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previously visited 090120 VACTERL ASSOCIATION WITH HIGH ANORECTAL MALFORMATION Random Case
Authored By: pedrad CSH, Radiologist, Chung Shan Medical University Hospital, Taiwan.
Patient: 1 day old male
History:

Chief complain:
Imperforate anus since birth; extradigit arising from right thumb

Caesarean section with gestational age 34+ weeks, twin B
Birthbody weight: 2020gm. G5P3AA2
Apgar score: 7 (at 1st min) to 8 (at 5th min).

Single umbilical artery was noted in prenatal ultrasound.
Premature rupture of membrane (PROM):+

Images:[small]mediumlargeas-submittedimages only

Fig. 1: X-ray prone cross-table beam with buttock up

Fig. 2: KUB

Fig. 3: CT scan pelvis, maximum-intensity projection, oblique coronal

Fig. 4: X-ray

Fig. 5: MR, sagittal, T1WI, T2WI with fat sat, tFISP (from left to right)

Fig. 6: MR, T2WI, axi (left), sag (right)

Fig. 7: Distal colostogram, lateral, AP

Fig. 8: Distal colostogram, lateral
Images:[small]mediumlargeas-submittedimages only
Findings:

X-ray prone cross-table beam with buttock up (figure 1):
1. No obvious rectal gas in pelvis.

KUB (figure 2):
1. Bowel loops dilatation with air content in abdomen,
   predominantly right abdomen, suspicious a distended
   sigmoid colon (arrow in figure 2).

Multidetector row CT scan of abdomen (focus on pelvis) with
 maximum-intensity projection and without intravenous contrast
 (figure 3):

1. Left side deviation of sacrum with lateral hemivertebra in S3
   (arrow in figure 3).

Plain film radiography of right hand (figure 4):
1. Duplication of right thumb (arrow in figure 4), in favor of
   preaxial (radial) polydactyly.

1.5 Tesla MRI of abdomen with T2 weighted echo train spin echo with
 and without fat saturation, T1 weighted echo train spin echo,
 T2/T1 true fast imaging with steady-state precession gradient
 echo with fat saturation, and without intravenous contrast
 (figure 5 and 6):

1. Dilatation of distal colon with somewhat bird beak appearance
   in lower abdomen, about 3.0cm in rectal pouch-perineal
   distance, suggesting high anorectal malformation (figure 5).
2. Presence of air-fluid level within the distal colon with some
   short T1 and T2 materials deposition (arrow in figure 5),
   suggesting meconium-packed pouch.
3. No abnormal finding in remaining abdomen, including both
   kidneys (white arrows in figure 6) and non-distended bladder
   (yellow arrow in figure 6).
4. No abnormal finding in spine (red arrow in figure 6) form
   current images.

Distal colostogram with water soluble contrast after diverting
 colostomy (figure 7 and 8):

1. Early opacity of bladder (arrows in figure 7), suggesting
   rectourinary fistula.
2. Distal tapering of colon with somewhat bird beak appearance
  (figure 8).

Diagnosis: Multiple abnormalities, including:
   (V)ertebral (hemivertebra, S3),
   (A)nal (high anorectal malformation),
   (R)rnal (rectourinary fistula), and
   (L)imb (preaxial polydactyly),
suggesting VACTERL association.
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Additional Details:

Case Number: 22953917Last Updated: 01-22-2009
Rating:

0 ratings
Anatomy: Gastrointestinal (GI)   Pathology: Congenital
Modality: CT, Conventional Radiograph, GI, MRAccess Level: Readable by all users
Keywords: vacterl, hemivertebra, anorectal malformation, rectourinary fistula, polydactyly, imperforate anusACR: 757.1433
Case has been viewed 264 times.

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