暂无摘要。

暂无摘要。

The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars\' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.

UNASSIGNED: This case report describes the successful use of a latissimus dorsi muscle flap attached to the femoral vessels to cover a large mass loss in a 27-year-old woman with bladder exstrophy. The two-stage reconstruction showed excellent results six months after surgery.
METHODS: A 27-year-old woman with bladder exstrophy and previous enterocystoplasty with Mitrofanoff-type urinary diversion combined with a late fascial flap presented 10 years later with disembowelment near the pubic symphysis. A two-stage reconstruction using a latissimus dorsi flap and polypropylene mesh was performed with excellent aesthetic and functional results.
UNASSIGNED: The latissimus dorsi muscle flap, described by J.M. Servant in 1984 as the \"apple turnover\" technique, is highly reliable for reconstructing large substance losses with minimal functional sequelae. The procedure in this case resulted in very satisfactory aesthetic and functional results at six months postoperatively. This approach provided a safe and effective technique of last resort.
CONCLUSIONS: the two-stage latissimus dorsi flap technique was a reliable, safe and effective solution for this complex reconstructive challenge.

UNASSIGNED: OEIS complex is a rare and complex anomaly of the genitourinary and intestinal tract. It includes Omphalocele, Exstrophy of cloaca, Imperforate anus and Spinal defects.
METHODS: We are reporting a case of OEIS complex.
UNASSIGNED: Cloacal exstrophy is considered the most severe ventral abdominal wall defect. Diagnosis is primarily antenatal based on the presenting features on ultrasonography. Management requires initial resuscitation and stabilization followed by either single staged closure or multistage procedures by multi-disciplinary surgical team.
CONCLUSIONS: The introduction of better diagnostic techniques and the detection of fetal malformations during pregnancy is it\'s the cornerstone of care.

METHODS: 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.

We present three new and six published infants with overlapping features of LUMBAR syndrome (lower body hemangioma, urogenital anomalies, spinal cord malformations, bony deformities, anorectal/arterial anomalies and renal anomalies) and OEIS complex (omphalocele, exstrophy, imperforate anus, and spinal defects), also known as cloacal exstrophy. OEIS is included under the recently proposed umbrella coined recurrent constellations of embryonic malformations (RCEMs). The RCEMs represent a phenotypically overlapping spectrum of rare disorders of caudal dysgenesis with unknown cause but likely shared pathogenesis. It has recently been proposed that LUMBAR be considered an RCEM. This report of infants with combined features of OEIS and LUMBAR is the first to demonstrate an overlap between LUMBAR and another RCEM, which supports LUMBAR\'s inclusion within the RCEM spectrum.

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.
METHODS: 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.

Variations from classic bladder exstrophy (BE) are extremely uncommon, resulting in distinctive challenges in both diagnosis and management. The supravesical fissure variant of BE is exceptionally rare and has only been reported in male patients to date. Herein, we report the case and surgical management of a supravesical fissure variant of BE presentation in a newborn female patient and provide a literature review of this exstrophy variant.

LUMBAR syndrome is rare with a multitude of features that requires a high index of suspicion for timely diagnosis and appropriate management. We present on a newborn female whose untreated segmental infantile hemangioma lead to poor healing of her bladder exstrophy closure. The objective of this report is to describe bladder exstrophy as a urogenital anomaly in patients with LUMBAR syndrome and the importance of balancing management of infantile hemangioma and time to surgery.