دورية أكاديمية
Atypical caudal regression syndrome with lumbar agenesis, hypoplastic sacrum without sacroiliac joints in the eastern Democratic Republic of Congo: a case report.
| العنوان: | Atypical caudal regression syndrome with lumbar agenesis, hypoplastic sacrum without sacroiliac joints in the eastern Democratic Republic of Congo: a case report. |
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| المؤلفون: | Mudekereza PS; Department of Surgery, Provincial General Reference Hospital of Bukavu, Bukavu, Democratic Republic of Congo. paternemude@yahoo.fr.; Faculty of Medicine, Université Catholique de Bukavu, Bukavu, Democratic Republic of Congo. paternemude@yahoo.fr., Balemba GM; Department of Radiology, Provincial General Reference Hospital of Bukavu, Bukavu, Democratic Republic of Congo.; Faculty of Medicine, Université Catholique de Bukavu, Bukavu, Democratic Republic of Congo., Murhega RB; Department of Surgery, Provincial General Reference Hospital of Bukavu, Bukavu, Democratic Republic of Congo.; Research Department, Association of Future African Neurosurgeons, Yaoundé, Cameroun.; Faculty of Medicine, Université Catholique de Bukavu, Bukavu, Democratic Republic of Congo., Murhula GB; Department of Surgery, Provincial General Reference Hospital of Bukavu, Bukavu, Democratic Republic of Congo.; Faculty of Medicine, Université Catholique de Bukavu, Bukavu, Democratic Republic of Congo., Nteranya DS; Research Department, Association of Future African Neurosurgeons, Yaoundé, Cameroun.; Department of Surgery, Official University of Bukavu, Bukavu, Democratic Republic of Congo., Lekuya HM; Department of Surgery, College of Health Sciences, Makerere University, Kampala, Uganda. |
| المصدر: | Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery [Childs Nerv Syst] 2023 Sep; Vol. 39 (9), pp. 2551-2556. Date of Electronic Publication: 2023 Jun 09. |
| نوع المنشور: | Case Reports; Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Springer International Country of Publication: Germany NLM ID: 8503227 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1433-0350 (Electronic) Linking ISSN: 02567040 NLM ISO Abbreviation: Childs Nerv Syst Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Berlin : Springer International, c1985- |
| مواضيع طبية MeSH: | Abnormalities, Multiple*/diagnostic imaging , Spinal Diseases* , Nervous System Malformations*, Infant ; Humans ; Female ; Sacrum/diagnostic imaging ; Sacrum/abnormalities ; Democratic Republic of the Congo ; Lumbosacral Region ; Sacroiliac Joint/diagnostic imaging ; Syndrome |
| مستخلص: | The agenesis of any segment of the lower spinal column referred to as "caudal regression syndrome" (CRS) is a rare congenital defect of the spine. This malformation is characterized by the absence of some or the entire lumbosacral vertebral segment. Etiological factors remain unknown. We report an atypical caudal regression syndrome with lumbar agenesis, disconnected from the remaining hypoplastic sacrum, in the Eastern part of the Democratic Republic of Congo (DRC).An 11-month-old female infant with no particular fetal or maternal history presented limb weakness with flexed knees and flanges in the popliteal fossae, sphincter atony, and a sensation of emptiness on palpation in the lumbosacral region. A 3D CT scan of the spine showed the absence of the lumbar spine and disconnection of the upper segment of the thoracic spine from the hypoplastic sacrum. We noted also the absence of the sacroiliac joints bilaterally and an unusual trigonal shape of the iliac bones. MRI and sonographic examination are required in the investigation of the disease. The management is multidisciplinary and depends upon the degree of the defect. Spine reconstruction has proven to be a valuable management technique but has many complications. We wanted to draw the medical world's attention to the existence of this extremely rare malformation in the east of the Democratic Republic of Congo, a mining area. (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.) |
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| فهرسة مساهمة: | Keywords: Birth defects; Caudal regression syndrome; Democratic Republic of Congo; Lumbar agenesis; Mining areas |
| تواريخ الأحداث: | Date Created: 20230609 Date Completed: 20230817 Latest Revision: 20230817 |
| رمز التحديث: | 20231215 |
| DOI: | 10.1007/s00381-023-05973-6 |
| PMID: | 37294350 |
| قاعدة البيانات: | MEDLINE |
Find this article in full text from Springer Verlag. 
Full Text Finder | تدمد: | 1433-0350 |
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| DOI: | 10.1007/s00381-023-05973-6 |