Presentation
We present a case of a 5-month-old girl who presented with macrocephaly, developmental delay, and failure to thrive. [pediatricneurosciences.com]
We also present a review of the literature about this entity. [jmedicalcasereports.biomedcentral.com]
Keywords Lateral meningocele multiple menigocoele spinal dysraphism INTRODUCTION Lateral meningocele is a rare presentation of spinal dysraphism characterized by extensions of the dura and arachnoid through an enlarged neural foramen.[1–10] In the present [clinicalimagingscience.org]
Case presentation: Colombian patient suspected of having lateral meningocele syndrome, with dysmorphic facies, multiple lateral meningoceles and dural ectasias. [medigraphic.com]
Here, we present a 2-year-old girl for whom an early genomic analysis allowed us to recognize the presence of lateral meningoceles and to begin early monitoring of her condition for possible neurological complications. [keio.pure.elsevier.com]
Entire Body System
- Short Stature
stature, scoliosis, and kyphosis. [pediatricneurosciences.com]
stature * Back pain * Wide set eyes * Droopy eyelids * Umbilical hernia * Ear abnormalities * Increased bone density in skull * Thickened calvaria * Flattened cheeks * Narrow palate * Wormian bones * Undescended testes * Loose joints * Reduced muscle [checkorphan.org]
- Asymptomatic
The patient may be asymptomatic or can present with back pain or paraparesis.[12] In contrast, in the setting of a small meningocoele, no symptoms can be recorded, and the lesion may be incidentally diagnosed on a routine chest radiograph. [clinicalimagingscience.org]
Perineural or Tarlov cysts are asymptomatic and are discovered incidentally through myelogram or MRI originally intended for other reasons [11]. [jmedicalcasereports.biomedcentral.com]
[…] presented with failure to thrive, indicating that LMS may rarely present with homeostatic symptoms of unknown origin possibly shared with other hereditary connective tissue disorders.[2] In contrast, when the meningoceles are small, the patient may be asymptomatic [pediatricneurosciences.com]
- Swelling
Article CAS PubMed Google Scholar Kaur N, Chandra Mishra S, Vijayaragvan P, Minocha VR: Lateral sacral meningomyelocele as a gluteal swelling: an unusual presentation. J Indian Med Assoc. 2005, 103 (10): 554-556. [jmedicalcasereports.biomedcentral.com]
Lateral sacral meningomyelocele as a gluteal swelling: An unusual presentation. J Indian Med Assoc. 2005;103:556. [Google Scholar] Reis C, Carneiro E, Fonseca J, Pereira P, Vaz R, Pinto R,. [clinicalimagingscience.org]
Lateral sacral meningomyelocele as a gluteal swelling: an unusual presentation. J Indian Med Assoc 2005;103:554-6. 11. Alves D, Sampaio M, Figueiredo R, Leão M. [pediatricneurosciences.com]
Respiratoric
- Pleural Effusion
Subsequently, the patient developed a pleural effusion requiring conversion of the meningocele-pleural shunt to a meningocele-peritoneal shunt. [karger.com]
Gastrointestinal
- Failure to Thrive
We present a case of a 5-month-old girl who presented with macrocephaly, developmental delay, and failure to thrive. [pediatricneurosciences.com]
One year later, Katz et al. [26] described a similar case in a 2-year-old male with multiple paraspinal masses and a widened spinal canal who initially presented for evaluation for failure to thrive. [karger.com]
- Progressive Dysphagia
The author described a 6-month-old female with progressive dysphagia and choking episodes [34]. [karger.com]
Musculoskeletal
- Macrocephaly
We present a case of a 5-month-old girl who presented with macrocephaly, developmental delay, and failure to thrive. [pediatricneurosciences.com]
Summary of neurosurgical management strategies in LMS patients Recently, Brown et al. [34] described a complicated case of LMS with a severe Chiari I malformation, ventriculomegaly, and macrocephaly. [karger.com]
Urogenital
- Cryptorchidism
The clinical characteristics included hypotonia, dysphagia, hypertension, lateral spinal meningocele, craniofacial anomaly, and cryptorchidism. Abnormal spinal MRI and brainstem evoked potential were also observed. [pesquisa.bvsalud.org]
Cryptorchidism and (rarely) renal anomaly Gastrointestinal. [ncbi.nlm.nih.gov]
Neurologic
- Irritability
Clinical follow-up 2 weeks later revealed resolution of her irritability and full strength in her lower extremities. [karger.com]
Workup
Pleura
- Pleural Effusion
Subsequently, the patient developed a pleural effusion requiring conversion of the meningocele-pleural shunt to a meningocele-peritoneal shunt. [karger.com]
Treatment
Treatment and prognosis Symptomatic treatment for lateral meningoceles may require surgical intervention for large lesions. History and etymology Lateral meningocele syndrome was first described in 1977 by Lehman et al 4. [radiopaedia.org]
[edit] Currently, there is no treatment for Lehman syndrome. [en.wikipedia.org]
Prognosis
Treatment and prognosis Symptomatic treatment for lateral meningoceles may require surgical intervention for large lesions. History and etymology Lateral meningocele syndrome was first described in 1977 by Lehman et al 4. [radiopaedia.org]
Etiology
Relevant literature up to February 2021, retrieved from PubMed, OMIM, CNKI, Wanfang, and CQVIP database with the terms of "lateral meningocele syndrome", " NOTCH3", were reviewed to summarize the clinical characteristics, pathogenesis, and genetic etiology [pesquisa.bvsalud.org]
Epidemiology
[…] is responsible for disintegration of the NCID after it has gone into the nucleus and completed its function.[4] The mutation causes the Notch 3 protein to be in the cell nucleus for a prolonged period of time continuing to affect gene activity.[14] Epidemiology [en.wikipedia.org]