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诊断患有弗林斯综合征和腭裂的新生儿膈膨出的挑战。

Challenges in diagnosing diaphragmatic eventration in a neonate with Fryns syndrome and cleft palate.

作者信息

Imdadoglu Timucin, Unal Irem, Yasar Elif, Alomari Omar, Guzel Bulent, Boyaci Erdogan, Altuncu Emel

机构信息

Department of Pediatrics, Sancaktepe Prof. Dr. Ilhan Varank Training and Research Hospital, Istanbul, Türkiye.

Department of Pediatrics, Division of Neonatology, Sancaktepe Prof. Dr. Ilhan Varank Training and Research Hospital, Istanbul, Türkiye.

出版信息

Radiol Case Rep. 2025 Aug 20;20(11):5632-5637. doi: 10.1016/j.radcr.2025.07.049. eCollection 2025 Nov.

Abstract

Diaphragmatic eventration is a rare congenital anomaly characterized by the replacement of diaphragmatic muscle with fibroelastic tissue, leading to diaphragm elevation and functional impairment. While it can occur in isolation, it is sometimes associated with congenital syndromes, including septo-optic dysplasia (SOD). This report presents a case of diaphragmatic eventration in a neonate with Fryns syndrome presenting with cleft palate and SOD, highlighting diagnostic challenges. A late preterm female neonate, born via cesarean at 36 weeks gestation, presented with respiratory distress and required intubation and surfactant therapy. Physical examination revealed features suggestive of Fryns syndrome, including coarse facies, microphthalmia, and cleft palate. Imaging studies, including chest X-ray and thoracic ultrasound, raised suspicion of diaphragmatic hernia, but subsequent MRI confirmed diaphragmatic eventration. Additional cranial imaging identified agenesis of the corpus callosum, colpocephaly, and optic nerve hypoplasia, leading to the diagnosis of SOD. Diaphragmatic eventration, though rare, can present in neonates with other congenital anomalies, making diagnosis challenging. In this case, the overlapping symptoms with cleft palate and SOD complicated the clinical picture. Early imaging, including MRI, is crucial for accurate diagnosis and timely management. Multidisciplinary follow-up is essential for optimal patient care.

摘要

膈膨升是一种罕见的先天性异常,其特征是膈肌被纤维弹性组织替代,导致膈肌抬高和功能受损。虽然它可单独发生,但有时与先天性综合征相关,包括视隔发育不良(SOD)。本报告介绍了一例患有腭裂和SOD的Fryns综合征新生儿的膈膨升病例,突出了诊断挑战。一名晚期早产儿女性新生儿,孕36周剖宫产出生,出现呼吸窘迫,需要插管和表面活性剂治疗。体格检查发现有提示Fryns综合征的特征,包括面容粗糙、小眼畸形和腭裂。影像学检查,包括胸部X线和胸部超声,怀疑有膈疝,但随后的MRI证实为膈膨升。额外的头颅影像学检查发现胼胝体发育不全、脑室扩张和视神经发育不全,从而诊断为SOD。膈膨升虽然罕见,但可出现在患有其他先天性异常的新生儿中,使诊断具有挑战性。在本病例中,与腭裂和SOD重叠的症状使临床情况复杂化。早期影像学检查,包括MRI,对于准确诊断和及时管理至关重要。多学科随访对于优化患者护理至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a29b/12396267/b5a2bd385f4e/gr1.jpg

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