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1.
JBJS Case Connect ; 14(1)2024 Jan 01.
Article in English | MEDLINE | ID: mdl-38452165

ABSTRACT

CASE: We present a case of an 8-year-old boy with classical bladder exstrophy and a neglected right hip dislocation, exemplifying the risk of missed developmental dysplasia of the hip (DDH) in patients with exstrophy requiring careful orthopaedic oversight. CONCLUSIONS: When treating patients with bladder exstrophy, physicians and surgeons should be vigilant not to miss associated DDH. If this condition requires surgical treatment, preoperative planning with computed tomography scans is vital to uncovering the complexities arising from abnormal pelvic and acetabular anatomy and ensuring successful treatment outcomes.


Subject(s)
Bladder Exstrophy , Developmental Dysplasia of the Hip , Hip Dislocation, Congenital , Male , Humans , Child , Bladder Exstrophy/complications , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/surgery , Acetabulum , Hip Dislocation, Congenital/complications , Hip Dislocation, Congenital/diagnostic imaging , Hip Dislocation, Congenital/surgery , Pelvis
2.
BMC Pregnancy Childbirth ; 24(1): 122, 2024 Feb 09.
Article in English | MEDLINE | ID: mdl-38336714

ABSTRACT

BACKGROUND: According to prenatal ultrasonographic studies, single umbilical artery may be present alone or in association with other fetal abnormalities. So far, the exact pathogenesis of bladder exstrophy is unclear. Some scholars believe that bladder exstrophy and cloacal exstrophy should be regarded as a disease spectrum to explore their pathogenesis. If bladder exstrophy and cloacal exstrophy are regarded as the same disease spectrum, then we can speculate that the single umbilical artery should have the probability of being accompanied by bladder exstrophy at the same time. CASE PRESENTATION: For the first time, we report a rare case of fetal bladder exstrophy with single umbilical artery in single pregnancy. This patient underwent targeted color Doppler ultrasound at 26 weeks of pregnancy which first suspected bladder exstrophy with single umbilical artery and fetal MRI for diagnosis at 38 + 3 weeks of pregnancy which confirmed the suspicion. After the diagnosis was confirmed, the patient was scheduled for a multidisciplinary discussion. Ultimately the patient opted for induced fetal demise at 38 + 5 weeks of pregnancy and the physical appearance of the fetal demise affirmed previous ultrasound and MRI examination results. CONCLUSIONS: Our report is the first finding of single umbilical artery combined with bladder exstrophy in a singleton pregnancy. Accordingly, our case enhances the evidence that cloacal exstrophy and bladder exstrophy should be treated as the same disease spectrum. In addition, we conducted a literature review on the diagnostic progress of single umbilical artery combined with bladder exstrophy, hoping to provide useful references for the diagnosis of this disease.


Subject(s)
Bladder Exstrophy , Single Umbilical Artery , Pregnancy , Female , Humans , Bladder Exstrophy/complications , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/pathology , Ultrasonography, Prenatal/methods , Prenatal Care , Fetal Death
3.
Urology ; 180: 235-241, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37473922

ABSTRACT

OBJECTIVE: To determine intrapelvic angles and distances in pure epispadias patients and compare them to normal values. METHODS: Using three-dimensional computed tomography, 17 intrapelvic dimensions (8 angles and 9 distances) were measured in 26 patients with pure epispadias (21 boys and 5 girls). These values were compared to normal values obtained from 6 patients (5 boys and 1 girl) who underwent either pelvic or abdominopelvic computed tomography for purposes unrelated to their bony pelvis. RESULTS: Significant differences were observed in five angles (sacroiliac joint angle, S1 tilt angle, sacral curvature, superior-inferior rotation of the pelvis, and pubococcygeal angle; P-value=.016, .044, .011, .020, and .001, respectively); these show less sacral rotation toward the axial plane, more sacral curvature, inferior rotation of the pelvis, and sacroiliac joints' rotation toward the coronal plane in epispadias compared to controls. Also, two distances (pubic diastasis and anterior segment length of the pelvis; P-value=.002 and .012, respectively) had significant differences, showing wider pubic diastasis and shorter anterior segment in epispadias. However, the differences between other intrapelvic angles and distances were not statistically significant between the two groups. CONCLUSION: In addition to the explanations hypothesized for the embryology of the exstrophy-epispadias complex, there can be other etiologies for both epispadias and bladder exstrophy to explain the differences between bony anatomies of the pelvis in these patients.


Subject(s)
Bladder Exstrophy , Epispadias , Pelvic Bones , Male , Female , Humans , Epispadias/diagnostic imaging , Epispadias/surgery , Pelvic Bones/diagnostic imaging , Tomography, X-Ray Computed/methods , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/surgery , Pelvic Floor
4.
J Orthop Surg Res ; 18(1): 329, 2023 May 02.
Article in English | MEDLINE | ID: mdl-37131198

ABSTRACT

BACKGROUND: Several types of pelvic osteotomy techniques have been reported and employed by orthopedic surgeons to enhance the approximation of symphyseal diastasis in bladder exstrophy patients. However, there is limited evidence on a long-term follow-up to confirm which osteotomy techniques provide the most suitable and effective outcomes for correcting pelvic deformities. This study aimed to describe the surgical technique of bilateral iliac bayonet osteotomies for correcting pelvic bone without using fixation in bladder exstrophy and to report on the long-term clinical and radiographic outcomes following the bayonet osteotomies. METHODS: We retrospectively reviewed patients with bladder exstrophy who underwent bilateral iliac bayonet osteotomies with the closure of bladder exstrophy between 1993 and 2022. Clinical outcomes and radiographic pubic symphyseal diastasis measurements were evaluated. From a total of 28 operated cases, eleven were able to attend a special follow-up clinic or were interviewed by telephone by one of the authors with completed charts and recorded data. RESULTS: A total of 11 patients (9 female and 2 male) with an average age at operation of 9.14 ± 11.57 months. The average followed-up time was 14.67 ± 9.24 years (0.75-29), with the average modified Harris Hip score being 90.45 ± 1.21. All patients demonstrated decreased pubic symphyseal diastasis distance (2.05 ± 1.13 cm) compared to preoperative (4.58 ± 1.37 cm) without any evidence of nonunion. At the latest follow-up, the average foot progression angle was externally rotated 6.25° ± 4.79° with full hips ROM, and no patients reported abnormal gait, hip pain, limping, or leg length discrepancy. CONCLUSIONS: Bilateral iliac wings bayonet osteotomies technique demonstrated a safe and successful pubic symphyseal diastasis closure with an improvement both clinically and radiographically. Moreover, it showed good long-term results and excellent patient's reported outcome scores. Therefore, it would be another effective option for pelvic osteotomy in treating bladder exstrophy patients.


Subject(s)
Bladder Exstrophy , Pelvic Bones , Pubic Symphysis Diastasis , Humans , Male , Female , Infant , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/surgery , Retrospective Studies , Ilium/surgery , Pelvic Bones/diagnostic imaging , Pelvic Bones/surgery , Pelvic Bones/abnormalities , Pubic Symphysis Diastasis/surgery , Osteotomy/methods
5.
J Pediatr Surg ; 58(10): 1949-1953, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37179209

ABSTRACT

INTRODUCTION: A single institutional study characterizes the rate of prenatal diagnosis of cloacal exstrophy (CE) and examines its role on successful primary closures. MATERIALS AND METHODS: An institutional database of 1485 exstrophy-epispadias patients was reviewed retrospectively for CE patients with confirmed presence/absence of prenatal diagnostics, primary exstrophy closure since 2000, institution of closure, and at least 1 year of follow up following closure. RESULTS: The cohort included 56 domestic patients and 9 international patients. Overall, 78.6% (n = 44) of domestic patients were prenatally diagnosed while 21.4% (n = 12) were diagnosed postnatally. A positive trend was observed in the rate of prenatal diagnosis across the study period, 56.3%, 84.2%, 88.9% respectively (p = 0.025). Confirmatory fMRI was obtained in 40.9% (n = 18) of prenatally diagnosed cases. Patients diagnosed prenatally were found to be more likely to undergo treatment at exstrophy centers of excellence (72.1% v 33.3%, p = 0.020). Prenatal diagnosis was not predictive of increased rate of successful primary closure (75.6% vs 75.0%; p = 1.00; OR: 1.03, 95% CI: 0.23-4.58). Primary closures undertaken at exstrophy centers of excellence were significantly more likely to be successful compared to outside hospitals (90.9% v 50.0%, p = 0.002). CONCLUSIONS: The rate of prenatal diagnosis of CE in patients referred for management to a high-volume exstrophy center is improving. Despite this improvement, patients continue to be missed in the prenatal period. While prenatal diagnosis offers the ideal opportunity to educate, counsel, and prepare expectant families, patients diagnosed at birth are not disadvantaged in their ability to receive a successful primary closure. Further research should investigate the benefit of patient referral to high-volume exstrophy centers of care to ensure optimal care and outcomes.


Subject(s)
Anorectal Malformations , Bladder Exstrophy , Epispadias , Infant, Newborn , Pregnancy , Female , Humans , Retrospective Studies , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/surgery , Prenatal Diagnosis
6.
J Clin Ultrasound ; 51(4): 644-651, 2023 May.
Article in English | MEDLINE | ID: mdl-36972256

ABSTRACT

Color Doppler (CD) is an established diagnostic modality for bladder exstrophy. We present two mid-trimester difficult-to-diagnose cases with no obvious infraumbilical mass bulge, assessed by CD in sagittal and axial pelvic views. The first case was a classical bladder exstrophy at 19 weeks nestled under the umbilical-cord and the second case was an omphalocele-exstrophy-imperforate-anus-spinal complex at 18 weeks 4 days gestation with exstrophic bladder embedded under the omphalocele. The altered course of umbilical arteries in relation to pelvic bony landmarks seen in these fetuses could be an objective approach to complement mid-trimester diagnosis of bladder exstrophy regardless of mass bulge.


Subject(s)
Abnormalities, Multiple , Bladder Exstrophy , Hernia, Umbilical , Pregnancy , Female , Humans , Bladder Exstrophy/diagnostic imaging , Umbilical Arteries/diagnostic imaging , Hernia, Umbilical/diagnosis , Abnormalities, Multiple/diagnosis , Ultrasonography, Prenatal , Pelvis
8.
Childs Nerv Syst ; 39(2): 517-526, 2023 02.
Article in English | MEDLINE | ID: mdl-36155841

ABSTRACT

BACKGROUND/IMPORTANCE: Gollop-Wolfgang complex is a rare skeletal dysplasia with only 200 cases reported in the literature. This disorder is usually associated with several extraosseous anomalies. This report describes the first case of a fatty filum terminale and a low-lying conus medullaris in a patient with this complex. A review of the current literature of the Gollop-Wolfgang complex accompanies this case, highlighting the documented extraosseous anomalies seen in this complex. CLINICAL PRESENTATION: We report a case of an 18-month-old patient with Gollop-Wolfgang complex who underwent cord untethering with release of the filum terminale after extensive workup showed the presence of a dyssynergic bladder and radiological evaluation revealed a fatty filum terminale and low-lying conus medullaris. CONCLUSION: Gollop-Wolfgang complex is a skeletal dysplasia usually associated with several extra skeletal anomalies. Our report describes the first case of a fatty filum terminale and low-lying conus medullaris in this complex, as well as provides an overview of the documented anomalies seen in this disorder. A multidisciplinary approach is recommended when treating these infants in order to ensure that occult manifestations of the complex are not missed.


Subject(s)
Abnormalities, Multiple , Cauda Equina , Femur , Hand Deformities, Congenital , Spinal Cord , Tibia , Cauda Equina/diagnostic imaging , Spinal Cord/diagnostic imaging , Spinal Cord/surgery , Abnormalities, Multiple/diagnostic imaging , Femur/abnormalities , Femur/diagnostic imaging , Hand Deformities, Congenital/diagnostic imaging , Tibia/abnormalities , Tibia/diagnostic imaging , Humans , Female , Infant , Radiography , Bladder Exstrophy/diagnostic imaging , Neurosurgical Procedures
9.
BMC Pregnancy Childbirth ; 22(1): 435, 2022 May 24.
Article in English | MEDLINE | ID: mdl-35610593

ABSTRACT

BACKGROUND: Penile duplication or diphallia is a rare congenital anomaly with unclear pathophysiological cause. Most cases of diphallia are reported postnatally; however, today with the use of a high-resolution ultrasound device, in-uterine diagnosis of many congenital anomalies is possible. CASE PRESENTATION: Herein we report a multiparous mother at 25 weeks of gestation who referred due to an abnormal cystic structure protruding from a large abdominal wall defect located below the umbilicus that was noted during a routine exam. Target scan revealed two penile-like protrusions with an empty scrotal sac and double bladder in an otherwise normal fetus, which was confirmed postnatally. Neonatal microarray study and karyotype were normal. CONCLUSION: Diphallia could be detected prenatally as an isolated anomaly, associated with caudal duplication syndrome, or as an exstrophy-epispadias complex. As this is a rare congenital anomaly, all sonographers should be familiar with prenatal ultrasound features and associated anomalies, an important issue in prenatal counseling with parents, delivery planning, psychological support of the family, and postnatal management.


Subject(s)
Bladder Exstrophy , Urogenital Abnormalities , Bladder Exstrophy/diagnosis , Bladder Exstrophy/diagnostic imaging , Female , Humans , Infant, Newborn , Male , Penis/abnormalities , Pregnancy , Prenatal Diagnosis , Ultrasonography, Prenatal , Urogenital Abnormalities/diagnosis
10.
Urology ; 165: 305-311, 2022 07.
Article in English | MEDLINE | ID: mdl-35038493

ABSTRACT

OBJECTIVE: To quantitatively measure the anatomical variations of the pelvic floor in children with exstrophy-epispadias complex using magnetic resonance imaging. MATERIALS AND METHODS: Six cases of classic bladder exstrophy (CBE), 5 cases of penile epispadias (PE) and 11 cases of penopubic epispadias (PPE) were included. Another 8 cases with the testicular tumor were taken as the controls. A series of measurements obtained from the pelvic floor magnetic resonance imaging were analyzed, and the measurements with significant differences were obtained by ANOVA. RESULTS: The pelvic floor of the CBE was significantly different from that of controls in measurements including wider pubic diastasis (P <.001), greater posterior anal distance (P = .019), greater posterior bladder neck distance (P = .004), larger iliac wing angle (P <.001), diminutive ischial angle (P <.001), bigger puborectalis angle (P <.001), larger ileococcygeous angle (P = .002) and shortened anterior corporal length (P <.001). For the PE, the posterior bladder neck distance (P = .038) was greater than that of controls. In the PPE, the posterior bladder neck distance (P = .001) and puborectalis angle (P = .026) was greater than that of controls, respectively. CONCLUSION: CBE shows severe anatomical variations of the pelvic floor. The bladder neck moves more anteriorly both in PE and PPE than the control. The enlarged puborectalis angle resulting from wider pubic diastasis and more anterior position of the anorectal canal is also noticed in PPE.


Subject(s)
Bladder Exstrophy , Digestive System Abnormalities , Epispadias , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/pathology , Bladder Exstrophy/surgery , Child , Epispadias/complications , Epispadias/diagnostic imaging , Epispadias/surgery , Humans , Magnetic Resonance Imaging , Male , Pelvic Floor/diagnostic imaging , Urinary Bladder
11.
J Clin Ultrasound ; 49(8): 834-837, 2021 Oct.
Article in English | MEDLINE | ID: mdl-34235755

ABSTRACT

Unlike classic exstrophy, covered bladder exstrophy is a rare variant characterized by a cycling bladder and intact abdominal wall. We present a case of covered bladder exstrophy diagnosed prenatally and associated persistent cloaca (PC) noted only after delivery. This case report demonstrates that prenatal diagnosis of covered bladder exstrophy is possible and PC can present without any abdominal cysts, bowel, or renal findings. Covered bladder exstrophy should be considered in the differential of cystic protrusion of the bladder to the abdominal wall.


Subject(s)
Bladder Exstrophy , Digestive System Abnormalities , Animals , Bladder Exstrophy/diagnostic imaging , Cloaca/diagnostic imaging , Female , Humans , Pregnancy , Prenatal Diagnosis , Ultrasonography, Prenatal
12.
J Pediatr Urol ; 17(3): 393.e1-393.e7, 2021 06.
Article in English | MEDLINE | ID: mdl-33583747

ABSTRACT

INTRODUCTION: Bilateral ureteral reimplantation at the time of the complete primary repair of bladder exstrophy (BUR-CPRE) has been proposed and has demonstrated favorable outcomes in the past. However, the potential benefits, including prevention of vesicoureteral reflux (VUR) and renal scarring must be tempered with any risks of reimplantation, persistent VUR, and the potential for overtreatment. We aimed to determine the impact of BUR-CPRE on reflux rates, renogram findings and bladder capacity. METHODS: An IRB approved registry of children treated for bladder exstrophy epispadias complex (BEEC) during a long-term international collaboration hosted in a region with high prevalence of BEEC was queried. Children undergoing primary CPRE for bladder exstrophy (BE) were identified. Surgical procedure and outcome measures nuclear medicine dimercaptosuccinic acid (DMSA) scintigraphy scans, voiding cystourethrogram (VCUG), and urodynamic study (UDS) were assessed for presence and degree of VUR, renogram abnormalities, and bladder capacity. RESULTS: A total cohort of 147 patients with BEEC was queried; 52 children (37 males, 71%) underwent primary CPRE for BE between 2009 and 2019 at median age of 1.1 years (IQR 0.6-1.9 years) with median follow up 4.4 years (IQR 2.4-6.4 years). BUR-CPRE was performed in 22/52 (42%). After BUR-CPRE, children were less likely to have VUR (any VUR present in 9 of 20 with imaging (45%) compared to 23 of 26 with imaging (82%) in the CPRE alone group (p = 0.007)). VUR in the BUR-CPRE group tended to be unilateral and lower grade in comparison to the CPRE alone group. DMSA abnormalities were less common in the BUR-CPRE group (4/19 (21%) vs.12/27 (44%)), although the difference did not reach statistical significance (p = 0.1). At 4 years follow-up, the BUR-CPRE group had a larger bladder capacity (p = 0.016). DISCUSSION: After BUR-CPRE, children had a lower rate of VUR, and when present, VUR was more often unilateral and lower grade compared to the CPRE alone group. Fewer numbers of children in the BUR-CPRE group depicted DMSA abnormalities. No children developed obstruction after BUR-CPRE and none have undergone repeat reimplantation. We documented a larger bladder capacity at the time of maximum follow-up available (4 years)-but further data are needed to confirm this observation. CONCLUSION: BUR-CPRE decreases the incidence and severity of VUR after CPRE, but the clinical significance of this remains unclear. We are encouraged by these initial results, but since BUR-CPRE does not uniformly eliminate VUR, we continue to proceed carefully in the well selected patient.


Subject(s)
Bladder Exstrophy , Vesico-Ureteral Reflux , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/surgery , Child , Humans , Infant , Male , Replantation , Retrospective Studies , Vesico-Ureteral Reflux/diagnostic imaging , Vesico-Ureteral Reflux/surgery
14.
J Pediatr Urol ; 16(6): 835.e1-835.e9, 2020 Dec.
Article in English | MEDLINE | ID: mdl-33023849

ABSTRACT

OBJECTIVE: Our aim was to evaluate the effectiveness of anterior osteotomy in the restoration of normal pelvic floor anatomy in classic bladder exstrophy repair using pelvic floor MRI as an imaging tool for evaluation. PATIENTS AND METHODS: This study is a pilot prospective randomized controlled study that included 20 infants with classic bladder exstrophy older than three months with no history of previous surgical operations. All patients underwent complete primary repair and were randomized into two groups, with or without osteotomy. Both groups were assessed preoperatively & postoperatively after three months with pelvic floor MRI and compared to age & gender-matched control group infants with normal pelvic anatomy infants who underwent pelvic MRI for other medical conditions. RESULTS: There was not any significant statistical difference between osteotomy and non-osteotomy groups in pre-operative demographic data and all pelvic floor MRI measurements, emphasizing that both groups were equal in all characters at the start point of the study before the operation. The mean change difference between pre-operative and post-operative pelvic floor MRI measurements were compared in both groups, and there was not any significant statistical difference in all pelvic floor MRI measurements. Both groups showed the same statistical significance when compared with the control group in all pelvic floor MRI measurements except the posterior bladder neck distance, which was in favor of the osteotomy group, closer to the normal pelvic floor anatomy. CONCLUSIONS: Anterior osteotomy did not have a significant difference in the restoration of the normal pelvic floor anatomy when combined with complete primary repair of classic bladder exstrophy in newly diagnosed neonates older than three months, except for a single measurement, posterior bladder neck distance. Future studies needed to determine the effect of osteotomy on continence.


Subject(s)
Bladder Exstrophy , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/surgery , Humans , Infant , Infant, Newborn , Magnetic Resonance Imaging , Osteotomy , Pelvic Floor/diagnostic imaging , Pelvic Floor/surgery , Pilot Projects , Prospective Studies
15.
J Pediatr Urol ; 16(6): 832.e1-832.e9, 2020 12.
Article in English | MEDLINE | ID: mdl-32981861

ABSTRACT

INTRODUCTION: Repair of classic bladder exstrophy (CBE) is known to alter dimensions of the bony pelvic ring. Pelvic volume and acetabular configuration are additional metrics which merit analysis in the reconstruction process. Advances in magnetic resonance imaging (MRI) allow for precise elucidation of such anatomy in pediatric patients, providing enhanced knowledge of how primary reconstruction may impact factors in pelvic health. METHODS: An IRB-approved exstrophy-epispadias database of 1337 patients was reviewed for patients with CBE who had pelvic MRI performed before and after repair. Pelvic MRIs were analyzed by a pediatric radiologist, and three-dimensional volumetric renderings of the true pelvis were calculated. Pre- and post-closure imaging were compared, in addition to imaging from age-matched controls without pelvic pathology. Cartilaginous acetabular index and version angles were also calculated and compared between groups. RESULTS: Eighteen patients with post-closure imaging, 14 of whom also had pre-closure imaging, and 23 control patients (ages 0-365 days) were included. The median ages at pre- and post-closure scan were 2 and 178 days, respectively. Osteotomy was performed in 6 (33%) of the closures. The median segmented pelvic volumes were 89 cm3 in the pre-closure group, 105 cm3 in the post-closure group, and 72 cm3 in the control group. At a given age, patients with CBE pre-closure had the largest pelvic volume, and those without bladder exstrophy demonstrated the smallest pelvic volumes (Summary Figure). CBE patients' pelvic volumes were overall lower following repair, relative to age (p = 0.007). Cartilaginous acetabular version angle increased following closure, with acetabular orientation converting from retroversion to anteversion in all cases. However, only acetabular version angles were significantly different between groups. DISCUSSION: This study found that pelvic volume significantly decreased relative to age following primary repair of CBE, but that it did not correct to control levels. Similarly, acetabular retroversion that is naturally seen in exstrophy patients was converted to anteversion post-closure but did not become completely normal. Knowledge of these persistent anatomical anomalies may be useful in treatment of future concerns related to exstrophy, such as continence achievement, pelvic organ prolapse, and potential gait disturbances. Lack of significant differences between study groups regarding acetabular index angles can give assurance to providers that hip dysplasia is neither a natural concern nor iatrogenically inflicted through reconstruction. CONCLUSIONS: The primary closure of exstrophy results in pelvic volumes and anteverted acetabula that more closely resemble those without bladder exstrophy, compared to pre-closure findings.


Subject(s)
Bladder Exstrophy , Acetabulum/diagnostic imaging , Acetabulum/surgery , Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/surgery , Child , Humans , Pelvis , Retrospective Studies , Treatment Outcome
16.
J Pediatr Urol ; 16(5): 665-671, 2020 Oct.
Article in English | MEDLINE | ID: mdl-32773250

ABSTRACT

INTRODUCTION: Prenatal determination of bladder exstrophy (BE) or cloacal exstrophy (CE), known also as the omphalocele-exstrophy-imperforate anus-spinal anomaly complex (OEIS), is challenging. Distinguishing between BE and CE is important because children with CE have many more challenges initially and during their lifetime. An accurate diagnosis is critical when counselling expectant parents. We hypothesized that there are key imaging features that can distinguish BE from CE, and that there are areas of diagnostic concordance and discordance between fetal ultrasound (fUS) and fetal MRI (fMRI) among these entities. MATERIALS AND METHODS: We queried a single institutional IRB-approved registry of children with BE and CE to identify those with accessible fetal imaging from 2000 to 2018, and formal interpretations were collected. Two pediatric radiologists performed independent retrospective blinded review of the images. Criteria evaluated included: genitalia, kidneys, bowel appearance, presence of anal dimple, location of insertion of umbilical cord into the abdomen relative to the abdominal wall defect, umbilical vessels, bladder protuberance, presence of omphalocele, and spine/neural cord abnormalities. We evaluated concordance between radiologic interpretations and postnatal diagnosis, as well as between specific findings in the two diagnostic modalities. RESULTS: Twenty-one infants born between 2000 and 2018 with BE or CE had fetal imaging for review: 15 had both fUS and fMRI, 2 had fUS alone, and 4 fMRI alone. There was 100% concordance between fUS and fMRI in evaluating kidneys, presence of anal dimple, location of abdominal insertion of umbilical cord relative to the defect, number of umbilical vessels, and spine abnormalities/level of neural cord termination. The following discrepancies were observed: 1) genitalia and bowel appearance, and bladder protuberance in 1/15 (6.7%); 2) presence of an omphalocele in 2/15 (13.3%). Of the initial radiologic interpretations, 4/17 (23.5%) of fUS and 2/19 (10.5%) of fMRI erroneously were interpreted as on the OEIS spectrum when the post-natal diagnosis was BE. Errors in diagnosis were due to a protuberant bladder plate extending beyond the plane of the abdominal wall with bowel loops posteriorly mimicking an omphalocele. In all of these BE cases, the abdominal wall defect was located inferior to the umbilical cord insertion on the abdominal wall. CONCLUSION: An everting bladder plate with bowel loops posterior to the plate in classic BE may be misdiagnosed as CE. Identification of the location of umbilical cord insertion relative to the abdominal wall defect, with fetal US or MRI, results in the correct differentiation between BE and CE.


Subject(s)
Anus, Imperforate , Bladder Exstrophy , Hernia, Umbilical , Anus, Imperforate/diagnostic imaging , Bladder Exstrophy/diagnostic imaging , Child , Female , Hernia, Umbilical/diagnostic imaging , Humans , Infant , Magnetic Resonance Imaging , Pregnancy , Prenatal Diagnosis , Retrospective Studies , Ultrasonography, Prenatal
17.
Clin Imaging ; 67: 55-57, 2020 Nov.
Article in English | MEDLINE | ID: mdl-32516694

ABSTRACT

Congenital pelvic anomalies include disorders of both the urinary and reproductive tracts. These disorders often coexist due to the overlapping embryological development of both systems. Magnetic resonance imaging (MRI) is a useful tool for characterization of the reproductive tract and can play an important role in the preoperative evaluation of patients with known bladder exstrophy. Accurate delineation of reproductive tract anomalies is critical as it can influence the surgical approach and can have implications for fertility. Here, we present a case of a 50-year-old female with uterine didelphys and congenital bladder exstrophy, which is an uncommon association. Our case is unique as the MR appearance of bladder exstrophy with concurrent uterine didelphys has not been reported. Additionally, this represents the first reported example of congenital herniation of both horns of the didelphys uterus through the mons pubis in the setting of bladder exstrophy.


Subject(s)
Bladder Exstrophy/diagnostic imaging , Urogenital Abnormalities/diagnostic imaging , Uterus/abnormalities , Bladder Exstrophy/complications , Bladder Exstrophy/surgery , Female , Humans , Magnetic Resonance Imaging , Middle Aged , Pelvis , Prolapse , Uterus/diagnostic imaging
18.
J Obstet Gynaecol Res ; 46(8): 1443-1449, 2020 Aug.
Article in English | MEDLINE | ID: mdl-32537902

ABSTRACT

We observed a case of classic cloacal exstrophy (CE) from 17 to 30 weeks' gestation. At 17 weeks, an omphalocele and single umbilical artery (SUA) were diagnosed with a normal female karyotype on amniocentesis. A pelvic cystic adjacent to SUA, considered to be the bladder at 17 weeks, became swollen to form double cysts at 25 weeks. A phallus-like structure along the lower abdomen was additionally detected, leading to the diagnosis of CE. Fetal magnetic resonance imaging (MRI) at 30 weeks confirmed that the phallus-like structure was of intestinal origin. The presence of a non-visualizable bladder together with abdominal wall defect or omphalocele and an elephant trunk-like deformity are key findings in the prenatal diagnosis of classic CE. Fetal MRI was useful in confirming ultrasonographic findings and obtaining additional findings for the diagnosis of CE. We have discussed clues and potential pitfalls in diagnosing CE, with a review of the literature.


Subject(s)
Bladder Exstrophy , Hernia, Umbilical , Bladder Exstrophy/diagnostic imaging , Female , Hernia, Umbilical/diagnostic imaging , Humans , Magnetic Resonance Imaging , Magnetic Resonance Spectroscopy , Pregnancy , Prenatal Diagnosis , Ultrasonography , Ultrasonography, Prenatal
19.
J Pediatr Orthop B ; 29(3): 209-213, 2020 May.
Article in English | MEDLINE | ID: mdl-30921248

ABSTRACT

The aim of this case series study is to describe the orthopedic management of bladder exstrophy and to report on the intermediate-term outcomes following bilateral horizontal Salter osteotomies with anterior symphyseal closure using a nylon tape. This retrospective study included seven patients (five boys and two girls) who underwent bilateral horizontal osteotomies after reconstruction of the urogenital deformity using the Cantwell-Ransley technique and symphyseal closure by nylon tape number 2-5. the age range was between 7 months and 8 years (median age = 17 months). Three patients had recurrent bladder exstrophy after they underwent "ramotomy" early in their life, whereas four were operated primarily. All patients were followed up over 2 years, with a mean follow-up duration of 3.27 years (2-5 years). Petrie cast was applied, with both legs abducted and internally rotated till bony union had been achieved. This allowed free handling of the wounds and catheters. Removal of k-wires was performed in an outpatient clinic after the complete union of bony osteotomies had been achieved (6-8 weeks). Satisfactory bladder closure was achieved in all patients. None of the patients had a bone infection or nonunion at the osteotomy sites. No postoperative wound dehiscence has occurred for up to 5 years. The foot progression angle improved from 37°-70° (median = 45°) preoperatively to 0°-15° (median = 5°) postoperatively. Symphyseal diastasis was closed well in all of our patients in postoperative plain radiography compared with 5.8-11 cm (median = 8.2 cm) diastasis preoperatively. One patient had a superficial wound infection. Another patient had a sutured bladder neck by a nylon tape during the symphyseal closure. Bilateral horizontal Salter osteotomies with anterior symphyseal closure using nylon tape are safe and effective in the management of bladder exstrophy, especially in older children and in extreme diastasis (> 6 cm); with improvement in the gait as it corrects the acetabular external rotation.


Subject(s)
Bladder Exstrophy/diagnostic imaging , Bladder Exstrophy/surgery , Nylons , Osteotomy/methods , Surgical Tape , Urologic Surgical Procedures/methods , Child , Child, Preschool , Female , Humans , Infant , Male , Retrospective Studies , Treatment Outcome
20.
Semin Pediatr Surg ; 28(5): 150839, 2019 Oct.
Article in English | MEDLINE | ID: mdl-31668296

ABSTRACT

Imaging of the reproductive tract is challenging and requires a general knowledge of congenital variations in anatomy. The anatomy of the developing fetus, whether a male phenotype or female phenotype, is also a dynamic process with many changes occurring during gestation. Families may ask details about the genitalia during prenatal imaging and when variations in what is thought to be normal are present, further investigation is sometimes needed to make sense of what is seen. This overview will describe categories of disorders of sex development (DSD), whether chromosomal or structural or both, and the current state of imaging of these anomalies.


Subject(s)
Disorders of Sex Development/diagnostic imaging , Fetus/diagnostic imaging , Magnetic Resonance Imaging , Ultrasonography, Prenatal , Adrenal Hyperplasia, Congenital/diagnostic imaging , Anus, Imperforate/diagnostic imaging , Bladder Exstrophy/diagnostic imaging , Cloaca/abnormalities , Diagnosis, Differential , Female , Hernia, Umbilical/diagnostic imaging , Humans , Hydrocolpos/diagnostic imaging , Hypospadias/diagnostic imaging , Infant, Newborn , Male , Pregnancy , Scoliosis/diagnostic imaging , Urogenital Abnormalities/diagnostic imaging
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