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[Budd-Chiari syndrome in children: case report]
L Tucciarone1, A Tomassini, P Stella
1Istituto di Clinica Pediatrica I, Università degli Studi di Roma La Sapienza, Roma.
Insights
This case report details a rare childhood Budd-Chiari syndrome in a young girl, co-occurring with caecum polyposis. The syndrome was classified as idiopathic, with the co-occurrence likely coincidental.
Area of Science:
- Pediatric Gastroenterology and Hepatology
- Rare Diseases
- Clinical Case Study
Background:
- Budd-Chiari syndrome is a rare hepatic vascular condition, particularly uncommon in pediatric populations.
- Caecum polyposis is a gastrointestinal condition involving abnormal growths in the large intestine's blind pouch.
Observation:
- A ten-year-old girl presented with clinical features of Budd-Chiari syndrome.
- The patient also exhibited significant polyposis of the caecum, a concurrent finding not previously reported in literature.
- The case was managed at the Pediatric Hospital of the University of Rome 'La Sapienza' over a 6-month period.
Findings:
- Budd-Chiari syndrome in this pediatric patient was classified as idiopathic.
- The co-occurrence of Budd-Chiari syndrome and caecum polyposis is considered a likely coincidence.
- Medical management and supportive care were administered; liver transplant remains a potential future intervention.
Implications:
- This case highlights the importance of considering rare conditions like Budd-Chiari syndrome in pediatric patients.
- The report contributes to the limited literature on childhood Budd-Chiari syndrome and its potential associations.
- Further research may elucidate any potential, albeit unlikely, pathogenetic links between these two conditions.
Abstract:
This work aims at describing a particular case of Budd-Chiari syndrome in a ten-year-old girl. In our patient, the subjective and objective features of this disease, which is already rare during childhood, are associated with a well-assessed polyposis in the blind intestine (intestinum caecum). In our opinion, a possible connection between these two pathological events, a connection unheard of in the literature so far, is an interesting issue to be dealt with in this case report. The follow-up of our patient took place at the Pediatric Hospital of the University of Rome "La Sapienza" and lasted an overall period of 6 months. The girl was hospitalized twice and she underwent a large number of radiological and laboratory tests. The therapy consisted only of medical treatment and support measures. Liver transplant is regarded as the only chance to obtain a full recovery, but it has not yet been performed on our patient. It is by comparing those researches carried out on similar cases with the outcome of our personal study, that we come to the conclusion that, as for our patient, the Budd-Chiari syndrome can be classified as an idiopathic syndrome. Moreover, the fact that this syndrome and the polyposis of caecum occur at the same time in a patient can probably be considered as a coincidence.