Situs ambiguus and sacral agenesis
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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 visualised on right; viscera in left upper abdomen; sacral agenesis.
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Cervical spine CT
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
- Bizarrely shaped pancreas
- Percutaneous tract, right lower abdomen
- Sacral agenesis type IV (total)
- Midline subcutaneous post-operational scar
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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