Anatomical Abnormalities Research Articles

P-440 Idiopathic infertility does not increase rates of placental abnormalities among singleton pregnancies conceived with either in-vitro fertilization (IVF) or ovulation induction±intrauterine insemination (OI±IUI)

Abstract Study question Is there an adverse impact of idiopathic infertility diagnosis on placental pathology among singleton pregnancies conceived with fertility treatments? Summary answer Idiopathic infertility does not increase rates of placental abnormalities and exhibits distinctive associations within the inflammatory and vascular spectrum of placental pathology. What is known already Pregnancies resulting from fertility treatments are often considered to be at higher risk for placenta-mediated obstetric complications and subsequent adverse perinatal outcomes. Treatment induced hormonal changes altering the endometrial milieu, along with embryo manipulation and culture conditions, may impact the processes of implantation, decidualization, trophoblast invasion, and placental vascularization. Nevertheless, the impact of infertility diagnoses on placental pathology among singleton pregnancies conceived with fertility treatments has not been elucidated, and whether idiopathic infertility alters the risk of placental abnormalities remains unknown. Study design, size, duration Retrospective review of placental pathology data from 1205 singleton livebirths conceived with fertility treatments (899 IVF, and 306 OI±IUI cycles) between 01/2004 and 04/2022. Placenta pathology was reviewed by one expert pathologist and classified as anatomic, inflammatory, infectious, and vascular [including any features of fetal (FVM) or maternal (MVM) vascular malperfusion], using the Amsterdam Workshop Consensus definitions. Placental abnormalities were compared between idiopathic infertility (IdI, n: 269) and all other, non-idiopathic infertility (non-IdI, n:936) diagnoses. Participants/materials, setting, methods Primary outcomes: anatomic, inflammatory, infectious, and vascular placental abnormalities. Parametric, and non-parametric tests were used as appropriately; odds ratios (OR) with 95% confidence intervals (95%CI) were assessed through logistic regression, adjusting for maternal age, body mass index (BMI, kg/m2), race, parity, gestational age, neonatal gender, treatment type, gestational diabetes, and hypertensive disorders. Analyses were further stratified by OI±IUI vs IVF treatments [the latter further stratified into fresh and frozen embryo transfers (FET)]. Main results and the role of chance Mean age, BMI, and AMH did not differ between groups, and neither did placental weight at term. Overall, unadjusted rates of inflammatory, infectious, and vascular abnormalities did not differ between groups (12.6% vs 11.4%, p 0.568; 22.0% vs 19.1%, p 0.256; 54.5% vs 58.6%, p 0.186, respectively). However, a higher rate of anatomic abnormalities was noted among IdI patients (32.8% vs 27.1%, p 0.044, IdI vs. non-IdI, respectively). When adjusting for potential confounders, no differences were noted between groups regarding inflammatory, infectious, and vascular abnormalities, and the observed difference in anatomic abnormalities lost its significance [adjOR(95%CI); inflammatory: 0.98(0.94-1.03), infectious: 1.03(0.98-1.09), vascular: 0.96(0.90-1.02), and anatomic: 1.02(0.96-1.08), non-IdI: ref]. Similarly, when separating OI±IUI from FET and fresh IVF cycles, adjOR(95%CI) revealed no differences in anatomic, infectious, and vascular abnormalities between groups [OI±IUI: 0.97(0.85-1.1), 0.97(0.87-1.08), 0.99(0.87-1.12); FET: 1.08(0.94-1.24), 1.07(0.94-1.23), 1.04(0.98-1.11) and, fresh IVF: 1.06(0.98-1.15), 1.04(0.96-1.13), 0.96(0.87-1.07), for anatomic, infectious, and vascular, respectively]. However, lower odds of inflammatory abnormalities were noted among fresh IVF but not among FET or OI±IUI cycles in IdI [adjOR (95%CI); fresh IVF: 0.95(0.91-0.99), FET: 1.09(0.98-1.21), OI±IUI: 0.97(0.87-1.08), non-IdI: ref]. Interestingly, among vascular abnormalities studied in the programmed FET cycles, MVM showed a difference between the groups [adjOR(95%CI) 0.90(0.85-0.96), non-IdI: ref]. Limitations, reasons for caution This study is limited by its retrospective design. The reported findings are from an infertile population undergoing treatments and thus not easily generalizable to natural conceptions. Other factors, such as environmental, nutritional, and lifestyle, might alter a patient’s individual pregnancy risk for placental abnormalities. Wider implications of the findings Overall, idiopathic infertility does not appear to increase the risk for placental abnormalities. However, it may impact placental pathology in a unique way, within the vascular and inflammatory spectrum, which might differ from that of other infertility diagnoses, and might be further altered by the treatment protocol. Trial registration number Not Applicable

O-156 Trophectoderm biopsy for pre-implantation genetic testing (PGT) does not increase rates of placental abnormalities among singleton pregnancies conceived with in-vitro fertilization (IVF)

Abstract Study question Does trophectoderm biopsy for PGT affect placental pathology? Summary answer Trophectoderm biopsy does not increase rates of placental abnormalities among singleton pregnancies conceived with in-vitro fertilization (IVF). What is known already PGT, routinely performed at the blastocyst stage, is used to screen embryos for aneuploidy, structural rearrangements, and single-gene disorders. The association of ART with abnormal placentation and adverse pregnancy outcomes is known. Whether, in a similar fashion, the disruption of the trophectoderm contributes to increased rates of abnormal placentation, remains unknown. Study design, size, duration Data from 987 singleton livebirths with available placental pathology, who conceived following IVF/±PGT at a single academic fertility center between 01/2004 and 12/2019, were retrospectively reviewed. Placental abnormalities were classified as anatomic, inflammatory, infectious, and vascular (any features of fetal or maternal vascular malperfusion), by one expert perinatal pathologist using the Amsterdam Workshop Consensus definitions. Placental pathology was compared between PGT (n = 76) and non-PGT (fresh and frozen, n = 911) cycles. Participants/materials, setting, methods Primary Outcome Measures: incidence of anatomic, vascular (maternal and fetal side), infectious, and inflammatory placental abnormalities. Odds ratios (OR) and 95% confidence intervals (95%CI) were calculated using generalized estimating equations regression to adjust for multiple deliveries per patient and for potential confounders [maternal age, body mass index (BMI:kg/m2), race, parity, infertility diagnosis, gestational age, infant gender, hypertensive disorders of pregnancy, IVF and FET protocol]. Main results and the role of chance Patients undergoing PGT were older than the non-PGT group [36.2(3.5) vs. 34.9(3.8) years, p = 0.006] and did not otherwise differ in AMH, BMI, and race. Overall, adjusted (Adj) OR (95%CI) did not reveal any differences between groups in anatomic [0.92(0.82-1.03)], inflammatory [1.03 (0.94-1.14)]), infectious [1.02 (0.9-1.16)], or vascular abnormalities [1.02(0.88-1.18)]. When limiting analysis to blastocyst biopsies/FET cycles only, adjOR did not show any differences in inflammatory [1.01 (0.89-1.13)]), infectious [1.02 (0.88-1.17)], or vascular abnormalities [1.01 (0.84-1.2)] between the PGT and non-PGT group. Interestingly, adjOR (95%CI) revealed lower odds for anatomic abnormalities in the PGT group (0.86 (0.76-0.98), p = 0.021, non-PGT: ref). A sub-analysis of programmed FET cycles revealed persistently lower odds of anatomic abnormalities among the PGT group [adjOR (95%CI): 0.77 (0.67-0.89), p < 0.001, non-PGT: ref]. Among anatomic abnormalities studied in the programmed FET cycles, marginal cord insertion showed a difference between the groups [adjOR(95%CI): 0.83 (0.73-0.95), p:0.007, non-PGT: ref ]. AdjOR(95%CI) showed no differences in inflammatory [ 1.01 (0.87-1.17)], infectious [1.01 (0.86-1.2)], and vascular [0.9 (0.73-1.1)] abnormalities between groups. Limitations, reasons for caution This study is limited by its retrospective nature. Although a large number of IVF placentas was included, the number of patients in the PGT compared to the non-PGT group was smaller potentially limiting our ability to detect subtle differences between groups. Wider implications of the findings Trophectoderm biopsy for PGT does not appear to increase risk for placental abnormalities, a reassuring finding given the increased incidence of abnormal placentation and adverse perinatal outcomes among IVF pregnancies. Trial registration number not applicable

Synaptic cell adhesion molecules contribute to the pathogenesis and progression of fragile X syndrome.

Fragile X syndrome (FXS) is the most common form of inherited intellectual disability and a monogenic cause of autism spectrum disorders. Deficiencies in the fragile X messenger ribonucleoprotein, encoded by the FMR1 gene, lead to various anatomical and pathophysiological abnormalities and behavioral deficits, such as spine dysmorphogenesis and learning and memory impairments. Synaptic cell adhesion molecules (CAMs) play crucial roles in synapse formation and neural signal transmission by promoting the formation of new synaptic contacts, accurately organizing presynaptic and postsynaptic protein complexes, and ensuring the accuracy of signal transmission. Recent studies have implicated synaptic CAMs such as the immunoglobulin superfamily, N-cadherin, leucine-rich repeat proteins, and neuroligin-1 in the pathogenesis of FXS and found that they contribute to defects in dendritic spines and synaptic plasticity in FXS animal models. This review systematically summarizes the biological associations between nine representative synaptic CAMs and FMRP, as well as the functional consequences of the interaction, to provide new insights into the mechanisms of abnormal synaptic development in FXS.

Role of MRI in Patellofemoral Pain Syndrome (Anterior Knee Pain)

Abstract Background MRI is a good diagnostic tool in identifying cartilage and soft tissue lesions as well as detecting underlying predisposing anatomical factors of patellar instability, it has a high specification for detecting the grades and the types of some of these diseases or factors that may predispose to them such as trochlear dysplasia and patella alta. Aim of the Work The aim of this study was to evaluate the role of MRI in diagnosis of Patellofemoralpain syndrome which is the most common cause of anterior knee pain. Patients and Methods This prospective study was done on patients referred from the orthopedic clinic to the department of radiodiagnosis, MRI unit at Ain Shams University hospitals.Thirty patients were included (18 females and 12 males) and their age ranged from 20 to 64 years. Conclusion This study shows that MRI is useful in detecting various predisposing anatomical factors of patellofemoral instability and associated lesions of knee joint thereby enabling orthopedic surgeons to treat joint pathology as well as correct underlying anatomical abnormality.

Utilization of SmartNav technology in cochlear implantation: optimizing efficiency in assessment of electrode placement

Objectives Proper electrode placement is essential for favorable hearing outcomes following cochlear implantation. Though often used, traditional intraoperative X-ray imaging is time consuming, exposes patients and staff to radiation, and poses interpretational challenges. The Nucleus® SmartNav System, utilizes electrode voltage telemetry (EVT) to analyze the positioning of the electrode array intraoperatively. This study investigates the efficacy of SmartNav in optimizing the efficiency and accuracy of assessing electrode placement. Methods This prospective clinical study analyzed placement of 50 consecutive Cochlear Corporation cochlear implants conducted at a single institution between March of 2022 and June of 2023. Placement check of electrode array using SmartNav and X-ray was completed and individually assessed. A comparative analysis of SmartNav and X-ray completion times for electrode placement assessment was conducted. Results Subjects included nine ears with abnormal anatomy and three reimplants. SmartNav placement check required a total time of 2.12 min compared to X-ray imaging at 14.23 min (p = 1.6E-16, CI 95%). Both SmartNav and X-ray had excellent sensitivity of 100% in identifying appropriate electrode position (p = 1.0). Tip fold-over was identified using both modalities in 3 cases with noted easier interpretation using SmartNav. Conclusion The Nucleus® SmartNav System significantly outperformed traditional X-ray imaging, offering a faster and more straightforward approach to assessing electrode positioning during cochlear implant surgery, thereby enhancing surgical efficiency and patient safety.

Creation and Validation of a Novel 3-Dimensional Pediatric Hip Ultrasound Model.

The aim of this study was to create and validate a 3-dimensional (3D) ultrasound model with normal and abnormal pediatric hip joint anatomy that is comparable to a pediatric hip joint in appearance and anatomy and replicates sonographic characteristics of a pediatric hip joint. A 3D rendering of the bone and soft tissue was created from a computed tomography pelvic scan of a pediatric patient. This rendering was modified to include a unilateral joint effusion. The bone was 3D printed with a photopolymer plastic, whereas the soft tissue was cast with a silicone mixture in a 3D-printed mold. The effusion was simulated by injecting saline into the soft tissue cavity surrounding the bone. The ultrasound model was validated by pediatric point-of-care ultrasonographers at an international pediatric ultrasound conference. A pediatric hip ultrasound model was developed that simulates both normal and abnormal pediatric hip joint anatomy, each with an appropriately sized, measurable joint effusion. Validation by pediatric point-of-care ultrasonographers showed that the key aspects of a normal pediatric hip joint (femoral physis, sloped femoral neck, and adequate soft tissue) with an identifiable and measurable effusion were included in the ultrasound model. In this study, we successfully created a cost-effective, reusable, and reproducible 3D pediatric hip ultrasound model. The majority of pediatric point-of-care ultrasonographers who evaluated the model agreed that this model is comparable to a pediatric patient for the purpose of teaching ultrasound skills and joint space measurement.

Assessment of Factors Responsible for Stone-Free Status After Retrograde Intrarenal Surgery.

Aim The study aimed to evaluate the predictive factors that determined stone-free rate (SFR) after retrograde intrarenal surgery (RIRS). Materials and methods This prospective study was conducted on 183 patients undergoing RIRS for renal stones. Patients were categorized into two groups, depending on stone-free status one month following the procedure. SFRwas defined as the complete absence of stones or stones <4 mm. The parameters studied included patient demographics, presence of hydronephrosis, presence of preoperative double J-stent, abnormal renal anatomy, and stone characteristics (stone burden, stone number, stone density, stone location, lower pole infundibulopelvic angle, and lower pole renal infundibular length (RIL)). Univariate and multivariate analyses were performed to identify risk factors for residual stones. We assessed the predictive ability of the RIRS score and Resorlu-Unsal stone score (RUSS) for evaluating SFR utilizing receiver operating characteristic (ROC) analysis. Results 183 patients were included inthe study with a median age of 51 years. 131 (71.6%) patients were declared stone-free after the procedure. The mean stone size and density were 16.9 SD±7.5 mm and 1038 SD±342 Hounsfield units (HU) respectively. Stone-free patients had lower stone size (14.3 mm vs. 23.6 mm, p<0.01) and stone density (970 HU vs. 1211 HU, p<0.01) compared to non-stone-free patients. Patients with residual stones had steeper lower pole renal infundibulopelvic angle (RIPA) (31.3° vs. 40.7°, p<0.01) and longer RIL(26.6 mm vs. 21.1 mm, p<0.01). Stone multiplicity (p<0.01), lower pole stone location (p<0.01), and renal malformations (p<0.01)were significant influencing factors for residual renal stones after RIRS. Multivariate analysis revealed stone size, stone density (HU), and stone location as independent predictors for SFR after RIRS. Among the scoring systems, the RIRS score had the highest diagnostic accuracy for SFR (area under the curve (AUC): -0.882, 95% CI-0.828-0.936). Conclusion Stone size, stone density (HU), and stone number are important predictors of SFR after RIRS. Lower pole stone location and abnormal renal anatomy play a substantial role in determining SFR after RIRS. In lower pole stones, a long RIL and acute RIPA negatively influence SFR. Additionally, the RIRS score was found to be a better predictor for SFR than the RUSS score.

Inter-rater agreement of CAUTI (catheter-associated urinary tract infections) diagnosis among Infectious disease physicians

Background: CAUTIs constitute forty percent of nosocomial infections, yet their direct link with mortality remains debated. In 2009, NHSN estimated the economic burden of CAUTIs in the U.S. to be over $340 million. Limited data exist on inter-physician concordance in diagnosing CAUTIs, especially in patients with abnormal genitourinary (GU) anatomy. Our study assessed inter-provider variability in diagnosing CAUTI in 50 such patients, including those meeting NHSN(National healthcare safety network) criteria. Methods: We included a random set of 50 adults (18+) with abnormal GU anatomy admitted to the University of Miami hospitals from January 2018 to November 2021 who had a urinary foley catheter and at least one positive urine culture during their hospitalization. Three Infectious disease fellows and three board-certified Infectious disease physicians independently reviewed each patient’s chart, classifying them as having or not having a CAUTI. Inter-physician reliability was assessed using kappa statistics. Results: Our findings highlight substantial variation in clinician-determined CAUTI incidence among the 50 patients with abnormal GU anatomy, ranging from 8% to 32% (Figures 2,3). Inter-rater agreement on CAUTI diagnosis was generally poor (Kappa Hollenbeak CS, et al. The attributable cost of catheter-associated urinary tract infections in the United States: A systematic review. Am J Infect Control. 2018 Jul;46(7):751-757. Trautner BW, et al. Development and validation of an algorithm to recalibrate mental models and reduce diagnostic errors associated with catheter-associated bacteriuria. BMC Med Inform Decis Mak. 2013 Apr 15;13:48. Gafary M, et al. Catheter Associated Urinary Tract Infections (CAUTI) in Bladder Cancer Patients Post Cystectomy With a Neobladder, Open Forum Infectious Diseases, Volume 2, Issue suppl_1, December 2015, 293.

Evaluation of the methanol extract of BaiYangJie: Toxicology and protective effect against acute kidney injury.

BaiYangJie (BYJ) is a terrestrial perennial plant commonly used as a Dai medicine and has therapeutic effects on liver and kidney diseases. Cisplatin (CP), a chemotherapy drug, has good therapeutic effects but causes many side effects, including nephrotoxicity. This article investigated the toxicology of the methanol extract of BYJ (ME-BYJ) and its protective effect on CP-induced acute kidney injury (AKI) through pharmacological experiments. The results showed that the treated mice had no toxicological symptoms and no anatomical, physiological, or histological abnormalities. The BYJ-high-dose group showed significantly attenuated CP-induced AKI. It is concluded that ME-BYJ has the most significant protective effect on AKI at a dose of 8 g/kg and BYJ was not toxic.

Modified Dunn Procedure for Open Reduction of Chronic Slipped Capital Femoral Epiphysis.

Abnormal femoral head anatomy following moderate-to-severe slipped capital femoral epiphysis (SCFE) can lead to femoroacetabular impingement and premature osteoarthritis4-10. Surgical correction at the deformity site through capital reorientation has the potential to fully ameliorate this but has traditionally been associated with high rates of osteonecrosis11-15. The modified Dunn procedure has the potential to restore anatomy in hips with SCFE while protecting the blood supply to the femoral head. A surgical dislocation of the hip is performed according to the technique described by Ganz et al.16. The remaining posterosuperior portion of the greater trochanter is trimmed to the level of the femoral neck by subperiosteal bone removal performed in an inside-out manner. The periosteum of the femoral neck is gradually elevated. The resulting soft-tissue flap, consisting of the retinaculum and external rotators, holds the blood vessels supplying the epiphysis. The femoral epiphysis is pinned in situ (in unstable cases) with threaded Kirschner wires, the ligamentum teres is transected, and the femoral head is dislocated. With the femoral neck exposed, the epiphysis is gradually mobilized from the metaphysis, allowing exposure of the residual femoral neck and inspection of any posteroinferior callus. To avoid tension on the retinacular vessels during reduction of the epiphysis, the posterior neck callus is completely excised. The remaining physis is removed with use of a burr while holding the epiphysis stable. The epiphysis is gently reduced onto the femoral neck, avoiding tension on the retinacular vessels. If tension is noted, the femoral neck is rechecked for residual callus, which is excised. If no callus is found, the neck may be carefully shortened in order to minimize tension. Epiphyseal fixation is achieved with use of a 3-mm fully threaded wire inserted antegrade through the fovea to the lateral cortex below the greater trochanter. A second wire is inserted retrograde under fluoroscopy. After reducing the hip, the capsule is closed and the greater trochanter is reattached with use of 3.5-mm cortical screws. Alternatives include nonoperative treatment, in situ fixation (e.g., pinning or screw fixation), gentle closed reduction with pinning, and triplanar trochanteric osteotomy (e.g., Imhauser or Southwick osteotomies). In situ pinning of mild-to-moderate, stable SCFE yields good long-term results with low rates of osteonecrosis9. Treatment of higher-grade SCFE without reduction aims to avoid osteonecrosis and assumes that the proximal femoral deformity will remodel; however, the head-neck offset will remain abnormal, risking impingement and early-onset osteoarthritis5,8. The procedure described in the present article allows anatomic reduction of the epiphysis with a low risk of osteonecrosis. Surgical dislocation of the hip16 with development of an extended retinacular soft-tissue flap17 provides extensive subperiosteal exposure of the circumferential femoral neck and preserves the vulnerable blood supply to the epiphysis18. The Dunn subcapital realignment procedure15 with callus removal and slip angle correction allows anatomic restoration of the proximal femur. Reported results of various centers performing the procedure vary greatly with regard to the number of hips treated and the follow-up time. Most studies have been retrospective and have lacked a control group. The reported risk of osteonecrosis ranges from 0% to 25.9%19, with the wide range most likely because of the challenging nature of the technique, the low number of cases per surgeon, and the long learning curve associated with the procedure. In centers with extensive experience in pediatric hip-preserving surgery, the reported rate of osteonecrosis is low3. Studies with mid to long-term follow-up have shown no conversion to total hip arthroplasty3,20,21, but residual deformities can persist, and subsequent surgery is possible. Extensive experience in surgical hip dislocation and retinacular flap development is a prerequisite for successful outcomes and low rates of osteonecrosis.Sufficient callus and physeal remnant resections are needed to avoid tension on the retinacular vessels during epiphyseal reduction.The skin incision should be centered over the greater trochanterThe Gibson interval must be carefully prepared for adequate release and to avoid injury.Tension on the periosteal flap should be avoided to prevent stress on the retinacular vessels. AP = anteroposteriorAVN = avascular necrosis (i.e., osteonecrosis)CI = confidence intervalCT = computed tomographyK-wire = Kirschner wireMRI = magnetic resonance imagingOA = osteoarthritisSHD = surgical hip dislocationTHA = total hip arthroplastyVTE = venous thromboembolism.

Avascular necrosis after surgery of developmental dysplasia of the hip in children after walking age: incidence and risk factors

Introduction Developmental dysplasia of the hip (DDH) is a common congenital anomaly in children. It includes a spectrum of anatomic abnormalities ranging from acetabular dysplasia to frank hip dislocation. Avascular necrosis (AVN) is one of the main complications associated with the treatment of DDH. It is a devastating problem that can lead to acetabular dysplasia and joint incongruity and cause premature osteoarthritis. The study aims to evaluate the incidence and risk factors of AVN after open reduction of developmental dislocation of the hip in children after walking age. Patients and methods Between the period of April 2014 and April 2019, a retrospective cohort evaluation of DDH cases was managed by surgery in the form of open reduction and pelvic osteotomy with or without femoral shortening. All grades of Tönnis classification were included. This study included 200 cases with an age range from 1 to 7 years, and the average age is 3.5 years at the time of last follow-up. The mean follow was 2.29 years, which ranged from 1 to 4 years. Plain radiography obtained in anteroposterior and frog lateral views after 1 year follow-up to diagnosis AVN as established in the literature. Results In this study, the mean time of intervention was 2.26 ± 0.97 years with an age range from 1 to 5 years. Twenty-six (13%) cases were managed only by open reduction and capsulorrhaphy, 174 (72.5%) cases had pelvic osteotomies, either Salter or Dega osteotomy, of whom 104 had femoral shortening and derotational osteotomy (59.77%). No AVN was reported in 159 (79.5%) cases, while AVN was evident in 41 (20.5%) cases. Discussion Incidence of AVN after open reduction depend on variable factor discussed in this study with total incidence of AVN 41 (20.5%) cases. This result is similar to other studies, with the incidence of AVN ranging from 15 to 30%. In the present study, the incidence of AVN increased 17 times in cases with postoperative immobilization in the hip spica more than or equal to 60° in the form of 21 cases showed AVN. Conclusion Postoperative hip immobilization in spica less than 60° as children with postoperative hip abduction more than or equal to 60° showed an increase incidence of AVN.

A Rare Case of Bilateral Retroperitoneal Ovaries: A Case Report and Review of the Literature

Introduction: Retroperitoneal gynecologic tumors and cysts are rare. They are often asymptomatic or present similarly to their intraperitoneal counterpart with vague abdominal discomfort and distention. Our literature review revealed that many retroperitoneal cysts are found intraoperatively due to their benign predominance consistent with their ultrasound findings. The rarity of retroperitoneal gynecologic tumors and cysts creates uncertainty regarding the best practice management for general gynecologists caught off guard by their location when found intraoperatively. Rarer still are retroperitoneal ovaries. There are few cases of retroperitoneal ovary displacement in the literature, most often associated with female neonatal incarcerated inguinal hernia repair surgery. We found one case report involving an intraoperatively found retroperitoneal ovarian cystadenofibroma in a patient with a hernia repair performed in infancy. To our knowledge, there are no bilateral retroperitoneal ovaries described in the literature. Objective: To report a rare case of bilateral retroperitoneal ovaries in an 11 year old female. Methods: Case report. Results: We present an 11-year-old female with a past medical history of Dandy-Walker malformation and hydrocephalus status post ventriculoperitoneal (VP) shunt with six laparoscopic VP shunt revisions who endorsed hirsutism and irregular, heavy, and painful menstrual periods. Her family history is significant for polycystic ovarian syndrome (PCOS), endometriosis, and Lynch syndrome. Physical exam revealed mild abdominal distention and lower abdominal tenderness. She had an elevated estradiol, luteinizing hormone (LH), and total and free testosterone. Normally, an elevated estradiol causes a negative feedback response that results in a low LH. Due to these abnormal results, a pelvic ultrasound was ordered to rule out a potential estrogen secreting ovarian tumor. Ultrasound revealed a large anechoic structure in the left adnexa not definitively separated from the ovary measuring 13.0 x 7.7 x 10.7 cm. Radiologic differential diagnosis included large simple cyst, hemorrhagic cyst, and serous neoplasm. A referral to an adolescent gynecologist was made where the patient presented five months later with 25-pound weight gain, constipation, and a hard belly. Repeat ultrasound was consistent with a left ovarian simple cyst with an increased size measuring 16.78 x 12.58 x 8.66 cm as well as a new finding consistent with a right ovarian simple cyst measuring 4.96 x 4.48 x 3.61 cm. Due to her history of severe abdominal adhesions secondary to multiple laparoscopic VP shunt revisions, likely endometriosis, and large ovarian cyst, she was referred to a gynecologic oncologist where she was consented for robotic assisted bilateral ovarian cystectomy and lysis of adhesions. In the operating room, extensive pelvic adhesions were lysed to reveal bilateral retroperitoneal ovaries. The right cyst was drained of clear fluid, revealing no internal complexity, and was completely excised. Persistent pelvic adhesions and cyst size prolonged the time required to excise the left ovarian cyst wall. Once open, papillary excrescences were seen inside the cyst raising concern for a borderline ovarian tumor. The decision was made to remove the cyst wall in its entirety regardless of increased risk of ovarian function compromise. Restoration of normal anatomy was not attainable, and her bilateral ovaries remained embedded in the side walls and superior to the iliac vessels. Pathology revealed a benign 8 cm right paratubal cyst and a benign 18 cm left serous cystadenoma with papillary excrescences. Conclusions: Pelvic anatomy can be greatly distorted secondary to prior abdominal surgeries or certain pathologies including endometriosis and congenital abnormalities. Ultrasound cannot easily discriminate between intraperitoneal and retroperitoneal ovarian cysts but is often the only preoperative imaging obtained prior to cystectomy. Thus, many retroperitoneal cysts are found intraoperatively resulting in diagnostic and therapeutic challenges. Therefore, general gynecologists must be aware of risk factors associated with abnormal pelvic anatomy and prepared to request intraoperative consultation if needed to ensure optimized patient care and safety.

A Rare Case of Bilateral Retroperitoneal Ovaries: A Case Report and Review of the Literature

Introduction: Retroperitoneal gynecologic tumors and cysts are rare. They are often asymptomatic or present similarly to their intraperitoneal counterpart with vague abdominal discomfort and distention. Our literature review revealed that many retroperitoneal cysts are found intraoperatively due to their benign predominance consistent with their ultrasound findings. The rarity of retroperitoneal gynecologic tumors and cysts creates uncertainty regarding the best practice management for general gynecologists caught off guard by their location when found intraoperatively. Rarer still are retroperitoneal ovaries. There are few cases of retroperitoneal ovary displacement in the literature, most often associated with female neonatal incarcerated inguinal hernia repair surgery. We found one case report involving an intraoperatively found retroperitoneal ovarian cystadenofibroma in a patient with a hernia repair performed in infancy. To our knowledge, there are no bilateral retroperitoneal ovaries described in the literature. Objective: To report a rare case of bilateral retroperitoneal ovaries in an 11 year old female. Methods: Case report. Results: We present an 11-year-old female with a past medical history of Dandy-Walker malformation and hydrocephalus status post ventriculoperitoneal (VP) shunt with six laparoscopic VP shunt revisions who endorsed hirsutism and irregular, heavy, and painful menstrual periods. Her family history is significant for polycystic ovarian syndrome (PCOS), endometriosis, and Lynch syndrome. Physical exam revealed mild abdominal distention and lower abdominal tenderness. She had an elevated estradiol, luteinizing hormone (LH), and total and free testosterone. Normally, an elevated estradiol causes a negative feedback response that results in a low LH. Due to these abnormal results, a pelvic ultrasound was ordered to rule out a potential estrogen secreting ovarian tumor. Ultrasound revealed a large anechoic structure in the left adnexa not definitively separated from the ovary measuring 13.0 x 7.7 x 10.7 cm. Radiologic differential diagnosis included large simple cyst, hemorrhagic cyst, and serous neoplasm. A referral to an adolescent gynecologist was made where the patient presented five months later with 25-pound weight gain, constipation, and a hard belly. Repeat ultrasound was consistent with a left ovarian simple cyst with an increased size measuring 16.78 x 12.58 x 8.66 cm as well as a new finding consistent with a right ovarian simple cyst measuring 4.96 x 4.48 x 3.61 cm. Due to her history of severe abdominal adhesions secondary to multiple laparoscopic VP shunt revisions, likely endometriosis, and large ovarian cyst, she was referred to a gynecologic oncologist where she was consented for robotic assisted bilateral ovarian cystectomy and lysis of adhesions. In the operating room, extensive pelvic adhesions were lysed to reveal bilateral retroperitoneal ovaries. The right cyst was drained of clear fluid, revealing no internal complexity, and was completely excised. Persistent pelvic adhesions and cyst size prolonged the time required to excise the left ovarian cyst wall. Once open, papillary excrescences were seen inside the cyst raising concern for a borderline ovarian tumor. The decision was made to remove the cyst wall in its entirety regardless of increased risk of ovarian function compromise. Restoration of normal anatomy was not attainable, and her bilateral ovaries remained embedded in the side walls and superior to the iliac vessels. Pathology revealed a benign 8 cm right paratubal cyst and a benign 18 cm left serous cystadenoma with papillary excrescences. Conclusions: Pelvic anatomy can be greatly distorted secondary to prior abdominal surgeries or certain pathologies including endometriosis and congenital abnormalities. Ultrasound cannot easily discriminate between intraperitoneal and retroperitoneal ovarian cysts but is often the only preoperative imaging obtained prior to cystectomy. Thus, many retroperitoneal cysts are found intraoperatively resulting in diagnostic and therapeutic challenges. Therefore, general gynecologists must be aware of risk factors associated with abnormal pelvic anatomy and prepared to request intraoperative consultation if needed to ensure optimized patient care and safety.

Accessory Right and Left Hepatic Arteries in a 69 year-old White Female Donor with a Duplicated Inferior Vena Cava: Anatomical and Surgical Significance

Hepatic artery variations are relatively common findings in patients with an array of findings that were categorized and quantified in the Michels classification system. Findings of an accessory right and left hepatic artery were found in a 69 year-old white female donor with a duplicated inferior vena cava during routine anatomical dissection (Michels type 7 variation). Research supports that the hepatic artery variations can be due to either failure of regression of ventral anastomosis between the dual aortas or failed regression of fetal hepatic blood flow. Given the concurrent development of the IVC and aortic branches during the fourth to seventh week of gestation, this patient’s duplicated IVC and abnormal CT and SMA anatomy are likely related, both a result of failure of regression of fetal vasculature. There are no known associated comorbidities with this vascular variation however it is clinically significant when undergoing any hepatobiliary surgical procedures and suggests an increased risk for hepatic ischemic events.

Primigravida with Fetal Malpresentation and Bicornuate Uterus: A Case Report

Introduction: Malpresentation of the fetal head in left oblique presentation is a rare obstetric complication but has the potential to cause birth difficulties. This condition can be worsened by anatomical abnormalities of the uterus such as a bicornuate uterus. Case presentation: An 18-year-old primigravida presented with complaints of labor and premature rupture of membranes. Examination revealed malpresentation of the fetal head in left oblique presentation, and further ultrasound examination revealed a possible bicornuate uterus. Conclusion: Careful labor management and appropriate surgical intervention, in this case, an emergency caesarean section, resulted in the birth of a healthy baby girl. This case emphasizes the importance of early diagnosis and a multidisciplinary approach in treating complex cases of fetal malpresentation.

Prevalence of small intestinal bacterial overgrowth in intestinal failure syndrome: A systematic review and meta-analysis.

Patients with intestinal failure (IF) have abnormal intestinal anatomy, secretion, and dysmotility, which impairs intestinal homeostatic mechanisms and may lead to small intestinal bacterial overgrowth (SIBO). We conducted a systematic review and meta-analysis to determine the prevalence of SIBO in patients with IF and to identify risk factors for SIBO. MEDLINE (PubMed) and Embase electronic databases were searched from inception to December 2023 for studies that reported the prevalence of SIBO in IF. The prevalence rates, odds ratio (OR), and 95% confidence intervals of SIBO in IF and the risk factors for SIBO in IF were calculated using random effects model. Final dataset included nine studies reporting on 407 patients with IF. The prevalence of SIBO in IF was 57.5% (95% CI 44.6-69.4), with substantial heterogeneity in this analysis (I2=80.9, P=0.0001). SIBO prevalence was sixfold higher in patients with IF who received parenteral nutrition (PN) compared with IF patients not on PN (OR=6.0, 95% CI 3.0-11.9, P=0.0001). Overall, the prevalence of SIBO in patients with IF using PPI/acid-suppressing agents (72.0%, 95% CI 57.5-83.8) was numerically higher compared with IF patients not using these agents (47.6%, 95% CI 25.7-70.2). This systematic review and meta-analysis suggests that there is an increased risk of SIBO in patients with IF and that PN, and potentially, the use of PPI/acid-suppressing agents is risk factors for SIBO development in patients with IF. However, the quality of evidence is low and can be attributed to lack of case-control studies and clinical heterogeneity seen in the studies.

Neuroanatomical Features of NAA10- and NAA15-Related Neurodevelopmental Syndromes.

NAA10-related (Ogden Syndrome) and NAA15-related neurodevelopmental syndromes present with varying degrees of intellectual disability, hypotonia, congenital cardiac abnormalities, seizures, and delayed speech and motor development. While there is much data on the clinical manifestations of these conditions, there are few radiologic reports describing the neuroanatomical abnormalities present on imaging. Our goal was to provide neuroimaging analyses for a subset of probands with NAA10- and NAA15-related neurodevelopmental symptoms and assess severity, common radiologic anomalies, and changes over time to better understand the pathophysiology of these disease processes. Neuroimaging studies from 26 probands (18 with pathogenic variants in NAA10, 8 with pathogenic variants in NAA15) were collected and analyzed. Size of the cerebrum, brainstem, and cerebellum, as well as myelination, brain malformations, globus pallidus hyperintensity, brain lesions, 4th ventricle size, tegmentovermian angle, cisterna magna size, pituitary size, olfactory tract, palate arch, and choroid plexus abnormalities were analyzed. In depth medical histories were also collected on all probands, including genetic testing results and social, cognitive, and developmental history. The Vineland 3 Adaptive Behavior Scale was also administered to the parents to assess functional status of the probands. On average, individuals with Ogden Syndrome had 5.7 anatomical abnormalities (standard deviation (SD) = 3.0), whereas those with NAA15 related neurodevelopmental syndrome had 2.8 (SD = 2.3) (p = .02). Probands who had more anatomical abnormalities tended to score worse on Vineland assessments, suggesting a possible correlation between the two. Structural-functional anatomic differences seen were preserved such that individuals with greater defects on, for example, motor regions of their scans tested worse on motor portions of the Vineland. Probands followed longitudinally demonstrated several changes between scans, most commonly in the cerebellum, brainstem, and degree of myelination. Such changes were only observed for probands with NAA10 variants in our cohort. Despite clinical imaging being reported as being predominantly "normal" during routine clinical care, this analysis of a cohort of patients with NAA10-related (Ogden Syndrome) and NAA15-related neurodevelopmental syndrome by one neuroradiologist has established a range of subtle abnormalities. We hope these findings guide future research and diagnostic studies for this patient population.

Medical management of anorectal malformations.

Anorectal malformations (ARMs) are congenital anomalies of the anorectum and the genitourinary system that result in a broad spectrum of hindgut anomalies. Despite surgical correction patients continue to have late postoperative genitourinary and colorectal dysfunction that have significant impact on quality of life. This paper will review the current evidence and discuss the evaluation and management of postoperative patients with ARMs who present with persistent defecation disorder. A literature search was conducted using PubMed/MEDLINE/EMBASE databases applying the following terms: ARMs, imperforate anus, constipation, faecal incontinence, neurogenic bowel, posterior sagittal anorectoplasty. Patients who present with postoperative defecation disorders require timely diagnostic and surgical evaluation for anatomic abnormalities prior to initiation of bowel management. Goals of management are to avoid constipation in young children, achieve faecal continence in early childhood and facilitate independence in older children and adolescents. Treatment options vary from high dose stimulant laxatives to high-volume retrograde and antegrade enemas that facilitate mechanical colonic emptying. Appropriate diagnostic work-up and implementation of treatment can decrease long-term morbidity and improve quality of life in postoperative patients with ARMs who presents with defecation disorders.

The Extended Utilization of Bulking Agents in Pediatric Urology.

Traditional surgical management for urinary incontinence and vesicoureteral reflux often requires complex reconstructive surgery and extended hospitalizations. Since the introduction of endoscopic bulking agents in 1973, there has been increasing interest in the use of endoscopic injection (EI) and bulking for the treatment of a variety of pediatric urologic disorders. The purpose of this review is to summarize the most recent literature addressing the use of bulking agents in pediatric urology. The most recent literature has focused primarily on the use of EI of bulking agents at the bladder neck for the treatment of urinary incontinence. Other uses of EI of bulking agents has focused on the treatment of vesicoureteral reflux (VUR) in patients with anatomic abnormalities or treatment of incontinence catheterizable channels. The development of advanced techniques for endoscopic injection along with safe, stable bulking agents has allowed for the treatment of a variety of urologic conditions. This minimally invasive procedure offers an additional tool for the pediatric urologist's armamentarium in the treatment of urinary incontinence and VUR.

FECALOME GEANT A PROPOS D’UNE OBSERVATION ET REVUE DE LA LITTERATURE A L’HOPITAL DONKA

Fecal impaction is a mass of hardened fecal matter impacted primarily in the rectum and sigmoid. The most common sites of fecal impaction are the sigmoid colon and the rectum. We report a case of giant ileocecal fecal impaction causing OIA which was treated surgically. Case report: 25-year-old patient, student, admitted to the department for progressive abdominal pain accompanied by cessation of materials and gases, and abdominal bloating lasting for two (2) weeks. As a history, we noted stubborn constipation and episodes of subocclusion. The clinical examination noted a deterioration in general condition WHO stage I, conjunctival hypocoloration, polypnea at 35 cycles/mm and tachycardia at 100/min. The abdomen was very distended and tympanic, intestinal peristalsis was inaudible. Rectal examination revealed vacuity of the rectal bulb. The diagnosis of OIA/VCP was made; the unprepared abdominal X-ray showed aerocolia; biological examination, normochromic and normocytic anemia at 10g/l. Intraoperatively, we found a giant ileocecal fecal impaction extending from the rectosigmoid hinge to the last ileal loops (50 cm from the ileocecal angle) on dolichomegacolon. (Figure 2) We carried out a sigmoidectomy to extract the fecal impaction which weighed 15 KG (figure 3), using the colostomy according to Hartmann. The operating part addressed to the anapath did not show any particularity. Three (3) months later we restored continuity. The ileal fecal masses were extracted manually. The postoperative course was simple; 16 weeks after the first intervention we restored continuity. Conclusion: Fecal impaction occurs when a large amount of fecal matter is compacted and cannot be evacuated spontaneously. It is significantly common in severe chronic constipation and in other at-risk patients with anatomical or functional anorectal abnormalities. Key words: giant fecal impaction, intestinal obstruction, Donka Hospital.