Splenic Torsion in Heterotaxy Syndrome with Left Isomerism: A Case Report and Literature Review

Cheang, I Nok; Fu, Yu-Wei; Chin, Taiwai; Hsu, Yao-Jen; Wu, Chin-Yen

doi:10.3390/diagnostics12122920

Cited by 2 publications

(4 citation statements)

References 39 publications

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“…Splenic infarction is an uncommon but well-recognized complication of polysplenia syndrome [ 3 ]. The symptoms of splenic infarction can mimic other conditions such as acute appendicitis, pneumonia, or urinary tract infection, making it a challenging diagnosis [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

“…The management of splenic infarction in polysplenia syndrome is mainly supportive, with antibiotics and pain management as the primary treatments [ 2 , 4 ]. The goals of therapy are to prevent complications such as sepsis, maintain hydration and electrolyte balance, and manage pain [ 3 ]. In this case, the patient was started on intravenous antibiotics and received pain management with acetaminophen and ibuprofen.…”

Section: Discussionmentioning

confidence: 99%

“…The prognosis for patients with polysplenia syndrome and splenic infarction is generally favorable, with most patients recovering without complications [ 3 ]. However, there is a risk of recurrent splenic infarction, which may require splenectomy, especially if there is a significant decrease in spleen function [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

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Polysplenia Syndrome Complicated by Splenic Infarction: A Report of a Rare Case

Abdullah¹,

Alharbi²,

Alnutaifi³

et al. 2023

Cureus

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Polysplenia syndrome is a rare congenital anomaly characterized by multiple spleens and associated organ anomalies, which can present with serious complications such as splenic infarction. Diagnosis and management of the disorder can be challenging due to the presence of associated anomalies and the condition is often diagnosed incidentally. We report a case of a six-year-old girl with no significant medical history who presented to the emergency department with fever, abdominal pain, and vomiting. Physical examination and laboratory investigations showed leukocytosis, anemia, and elevated levels of C-reactive protein. A computed tomography scan revealed splenic infarction with polysplenia syndrome. The patient received intravenous antibiotics and pain management and was closely monitored for complications such as sepsis. Early diagnosis and appropriate management are essential to prevent complications, and close monitoring and follow-up are necessary for long-term management.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Polysplenia Syndrome Complicated by Splenic Infarction: A Report of a Rare Case

Abdullah¹,

Alharbi²,

Alnutaifi³

et al. 2023

Cureus

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“…Left atrial isomerism is a heterotaxy syndrome that may associate omphalocele with polysplenia, congenital heart defects (ventricular noncompaction, ventricular hypertrophy, bradycardia, cardiomegaly, and heart block), bilateral bilobed lungs, and situs abnormalities of abdominal organs [ 53 ]. Splenic torsion is an unusual but dangerous condition that may occur in these patients, and physicians should be vigilant of the differential diagnosis of the acute abdomen [ 54 ].…”

Section: Chromosomal Abnormalities and Syndromesmentioning

confidence: 99%

Omphalocele and Cardiac Abnormalities—The Importance of the Association

Ţarcă

Namat²,

Luca

et al. 2023

Diagnostics

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Omphalocele is the most common ventral abdominal wall defect. Omphalocele is associated with other significant anomalies in up to 80% of cases, among which the cardiac ones are the most frequent. The aim of our paper is to highlight, through a review of the literature, the importance and frequency of association between the two malformations and what impact this association has on the management and evolution of patients with these pathologies. We reviewed the titles, the available abstracts, and the full texts of 244 papers from the last 23 years, from three medical databases, to extract data for our review. Due to the frequent association of the two malformations and the unfavorable effect of the major cardiac anomaly on the prognosis of the newborn, the electrocardiogram and echocardiography must be included in the first postnatal investigations. The timing of surgery for abdominal wall defect closure is mostly dictated by the cardiac defect severity, and usually the cardiac defect takes priority. After the cardiac defect is medically stabilized or surgically repaired, the omphalocele reduction and closure of the abdominal defect are performed in a more controlled setting, with improved outcomes. Compared to omphalocele patients without cardiac defects, children with this association are more likely to experience prolonged hospitalizations, neurologic, and cognitive impairments. Major cardiac abnormalities such as structural defects that require surgical treatment or result in developmental delay will significantly increase the death rate of patients with omphalocele. In conclusion, the prenatal diagnosis of omphalocele and early detection of other associated structural or chromosomal anomalies are of overwhelming importance, contributing to the establishment of antenatal and postnatal prognosis.

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Splenic Torsion in Heterotaxy Syndrome with Left Isomerism: A Case Report and Literature Review

Cited by 2 publications

References 39 publications

Polysplenia Syndrome Complicated by Splenic Infarction: A Report of a Rare Case

Polysplenia Syndrome Complicated by Splenic Infarction: A Report of a Rare Case

Omphalocele and Cardiac Abnormalities—The Importance of the Association

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