mundlos S et al. Cleidocranial dysplasia: clinical and molecular genetics. J Med Genet. 1999 Mar;36(3): CCD is highly polymorphic; therefore, absolute 177-182. verification of the diagnosis of CCD can only be 5. Shaikh R, Shusterman S. Delayed dental maturation in 1–3 obtained by molecular genetic analysis . This cleidocranial dysplasia. ASDC J Dent Child. 1988 patient had not been previously diagnosed, a Sep-Oct;65(5):325-329. On the basis of history, clinical examinations and it was soon found that labile factor and factor V were laboratory investigations- a diagnosis of Parahaemophilia one and same factor.1,3 was made. Patient was treated with FFP and The coagulation factors that function as enzymatically haematinics. Two weeks later, follow up physical active serine protease activators, factor VIII and examination and investigations showed disappearance factor V, are cofactors in the coagulation cascade. of ecchymosis, stoppage of per rectal bleeding and epistaxis with factor v level increasing up to 20% of Activated factor V is a cofactor in the conversion of normal range. She completely recovered after 04 prothrombin to thrombin by factor Xa in an action weeks having factor V level 30% and has been that are phospholipid and calcium dependent. The followed up at the out patient department. Family activator complex formed by factor Xa, activated screening of other family membersrevealed no factor V, phospholipid and calcium was called the coagulation abnormality. Due to non-availability, prothrombinase complex. Activation of factor V by mutation study was not performed. thrombin is achieved through a series of proteolytic cleavages. Activated factor V was inactivated by Discussion: activated protein C in a surface dependent reaction In 1943, Owren described a female patient who had a that requires calcium ion and protein S.9 bleeding diathesis attributable to the lack of single blood clotting factor, namely factor V or labile factor. Our patient presented with history and features which Owren designated the disease “parahemophilia,” to overlapped with the features of Haemophilia B and emphasize the clinical similarity to hemophilia. At Von Willebrand Disease. These overlapping features that time, only four coagulation factors were posed a diagnostic dilemma. Parahaemophilia is a considered: fibrinogen, calcium, prothrombin, milder disorder than other congenital haemorrhagic thrombokinase. After owren reported parahemophilia, disorder. Even though patient may develop

Vesicouterine fistula – a rare delayed complication of cesarean section S Akter et al complications, likeanaemia. If not timely diagnosed; 2. Charles WB: Parahaemophilia (Congenital Factor V did not complain of dysuria or lower abdominal pain. bladder either during uterotomy or uterine closure. b) In our case the supratrigonal vesicouterine fistula was 10. jόzwik M jόzwik M. clinical classification of 20. Moon SG , kim SH, Lee HJ, Moon MH, Myung JS. difficulty communicating with her classmates. Failed Written consent was taken from patient’s guardian to Deficiency). Can Med Assoc J. 1965; 92:979-981. it would have been fatal. She also did not give any history of urinary Inadequate downward mobilization of bladder or repaired through intraperitonial approach. In bladder, vesicouterine fistula. Int j gynecol Obst. 2000;70:353-7. Pelvic fistulas complicating pelvic surgery or diseases: eruption of her permanent teeth was the reason that publish her disease features and photographs. https://doi.org/10.1016/S0020-7292(00)00247-2 Factor V is labile coagulation factor which appears to 3. Gilbert C, White II. Coagulation factors V and VIII incontinence. Then she was given progesterone to direct injury to that portion of the bladder which spectrum of imaging findings. Korean J radiol the patient sought treatment. Her parents reported an Normal function and clinical disorder. In: Robert IH, fistula opening was closed in two layers after be necessary both for the production of fully potent stop menstruation for 2 months. At that time there sometimes adherent to the anterior vaginal wall or 11. Porcaro AB, Zicari M, Zecchini Antniolli S, Pianon R, (2001)2:97-104. https://doi.org/10.3348/kjr.2001.2.2.97, unremarkable medical history. She was in good Samuel EL, Thomas PS, eds. Blood, Principles & mobilization of uterine wall from bladder by intrinsic thromboplastin and for the rapid conversion lower segment wall5 , c) aberrant sutures placed so Monaco C, Migliorini F, et al. Vesicouterine fistulas PMid:11752977 PMCid:PMC2718108 health with no medications and no known allergies. practice of Hematology. 1995;1151-1179. was no per vaginal bleeding or passage of bloody dissection and uterine opening was repaired in single following cesarean section: report on a case, review and of prothrombin to thrombin in the presence of tissue that a knuckle of bladder is caught in the sutures5. 21. Ali-El-Dein, El- Tabey N, El- Hefnawy A, Osman Y, She had no history of trauma to the mouth, teeth, or 4. Ruth AS: Parahemophilia. Medical Clinics of North urine. USG showed normal uterine and ovarian layer suture. There was no menouria after repair and update of the literature. Int urol Nephrol (2002) thromboplastin. Thus, the patients with Factor V Repeated Cesarean sections may result in progressive Soliman S, Shaaban AA, Diagnosis , treatment and need jaws. America 1972;56:119-125. https://doi.org/10.1016/ S0025- structure, no stone in urinary bladder. Urine routine menses was commenced normally. 34:335-44. https://doi.org/10.1023/A:1024443822378, deficiency manifest impaired thromboplastin 7125(16)32427-0 devitalization and scarring of the uterus and bladder PMid:12899224 for hysterectomy in management of post cesarean section A general physical examination revealed a short- 10 microscopic examination revealed normal. vesico uterine fistula. Scand J Urol (2014) 48:460-5., generation and prolongation of prothrombin time. 5. Miletich JP, Majerus DW, Majerus PW. Patients with base by damaging their vascular network, thus After correction of VUF, successful pregnancy 12. B.K. Park, S. H. Kim, J.Y. Cho, J.S.Sim and C.K. Seong, statured, well-oriented young girl with narrow, 11 https://doi.org/10.3109/21681805.2014.903511, Diagnosis is based on prolonged prothrombin (PT) congenital factor V deficiency have decreased factor Xa AFB was not found in endometrial tissue. Then predisposing to delayed fistula formation . But to outcome was also reported. "Vesicouterine fistula after cesarean section: drooped shoulders (Figure-1). Further examination binding sites on their platelets. J Clinic invest cystoscopic evaluation was done . Trigone was develop vesicouterine fistula after 8 years of LUCS ultrasonographic findings in two cases," Journal of PMid:24694181 and activated partial thromboplastin times (APTT) Conclusion: showed an abnormal facility in opposing her 1978;62:824-831. https://doi.org/10.1172/JCI109194, normal . There was a small opening about 1 mm in is most unlikely and rare. Her endometrial tissue and Ultrasound in Medicine, vol.18, no. 6, pp. 441443,1999. 22. Tancer ML. Observation on prevention and management and on low plasma Factor V levels.Bleeding time PMid:701480; PMCid:PMC371834 https://doi.org/10.7863/jum.1999.18.6.441, shoulders (Figure 2) due to malformed or absent Vesicouterine fistula is a rare variety of genitourinary of vesicovaginal fistula after total hysterectomy. Surg (BT) can be prolonged in severe cases.11 supratrigone region of bladder. No feature of fistula margin was send for histopathology but no PMid:10361852 clavicles, which was later confirmed with a small 6. Moret A: Next generation sequencing in bleeding granulomatous lesion was found. AFB stain was also fistula. Previously it was occurred after complicated Gynecol Obstet (1992) 175:501-6. Figure 3: X-ray Chest P/A view shows malformed Differential diagnoses include vWD, Haemophilia disorders: two novel variants in the F5 gene (Valencia-1 granuloma was found. 13. Hadzi- Djokic JB, Pejcic TP, Colovic VC. Vesico-uterine malformed clavicle in chest radiograph (Figure 3). negative. vaginal delivery. Due to increasing trend of lower small clavicle. (homozygous type), inhibitors to coagulation factor, and Valencia-2) associated with mild factor V deficiency. fistula: Report of 14 cases. BJU int 2007;100:1361-3. 23. P. Narayanan, M. Nobbenhuis, K. M. Reynolds, A. Facial examination showed a brachycephalic head Methylene blue dye was pushed through cervix and it segment cesarean section, incidence of VUF also Sahdeb, R. H. Reznek, and A.G. Rockall, " fistulous in and very rarely combined deficiency of factor V and J Thromb Thrombolysis 2019 Nov;48(4). https://doi.org/ If the diagnosis of VUF is early and the fistula https://doi.org/10.1111/j.1464-410X.2007.07067.x, with frontal and parietal bossing, hypoplasticzygomatic Figure 5: CBCT frontal view shows multiple 10.1007/s11239-019-01911-z, PMid:31267299 passed through the bladder opening. Then decision of rise. So surgeons should be more careful during PMid:17590179 malignant gynecologic disease; etiology, imaging and factor VIII. presumed to be small, conservative measures with bones, bulging calvarium, and depressed nasal bridge laparotomy was taken. After opening the abdomen management," Radiographics, (2009) vol. 29;4:107383. impacted teeth. 7. Ding H:Phenotypic and genetic analysis of a pedigree prolonged catheterization with antibiotics and cesarean section to avoid complication. Patient must 14. El- Lamie I. urogenital fistulae: changing trends and with a broad alar base (Figure 2). Intraoral The level of factor V activity in thepatients’ plasma is bladder was found adherent with anterior uterine https://doi.org/10.1148/rg.294085223 PMid:19605657 Figure 8 X-ray Lateral Cephalogram shows normal affected with hereditary FV deficiency due to a novel anticholinergic for about 4 weeks have been reported be councelled about the consequence of repeat personal experience of 46 cases. Int urogynecol j Pelvic examination revealed persistence of the primary usually < 5% of that in normal plasma. Most bleeding deletional variant of F5 gene. Zhonghua Yi Xue Yi Introduction: has been known as Youssef’s syndrome; Type ii is floor Dysfunct (2008) 19:26772. https://doi.org/ maxilla with prognathic mandible. wall. About 1 mm of opening was found in body of to be effective and provides a cure rate in about 5% of cesarean section. 24. G. L. Brodhead . Spontaneous closure of large vesico-uterine dentition, delayed eruption of the permanent teeth, an episodes in patients with factor V deficiency can be Chuan Za Zhi 2019 Nov; 36(11). Vesicouterine fistula is a rare form of urogynaecological associated with dual menstrual flow via both the 10.1007/s00192-007-0426-5, PMid:17639345 uterus which communicated with base of bladder. 12,18,19 fistula, Medication reconciliation. (1920);98:437. Angle class III malocclusion, negative overjet, treated with sufficient fresh frozen plasma to raise the cases . Our patient had developed VUF , 8 References: 8. Tabibian S: A Comprehensive Overview of Coagulation fistula when compared with vesicovaginal fistula. It bladder and vagina and type iii is associated with Fistula was repaired. Catheter was kept in bladder for 15. Józwik M, Józwik M, LotockiW. Vesicouterine fistula-an bilateral posterior crossbite (Figure 4). 12 10 years after lower uterine cesarean section. Diagnosis 1. Iloabachie GC, Njoku O. Vesicouterine fistula. BRITISH 25. Rubino SM. Medical treatment of uterovesical fistula. plasma factor V level to 30%. Factor V and Congenital Factor V Deficiency. Semin represents about 1-4% of all cases of normal menses and lack of menouria . Usually, analysis of 24 cases from Poland. Int J Gynaecol Obstet Introduction: fontanelle and metopic sutures results in frontal Thromb Hemost 2019 July; 45(5). https://doi.org/ 1 21 days. Progesterone was given for 1 month to was made from history, clinical feature of Type 1 J Urol 1985;57:438-9. https://doi.org/10.1111/ j.1464-410X. Lancet 1977;309:900-0. https://doi.org/10.1016/ S0140-6736 urogynaecological fistula . About 83-93% of cases, it 1997;57(02): 169-172. https://doi.org/10.1016/ S0020-7292 1-3 Fresh frozen plasma (FFP) is the only treatment as immediate presentation occurs when there is direct 9 Cleidocranial dysplasia (CCD) is very uncommon bossing . 10.1055/s-0039-1687906, PMid:31121608 2,3,4 induce amenorrhoea. Urine colour was normal. Her Youssef’s syndrome and confirmed by cystoscopy 1985.tb06305.x, PMid:4027516 (97)02837-3 (77)91218-1 Factor V concentrates are not available. In acute is usually iatrogenic following cesarean section . injury to the bladder during surgery. Patients can autosomal inheritant congenital skeletal defect that 9. Desage S: The potential value of thrombin generation menstruation started 4 months after surgery. She also Figure 2- after excision of vesicouterine fistula which showed about 1 mm of fistula in supratrigone 2. Eisenkop SM, Richman R, Platt LD, Paul RH. Urinary The different clinical manifestations reflect the basic cases of severe bleeding, the addition of platelet Other causes of fistula may be due to following have early hematuria and/or urinary leakage, voiding 16. Yip SK, Leung TY. Vesicouterine fistula: an updated 26. Birge O, Ozbey EG, Erkam MM, Arslan D, Kayar i. primarily affects the bones and teeth. CCD is assay in the diagnosis of FV inhibitors. Int J lab hematol (VUF) Discussion : region of urinary bladder and dye test was positive. tract injury during cesarean section. Obst Gynecol mechanisms of skeletal development, patterning, induced abortion, obstructed labour, instrumental difficulty, low-grade pyrexia, urinary sepsis or be relieved of haematuria after surgery. review. Int urogynecol j pelvic Floor Dysfunct 1998;9:252-6. Youssef's syndrome following cesarean section. Case concentrates may be helpful.Prognosis is good with 2019 Oct; 41(5). https://doi.org/10.1111/ijlh.13027 1982;60:591-6 https://doi.org/10.1007/BF01901500, PMid:9849756 characterized by delayed closure of fontanelles, 8, 13 7 Other imaging procedure can be useful to fully reports. Obstet Gynecol 2015;2015:1- https://doi.org/ bone and cartilage formation, growth and early diagnosis and adequate treatment. delivery, vaginal birth after cesarean section, completely asymptomatic . Delayed presentation can The world health organization estimates 130,000 new presence of open skull sutures, hypoplastic or 4 10. Billoir P: Thrombin generation assay is a useful cases of urogenital fistula occuring per year. characterize the fistulous tract. But there is no clear 3. Buchholz NP, Daly- Grandeau E, Huber - buccholz MM. 17. Park OR, kim TS, Kim HJ. Sonographic diagnosis of 10.1155/2015/605325, PMid:26457214, PMCid:PMC4589585 homeostasis . The oral manifestations of CCD pre-operative tool to predict non-bleeding risk in a patient placenta percreta, brachytherapy, migrated intra occur when there is an infection or a progressive 20 aplastic clavicles, supernumerary teeth, delayed Figure 4: Intra-oral photograph shows bilateral Conclusion: 5 consensus on the best modality . Cystography, Urological complications associated with caesarean vesicouterine fistula . Ultrasound Obstet Gynecol (2003) include an underdeveloped maxilla with a high, with mild factor V deficiency.Transfus med 2020 Apr 6. uterine contraceptive devices . Less frequent causes devitalization of the posterior wall of the bladder11. Vesicouterine fistula represents only 2-9% of all 27. Bettez M, Breault G, Carr L, Tu Lm. Early versus crossbite, multiple retained deciduous teeth with hysterography, excretory urography, also ultrasonography section. Eur J obstet gynecol Biol 1994:56:161-3. 22:82-4. https://doi.org/10.1002/uog.161, PMid:12858310 eruption of permanent dentition, wide pubic narrow arched palate, prolonged retention of This is the first reported case on Parahaemophilia in https://doi.org/10.1111/tme.12680, PMid:32250000 are inflammatory bowel disease, endometriosis and urogenital delayed of vesicouterine fistula. J Can urol Assoc 2011; Patients with delayed presentation often have 21,22 https://doi.org/10.1016/0028-2243(94)90163-5 symphysis, short stature and a variety of other caries. 6,7 with or without intra uterine saline infusion can 18. Kilinc F , Bagis T, Guvel S, Egilmez T, ozkardes H. 5(4):457-9. https://doi.org/10.5489/cuaj.10065, PMid:21806894 deciduous teeth, failure of the secondary dentition to Bangladesh. Parahaemophilia is a very rare inherited bladder tuberculosis . Vesicouterine fistula was first 13, 14 11. Walker FJ, Sexton PW, Esmon CT. The inhibition of symptoms of urinary leakage from the vagina if the fistula . The frequency of vesicouterine fistulae in Unusual case of post- cesarean vesicputerine fistula skeletal changes. Delayed closure of the anterior erupt, delayed maturation among the permanent teeth Figure 6: CBCT occluso-palatal view shows coagulation disorder. If not investigated thoroughly, 8 complete the diagnostic workup. MRI has now 4. Yossepwitch O, Baniel J, Livne PM. Urological injuries PMCid:PMC3148396 A CBCT & panoramic X-ray show the presence of blood coagulation by activated protein C through the reported in the literature in 1908 by knipe . It was cervix is incompetent, cyclic hematuria (menouria), (Youssef's syndrome) int J Urol 2003; 10:236-8., 1, 5-7 relation to other urogenital fistula, as previously become the first choice in the investigation of during cesarean section: Intraoperative diagnosis and and multiple impacted supernumerary teeth. multiple supernumerary teeth in both jaw which are numerous supernumerary teeth which causes patients can be misdiagnosed. Screening test and selective inactivation of activated factor V. Biochi described by Youssef in 1957 as a clinical syndrome 11 15, 16 https://doi.org/10.1046/j.0919-8172.2003.00599.x, 28. K. Parveen, R. Gupta, Al=Badr and A.K. Hemal. Robot amenorrhea, infertility or first trimester abortion . reported, varies from 1% to 16.4% . It is very 23,24 management. J Urol 2004;172:196-9. https://doi.org/ impaction of multiple permanent tooth. associated coagulation factor assay should be done Biophys Acta 1979;71:333-342. https://doi.org/10.1016/ fistulas . PMid:12657106 assisted laparoscopic repair of rare post-cesarean section The incidence of CCD is one per million births. It 16 in number; 3 in both side of upper jaw, 6 in lower 0005-2744(79)90103-7 consisting of cyclic haematuria (menouria), amenorrhoea, Most of the cases present in delayed fashion, from likely that there is an increase in the relative 10.1097/01.ju.0000128632.29421.87, PMid:15201771 Figure 1: Frontal view shows narrow & dropped for proper diagnosis. Although factor V deficiency is 12 vesicocervical and vesicouterine fistula: a case series of a affects men and women with equal frequency. CCD left side & 4 in lower right side (Figure 5-7), and complete urinary incontinence in a patient who weeks to years after the inciting event . In such frequency of this type of fistula, because of the The definitive treatment for Youssef’s syndrome is 19. Bastakoti R, Saha R. Congervative management of shoulders, smile shows retained carious upper a milder disorder than Haemophilia A/B, patients 12. William HK, Earl WD. Blood coagulation Factor V and 9 5. Tancer ML. Vesico uterine fistula- a review. Obstet novel technique. Urology (2012) 80:477-82. https://doi.org/ appears spontaneously with no apparent genetic impaction of the most maxillary & mandibular had lower segment cesarean section . A suggested cases diagnosis can be done by detection of urine increase in cesarean section rates. Typically patient surgical. Conservative measure such as prolonged uterovesical fistula following primary cesarean section. anterior teeth resulting unpleasant smile. with factor V deficiency may sometimes require VIII: Structural and Functional Similarities and Their 18, 19 gynaecol Surv 1986;41:743-53. https://doi.org/10.1097/ 10.1016/j.urology.2012.04.027, PMid:22705111 nd classification of VUF, based on the routes of catheterization and medical management to JKMC 2014: 2:211-3. https://doi.org/10.3126/jkmc.v2i4.11799 cause in approximately 40% of affected patients, and permanent teeth, ectopic position of upper left 2 Relationship to Hemorrhagic and Thrombotic Disorders. colour after dye passing through cervical external os presents with cyclic haematuria or menouria, 00006254-198612000-00001, PMid:3540759 6,8 replacement therapy. With proper treatment; 10 one in three patients has unaffected parents . We nd Blood 1988;71:539-555. https://doi.org/10.1182/ blood.V71.3.539. menstrual flow proposed by Jόzwik and Jόzwik or by HSG or cystoscopy. Conservative treatment amenorrhoea and urinary continence, as described in cause induced amenorrhoea to help fistula healing premolar, dilacerations of lower left 2 molar and prognosis is good. If untreated, it can be fatal. 6. DiMarco CS, DiMarco DS, Klingel CL, Gebhart JB. herein report a case of CCD of a 13-year-old Figure 9: X-ray Frontal cephalogram shows bulging bloodjournal713539 in2000, divides vesicouterine fistula (VUF) into three may be approachable but surgery is the best option. 9 with oral contraceptive, progesterone and GnRH caries in several deciduous teeth. The Yossef’s syndrome . Our patient presented with Vesicouterine fistula: A review of eight cases. Int References: 13. Takemoto K:Challenges in management of unusual types. Type 1 is characterized by the triad of amenorrhoea analog have been reported in the literature with Bangladeshi girl& probably the first case from calvarium, narrow maxilla. We report a case of successful surgical repair of VUF these classical clinical features. Other presenting Urogynecol j Pelvic Floor Dysfunct 2006; 17:395-9., anterior-posterior (Figure 8) and lateral cephalogram 1. Owren PA: Parahemophilia, Hemorrhagic Diathesis Due acquired factor V deficiency: A case report. Medicine successful outcomes 25, 26 . Bangladesh to be documented in perspective to and menouria and the complete continence of urine which was developed after many years of cesarean complaints can include perineal irritation, vaginal https://doi.org/10.1007/s00192-005-0025-2, (Figure 9) show open sutures of the skull, large Discussion: to absence of previously unknown clotting factor. Lancet (Baltimore) 2019 Apr; 98(7). https://doi.org/10.1097/ dental surgery and orthodontics. 1947;5:446-448.https://doi.org/10.1016/S0140-6736(47) MD.0000000000015259.PMid:31027075 section. fungal infection, and recurrent urinary tract Surgery can be performed transabdominally, endoscopically PMid:16523247 fontanelles, small maxillary sinuses, and a prognathic CCD is diagnosed by the clinical and radiological 17 Case Presentation: findings. The clinical findings of CCD, although 91941-7 PMCid:PMC6831250 Case report: infections . or robotically. Transvaginal approach is less favored 7. Alkatib M , Franco AV, Fynes MM. Vesiciuterine fistula mandible. The cephalometric analysis confirmed the A 13-year-old Bangladeshi girl was referred to the present at birth, could be easily missed because of Mrs “X”, 37 year old multipara came to our clinic Previously she had two lower segment cesarean because of higher location of the fistula and its following cesarean delivery-ultrasound diagnosis and class III skeletal Malocclusion. The anterior and 16 department of orthodontic, Sapporo dental College, their extremely low frequency, rare manifestation of with complaints of passage of bloody urine during section which is one of the important iatrogenic cause complexity . surgical management. Ultrasound Obstet Gynecol 2005; posterior cranial base are significantly reduce. 26:183-5. https://doi.org/10.1002/uog.1925, PMid:15997459 Dhaka, Bangladesh by a local dental surgeon. She the typical extraoral symptoms in early childhood, menstruation for 2 episodes following 3 months of of vesicouterine fistula9 . Probable factors which may Hysterectomy is not indicated for this treatment27. Increased horizontal mandibular growth is found presented with her mother with the chief complaint of and the clinical variety of the disorder 3, as was true amenorrhoea. She had H/O repeat cesarean section 8 lead to formation of vesicouterine fistula after LUCS Disadvantage of the trans abdominal route are 8. Knipe WHW. Vesico-uterine fistula Am J Obstet Gynecol. along with anterior rotation of the mandible in delayed eruption of thepermanent anterior teeth. in the present case. years back. There was no post operative complication are as follows – a) an inadequate reflection of the increased morbidity, long hospital stay and increased 1908;57:211-7. relation to the cranial base. The chest radiograph Figure 7: CBCT occluso-baccal view shows multiple Retained anterior deciduous teeth were carious & after surgery. She had no history of induced abortion bladder from the lower uterine segment during blood loss. These can be overcome by using 9. Youssef AF. Menouria following lower segment cesarean looked ugly that's why she was often teased at school. Figure 2: Close approximation of her shoulders in shows a very narrow thorax with oblique ribs and supernumerary teeth which causes impaction of A characteristic feature of CCD is aplastic or 28 or any other surgery in uterus after last LUCS. She Figure 1 - fistula between bladder base and uterus cesarean section is the main cause of injury of the endoscopic and robotic approach . section: a syndrome. Am J Obstet Gynecol. 1957;73:759-67 Her smile was very unpleasant, which resulted in front of the chest hypoplastic clavicles (Figure 3). permanent teeth. hypoplastic clavicleswhich is responsible for the 210

Journal of Bangladesh College of Physicians and Surgeons Vol. 38, No. 4, October 2020 complications, likeanaemia. If not timely diagnosed; 2. Charles WB: Parahaemophilia (Congenital Factor V did not complain of dysuria or lower abdominal pain. bladder either during uterotomy or uterine closure. b) In our case the supratrigonal vesicouterine fistula was 10. jόzwik M jόzwik M. clinical classification of 20. Moon SG , kim SH, Lee HJ, Moon MH, Myung JS. difficulty communicating with her classmates. Failed Written consent was taken from patient’s guardian to Deficiency). Can Med Assoc J. 1965; 92:979-981. it would have been fatal. She also did not give any history of urinary Inadequate downward mobilization of bladder or repaired through intraperitonial approach. In bladder, vesicouterine fistula. Int j gynecol Obst. 2000;70:353-7. Pelvic fistulas complicating pelvic surgery or diseases: eruption of her permanent teeth was the reason that publish her disease features and photographs. https://doi.org/10.1016/S0020-7292(00)00247-2 Factor V is labile coagulation factor which appears to 3. Gilbert C, White II. Coagulation factors V and VIII incontinence. Then she was given progesterone to direct injury to that portion of the bladder which spectrum of imaging findings. Korean J radiol the patient sought treatment. Her parents reported an Normal function and clinical disorder. In: Robert IH, fistula opening was closed in two layers after be necessary both for the production of fully potent stop menstruation for 2 months. At that time there sometimes adherent to the anterior vaginal wall or 11. Porcaro AB, Zicari M, Zecchini Antniolli S, Pianon R, (2001)2:97-104. https://doi.org/10.3348/kjr.2001.2.2.97, unremarkable medical history. She was in good Samuel EL, Thomas PS, eds. Blood, Principles & mobilization of uterine wall from bladder by intrinsic thromboplastin and for the rapid conversion lower segment wall5 , c) aberrant sutures placed so Monaco C, Migliorini F, et al. Vesicouterine fistulas PMid:11752977 PMCid:PMC2718108 health with no medications and no known allergies. practice of Hematology. 1995;1151-1179. was no per vaginal bleeding or passage of bloody dissection and uterine opening was repaired in single following cesarean section: report on a case, review and of prothrombin to thrombin in the presence of tissue that a knuckle of bladder is caught in the sutures5. 21. Ali-El-Dein, El- Tabey N, El- Hefnawy A, Osman Y, She had no history of trauma to the mouth, teeth, or 4. Ruth AS: Parahemophilia. Medical Clinics of North urine. USG showed normal uterine and ovarian layer suture. There was no menouria after repair and update of the literature. Int urol Nephrol (2002) thromboplastin. Thus, the patients with Factor V Repeated Cesarean sections may result in progressive Soliman S, Shaaban AA, Diagnosis , treatment and need jaws. America 1972;56:119-125. https://doi.org/10.1016/ S0025- structure, no stone in urinary bladder. Urine routine menses was commenced normally. 34:335-44. https://doi.org/10.1023/A:1024443822378, deficiency manifest impaired thromboplastin 7125(16)32427-0 devitalization and scarring of the uterus and bladder PMid:12899224 for hysterectomy in management of post cesarean section A general physical examination revealed a short- 10 microscopic examination revealed normal. vesico uterine fistula. Scand J Urol (2014) 48:460-5., generation and prolongation of prothrombin time. 5. Miletich JP, Majerus DW, Majerus PW. Patients with base by damaging their vascular network, thus After correction of VUF, successful pregnancy 12. B.K. Park, S. H. Kim, J.Y. Cho, J.S.Sim and C.K. Seong, statured, well-oriented young girl with narrow, 11 https://doi.org/10.3109/21681805.2014.903511, Diagnosis is based on prolonged prothrombin (PT) congenital factor V deficiency have decreased factor Xa AFB was not found in endometrial tissue. Then predisposing to delayed fistula formation . But to outcome was also reported. "Vesicouterine fistula after cesarean section: drooped shoulders (Figure-1). Further examination binding sites on their platelets. J Clinic invest cystoscopic evaluation was done . Trigone was develop vesicouterine fistula after 8 years of LUCS ultrasonographic findings in two cases," Journal of PMid:24694181 and activated partial thromboplastin times (APTT) Conclusion: showed an abnormal facility in opposing her 1978;62:824-831. https://doi.org/10.1172/JCI109194, normal . There was a small opening about 1 mm in is most unlikely and rare. Her endometrial tissue and Ultrasound in Medicine, vol.18, no. 6, pp. 441443,1999. 22. Tancer ML. Observation on prevention and management and on low plasma Factor V levels.Bleeding time PMid:701480; PMCid:PMC371834 https://doi.org/10.7863/jum.1999.18.6.441, shoulders (Figure 2) due to malformed or absent Vesicouterine fistula is a rare variety of genitourinary of vesicovaginal fistula after total hysterectomy. Surg (BT) can be prolonged in severe cases.11 supratrigone region of bladder. No feature of fistula margin was send for histopathology but no PMid:10361852 clavicles, which was later confirmed with a small 6. Moret A: Next generation sequencing in bleeding granulomatous lesion was found. AFB stain was also fistula. Previously it was occurred after complicated Gynecol Obstet (1992) 175:501-6. Figure 3: X-ray Chest P/A view shows malformed Differential diagnoses include vWD, Haemophilia disorders: two novel variants in the F5 gene (Valencia-1 granuloma was found. 13. Hadzi- Djokic JB, Pejcic TP, Colovic VC. Vesico-uterine malformed clavicle in chest radiograph (Figure 3). negative. vaginal delivery. Due to increasing trend of lower small clavicle. (homozygous type), inhibitors to coagulation factor, and Valencia-2) associated with mild factor V deficiency. fistula: Report of 14 cases. BJU int 2007;100:1361-3. 23. P. Narayanan, M. Nobbenhuis, K. M. Reynolds, A. Facial examination showed a brachycephalic head Methylene blue dye was pushed through cervix and it segment cesarean section, incidence of VUF also Sahdeb, R. H. Reznek, and A.G. Rockall, " fistulous in and very rarely combined deficiency of factor V and J Thromb Thrombolysis 2019 Nov;48(4). https://doi.org/ If the diagnosis of VUF is early and the fistula https://doi.org/10.1111/j.1464-410X.2007.07067.x, with frontal and parietal bossing, hypoplasticzygomatic Figure 5: CBCT frontal view shows multiple 10.1007/s11239-019-01911-z, PMid:31267299 passed through the bladder opening. Then decision of rise. So surgeons should be more careful during PMid:17590179 malignant gynecologic disease; etiology, imaging and factor VIII. presumed to be small, conservative measures with bones, bulging calvarium, and depressed nasal bridge laparotomy was taken. After opening the abdomen management," Radiographics, (2009) vol. 29;4:107383. impacted teeth. 7. Ding H:Phenotypic and genetic analysis of a pedigree prolonged catheterization with antibiotics and cesarean section to avoid complication. Patient must 14. El- Lamie I. urogenital fistulae: changing trends and with a broad alar base (Figure 2). Intraoral The level of factor V activity in thepatients’ plasma is bladder was found adherent with anterior uterine https://doi.org/10.1148/rg.294085223 PMid:19605657 Figure 8 X-ray Lateral Cephalogram shows normal affected with hereditary FV deficiency due to a novel anticholinergic for about 4 weeks have been reported be councelled about the consequence of repeat personal experience of 46 cases. Int urogynecol j Pelvic examination revealed persistence of the primary usually < 5% of that in normal plasma. Most bleeding deletional variant of F5 gene. Zhonghua Yi Xue Yi Introduction: has been known as Youssef’s syndrome; Type ii is floor Dysfunct (2008) 19:26772. https://doi.org/ maxilla with prognathic mandible. wall. About 1 mm of opening was found in body of to be effective and provides a cure rate in about 5% of cesarean section. 24. G. L. Brodhead . Spontaneous closure of large vesico-uterine dentition, delayed eruption of the permanent teeth, an episodes in patients with factor V deficiency can be Chuan Za Zhi 2019 Nov; 36(11). Vesicouterine fistula is a rare form of urogynaecological associated with dual menstrual flow via both the 10.1007/s00192-007-0426-5, PMid:17639345 uterus which communicated with base of bladder. 12,18,19 fistula, Medication reconciliation. (1920);98:437. Angle class III malocclusion, negative overjet, treated with sufficient fresh frozen plasma to raise the cases . Our patient had developed VUF , 8 References: 8. Tabibian S: A Comprehensive Overview of Coagulation fistula when compared with vesicovaginal fistula. It bladder and vagina and type iii is associated with Fistula was repaired. Catheter was kept in bladder for 15. Józwik M, Józwik M, LotockiW. Vesicouterine fistula-an bilateral posterior crossbite (Figure 4). 12 10 years after lower uterine cesarean section. Diagnosis 1. Iloabachie GC, Njoku O. Vesicouterine fistula. BRITISH 25. Rubino SM. Medical treatment of uterovesical fistula. plasma factor V level to 30%. Factor V and Congenital Factor V Deficiency. Semin represents about 1-4% of all cases of normal menses and lack of menouria . Usually, analysis of 24 cases from Poland. Int J Gynaecol Obstet Introduction: fontanelle and metopic sutures results in frontal Thromb Hemost 2019 July; 45(5). https://doi.org/ 1 21 days. Progesterone was given for 1 month to was made from history, clinical feature of Type 1 J Urol 1985;57:438-9. https://doi.org/10.1111/ j.1464-410X. Lancet 1977;309:900-0. https://doi.org/10.1016/ S0140-6736 urogynaecological fistula . About 83-93% of cases, it 1997;57(02): 169-172. https://doi.org/10.1016/ S0020-7292 1-3 Fresh frozen plasma (FFP) is the only treatment as immediate presentation occurs when there is direct 9 Cleidocranial dysplasia (CCD) is very uncommon bossing . 10.1055/s-0039-1687906, PMid:31121608 2,3,4 induce amenorrhoea. Urine colour was normal. Her Youssef’s syndrome and confirmed by cystoscopy 1985.tb06305.x, PMid:4027516 (97)02837-3 (77)91218-1 Factor V concentrates are not available. In acute is usually iatrogenic following cesarean section . injury to the bladder during surgery. Patients can autosomal inheritant congenital skeletal defect that 9. Desage S: The potential value of thrombin generation menstruation started 4 months after surgery. She also Figure 2- after excision of vesicouterine fistula which showed about 1 mm of fistula in supratrigone 2. Eisenkop SM, Richman R, Platt LD, Paul RH. Urinary The different clinical manifestations reflect the basic cases of severe bleeding, the addition of platelet Other causes of fistula may be due to following have early hematuria and/or urinary leakage, voiding 16. Yip SK, Leung TY. Vesicouterine fistula: an updated 26. Birge O, Ozbey EG, Erkam MM, Arslan D, Kayar i. primarily affects the bones and teeth. CCD is assay in the diagnosis of FV inhibitors. Int J lab hematol (VUF) Discussion : region of urinary bladder and dye test was positive. tract injury during cesarean section. Obst Gynecol mechanisms of skeletal development, patterning, induced abortion, obstructed labour, instrumental difficulty, low-grade pyrexia, urinary sepsis or be relieved of haematuria after surgery. review. Int urogynecol j pelvic Floor Dysfunct 1998;9:252-6. Youssef's syndrome following cesarean section. Case concentrates may be helpful.Prognosis is good with 2019 Oct; 41(5). https://doi.org/10.1111/ijlh.13027 1982;60:591-6 https://doi.org/10.1007/BF01901500, PMid:9849756 characterized by delayed closure of fontanelles, 8, 13 7 Other imaging procedure can be useful to fully reports. Obstet Gynecol 2015;2015:1- https://doi.org/ bone and cartilage formation, growth and early diagnosis and adequate treatment. delivery, vaginal birth after cesarean section, completely asymptomatic . Delayed presentation can The world health organization estimates 130,000 new presence of open skull sutures, hypoplastic or 4 10. Billoir P: Thrombin generation assay is a useful cases of urogenital fistula occuring per year. characterize the fistulous tract. But there is no clear 3. Buchholz NP, Daly- Grandeau E, Huber - buccholz MM. 17. Park OR, kim TS, Kim HJ. Sonographic diagnosis of 10.1155/2015/605325, PMid:26457214, PMCid:PMC4589585 homeostasis . The oral manifestations of CCD pre-operative tool to predict non-bleeding risk in a patient placenta percreta, brachytherapy, migrated intra occur when there is an infection or a progressive 20 aplastic clavicles, supernumerary teeth, delayed Figure 4: Intra-oral photograph shows bilateral Conclusion: 5 consensus on the best modality . Cystography, Urological complications associated with caesarean vesicouterine fistula . Ultrasound Obstet Gynecol (2003) include an underdeveloped maxilla with a high, with mild factor V deficiency.Transfus med 2020 Apr 6. uterine contraceptive devices . Less frequent causes devitalization of the posterior wall of the bladder11. Vesicouterine fistula represents only 2-9% of all 27. Bettez M, Breault G, Carr L, Tu Lm. Early versus crossbite, multiple retained deciduous teeth with hysterography, excretory urography, also ultrasonography section. Eur J obstet gynecol Biol 1994:56:161-3. 22:82-4. https://doi.org/10.1002/uog.161, PMid:12858310 eruption of permanent dentition, wide pubic narrow arched palate, prolonged retention of This is the first reported case on Parahaemophilia in https://doi.org/10.1111/tme.12680, PMid:32250000 are inflammatory bowel disease, endometriosis and urogenital delayed of vesicouterine fistula. J Can urol Assoc 2011; Patients with delayed presentation often have 21,22 https://doi.org/10.1016/0028-2243(94)90163-5 symphysis, short stature and a variety of other caries. 6,7 with or without intra uterine saline infusion can 18. Kilinc F , Bagis T, Guvel S, Egilmez T, ozkardes H. 5(4):457-9. https://doi.org/10.5489/cuaj.10065, PMid:21806894 deciduous teeth, failure of the secondary dentition to Bangladesh. Parahaemophilia is a very rare inherited bladder tuberculosis . Vesicouterine fistula was first 13, 14 11. Walker FJ, Sexton PW, Esmon CT. The inhibition of symptoms of urinary leakage from the vagina if the fistula . The frequency of vesicouterine fistulae in Unusual case of post- cesarean vesicputerine fistula skeletal changes. Delayed closure of the anterior erupt, delayed maturation among the permanent teeth Figure 6: CBCT occluso-palatal view shows coagulation disorder. If not investigated thoroughly, 8 complete the diagnostic workup. MRI has now 4. Yossepwitch O, Baniel J, Livne PM. Urological injuries PMCid:PMC3148396 A CBCT & panoramic X-ray show the presence of blood coagulation by activated protein C through the reported in the literature in 1908 by knipe . It was cervix is incompetent, cyclic hematuria (menouria), (Youssef's syndrome) int J Urol 2003; 10:236-8., 1, 5-7 relation to other urogenital fistula, as previously become the first choice in the investigation of during cesarean section: Intraoperative diagnosis and and multiple impacted supernumerary teeth. multiple supernumerary teeth in both jaw which are numerous supernumerary teeth which causes patients can be misdiagnosed. Screening test and selective inactivation of activated factor V. Biochi described by Youssef in 1957 as a clinical syndrome 11 15, 16 https://doi.org/10.1046/j.0919-8172.2003.00599.x, 28. K. Parveen, R. Gupta, Al=Badr and A.K. Hemal. Robot amenorrhea, infertility or first trimester abortion . reported, varies from 1% to 16.4% . It is very 23,24 management. J Urol 2004;172:196-9. https://doi.org/ impaction of multiple permanent tooth. associated coagulation factor assay should be done Biophys Acta 1979;71:333-342. https://doi.org/10.1016/ fistulas . PMid:12657106 assisted laparoscopic repair of rare post-cesarean section The incidence of CCD is one per million births. It 16 in number; 3 in both side of upper jaw, 6 in lower 0005-2744(79)90103-7 consisting of cyclic haematuria (menouria), amenorrhoea, Most of the cases present in delayed fashion, from likely that there is an increase in the relative 10.1097/01.ju.0000128632.29421.87, PMid:15201771 Figure 1: Frontal view shows narrow & dropped for proper diagnosis. Although factor V deficiency is 12 vesicocervical and vesicouterine fistula: a case series of a affects men and women with equal frequency. CCD left side & 4 in lower right side (Figure 5-7), and complete urinary incontinence in a patient who weeks to years after the inciting event . In such frequency of this type of fistula, because of the The definitive treatment for Youssef’s syndrome is 19. Bastakoti R, Saha R. Congervative management of shoulders, smile shows retained carious upper a milder disorder than Haemophilia A/B, patients 12. William HK, Earl WD. Blood coagulation Factor V and 9 5. Tancer ML. Vesico uterine fistula- a review. Obstet novel technique. Urology (2012) 80:477-82. https://doi.org/ appears spontaneously with no apparent genetic impaction of the most maxillary & mandibular had lower segment cesarean section . A suggested cases diagnosis can be done by detection of urine increase in cesarean section rates. Typically patient surgical. Conservative measure such as prolonged uterovesical fistula following primary cesarean section. anterior teeth resulting unpleasant smile. with factor V deficiency may sometimes require VIII: Structural and Functional Similarities and Their 18, 19 gynaecol Surv 1986;41:743-53. https://doi.org/10.1097/ 10.1016/j.urology.2012.04.027, PMid:22705111 nd classification of VUF, based on the routes of catheterization and medical management to JKMC 2014: 2:211-3. https://doi.org/10.3126/jkmc.v2i4.11799 cause in approximately 40% of affected patients, and permanent teeth, ectopic position of upper left 2 Relationship to Hemorrhagic and Thrombotic Disorders. colour after dye passing through cervical external os presents with cyclic haematuria or menouria, 00006254-198612000-00001, PMid:3540759 6,8 replacement therapy. With proper treatment; 10 one in three patients has unaffected parents . We nd Blood 1988;71:539-555. https://doi.org/10.1182/ blood.V71.3.539. menstrual flow proposed by Jόzwik and Jόzwik or by HSG or cystoscopy. Conservative treatment amenorrhoea and urinary continence, as described in cause induced amenorrhoea to help fistula healing premolar, dilacerations of lower left 2 molar and prognosis is good. If untreated, it can be fatal. 6. DiMarco CS, DiMarco DS, Klingel CL, Gebhart JB. herein report a case of CCD of a 13-year-old Figure 9: X-ray Frontal cephalogram shows bulging bloodjournal713539 in2000, divides vesicouterine fistula (VUF) into three may be approachable but surgery is the best option. 9 with oral contraceptive, progesterone and GnRH caries in several deciduous teeth. The Yossef’s syndrome . Our patient presented with Vesicouterine fistula: A review of eight cases. Int References: 13. Takemoto K:Challenges in management of unusual types. Type 1 is characterized by the triad of amenorrhoea analog have been reported in the literature with Bangladeshi girl& probably the first case from calvarium, narrow maxilla. We report a case of successful surgical repair of VUF these classical clinical features. Other presenting Urogynecol j Pelvic Floor Dysfunct 2006; 17:395-9., anterior-posterior (Figure 8) and lateral cephalogram 1. Owren PA: Parahemophilia, Hemorrhagic Diathesis Due acquired factor V deficiency: A case report. Medicine successful outcomes 25, 26 . Bangladesh to be documented in perspective to and menouria and the complete continence of urine which was developed after many years of cesarean complaints can include perineal irritation, vaginal https://doi.org/10.1007/s00192-005-0025-2, (Figure 9) show open sutures of the skull, large Discussion: to absence of previously unknown clotting factor. Lancet (Baltimore) 2019 Apr; 98(7). https://doi.org/10.1097/ dental surgery and orthodontics. 1947;5:446-448.https://doi.org/10.1016/S0140-6736(47) MD.0000000000015259.PMid:31027075 section. fungal infection, and recurrent urinary tract Surgery can be performed transabdominally, endoscopically PMid:16523247 fontanelles, small maxillary sinuses, and a prognathic CCD is diagnosed by the clinical and radiological 17 Case Presentation: findings. The clinical findings of CCD, although 91941-7 PMCid:PMC6831250 Case report: infections . or robotically. Transvaginal approach is less favored 7. Alkatib M , Franco AV, Fynes MM. Vesiciuterine fistula mandible. The cephalometric analysis confirmed the A 13-year-old Bangladeshi girl was referred to the present at birth, could be easily missed because of Mrs “X”, 37 year old multipara came to our clinic Previously she had two lower segment cesarean because of higher location of the fistula and its following cesarean delivery-ultrasound diagnosis and class III skeletal Malocclusion. The anterior and 16 department of orthodontic, Sapporo dental College, their extremely low frequency, rare manifestation of with complaints of passage of bloody urine during section which is one of the important iatrogenic cause complexity . surgical management. Ultrasound Obstet Gynecol 2005; posterior cranial base are significantly reduce. 26:183-5. https://doi.org/10.1002/uog.1925, PMid:15997459 Dhaka, Bangladesh by a local dental surgeon. She the typical extraoral symptoms in early childhood, menstruation for 2 episodes following 3 months of of vesicouterine fistula9 . Probable factors which may Hysterectomy is not indicated for this treatment27. Increased horizontal mandibular growth is found presented with her mother with the chief complaint of and the clinical variety of the disorder 3, as was true amenorrhoea. She had H/O repeat cesarean section 8 lead to formation of vesicouterine fistula after LUCS Disadvantage of the trans abdominal route are 8. Knipe WHW. Vesico-uterine fistula Am J Obstet Gynecol. along with anterior rotation of the mandible in delayed eruption of thepermanent anterior teeth. in the present case. years back. There was no post operative complication are as follows – a) an inadequate reflection of the increased morbidity, long hospital stay and increased 1908;57:211-7. relation to the cranial base. The chest radiograph Figure 7: CBCT occluso-baccal view shows multiple Retained anterior deciduous teeth were carious & after surgery. She had no history of induced abortion bladder from the lower uterine segment during blood loss. These can be overcome by using 9. Youssef AF. Menouria following lower segment cesarean looked ugly that's why she was often teased at school. Figure 2: Close approximation of her shoulders in shows a very narrow thorax with oblique ribs and supernumerary teeth which causes impaction of A characteristic feature of CCD is aplastic or 28 or any other surgery in uterus after last LUCS. She Figure 1 - fistula between bladder base and uterus cesarean section is the main cause of injury of the endoscopic and robotic approach . section: a syndrome. Am J Obstet Gynecol. 1957;73:759-67 Her smile was very unpleasant, which resulted in front of the chest hypoplastic clavicles (Figure 3). permanent teeth. hypoplastic clavicleswhich is responsible for the 211