Absence Of Right Kidney Research Articles

8356 Robotic Surgery: Pelvic Mass Excision, Resection of Non-Communicating Uterine Horn, and Fulguration of Endometriosis

<h3>Study Objective</h3> To demonstrate surgical techniques for approaching an advanced case of endometriosis in a patient with a congenital uterine anomaly, large pelvic mass, and severe adhesive disease. <h3>Design</h3> A step-by-step explanation of the surgery. <h3>Setting</h3> Community Based Teaching Hospital, Operating Room. <h3>Patients or Participants</h3> 33-yo G0 with known unicornuate uterus with non-communicating uterine horn, dysmenorrhea, suspected endometriosis, infertility, congenital absence of right kidney. <h3>Interventions</h3> Chromotubation was first performed to evaluate if the patient had patent fallopian tubes. The pelvic mass was then decompressed and drained revealing multiple right ovarian cysts. Anatomical landmarks were identified with a retroperitoneal dissection and extensive lysis of adhesions. The bladder was backfilled intermittently to help identify surgical planes. A Keith needle was temporarily placed in the abdomen to elevate the pelvic mass and aid in visualization. The pelvic mass and non-communicating uterine horn were resected. Hemostasis was achieved with electrocautery and suturing. Endometriotic implants in the pelvis and diaphragm were fulgurated and an appendectomy was performed due to extensive damage from endometriosis. All specimens were placed in a bag and removed through the umbilical port site. Post-operatively, the patient was started on progesterone therapy. <h3>Measurements and Main Results</h3> Successful completion of surgery, no complications, the patient reported improvement in her symptoms. <h3>Conclusion</h3> When dealing with severe adhesive disease, abnormal anatomy, and limited space due to a pelvic mass it is key to approach these cases in a stepwise fashion. Visualization can be optimized via temporary measures such as using a Keith needle. If irregular anatomy is noted, landmarks can be identified via the retroperitoneal dissection.

OHVIRA Syndrome: A Case Report

OHVIRA syndrome or Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of female urogenital tract which is represented by the triad of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. This rare variant of Müllerian duct anomalies represent failure of vertical and the lateral fusion of Müllerian ducts around 9 weeks of gestation. OHVIRA syndrome comprises about 2-3% of Müllerian abnormalities. A 13 years old girl got admission in FMCH on 15.3.2018 with lower abdominal pain and acute retention of urine. Her menarche was established 6 months back with regular cycle and associated with dysmenorrhoea. Imaging studies revealed uterine didelphys, highly dilated cervix with hematocolpos and absence of right kidney. The vaginal septum was excised. The patient recovered fully and was under follow up for 6 months. There was no evidence of further outflow tract obstruction. Faridpur Med. Coll. J. Jan 2019;14(1): 54-56

Síndrome de Herlyn-Wener-Wunderlich

Introducción: El Síndrome de Herlyn-Wener-Wunderlich es una malformación de los conductos de Müller y de Wolf, debido a una falta de fusión de los mismos, es muy rara y se caracteriza por presentar un útero didelfo, con una hemivagina obstruida y agenesia renal ipsilateral, más frecuente del lado derecho.&#x0D; Objetivo: Describir un caso clínico de una paciente adolescente con cuadro de dismenorrea secundario a Sindrome de Herlyn-Wener-Wunderlich.&#x0D; Material y métodos: Se realizó un estudio descriptivo, retrospectivo y documental sobre dismenorrea secundaria, malformaciones mullerianas y presentación de un caso clínico.&#x0D; Resultados: Se describe el caso de una paciente de 13 años tratada en la Unidad Materno Infantil del Hospital Teodoro Maldonado Carbo, con cuadro de dismenorrea severa desde el inicio de la menarca, presenta masa pélvica dolorosa a la palpación, se realiza ecografía abdominal y pélvica encontrando dos tumoración de contenido líquido con sedimento que se extienden hasta canal vaginal y que compromete cérvix bilobulado, la ecografía abdominal reporta ausencia de riñón derecho por lo que fue sometida a laparotomía exploratoria confirmando el diagnóstico de malformación uterina con útero didelfo, hemivagina derecha ciega y hematocolpos. &#x0D; Conclusiones: Las malformaciones Mullerianas corresponde a anomalías congénitas poco comunes y el Síndrome de Herlyn-Wener-Wunderlich es una entidad mucho menos común, causante de cuadros de dismenorrea severa, abdomen agudo, su diagnóstico clínico es difícil y requiere estudios complementarios. El tratamiento es conservador con el fin de preservar la función reproductiva de la paciente

MRI image features and differential diagnoses of Herlyn–Werner–Wunderlich syndrome

This study aims to explore how Herlyn–Werner–Wunderlich Syndrome (HWWS) manifests in diagnostic images and analyze the imaging specifications and differential diagnoses thus improving the understanding of the disease. The preoperative magnetic resonance imaging (MRI) findings and clinical data of 19 patients with HWWS were retrospectively analyzed, taking the intraoperative findings and postoperative pathological results of laparoscopic or hysteroscopy surgery as the diagnostic criteria. In this study, all the 19 patients underwent laparoscopic or hysteroscopic excision of vaginal oblique septum, and the preoperative MRI diagnosis of HWSS was consistent with the clinical postoperative diagnosis, with a diagnosis coincidence rate of 19/19 (100%). According to the classification of vaginal oblique septum syndrome, 16 cases were vaginal oblique septum type I and 3 cases were vaginal oblique septum type II. MRI showed a double uterus and double cervix, blood accumulation and dilatation of the vagina connected with the cervix of the affected side, T1WI showed high signal intensity and T2WI showed low or mixed signal intensity. MRI coronal view of 19 patients clearly showed ipsilateral renal absence with obstruction, including 5 cases on the left and 14 cases on the right. Other pelvic complications included: 1 patient with cervical dysplasia and the absence of right kidney and ureter, 3 patients with right ovarian endometriosis cyst, 1 patient with complicated with adenomyosis, 1 patient with right ovarian follicular cyst, 1 patient with right ovarian fallopian tube and pelvic abscess, and 1 patient with right ovarian hematoma. The MRI image of HWWS has its own features and MRI can more accurately evaluate type and related complications of HWWS, therefore it can be used as the best and effective examination method for preoperative imaging evaluation, thus better providing help for clinical comprehensive preoperative evaluation and guidance of surgery.

SAT-292 Superimposed Peripheral Precocious Puberty Caused by Autonomous Ovarian Follicular Cyst in Being Treated Central Precocious Puberty Patient: Underlying Condition of Herlyn-Werner-Wunderlich Syndrome

Autonomous ovarian follicular cysts are known as the most common cause of peripheral precocious puberty(PPP) in prepubertal girls, and relapses of autonomous ovarian cysts resulting in prolonged estrogen exposure can precipitate early maturation of the hypothalamic-pituitary-gonadal axis, resulting in progression from PPP to central precocious puberty(CPP), which is called “combined precocious puberty”, an evolution which may require GnRH therapy. In present case, PPP caused by autonomous ovarian follicular cyst was developed concomitantly during the treatment course of CPP, with suppressed status of hypothalamic-hypophyseal-axis. In addition, a rare congenital Müllerian anomaly known as Herlyn-Werner-Wunderlich syndrome(HWWS), consisting of uterus didelphys, obstructed hemivagina and unilateral renal agenesis was incidentally diagnosed as an underlying condition. A 7-year-old girl was presented to our clinic with the development of breast buds which was examined as Tanner stage 2-3. Right renal agenesis was diagnosed in infancy. Her bone age was advanced as 10 years of age, and Luteinizing hormone(LH) showed pubertal response in LHRH stimulation test. The pelvic US revealed absence of right kidney and uterine enlargement. Brain MRI showed no specific abnormalities. Based on these results, We diagnosed idiopathic central precocious puberty. Thereafter, we commenced the treatment with Leuprolide acetate 3.75mg every 4 weeks. After the 11th injection of Leuprolide acetate, however, the patient complained of sudden breast engorgement and vagnal bleeding. Being considered as unsuccessful suppression of HPG axis, Leuprolide acetate was boosted. Hormonal analysis results revealed an extremely elevated level of estradiol and suppressed baseline gonadotropin levels. The pelvic US revealed double uterus, which had not been detected to that point. Abdominopelvic MRI demonstrated right renal agenesis and uterus didelphys with clearly separated uterine body, cervix and vagina. About 3cm sized cystic lesion with several internal daughter cysts is also discovered in left adnexa area. Based on all examinations, we concluded that PPP caused by autonomous ovarian cyst was superimposed on treatment course of CPP and HWWS was incidentally found as underlying disease. Maintaining GnRH agonist treatment, the progression of secondary sexual characteristics was halted and left ovarian cyst disappeared. In couclusion, clinician should be aware of possibility of autonomous ovarian follicular cyst when sudden progression of secondary sexual characteristics is shown in being treated CPP patients, and discriminate PPP from unsuccessfully treated CPP. Additionally, careful suspicion for HWWS that is tied to indications of renal agenesis in children may be the key for successful early diagnosis and immediate treatment intervention, which could prevent long-term complications.

Zinner's Syndrome:Report of two cases with different symptoms managed by minimally invasive approach

Abstract Background: Zinner’s syndrome is characterized by triad of seminal vesicle cysts with ipsilateral renal agenesis and ejeculatory duct obstruction. It sometimes becomes symptomatic during reproductive time of the patient. In this article,we present a minimally invasive approach for two patients with Zinner syndrome. Case presentation: First patient was 37 years old man with 5 years history of infertility. Semen analysis was typical for obstructive azoospermia and ultrasonography revealed the absence of left kidney and anechoic pelvic mass with a thick wall. MRI confirmed Zinner’s syndrome showing 9 cm sized seminal vesicle cyst on the posterior of the bladder. The second patient was 18 years old boy with one year history of abdominal-pelvic pain and lower urinary tract symptoms. Ultrasonography revealed the absence of right kidney and fluid-filled cystic lesion behind the bladder. Contrast enhanced CT confirmed Zinner’s syndrome diagnosis showing solitary left kidney with 8 cm sized seminal vesicle cyst on the posterior of the bladder. We performed laparoscopic seminal vesicle cyst excision for both patient successfully. The procedure was performed with transperitoneal access using four trocars for two patients. Conclusions: Laparoscopic approach for seminal vesicle cysts is feasible because it provides quicker recovery and less morbidity. Therefore it should be considered for treating seminal vesicle cysts.

Renal adysplasia in fetus: A rare autopsy case report

Renal adysplasia is rare, occurring in only one per 10,000 births. Ultrasonography at 21 weeks of gestation in 33 years woman showed cystically enlarged left kidney in fetus and absent right kidney in fetus for which the pregnancy was terminated. On fetal autopsy, left kidney showed multiple tiny cysts. Microscopy of the left kidney showed features of dysplasia and confirmed the absence of right kidney and bilateral ureters. The diagnosis of renal adysplasia (multicystic dysplasia of left kidney and agenesis of right kidney) was made. This case is presented for its rarity. DOI: 10.21276/APALM.1441

English

Cystic malformation of the seminal vesicle is rare congenital urogenital tract anomaly. It can be isolated anomaly. It can be associated with other urinary tract anomalies. Clinical presentation may be extremely variable. In symptomatic patients surgical intervention is required so radiological features and clinical aspects are very important for patient's management. CASE REPORT: A 32 yr male patient was admitted with complaints of dysuria, frequency of micturition and intermittent febrile episodes since last two months. A long course of antibiotics was given to him with clinical diagnosis of urinary tract infection. At the time of admission urine examination was normal. Per rectal examination revealed a firm mass located just superior to the prostate. Ultrasound of the abdomen and pelvis was performed which revealed absence of right kidney and hypertrophy of contralateral kidney which was also malrotated and improperly ascended. There was a cystic lesion at the anatomical site of seminal vesicle which is not seen separately, the prostate also appeared smaller in size. Transrectal ultrasound was performed for further characterization which revealed a large tubular cystic lesion entirely replacing the seminal vesicle. There was no solid component. The prostate was well visualized however it was smaller in size. Patient was further investigated with IVU, CT scan and MRI. Intravenous urography revealed absent right kidney and compensatory hypertrophy of left kidney. Left kidney was malrotated and incompletely ascended however it showed prompt and normal excretion. The left ureter and urinary bladder were normal. Plain and post intravenous contrast CT scan of the abdomen was performed. There was hypodense mass lesion of water attenuation in the pelvis at the bladder base more to the right side with non visualization of the seminal vesicles separately. There was no contrast enhancement. Plain MRI was performed using dedicated pelvic coil on 1.5 T GE units. Axial, coronal and sagittal T1W and T2 W sequences were performed. It showed a tortuous tubular structure of fluid signal intensity entirely replacing the seminal vesicles. It measured approximately 8x7x6 cms in size. There was no solid component. The prostate was small in size however appeared normal in morphology and signal characteristics. The rest of the pelvic structures were normal. So based on the imaging findings diagnosis of congenital cystic anomaly of the seminal vesicle was made. DISCUSSION: Congenital cysts of seminal vesicles can be subcategorized as isolated cysts, cysts associated with upper urinary tract anomalies and cysts associated with autosomal dominant polycystic kidney disease. Cysts of seminal vesicle usually become apparent during second to third decade of life, perhaps related to onset of sexual activity at this age(1). Cysts are mostly smaller than 5 cm in diameter and are often discovered incidentally, but they may present with a wide variety of

Prenatal diagnosis of deletion of chromosome 6p presenting with hydrops fetalis

To report the first known case of 6p deletion presenting in utero with hydrops fetalis and multiple anomalies in the second trimester of pregnancy. A thirty-year-old woman (gravida 3 para 1 abortion 1) was referred to our hospital at 18 weeks of gestation because of suspicion of fetal anomaly on routine ultrasound examination. A detailed anomaly scan revealed a single viable fetus with marked skin edema, marked ascites, pleural effusion, hydronephrosis of left kidney, absence of right kidney, cardiac anomaly and oligohydramnios. The fetal face was not visible due to the fetal position. Fetal karyotyping revealed 46,XX,del(6)(p21.3). The couple opted to terminate the pregnancy. A hydropic female fetus was aborted and the autopsy revealed hydrops fetalis with bilateral cleft lips, hydronephrosis of left kidney, absence of right kidney, spleen, and thymus gland, truncus arteriosus, and single umbilical artery. Cord blood and tissue culture confirmed that the fetus had deletion of chromosome 6p. Deletion of short arm of chromosome 6 can result in hydrops fetalis in early pregnancy.

Congenital absence of right kidney with cystic formation of lower end of ureter

Congenital absence of right kidney with cystic formation of lower end of ureter

Retroperitoneal Cyst Arising in Persistent Meta-Nephros with Congenital Absence of Right Kidney and Ureter

Retroperitoneal Cyst Arising in Persistent Meta-Nephros with Congenital Absence of Right Kidney and Ureter