Presentation
Newborn with difficult breathing, dropping oxygen saturation, and cyanosis. Delivered by emergency C-section at 35 weeks of gestational age, due to fetal distress.
Patient Data
- dextrocardia
- gastric bubble to the right of the midline with a feeding tube in situ
- 5th dorsal hemivertebra
Umbilical catheters are seen in place; the tip of the umbilical venous catheter (UVC) is in the right hypochondrium (likely within the liver), and the tip of the umbilical arterial catheter (UAC) is lying at about D5-6 level.
5th dorsal hemivertebra; otherwise, no gross abnormality (particularly no periosteal reaction) is seen in the visualized skeleton.
FINDINGS: Centrally located liver (bridging liver) with smooth outlines. A few linear hyperechoic foci are seen in the left lobe of liver, which likely represents air (likely related to UVC insertion). No suspicious focal hepatic pathology or biliary dilatation is noted. The gallbladder is seen on the right side of the midline. Spleen is not confidently identified. Stomach air is seen in the right hypochondrium and epigastric region. Right-sided abdominal IVC and left-sided abdominal aorta. Umbilical arterial and venous catheters are seen in place. Gross morphology of kidneys and adrenal gland is within normal limits.
CONCLUSION: Mal-positioned liver, stomach and non-visualized spleen.
Diffuse symmetrical periosteal reaction or cortical thickening (also known as cortical hyperostosis) is noted along the diaphysis of long bones of both upper and lower extremities, with sparing of the metaphysis. No periosteal reaction is sen along the ribs, clavicles, or mandibles. No bone fracture or dislocation is noted. The gross morphology of small bones of the hands and feet and skull is also within normal limits.
Case Discussion
- ECHOCARDIOGRAM: Complex congenital heart disease. Dextrocardia, unbalanced AVSD, hypoplastic RV, pulmonary atresia, small tortuous PDA, unobstructed left aortic arch, superior vena cava duplication with no bridging vein, total anomalous pulmonary venous return.
- Radiographic, sonographic & echocardiographic features are suggestive of heterotaxy syndrome (right isomerism/asplenia).
- Prostaglandin E (PGE1 0.05 mic/kg/min) infusion was started within first hour of birth. Case was discussed in multidisciplinary meeting at pediatric cardiac center and offered no cardiac intervention due to complexity of the disease with grave prognosis. Patient was managed conservatively with low dose prostaglandins for three months, which were later on discontinued due to clinical deterioration of the patient. Patient died after one week of discontinuation of prostaglandins.
- Based on above mentioned clinical history, prostaglandin therapy, and imaging findings; diffuse periosteal reaction / thickening noted along the long bones of the bilateral upper and lower extremities on 2nd skeletal survey, is suggestive of prostaglandin-induced cortical hyperostosis.
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