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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5514_Библиотеки_им_академика_М_И_Перельмана.pdf
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Fig. 4.15 Renal anomalies in the exstrophy-epispadias complex. (a) 2-day-old boy with covered
exstrophy variant. Transverse midline grayscale image from a screening ultrasound (US) shows a
midline pelvic, fused kidney with multiple cysts (C=largest cyst). S=spine; P=psoas muscle.
(b) 3-day-old boy with cloacal exstrophy. Sagittal grayscale US image of the pelvis, to the right of
the midline, shows the right kidney in an ectopic, pelvic position with normal morphology. Note
the lower lumber spine and sacrum posterior to the kidney (arrows)
E. R. Oliver and S. J. Back
Fig. 4.16 UTD following bladder exstrophy repair. A 4-month-old boy with bladder exstrophy.
1month after stage 1 repair, a sagittal grayscale ultrasound image of the left kidney (a) and left
ureter (b) showed UTD including central calyceal dilation, renal pelvic (P) and ureteral (U) dilation developed. Initial renal bladder ultrasound was normal (not shown). The postoperative UTD
ultimately resolved
In the setting of recurrent urinary tract infections, dimercaptosuccinic acid
(DMSA) and mercapto-actyl-triglycine (MAG3) nuclear medicine exams can be
valuable to assess renal parenchymal scarring and function. One series found that
male patients were at greater risk for UTD, both presence and degree, as well as
compromised renal function [32]. However, lower tract surgical and clinical status
(e.g., ureteral reimplantation, continence, bladder pressure) and risk of high blood
pressure were not associated with upper tract outcomes (e.g., dilation, function)
[32, 33].
4 Prenatal and Postnatal Imaging of the Bladder Epispadias-Exstrophy Complex
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Fig. 4.17 Two boys with epispadias. (a) 2.5-year-old boy with epispadias. Right oblique uoro-
scopic image from a voiding cystourethrogram (VCUG) shows bilateral vesicoureteral reux
(VUR), a smooth-walled bladder, and a shortened urethral length. The arrow indicates contrast at
the distal end of the urethra. (b) Left oblique uoroscopic image from a VCUG of a 7-month-old
boy shows bilateral VUR, a smooth-walled bladder, and shortened urethral length. The arrow indicates the location of the meatus with contrast directed cephalad during the voiding phase
During closure of bladder exstrophy, ureteral stents and suprapubic cystostomy
catheters are placed. Some centers perform “stentograms,” where contrast is instilled
through the ureteral stents by gravity into the upper tracts and ureteral drainage is
assessed prior to removing the stents (Fig.4.18). The contrast infusion is administered by gravity and is positioned at a height of 30cm or less above the level of the
kidneys so that it is administered at low pressure. A cystogram through the suprapubic catheter can demonstrate contrast extravasation, contour of the bladder, and
bladder capacity (Fig.4.19). Prior to reimplantation, the distal ureters have been
described as having a “J-shape” [34]. The ureters are sometimes reimplanted at the
time of primary closure and are more often reimplanted at the time of bladder neck
reconstruction.
Fluoroscopic imaging can also be used to assess for complications, when
suspected, including of the urethra, by retrograde urethrography or voiding
cystogram. Following epispadias repair, the urethra may have an irregular
appearance.
56
E. R. Oliver and S. J. Back
a
b
cde
Fig. 4.18 Stentograms in two patients. (a) 4-month-old boy, 2 weeks after bladder exstrophy
repair. Abdominal radiograph showing right ureteral stent (open black arrowhead), left ureteral
stent (open white arrowhead), and transurethral bladder (solid white arrowhead) catheters are present. A cast is partially seen due to iliac osteotomies. (b) Fluoroscopic image from the left stentogram. Contrast is instilled into the left ureteral stent with lling of the left renal calyces and ureter
(open white arrowhead). Transurethral bladder catheter (solid white arrowhead). (c) Moments
later, the contrast is seen draining into the irregularly shaped bladder (asterisks). (d, e) 4-month-old
girl, 2 weeks following bladder exstrophy closure with bilateral ureteral reimplantation. Right
(open black arrowhead) and left (open white arrowhead) ureteral stents and a transurethral bladder
catheter (solid white arrowhead) are present. Contrast was administered into the right stent with
prompt contrast drainage into the bladder (not shown). Next, the contrast was administered to the
left stent. There is left ureteral (open white arrowhead), renal pelvic, and calyceal dilation. No
contrast drained into the bladder during the examination. A cast is seen over the upper abdomen
4 Prenatal and Postnatal Imaging of the Bladder Epispadias-Exstrophy Complex
Fig. 4.19 Cystogram of a
3-month-old boy, 1month
following complete
primary repair of exstrophy
with bladder neck
reconstruction. Contrast
was instilled by gravity
infusion into the
suprapubic cystostomy
catheter (black arrowhead).
The irregularly shaped
bladder (asterisks) lls
with contrast. There is
prompt right and left
vesicoureteral reux. A
cast is present over the
abdomen supporting iliac
osteotomies
57
Musculoskeletal System
Radiography is an excellent tool to examine the skeletal system and is often the rst
radiologic imaging done on a child with EEC to assess pubic diastasis (Fig.4.20).
When cross-sectional imaging is indicated to evaluate the musculoskeletal anatomy,
either MRI or CT can be used. Each modality has its advantages and disadvantages.
While MRI is radiation-free, a young child usually requires sedation or anesthesia
to remain still during image acquisition. However, MRI is preferred for soft tissue
evaluation due to the high spatial resolution and better tissue contrast in those without external xation hardware or MRI-incompatible implants. Even though CT
exposes a child to radiation, current CT technology can obtain diagnostic images
with low doses and can provide valuable information about the osseous pelvis.
Three-dimensional (3D) reconstructions can be generated from either modality
(Fig.4.21). 3D-computerized and printed models can aid surgical planning, multidisciplinary care discussions, and patient and family education, especially in more
complex or variant anatomy [35]. Some centers routinely perform dedicated imaging of the pelvis before and after surgical repair; however, there are no set guidelines [36].
58
Fig. 4.20 Pubic diastasis
assessment. Anterior
posterior pelvic radiograph
in a newborn boy with
bladder exstrophy on day
of life one. There is wide
separation of the pubic
symphysis (dashed line),
consistent with pubic
diastasis and a typical
nding associated with
bladder exstrophy
E. R. Oliver and S. J. Back
Imaging has afforded a better understanding of the pelvic musculoskeletal anatomy in children with EEC.Studies of CT and MRI of the pelvis prior to and following exstrophy repair have described the structure of the pelvis, including angular
and linear distances between bones and pelvic oor muscles, as well as how the
surgeries affect these anatomic relationships and the potential sequelae on pelvis
shape, acetabular morphology, and continence [37, 38]. The focus of these studies
is on classic bladder exstrophy (CBE), noting that within the EEC spectrum, the
pelvic structure of children with epispadias is closer to normal, and those with cloacal exstrophy can have greater asymmetry between each side of the pelvis compared
with CBE.
Children with CBE have shorter pubic bones, external rotation of the pelvis and
retroversion of the acetabulae, as well as alteration in the muscles of the pelvic diaphragm, or levator ani muscle group [39–41]. The levator ani muscle group, including the iliococcygeus, pubococcygeus, and puborectalis sling muscles, is oriented in
an anterior to posterior direction and attaches to the pubis and coccyx. The levator
ani muscles are further supported through lateral attachments to the obturator internus muscles, which originate along the inferior aspect of the superior pubic ramus
and insert on the greater trochanter of the femur. The muscular diaphragm supports
the pelvic genitourinary anatomy and has sphincteric function. The levator ani in
patients with CBE are wider and atter than normal with increased dome-shaped
contour, which is disproportionately pulled anteriorly. As a result, the puborectalis
sling is not centered on the genitourinary structures and anus, which can affect continence [40, 41] (Fig.4.22).
4 Prenatal and Postnatal Imaging of the Bladder Epispadias-Exstrophy Complex
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59
c
d
ef
Fig. 4.21 CT of the pelvis of a boy with bladder exstrophy prior to and following stage 1 repair
in bladder exstrophy. (a–c) Images from preoperative CT, at 3months of age, with images at the
level of the pubis (a) and S1 (b) showing the expected osseous conguration in a patient with bladder exstrophy. The pubic bones are shortened and pubic diastasis is present (dashed line). The
pelvis is externally rotated with an “open-book” appearance. (c) 3D-reconstruction rendered from
2D-preoperative images. (d–f) One month following surgery, (d) the pubic bones are more approximated (dashed line), and (e) posterior iliac osteotomies have been done to help close the midline
repair. Healing bone is present at the osteotomies (arrows). The cystostomy catheter is partially
visualized in (d) and the cystostomy and right ureteral stent are partially visualized in (d) and (e).
(f) 3D-reconstruction rendered from 2D-postoperative images
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Fig. 4.22 Pelvic oor musculature in bladder exstrophy. Axial T2 SPACE MRI image of the pelvis (a) through the level of the pubic bones in an 11-month-old boy with skin-covered exstrophy
variant compared to a normal infant (b). There is pubic diastasis (dashed line). The levator ani
muscle group (arrows) is disproportionately positioned anteriorly. The rectum and urethra are relatively anterior. O=obturator internus muscles
E. R. Oliver and S. J. Back
Because of the origins and insertions of the pelvic oor musculature, it would
follow that the skeletal differences in children with EEC would portend muscular differences. Analyzing musculoskeletal relationships along with the clinical
outcomes may improve attainment of the surgical goals of abdominal wall closure and urinary continence while reducing morbidity [42]. However, understanding which musculoskeletal relationships correlate with continence has
been challenging as children continue to grow and develop after surgery and
normative relationships are not established. Those who have attained continence
have been described as having a pelvic shape closer to that of age-matched unaffected children [43].
In order to reduce tension on the midline closure, the pubic bones are reapproximated. Depending on the age of the child at the time of surgery and the
degree of diastasis, the pubis may be reapproximated with tension sutures alone
in newborn infants or pelvic osteotomies in older infants and children. The consideration of which type of osteotomy is addressed in another chapter. Pubic
diastasis is most commonly observed by radiographs in a newborn with classic
bladder exstrophy or cloacal exstrophy. A transverse linear measurement
between the midportion of the right and left pubic bones denes the distance of
the diastasis. In skeletally immature children, only the ossied bone, and not the
cartilage, is visible by radiograph. Diastasis can also be measured on CT and
MRI.Following osteotomies, bone healing and hardware can be monitored by
radiographs or CT.
4 Prenatal and Postnatal Imaging of the Bladder Epispadias-Exstrophy Complex
61
Spine
Of children with EEC, those with cloacal exstrophy most commonly have spine
anomalies associated with the OEIS complex, which may take the form of vertebral
segmentation anomalies and/or closed (i.e., skin-covered) spinal dysraphisms.
Radiography can depict vertebral anomalies, and spinal US is used as a rst-line
screening evaluation of the spinal cord (Figs.4.23 and 4.24). Spine US in the neonate is a non-invasive, well-tolerated examination and readily detects anomalies of
the spinal canal, which can be visualized commonly up to 6months of age due to
the timing of spine ossication [44]. It is ideally performed with the infant in the
prone position. If additional imaging is warranted, a spine MRI can be obtained to
further classify the anomaly, which can range from closed to open dysraphism
(Fig.4.24) [45].
Fig. 4.23
Thoracoabdominal
radiograph in a newborn
girl with cloacal exstrophy
shows mid-thoracic
vertebral anomalies
(arrows) and an
omphalocele (asterisks)
projecting over the pelvis
62
E. R. Oliver and S. J. Back
a
c
bd
Fig. 4.24 Spine US and MRI in two patients with cloacal exstrophy. (a) Sagittal ultrasound (US)
image of the spine with the infant in the prone position on the day of life 0. The vertebral levels are
numbered. The low-lying conus is associated with a round, echogenic lesion (asterisk) suspicious
for a lipoma. The lum terminale is thickened (arrow). These features are concerning for tethered
cord. (b) Sagittal T2 TSE MRI images at 1week of life, in the midline showing the same ndings
as seen in US. (c) Spine ultrasound of the second child on the day of life 3 with the conus and nerve
roots communicating with a uid-lled sac in the inferior soft tissues (asterisk) at the level of the
hypoplastic sacrum (arrows). Skin overlies the uid-lled sac. (d) Sagittal T2 TSE image of the
spine just to the left of the midline at 1month of age showing the abnormal conguration of the
sacrum (arrow) and low-lying cord. On MRI tissue with fat signal characteristics was identied
associated with the uid-lled sac (asterisk), consistent with a lipomyelomeningocele
Conclusions
Prenatal and postnatal imaging are each important in the care of children with EEC
but serve two distinct roles. Prenatal imaging primarily serves to make an accurate
diagnosis in order to facilitate prenatal counseling and prepare for postnatal care.
Postnatal imaging, however, helps to more completely dene the child’s anatomy
and monitors for post-surgical complications. Understanding the prenatal imaging
manifestations, pitfalls, associated anatomic anomalies, and potential complications
of care strengthens the communication between the radiologist, urologist, and
medical team.
4 Prenatal and Postnatal Imaging of the Bladder Epispadias-Exstrophy Complex
63
References
1. American Institute of Ultrasound in Medicine. AIUM-ACR-ACOG-SMFM-SRU practice
parameter for the performance of standard diagnostic obstetric ultrasound examinations. J
Ultrasound Med. 2018;37(11):E13–24.
2. American Institute of Ultrasound in Medicine. AIUM practice parameter for the performance of detailed second- and third-trimester diagnostic obstetric ultrasound examinations. J
Ultrasound Med. 2019;38(12):3093–100.
3. American Institute of Ultrasound in Medicine. AIUM practice parameter for the performance of detailed diagnostic obstetric ultrasound examinations between 12 weeks 0 days and
13 weeks 6 days. J Ultrasound Med. 2021;40(5):E1–16.
4. Gearhart JP, Ben-Chaim J, Jeffs RD, Sanders RC.Criteria for the prenatal diagnosis of classic
bladder exstrophy. Obstet Gynecol. 1995;85(6):961–4.
5. Mirk P, Calisti A, Fileni A.Prenatal sonographic diagnosis of bladder extrophy. J Ultrasound
Med. 1986;5(5):291–3.
6. Antomarchi J, Moeglin D, Laurichesse H, Combourieu D, Bigi N, Maisonneuve E, etal. The
pubic diastasis measurement, a key element for the diagnosis, management, and prognosis of
the bladder exstrophy. Fetal Diagn Ther. 2019;45(6):435–40.
7. Mallmann MR, Mack-Detlefsen B, Reutter H, Pohle R, Gottschalk I, Geipel A, etal. Isolated
bladder exstrophy in prenatal diagnosis. Arch Gynecol Obstet. 2019;300(2):355–63.
8. Pakdaman R, Woodward PJ, Kennedy A.Complex abdominal wall defects: appearances at
prenatal imaging. Radiographics. 2015;35(2):636–49.
9. Manganaro L, Antonelli A, Bernardo S, Capozza F, Petrillo R, Satta S, etal. Highlights on
MRI of the fetal body. Radiol Med. 2018;123(4):271–85.
10. Marine MB, Forbes-Amrhein MM.Magnetic resonance imaging of the fetal gastrointestinal
system. Pediatr Radiol. 2020;50(13):1895–906.
11. Weiss DA, Oliver ER, Borer JG, Kryger JV, Roth EB, Groth TW, et al. Key anatomic ndings on fetal ultrasound and MRI in the prenatal diagnosis of bladder and cloacal exstrophy. J
Pediatr Urol. 2020;16(5):665–71.
12. Dannull KA, Browne LP, Meyers MZ.The spectrum of cloacal malformations: how to differentiate each entity prenatally with fetal MRI.Pediatr Radiol. 2019;49(3):387–98.
13. Carey JC, Greenbaum B, Hall BD.The OEIS complex (omphalocele, exstrophy, imperforate
anus, spinal defects). Birth Defects Orig Artic Ser. 1978;14(6B):253–63.
14. Carey JC.Exstrophy of the cloaca and the OEIS complex: one and the same. Am J Med Genet.
2001;99(4):270.
15. Feldkamp ML, Botto LD, Amar E, Bakker MK, Bermejo-Sánchez E, Bianca S, etal. Cloacal
exstrophy: an epidemiologic study from the International Clearinghouse for Birth Defects
Surveillance and Research. Am J Med Genet C Semin Med Genet. 2011;157C(4):333–43.
16. Coleman PW, Marine MB, Weida JN, Gray BW, Billmire DF, Brown BP.Fetal MRI in the
identication of a fetal ventral wall defect spectrum. AJP Rep. 2018;8(4):e264–76.
17. Hamada H, Takano K, Shiina H, Sakai T, Sohda S, Kubo T. New ultrasonographic criterion for the prenatal diagnosis of cloacal exstrophy: elephant trunk-like image. J Urol.
1999;162(6):2123–4.
18. Goncalves LF, Hill H, Bailey S.Prenatal and postnatal imaging techniques in the evaluation of
disorders of sex development. Semin Pediatr Surg. 2019;28(5):150839.
19. Coblentz AC, Teixeira SR, Mirsky DM, Johnson AM, Feygin T, Victoria T.How to read a fetal
magnetic resonance image 101. Pediatr Radiol. 2020;50(13):1810–29.
20. Zalel Y, Lehavi O, Aizenstein O, Achiron R.Development of the fetal spinal cord: time of
ascendance of the normal conus medullaris as detected by sonography. J Ultrasound Med.
2006;25(11):1397–401; quiz 1402.
21. Bronshtein M, Riechler A, Zimmer EZ.Prenatal sonographic signs of possible fetal genital
anomalies. Prenat Diagn. 1995;15(3):215–9.
22. Valioulis I, Levard G, Lottmann H, Hameury F, Cendron J.A rare complex bladder exstrophy
variant. BJU Int. 2000;86(3):398–400.