Situs ambiguus and sacral agenesis

Case contributed by Dr Yair Glick

Presentation

Abdominal pain.

Patient Data

Age: 25-35 years
Gender: Male
X-ray

Chest (PA erect):
Technical: Taken during expiration and mildly rotated to left.
Left-sided heart and aorta. Vertical stripe just left of aortic knob represents left mediastinal border13 pairs of ribsThoracolumbar scoliosis.
Abdomen ( AP supine & PA erect): Liver not visualised on right; viscera in left upper abdomen; sacral agenesis.

Cervical spine CT

Neck CTA (part of head & neck CTA)

Lumbar spine CT

  • Left kidney removed
  • Left-sided liver with gallbladder
  • Polysplenia
  • Bizarrely shaped pancreas
  • Percutaneous tract, right lower abdomen
  • Sacral agenesis type IV (total)
  • Midline subcutaneous post-operational scar

Case Discussion

Known sacral agenesis and bilateral clubfoot. Underwent MACE (Malone Antegrade Continence Enema) and synchronous Mitrofanoff procedure due to faecal incontinence and neurogenic bladder: self-administers percutaneous enemas and self-intubates his bladder via a suprapubic tract, respectively. Underwent left nephroureterectomy due to nonfunctioning left kidney as a consequence of vesicoureteral reflux.

The complete 'picture' was constructed from several radiologic examinations performed for various clinical indications:

  • Sacral agenesis first noted on abdominal radiographs
  • Cervical block vertebrae on cervical CT
  • Situs ambiguus noted on lumbar spine CT
  • Hemiazygos draining into persistent left superior vena cava, which in turn drains into left atrium noted at lower part of head-neck CTA

To sum up, this is a very rare and unusual case of both sacral agenesis and situs ambiguus; no report found on Pubmed of a single patient with both conditions.

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Case information

rID: 48447
Case created: 7th Oct 2016
Last edited: 11th May 2017
Inclusion in quiz mode: Included

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