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Situs ambiguus and sacral agenesis

Case contributed by Yaïr Glick
Diagnosis certain

Presentation

Abdominal pain.

Patient Data

Age: 30 years
Gender: Male

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 border. 13 pairs of ribs. Thoracolumbar scoliosis.
Abdomen ( AP supine & PA erect): Liver not visualized on right; viscera in left upper abdomen; sacral agenesis.

Cervical spine CT

  • C2-C3 and C4-C5 block vertebrae
  • Spina bifida occulta at C6 level

Neck CTA (part of head & neck CTA)

  • Hemiazygos continuation drains into persistent left superior vena cava (PLSVC), which drains into left atrium
  • Anterior pseudoarthrosis of 1st and 2nd right ribs (not shown)

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 Malone antegrade continence enema (MACE) and synchronous Mitrofanoff procedure due to fecal 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 the left atrium noted at lower part of head-neck CTA

To sum up, this is a very rare and unusual case of total sacral agenesis coexisting with situs ambiguus; no report found on PubMed of any such case.

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