Prenatal counseling for cloaca and cloacal exstrophy—challenges faced by pediatric surgeons

Bischoff, Andréa; Calvo-Garcia, Maria A.; Baregamian, Naira; Levitt, Marc A.; Lim, Foong Yen; Hall, Jennifer; Peña, Alberto

doi:10.1007/s00383-012-3133-3

Cited by 39 publications

(19 citation statements)

References 34 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Indeed, defects in the lower abdominal wall, as well as abnormal traction of the umbilical cord, seen in omphalocele, were most likely to have a deleterious effect on normal development from the urogenital sinus to the bladder [1011]. Recent advances in ultrasound techniques revealed urachus defects in fetuses in utero [1213]. However, abnormalities in the vas deferens and/or colliculus seminalis, even if present, would be found after puberty.…”

Section: Discussionmentioning

confidence: 99%

Descent of mesonephric duct to the final position of the vas deferens in human embryo and fetus

et al. 2016

View full text Add to dashboard Cite

Because the ureter arises from the mesonephric or Wolffian duct (WD), the WD opening should migrate inferiorly along the urogenital sinus or future urethra. However, this process of descent has not been evaluated morphometrically in previous studies and we know little about intermediate morphologies for the descent. In the present work, serial sagittal sections of 15 specimens at gestational age 6–12 weeks and serial horizontal sections of 20 specimens at 6–10 weeks were analyzed. Monitoring of horizontal sections showed that, until 9 weeks, a heart-, lozenge- or oval-shape of the initial urogenital sinus remained in the bladder and urethra. Thus, the future bladder and urethra could not be distinguished by the transverse section or plane. The maximum width of the urogenital sinus or bladder at 6–10 weeks was 0.8 mm, although its supero-inferior length reached 5 mm at 10 weeks. During earlier stages, however, the medial shift of the WD was rather evident. Depending on the extent of upward growth of the bladder smooth muscle, the descent of the vas deferens became evident at 10–12 weeks. Development of the urethral rhabdosphincter likely resulted in the differentiation of urogenital sinus into the urethra and bladder before formation of the bladder neck with 3-layered smooth muscles. Development of the prostate followed these morphological changes, later accelerating the further descent of the WD opening. Because of their close topographical relationships, slight anomalies or accidents of the umbilical cord at 10–12 weeks may have a significant effect on normal anatomy.

show abstract

Section: Discussionmentioning

confidence: 99%

Descent of mesonephric duct to the final position of the vas deferens in human embryo and fetus

et al. 2016

View full text Add to dashboard Cite

show abstract

“…Counseling for parents should include a discussion of bowel and urinary control and sexual function (44). Most patients with cloacal exstrophy will not have normal bowel Ax ial US image through the pelvis shows a soft-tissue mass distorting the contour of the fetal abdominal wall (white arrows), an absent bladder (arrowhead), and a subcutaneous mass over the distal spine (black arrows).…”

Section: Cloacal Exstrophymentioning

confidence: 98%

“…Both bowel and bladder control are adversely affected by the presence of a myelomeningocele or tethered cord (44). Sex assignment or reassignment can be challenging in the context of cloacal exstrophy because of genital ambiguity (44). Sexual function in females may require vaginal reconstruction; normal male sexual function is more difficult to achieve.…”

Section: Cloacal Exstrophymentioning

confidence: 98%

“…Over time, the distal bowel may grow enough to absorb water and form solid stool and thus allow individuals to participate in a bowel management program, rather than have a permanent stoma. Both bowel and bladder control are adversely affected by the presence of a myelomeningocele or tethered cord (44). Sex assignment or reassignment can be challenging in the context of cloacal exstrophy because of genital ambiguity (44).…”

Section: Cloacal Exstrophymentioning

confidence: 99%

See 1 more Smart Citation

Complex Abdominal Wall Defects: Appearances at Prenatal Imaging

Pakdaman

Woodward²,

Kennedy³

2015

RadioGraphics

View full text Add to dashboard Cite

Abdominal wall defects are a complex group of anomalies, and many are incorrectly diagnosed. Evaluation of the defect relative to the umbilical cord insertion site is fundamentally important in differentiating among the various malformations. The two most common abdominal wall defects are gastroschisis, in which the defect is on the right side of the normally inserting cord and free-floating bowel loops are present, and omphalocele, in which the cord inserts on a membrane-covered midline defect. Omphalocele may also form a portion of a more complex defect that may remain undiagnosed without thorough evaluation. In cloacal exstrophy, the defect extends inferiorly and the bowel loops extrude between the two bladder halves. In pentalogy of Cantrell, the defect extends superiorly and is typically associated with ectopia cordis. Bladder exstrophy is a lower abdominal defect in which the hallmark finding is absence of a fluid-filled bladder. The cord insertion site is normal to low but does not form part of the defect. Both body stalk anomaly and abdominoschisis due to amniotic bands cause severe malformations, often involving extrusion of solid organs and the bowel. Although these two entities have many overlapping features, body stalk anomaly may be recognized on the basis of absence of a free-floating umbilical cord. With use of an algorithmic approach beginning with discovery of the location of the defect, a more precise diagnosis can be determined that may directly affect pre- and postnatal management decisions.

show abstract

“…Also, upper urinary tract anomalies can be seen in 60% of cases as pelvic kidney, horseshoe kidney, hypoplastic kidney and solitary kidney, with subsequent hydronephrosis and oligohydramnios [89]. The US diagnosis can also describe "the elephant trunk sign", which is the protrusion of the ileum in the amniotic fluid, resembling the trunk of an elephant [90]. Typically, CE does not associate aneuploidies, but invasive diagnosis can be offered.…”

Section: Cloacal and Bladder Exstrophymentioning

confidence: 99%

Fetal Abdominal Wall Defects

Dragusin¹,

Şorop-Florea²,

LaurențiuPătru³

et al. 2018

Congenital Anomalies - From the Embryo to the Neonate

View full text Add to dashboard Cite

Abdominal wall defects (AWDs) represent a group of congenital anomalies that can be diagnosed early during pregnancy even at the time of the first trimester assessment, with direct impact on pre-and postnatal fetal prognosis and management decisions. The most frequent anomalies in this group are gastroschisis and omphalocele. The key method available, that allows the detection of any deviation from the physiologic midgut herniation, is the ultrasound (US) assessment. A precise algorithmic scan approach is imposed not only for an accurate detection of any abdominal wall defect, but also for a proper location of the defect and of the spatial relation to the umbilical cord insertion, fundamentally important in differentiating among various malformations. Other structural or chromosomal anomalies should be excluded. Suitable multidisciplinary counseling should be considered. Unfortunately, in utero surgery, in these cases, has not been yet successful. Postnatal early interventions are usually required in specialized pediatric centers.

show abstract

Prenatal counseling for cloaca and cloacal exstrophy—challenges faced by pediatric surgeons

Cited by 39 publications

References 34 publications

Descent of mesonephric duct to the final position of the vas deferens in human embryo and fetus

Descent of mesonephric duct to the final position of the vas deferens in human embryo and fetus

Complex Abdominal Wall Defects: Appearances at Prenatal Imaging

Fetal Abdominal Wall Defects

Contact Info

Product

Resources

About