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Vitamin K deficiency-induced spontaneous haemopericardium and cardiac tamponade in an infant with alpha-1 antitrypsin deficiency: a case report.

ABSTRACT:

Background

Vitamin K deficiency bleeding is a life-threatening complication in early infancy. Exclusive breastfeeding and neonatal cholestasis syndromes, most notable α-1-antitrypsin deficiency, have been reported to be risk factors. Intracranial haemorrhage is most common. No association to haemopericardium has been reported before.

Case summary

We report on an 11 weeks old at term-born infant, who presented with severe anaemia and signs of cardiogenic shock. Immediately echocardiography was done and depicted cardiac tamponade. Pericardiocentesis was performed and a significant amount of haemorrhagic fluid was removed. Further workup revealed deranged coagulation parameters, cholestatic liver disease, and reduced α-1-antitrypsin levels. Despite normal brain sonography, a small cerebral haemorrhage was detected on magnetic resonance imaging. A genetic test finally proofed homozygotic mutation of the SERPINA1-gene and confirmed the diagnosis of α-1-antitrypsin deficiency as the underlaying cause. After initial replacement of coagulation factors, erythrocytes and vitamin K, the infant recovered. Eighteen weeks after discharge, the infant is still on vitamin K supplementation. She did not have any further bleedings and no neurologic or developmental impairment.

Discussion

Alpha-1-antitrypsin deficiency can lead to vitamin K deficiency in young infants even with adequate prophylaxis. Spontaneous haemorrhagic pericardial effusion was a new manifestation of vitamin K deficiency bleeding in our patient and should be considered and ruled out in young infants who present with acute anaemia and poor clinical condition.

SUBMITTER: Bauer C

PROVIDER: S-EPMC7898583 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundPericardiocentesis is an effective treatment for cardiac tamponade, but there are risks, including haemorrhagic events, cardiac perforation, pneumothorax, arrhythmia, acute pulmonary oedema and so on. Mediastinal effusion caused by puncture is rarely reported.Case presentationA 47-year-old man who had a history of right leg deep vein thrombosis and pulmonary artery embolism with implantation of an inferior vena cava filter presented for inferior vena cava filter removal. Within 30 min after the procedure, he developed chest pain, nausea, vomiting and presyncope with shock. Echocardiography confirmed massive pericardial effusion with evidence of cardiac tamponade. Emergency pericardiocentesis was performed. Confusingly, only 3 mL of bloody pericardial effusion was drained in total, and subsequently, the patient's symptoms rapidly improved with stable haemodynamics. Repeat echocardiography showed that the pericardial effusion had disappeared. Urgent computed tomography pulmonary angiography demonstrated localized effusion, which was not seen the previous computed tomography results and was noted around the left ventricle in the mediastinal apace. No intervention was performed, given that there was no bleeding tendency or further adverse events related to the mediastinal effusion. The patient was subsequently discharged in a stable condition a few days later, and outpatient follow-up was advised.ConclusionsMediastinal effusion is a rare complication of pericardiocentesis. In the case described herein, the most likely cause was pericardial effusion extravasated into the mediastinum through the needle insertion site in the puncture process due to large pressure variations in the intrapericardial space with tamponade, differing from cases of over-anticoagulation reported in the previous literature. Just as our case demonstrates that conservative treatment of an hemodynamic insignificant mediastinal effusion may be appropriate. Echocardiography is useful and effective to minimize complication rates.