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病例报告:丹迪-沃克畸形合并枕部脑膨出及叠加性脑膜炎。

Case report: Dandy-Walker malformation with occipital encephalocele and superadded meningitis.

作者信息

Bano Saher, Faizan Muhammad Aqib, Rehman Tooba, Kaur Jasleen, Singh Jeevanjyot

机构信息

Ayub Medical College, Khyber Medical University, Khyber Pakhtunkhwa, Pakistan.

Gomal Medical College, Khyber Medical University, Khyber Pakhtunkhwa, Pakistan.

出版信息

Heliyon. 2025 Jan 10;11(2):e41883. doi: 10.1016/j.heliyon.2025.e41883. eCollection 2025 Jan 30.

Abstract

UNLABELLED

Dandy-Walker malformation is a relatively uncommon abnormality known as cerebellar dysgenesis. This condition is characterized by cerebellar vermis hypoplasia, an upwardly rotated and malrotated vermis, an enlarged fourth ventricle, and an enlarged posterior fossa. This syndrome is thought to affect roughly one in every 35000 live births. Here we present a case of a preterm male of 33 + 4 weeks period of gestation who presented to us with a posterior midline cystic swelling at the occipital neck region. While there was no bruit in the transillumination of the cyst, there was a bruit in the transillumination of the skull.Due to his grunting respiratory rate of 44 breaths per minute and heart rate of 140 breaths per minute at presentation, he was admitted to the newborn critical care unit. The occipital cyst was excised following neurosurgery on board after getting a CT scan brain done which showed dilated ventricles with normal pressure hydrocephalus, cystic cerebellar changes, communicating with 4th ventricle and occipital encephalocele. Brain parenchymal changes were also noted and a CSF R/E was sent which showed meningitis and the patient was put on IV antibiotics empirically along with fluid supplementation.

OBJECTIVE

This case report aims to highlight the clinical presentation, diagnostic approach, and management of Dandy-Walker Malformation (DWM) in a preterm neonate, emphasizing the importance of early recognition and intervention in similar cases.

RATIONALE

This case underscores the need for careful evaluation of neonatal cystic lesions, particularly in preterm infants, where timely diagnosis and appropriate management can significantly affect outcomes. By documenting this case, we aim to contribute to the understanding of Dandy-Walker Malformation and improve clinical practices in neonatal care.

摘要

未标注

丹迪-沃克畸形是一种相对罕见的异常,称为小脑发育不全。这种病症的特征是小脑蚓部发育不全、蚓部向上旋转和旋转异常、第四脑室扩大以及后颅窝扩大。据认为,这种综合征在每35000例活产中约有1例受影响。在此,我们报告一例孕33 + 4周的早产男婴,其枕颈部出现后正中囊性肿胀。囊肿透照时无杂音,但颅骨透照时有杂音。由于其就诊时呼吸急促,每分钟44次呼吸,心率每分钟140次,他被收治入新生儿重症监护病房。在进行脑部CT扫描显示脑室扩张伴正常压力脑积水、小脑囊性改变、与第四脑室相通以及枕部脑膨出后,在船上进行神经外科手术后切除了枕部囊肿。还注意到脑实质改变,并送检了脑脊液常规及生化检查,结果显示为脑膜炎,患者经验性地接受了静脉抗生素治疗并补充了液体。

目的

本病例报告旨在强调早产新生儿丹迪-沃克畸形(DWM)的临床表现、诊断方法和管理,强调在类似病例中早期识别和干预的重要性。

原理

本病例强调了对新生儿囊性病变进行仔细评估的必要性,特别是在早产儿中,及时诊断和适当管理可显著影响预后。通过记录本病例,我们旨在促进对丹迪-沃克畸形的理解并改善新生儿护理的临床实践。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee44/11787474/259760a9e471/gr1.jpg

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