<strong>Introduction: </strong><span style="font-family:""><span style="font-family:Verdana;">OEIS syndrome, the most severe form of exstrophy-epispadias complex, is ch...<strong>Introduction: </strong><span style="font-family:""><span style="font-family:Verdana;">OEIS syndrome, the most severe form of exstrophy-epispadias complex, is characterized by the existence of an omphalocele, a bladder exstrophy, an anal imperforation and a spina bifida. </span><b><span style="font-family:Verdana;">Case Presentation:</span></b><span style="font-family:Verdana;"> Two clinical cases admitted at the first day of life in neonatology department are reported;one was born by caesarean section, weighing 2500 g and the other one vaginally with a weight at 1890 g. The first child’s mother was a primigravid, primiparous and the second mother was multiparous. Prenatal ultrasound was performed in one case. The clinical examination found two exstrophied hemi-bladders with two productive ureteral meatus, separated by a double intestinal orifice, a manifestation of anal imperforation, omphalocele, spina bifida and lumbar vertebral malformations;which corresponds to a typical form of OEIS syndrome. One of the babies had an associated clubfoot. The karyotype could not be performed. Both were dead;the first in the early postoperative period, the second one at 16 days of life without surgery. </span><b><span style="font-family:Verdana;">Conclusion:</span></b><span style="font-family:Verdana;"> OEIS syndrome is a rare condition and represents a therapeutic challenge in developing countries. Promoting prenatal diagnosis is essential.</span></span>展开更多
Background: Bladder exstrophy is a rare congenital malformation of the genitourinary system, with an estimated incidence of approximately 1 per 50,000 live births. Clinically, patients do not have capacity to accumula...Background: Bladder exstrophy is a rare congenital malformation of the genitourinary system, with an estimated incidence of approximately 1 per 50,000 live births. Clinically, patients do not have capacity to accumulate urine and urine continously leak. We present patient with partial storing capacity from the dialated distal ureters. A case of dialated distal ureters from an 8-year-old female patient with ectopic vesicae is described. The dialated ureters act as reservoir of urine where the patient is partially continent in the night time. These dialated ureters are the compensation for the literally absent bladder. During reconstruction, we observed that they can be used as an additional bladder volume reducing risk of reconstruction failure from tension.展开更多
Ureterosigmoidostomy was commonly utilized as a procedure for continent urinary diversion.However,ureterosigmoidostomy is associated with complications such as infection,electrolyte disturbances,and neoplasia developm...Ureterosigmoidostomy was commonly utilized as a procedure for continent urinary diversion.However,ureterosigmoidostomy is associated with complications such as infection,electrolyte disturbances,and neoplasia development.A 40-year-old Caucasian male presented with acute left flank pain.Past medical history was significant for bladder exstrophy for which ureterosigmoidostomy urinary diversion was performed during childhood.On physical exam,multiple circular erythematous patches were scattered across the forearms that had been presented for 2 years.Cross-sectional imaging demonnttated an ill-defined mass at the site of ureteral implantation with associated severe left hydroureteronephrosis.Endoscopy revealed a mass at the site of ureteral implantation and biopsy demonstrated invasive,poorly differentiated adenocarcinoma.The dermatosis was diagnosed as interstitial granulomatous dermatitis,a rare inflammatory skin condition associated with underlying autoimmune disease or malignancy.Patient elected operative management with left nephrectomy,sigmoidectomy,and ileal conduit diversion.Ihis case demonnttates a rare presentation of cutaneous paraneoplastic syndrome after development of colon cancer after ureterosigmoidostomy.Ureterooolonic urinary diversion has a demonnttaaie risk of neoplasia development at the anastomotic site,requiring routine endoscopic surveillance.展开更多
文摘<strong>Introduction: </strong><span style="font-family:""><span style="font-family:Verdana;">OEIS syndrome, the most severe form of exstrophy-epispadias complex, is characterized by the existence of an omphalocele, a bladder exstrophy, an anal imperforation and a spina bifida. </span><b><span style="font-family:Verdana;">Case Presentation:</span></b><span style="font-family:Verdana;"> Two clinical cases admitted at the first day of life in neonatology department are reported;one was born by caesarean section, weighing 2500 g and the other one vaginally with a weight at 1890 g. The first child’s mother was a primigravid, primiparous and the second mother was multiparous. Prenatal ultrasound was performed in one case. The clinical examination found two exstrophied hemi-bladders with two productive ureteral meatus, separated by a double intestinal orifice, a manifestation of anal imperforation, omphalocele, spina bifida and lumbar vertebral malformations;which corresponds to a typical form of OEIS syndrome. One of the babies had an associated clubfoot. The karyotype could not be performed. Both were dead;the first in the early postoperative period, the second one at 16 days of life without surgery. </span><b><span style="font-family:Verdana;">Conclusion:</span></b><span style="font-family:Verdana;"> OEIS syndrome is a rare condition and represents a therapeutic challenge in developing countries. Promoting prenatal diagnosis is essential.</span></span>
文摘Background: Bladder exstrophy is a rare congenital malformation of the genitourinary system, with an estimated incidence of approximately 1 per 50,000 live births. Clinically, patients do not have capacity to accumulate urine and urine continously leak. We present patient with partial storing capacity from the dialated distal ureters. A case of dialated distal ureters from an 8-year-old female patient with ectopic vesicae is described. The dialated ureters act as reservoir of urine where the patient is partially continent in the night time. These dialated ureters are the compensation for the literally absent bladder. During reconstruction, we observed that they can be used as an additional bladder volume reducing risk of reconstruction failure from tension.
文摘Ureterosigmoidostomy was commonly utilized as a procedure for continent urinary diversion.However,ureterosigmoidostomy is associated with complications such as infection,electrolyte disturbances,and neoplasia development.A 40-year-old Caucasian male presented with acute left flank pain.Past medical history was significant for bladder exstrophy for which ureterosigmoidostomy urinary diversion was performed during childhood.On physical exam,multiple circular erythematous patches were scattered across the forearms that had been presented for 2 years.Cross-sectional imaging demonnttated an ill-defined mass at the site of ureteral implantation with associated severe left hydroureteronephrosis.Endoscopy revealed a mass at the site of ureteral implantation and biopsy demonstrated invasive,poorly differentiated adenocarcinoma.The dermatosis was diagnosed as interstitial granulomatous dermatitis,a rare inflammatory skin condition associated with underlying autoimmune disease or malignancy.Patient elected operative management with left nephrectomy,sigmoidectomy,and ileal conduit diversion.Ihis case demonnttates a rare presentation of cutaneous paraneoplastic syndrome after development of colon cancer after ureterosigmoidostomy.Ureterooolonic urinary diversion has a demonnttaaie risk of neoplasia development at the anastomotic site,requiring routine endoscopic surveillance.
