We report a case of non-identical twins who presented with identical neonatal intestinal obstruction with features of anorectal stenosis, presacral mass and sacral anomaly consistent with Currarino's syndrome or triad. Plain sacral radiograph, contrast enema and MRI were diagnostic. Initial management involved a defunctioning colostomy followed by a posterior sagittal anorectoplasty with excision of the teratoma ± anterior sacral meningocele and finally closure of colostomy in a staged multidisciplinary approach. The twins' father is also affected with features of Currarino's syndrome but was diagnosed during family screening. Currarino's syndrome presenting with identical neonatal low intestinal obstruction in a non-identical set of twins is rare and interesting. Antenatal diagnosis of Currarino's syndrome is difficult and may prove to be a challenge even in the postnatal period. Sacral spine radiograph, contrast enema and MRI are diagnostic. Management requires high index of suspicion, low threshold for MRI and multidisciplinary staged approach.
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http://dx.doi.org/10.1136/bcr-2014-204276 | DOI Listing |
JTCVS Tech
December 2024
Department of Cardiovascular and Thoracic Surgery, Mayo Clinic, Phoenix, Ariz.
Objective: Pectus arcuatum is a rare variant of pectus deformities that can cause varying degrees of cardiac compression. A review of the evaluation, surgical repair, and outcomes of pectus arcuatum is presented.
Methods: A retrospective review of all patients undergoing surgical treatment of pectus arcuatum at a single institution was conducted between January 1, 2010, and May 31, 2024.
J Cancer Res Ther
April 2024
Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
Currarino syndrome (CS) is a rare congenital syndrome characterized by a triad of anorectal malformation, sacral deformity, and presacral mass. In about 50% of cases, it is caused by HLXB9 gene mutation in chromosome 7q36. A 13-month-male child presented with presacral discharging sinus with a history of surgery for anorectal malformation and perineal fistula at the time of birth.
View Article and Find Full Text PDFCancers (Basel)
June 2024
Department of Pediatric Hematology and Oncology, University Hospital Bonn, 53127 Bonn, Germany.
GCTs are developmental tumors and are likely to reflect ontogenetic and teratogenetic determinants. The objective of this study was to identify syndromes with or without congenital anomalies and non-syndromic defects as potential risk factors. Patients with extracranial GCTs (eGCTs) registered in MAKEI 96/MAHO 98 between 1996 and 2017 were included.
View Article and Find Full Text PDFPediatr Surg Int
May 2024
Department of General Surgery, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, No.56 Nanlishi st, Xicheng District, Beijing, 100045, China.
Purpose: To describe the long-term bowel function of anorectal malformation (ARM) patients and explore the potential influence factors.
Methods: ARM patients with follow-up data > 10 years were included. Cases of cloaca, Currarino syndrome, and VACTERL syndrome were excluded.
J Pediatr Surg
August 2024
Department of Surgery, UC Davis Children's Hospital, University of California Davis, Sacramento, CA, USA; Department of Surgery, Shriners Hospital for Children-Northern California, Sacramento, CA, USA. Electronic address:
Purpose: Congenital anorectal stenosis is managed by dilations or operative repair. Recent studies now propose use of dilations as the primary treatment modality to potentially defer or eliminate the need for surgical repair. We aim to characterize the management and outcomes of these patients via a multi-institutional review using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry.
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