Polysplenia: Difference between revisions
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{{Infobox medical condition | |||
| name = Polysplenia | |||
| synonyms = [[Ivemark syndrome]], [[Bilateral left-sidedness]] | |||
| field = [[Medical genetics]], [[Pediatrics]] | |||
| symptoms = Multiple [[spleens]], [[congenital heart defects]], [[abnormal organ placement]] | |||
| complications = [[Heart failure]], [[infections]] | |||
| onset = [[Congenital]] | |||
| duration = [[Lifelong]] | |||
| causes = [[Genetic mutations]], [[environmental factors]] | |||
| risks = [[Family history]], [[genetic syndromes]] | |||
| diagnosis = [[Imaging studies]], [[echocardiogram]], [[genetic testing]] | |||
| differential = [[Asplenia]], [[heterotaxy syndrome]] | |||
| prevention = None | |||
| treatment = [[Surgical intervention]], [[antibiotic prophylaxis]] | |||
| prognosis = Variable, depends on associated anomalies | |||
| frequency = Rare | |||
}} | |||
'''Polysplenia''' is a rare congenital condition characterized by the presence of multiple [[spleen]]s of varying sizes in the body. It is often associated with complex [[congenital heart disease]] and abnormalities in other organ systems. | '''Polysplenia''' is a rare congenital condition characterized by the presence of multiple [[spleen]]s of varying sizes in the body. It is often associated with complex [[congenital heart disease]] and abnormalities in other organ systems. | ||
==Etiology== | ==Etiology== | ||
The exact cause of polysplenia is unknown. However, it is believed to be a result of abnormal [[embryogenesis]] during the fifth week of [[gestation]]. This is when the spleen and other organs are forming. | The exact cause of polysplenia is unknown. However, it is believed to be a result of abnormal [[embryogenesis]] during the fifth week of [[gestation]]. This is when the spleen and other organs are forming. | ||
==Clinical Features== | ==Clinical Features== | ||
Polysplenia is often diagnosed during infancy or early childhood. The condition is usually identified during investigations for associated anomalies, particularly cardiac defects. Symptoms may vary depending on the associated anomalies. | Polysplenia is often diagnosed during infancy or early childhood. The condition is usually identified during investigations for associated anomalies, particularly cardiac defects. Symptoms may vary depending on the associated anomalies. | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis of polysplenia is usually made through [[medical imaging]] techniques such as [[ultrasound]], [[computed tomography (CT) scan]], or [[magnetic resonance imaging (MRI)]]. These imaging studies can reveal the presence of multiple spleens and any associated anomalies. | Diagnosis of polysplenia is usually made through [[medical imaging]] techniques such as [[ultrasound]], [[computed tomography (CT) scan]], or [[magnetic resonance imaging (MRI)]]. These imaging studies can reveal the presence of multiple spleens and any associated anomalies. | ||
==Treatment== | ==Treatment== | ||
Treatment for polysplenia is usually supportive and depends on the associated anomalies. In cases where there are severe cardiac defects, [[surgery]] may be required. | Treatment for polysplenia is usually supportive and depends on the associated anomalies. In cases where there are severe cardiac defects, [[surgery]] may be required. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis for individuals with polysplenia varies widely and depends largely on the severity of the associated anomalies. | The prognosis for individuals with polysplenia varies widely and depends largely on the severity of the associated anomalies. | ||
==See Also== | ==See Also== | ||
* [[Asplenia]] | * [[Asplenia]] | ||
* [[Heterotaxy syndrome]] | * [[Heterotaxy syndrome]] | ||
* [[Situs inversus]] | * [[Situs inversus]] | ||
==References== | ==References== | ||
{{reflist}} | {{reflist}} | ||
[[Category:Congenital disorders]] | [[Category:Congenital disorders]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
Latest revision as of 05:36, 4 April 2025
| Polysplenia | |
|---|---|
| Synonyms | Ivemark syndrome, Bilateral left-sidedness |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Multiple spleens, congenital heart defects, abnormal organ placement |
| Complications | Heart failure, infections |
| Onset | Congenital |
| Duration | Lifelong |
| Types | N/A |
| Causes | Genetic mutations, environmental factors |
| Risks | Family history, genetic syndromes |
| Diagnosis | Imaging studies, echocardiogram, genetic testing |
| Differential diagnosis | Asplenia, heterotaxy syndrome |
| Prevention | None |
| Treatment | Surgical intervention, antibiotic prophylaxis |
| Medication | N/A |
| Prognosis | Variable, depends on associated anomalies |
| Frequency | Rare |
| Deaths | N/A |
Polysplenia is a rare congenital condition characterized by the presence of multiple spleens of varying sizes in the body. It is often associated with complex congenital heart disease and abnormalities in other organ systems.
Etiology[edit]
The exact cause of polysplenia is unknown. However, it is believed to be a result of abnormal embryogenesis during the fifth week of gestation. This is when the spleen and other organs are forming.
Clinical Features[edit]
Polysplenia is often diagnosed during infancy or early childhood. The condition is usually identified during investigations for associated anomalies, particularly cardiac defects. Symptoms may vary depending on the associated anomalies.
Diagnosis[edit]
Diagnosis of polysplenia is usually made through medical imaging techniques such as ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI). These imaging studies can reveal the presence of multiple spleens and any associated anomalies.
Treatment[edit]
Treatment for polysplenia is usually supportive and depends on the associated anomalies. In cases where there are severe cardiac defects, surgery may be required.
Prognosis[edit]
The prognosis for individuals with polysplenia varies widely and depends largely on the severity of the associated anomalies.
See Also[edit]
References[edit]
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