“…Subtotal resection of CCL is recommended in symptomatic patients for less morbimortality. [ 15 , 19 , 22 , 23 ]…”
Section: Discussionmentioning
confidence: 99%
“…Subtotal resection of CCL is recommended in symptomatic patients for less morbimortality. [15,19,22,23] Ethical approval e Institutional Review Board approval is not required.…”
Background:
Intracranial lipomas are a rare clinical entity. These lesions are frequently asymptomatic and originate in the pericallosal area. As they are fat-containing lesions which are intimately attached to the surrounding structures, surgery is not recommended. In some individual reports, subtotal resection is recommended to lessen complications. There have been no previous reports of corpus callosum lipoma (CCL) associated with limited dorsal myeloschizis (LDM).
Case Description:
We describe the case of a combination of CCL and bilateral choroid plexus lipoma discovered incidentally during the investigation of LDM in a 3-month-old male child. Given the asymptomatic behavior of the lipoma and the vascular elements of the pericallosal area, it was decided to monitor it regularly. Thus, the patient underwent surgery only for LDM. Histological examination confirmed the diagnosis, and postoperative follow-up 1 year after showed good evolution. To the best of our knowledge, this association has never been described in the literature.
Conclusion:
This case suggests a possible developmental relationship between CCL and spinal dysraphism.
“…Subtotal resection of CCL is recommended in symptomatic patients for less morbimortality. [ 15 , 19 , 22 , 23 ]…”
Section: Discussionmentioning
confidence: 99%
“…Subtotal resection of CCL is recommended in symptomatic patients for less morbimortality. [15,19,22,23] Ethical approval e Institutional Review Board approval is not required.…”
Background:
Intracranial lipomas are a rare clinical entity. These lesions are frequently asymptomatic and originate in the pericallosal area. As they are fat-containing lesions which are intimately attached to the surrounding structures, surgery is not recommended. In some individual reports, subtotal resection is recommended to lessen complications. There have been no previous reports of corpus callosum lipoma (CCL) associated with limited dorsal myeloschizis (LDM).
Case Description:
We describe the case of a combination of CCL and bilateral choroid plexus lipoma discovered incidentally during the investigation of LDM in a 3-month-old male child. Given the asymptomatic behavior of the lipoma and the vascular elements of the pericallosal area, it was decided to monitor it regularly. Thus, the patient underwent surgery only for LDM. Histological examination confirmed the diagnosis, and postoperative follow-up 1 year after showed good evolution. To the best of our knowledge, this association has never been described in the literature.
Conclusion:
This case suggests a possible developmental relationship between CCL and spinal dysraphism.
“…MRI is useful also to define the lesion’s dimensions and associated anomalies of the corpus callosum [ 23 ]. Shinar et al have recently published a paper regarding the clues to diagnose pericallosal lipoma prenatally [ 40 ]. Fig.…”
Background
Pai syndrome is a rare idiopathic developmental condition characterized by midline craniofacial abnormalities. It was originally described as the presence of a median cleft lip, cutaneous polyps of the nasal mucosa and face, and midline lipomas of the central nervous system, mostly at the corpus callosum. However, there is great phenotypical variability and these characteristics are rarely all present at once.
Objective
The aim of this review was to analyze the available evidence regarding Pai syndrome in order to better delineate this rare condition and its features.
Methods
We analyzed the PubMed database using the words “Pai syndrome”, “frontonasal dysplasia”, “cleft lip”, “nasal polyp”, “facial polyp”, and “corpus callosum lipoma”, including reviews, case reports and case series.
Conclusion
There is no consensus regarding the diagnostic criteria of Pai syndrome up to date. It is usually diagnosed at birth, and its incidence is often underestimated. At present, the etiology of Pai syndrome is unknown. Several hypotheses regarding its genetic background have been made; however, there are not enough data yet to elucidate this point. An improved awareness could help in diagnosing the condition and performing the necessary investigations. These patients should have a multidisciplinary follow-up.
“…Furthermore, among all intracranial lipomas; PCLs are the most frequently occurring lipomas (14,16). PCLs are located within the interhemispheric fissure and they are defined by ultrasonography as hyperechoic, solid and well-demarcated masses (11,14).…”
AIM: To determine the clinical and radiographical aspects of six patients diagnosed with pericallosal lipomas (PCL). MATERIAL and METHODS: A retrospective analysis of patients who presented to the neurosurgery outpatient clinics of Selcuk Faculty of Medicine between 2009 and 2019, revealed that six patients were diagnosed with PCL. The clinical and magnetic resonance imaging (MRI) data were obtained by reviewing patients' records. RESULTS: A total of six patients (two girls and four boys), with a mean age of 53.8 months (38-72 months), were included in this study. They were followed up for a mean period of 36.5 months (32-41 months). PCL were detected on MRIs, which were obtained to investigate headache in two patients, epilepsy in one patient, frontal dermal sinus tract and left frontal epidermoid tumor in one patient, and subcutaneous lipoma associated with PCL in one patient. Five patients displayed tubulonodular lipomas and one patient displayed curvilinear lipomas. Agenesis or dysgenesis of the corpus callosum (CC) was observed in four (66%) patients. Two patients received surgical treatment for cosmetic skin problem. CONCLUSION: Because of the benign course of PCL, i.e. no growth or very slow growth, and close proximity to the surrounding neurovascular structures, surgical removal should be considered only in symptomatic PCL. Furthermore, other malformations and anomalies may accompany PCL.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
