Management and Outcome of a Neurologically Intact Infant with Pierre Robin Sequence and Dandy-Walker Variant Diagnosed with Large Hemispheric Brain Abscess ­ Case Report

Pierre Robin sequence (PRS) is a condition consisting of three essential components: micrognathia or retrognathia, cleft palate, and glossoptosis. It can be part of multiple congenital anomalies. We present the case and outcome of a 3-month-old clinically stable patient who has PRS with Dandy-Walker variant ­ which is a rare presentation in the literature ­ with a large right hemispheric brain abscess, treated with multiple minimally-invasive surgical drainage procedures with adjuvant antibiotics.

Buckling of the Ligamentum Flavum as a Rare Complication of Anterior Cervical Corpectomy and Fusion: A Case Report

Introduction and Importance Neurological deterioration due to buckling of the ligamentum flavum (LF) is an uncommon complication after anterior cervical corpectomy or discectomy with fusion. Case Presentation In this report, we present the case of a 66-year-old male who underwent anterior cervical partial corpectomy of C5 and discectomy of prolapsed C5- C6 with fusion. Postsurgery, the patient displayed signs of neurological deterioration. Upon immediate cervical magnetic resonance imaging (MRI), posterior canal stenosis and severe compression with cord signal due to LF buckling were detected. A posterior laminectomy procedure and canal decompression at the C5-C6 level with bone fusion were performed. Clinical Discussion Patient presented with walking difficulty, then walking disability, followed by bilateral upper and lower limb paresthesia with burning sensation. Examination showed ⅘ muscle strength in both handgrips. Further investigation showed brisk deep tendon reflexes, positive Hoffman sign unilaterally, equivocal Babinski sign, and progressive quadriparesis. Magnetic resonance imaging showed mild and diffuse building of some cervical discs, with spinal cord progression. We performed an anterior cervical corpectomy and fusion (ACCF) and anterior cervical discectomy and fusion (ACDF); a titanium mesh with plates and screws was used for fusion, with removal of a calcified and herniated subligamentous disc. Postoperatively, upper and lower limb strength deteriorated; immediate cervical and thoracic MRI showed LF buckling, which caused canal stenosis and severe compression. Urgent posterior laminectomy and canal decompression with bone fusion was scheduled on the same day. The patient underwent physiotherapy and regained upper and lower limb strength and his ability to walk. Conclusion This indicates the possibility of neurological deterioration as a result of LF buckling, whichmay be a result of LF thickening accompanied by hyperextension in the cervical region. In this regard, immediate imaging following signs of neurological complications after anterior cervical corpectomy or discectomy warrants early detection, which results in a better prognosis.

Maternal and fetal serum leptin levels and their association with maternal and fetal variables and labor: A cross-sectional study.

BACKGROUND: Leptin is a polypeptide hormone that may be implicated in the pathogenesis of various disorders during pregnancy. We sought to determine serum leptin levels among pregnant women and their fetuses and to investigate their association with fetal and maternal variables. METHOD: 452 pregnant women who attended to labor ward between January 2020 and August 2020 were included in the study. Serum leptin concentrations were measured using enzyme-linked immunosorbent assay method. Mann-Whitney U test and Spearman's correlation test were used for statistical analysis. A multivariate linear regression analysis was then performed. Significance level was considered at alpha <0.05. RESULTS: The median maternal and fetal serum leptin levels were 6.42 [4.16-8.51] ng/mL and 2.9 [1.03-5.36] ng/mL respectively. There was no significant correlation between maternal and fetal serum leptin levels (p = 0.064). Maternal serum leptin levels correlated positively with maternal body mass index (BMI) (r = 0.117, p = 0.005). Besides, maternal serum leptin levels were significantly higher in nulliparous women (7.57 [4.45-9.30] ng/mL vs. 6.22 [4.02-8.30] ng/mL, p = 0.037) and in women who were in active labor (6.83 [4.39-8.92] ng/mL vs. 6.25 [4.04-8.30] ng/mL, p = 0.047). Fetal serum leptin levels were significantly higher in large for gestational age (LGA) fetuses (4.81 [2.13-7.22] ng/mL vs. 2.80 [0.96-5.16] ng/mL, p = 0.003) and in fetuses with preterm premature ruptures of membranes (PPROM) (5.23 [2.42-8.07] ng/mL vs. 2.86 [1.00-5.23] ng/mL, p = 0.021). CONCLUSION: Maternal serum leptin levels were influenced by maternal BMI, parity and labor. Fetal serum leptin levels were higher among LGA fetuses and in fetuses with PPROM.

Primary cervical glioblastoma multiforme as a presentation of constitutional mismatch repair deficiency: Case report and literature review.

INTRODUCTION AND IMPORTANCE: Primary Glioblastoma Multiforme(GBM) of cervical spinal cord represent an extremely rare type of tumors in the pediatric age group. Constitutional mismatch repair deficiency (CMMRD) patients are known to develop uni- or multiple synchronous-high grade gliomas in the brain. CASE PRESENTATION: The authors report a 23 month old child presented with bilateral upper limb weakness for 7 days with imaging evidence of intramedullary mass lesion that extends from the level of the C3 to C7. The patient underwent excisional biopsy from C3 to C7 and laminoplasty. Immunohistology confirmed primary cervical GBM. CLINICAL DISCUSSION: Constitutional mismatch repair deficiency is cancer tendent syndrome associated with broad spectrum of malignancies. Screening for CMMRD is not a daily practice in oncology and thus prevalence might be underestimated. To authors' knowledge, no prior primary cervical GBM in CMMRD syndrome. CONCLUSION: This report highlights the challenges of CMMRD polymorphic presentations, diagnosis, complications, management and surveillance.

Paraparesis As a Rare First Presentation Of Primary Hyperparathyroidism-Related Brown Tumor in The Thoracic Spine: Case report and literature review.

INTRODUCTION AND IMPORTANCE: Brown tumor (BT) is defined as osteolytic lesion of an underlying state of hyperparathyroidism. Hyperparathyroidism will activate osteoclasts which initiate active bone resorption foci of lytic-cysts with hemosiderin depositions that pigment it with its characteristic brown pathologic gross appearance. Devastating fractures and injuries can occur to affected bones and surrounding tissue that require emergent intervention and correction. CASE PRESENTATION: We present a case of a medically free 31-year-old female patient, who presented complaining of unsteadiness and progressive lower limbs weakness over 40 days of duration. Subsequent lab tests showed elevated PTH levels, along with 3.5 × 1.8 cm heterogeneous soft tissue mass involving the right pedicle on T7 level compressing the corresponding level of the spinal cord. Surgical management aimed to decompress the spinal cord and to obtain a biopsy for histopathologic examination which revealed a brown tumor. Neck ultrasound and Sestamibi scan indicated the presence of hyperactive and hyperplastic parathyroid tissue most suggestive of parathyroid adenoma. CLINICAL DISCUSSION: Various presentations of Brown Tumor depend on the bone affected, despite the rarity of spinal involvement, yet expanding tumors can manifest either with back pain, radicular pain, paresthesia, weakness, paralysis, or incontinence. The highest incidence rates of spinal brown tumors affect adults over the age of 40. Management goals are to decompress the neuronal tissue emergently and to prevent further bony lytic deterioration. CONCLUSION: The objective of this study is to provide an overview of primary hyperparathyroidism-related spinal brown tumors, presentation, and summary of previously reported similar cases in the literature.