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Thoracoabdominal ectopia cordis

MedGen UID:
868872
•Concept ID:
C4023283
•
Congenital Abnormality
HPO: HP:0011586

Definition

Congenital malformation of the ventral wall with partial or total evisceration of the heart outside the thoracic cavity and displacement partially into the abdominal cavity. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThoracoabdominal ectopia cordis

Professional guidelines

PubMed

Ectopia cordis: prenatal diagnosis, perinatal outcomes, and postnatal follow-up of an international multicenter cohort case series.
Araujo Júnior E, Coutinho LG, Bravo-Valenzuela NJ, Aquino P, Rocha LAD, Rizzo G, Tonni G, Respondek-Liberska M, Slodki M, Wolter A, Axt-Fliedner R
J Matern Fetal Neonatal Med 2023 Dec;36(1):2203791. doi: 10.1080/14767058.2023.2203791. PMID: 37080921

Recent clinical studies

Etiology

Chest wall reconstruction in thoracoabdominal ectopia cordis: using the pedicled osteomuscular latissimus dorsi composite flap.
Lampert JA, Harmaty M, Thompson EC, Sett S, Koch RM
Ann Plast Surg 2010 Nov;65(5):485-9. doi: 10.1097/SAP.0b013e3181d376a3. PMID: 20948422
Successful staged-Fontan operation in a patient with ectopia cordis.
Tokunaga S, Kado H, Imoto Y, Shiokawa Y, Yasui H
Ann Thorac Surg 2001 Feb;71(2):715-7. doi: 10.1016/s0003-4975(00)02224-4. PMID: 11235741
Ectopia cordis and other midline defects.
Morales JM, Patel SG, Duff JA, Villareal RL, Simpson JW
Ann Thorac Surg 2000 Jul;70(1):111-4. doi: 10.1016/s0003-4975(00)01388-6. PMID: 10921692
Immediate operation for ectopia cordis.
Jones AF, McGrath RL, Edwards SM, Lilly JR
Ann Thorac Surg 1979 Nov;28(5):484-6. doi: 10.1016/s0003-4975(10)63158-x. PMID: 159022

Diagnosis

Chest wall reconstruction in thoracoabdominal ectopia cordis: using the pedicled osteomuscular latissimus dorsi composite flap.
Lampert JA, Harmaty M, Thompson EC, Sett S, Koch RM
Ann Plast Surg 2010 Nov;65(5):485-9. doi: 10.1097/SAP.0b013e3181d376a3. PMID: 20948422
Prenatal diagnosis of ectopia cordis at 10 weeks of gestation using two-dimensional and three-dimensional ultrasonography.
Liang RI, Huang SE, Chang FM
Ultrasound Obstet Gynecol 1997 Aug;10(2):137-9. doi: 10.1046/j.1469-0705.1997.10020137.x. PMID: 9286025
Trisomy 18 with ectopia cordis, omphalocele, and ventricular septal defect: case report.
Soper SP, Roe LR, Hoyme HE, Clemmons JJ
Pediatr Pathol 1986;5(3-4):481-3. doi: 10.3109/15513818609068872. PMID: 3537999
Thoracoabdominal ectopia cordis with mosaic Turner's syndrome: Report of a case.
Garson A Jr, Hawkins EP, Mullins CE, Edwards SB, Sabiston DC Jr, Cooley DA
Pediatrics 1978 Aug;62(2):218-21. PMID: 151256

Prognosis

Thoracoabdominal ectopia cordis with good outcome.
Acco FS, Bortoli AF, Capaverde VB, Zen PRG, Rosa RFM
Pediatr Int 2019 Nov;61(11):1179-1180. Epub 2019 Nov 13 doi: 10.1111/ped.13999. PMID: 31724283
Chest wall reconstruction in thoracoabdominal ectopia cordis: using the pedicled osteomuscular latissimus dorsi composite flap.
Lampert JA, Harmaty M, Thompson EC, Sett S, Koch RM
Ann Plast Surg 2010 Nov;65(5):485-9. doi: 10.1097/SAP.0b013e3181d376a3. PMID: 20948422
Images in cardiovascular medicine: Pentalogy of Cantrell associated with thoracoabdominal ectopia cordis.
Gao Z, Duan QJ, Zhang ZW, Ying LY, Ma LL
Circulation 2009 Apr 21;119(15):e483-5. doi: 10.1161/CIRCULATIONAHA.108.811000. PMID: 19380628
Ectopia cordis and other midline defects.
Morales JM, Patel SG, Duff JA, Villareal RL, Simpson JW
Ann Thorac Surg 2000 Jul;70(1):111-4. doi: 10.1016/s0003-4975(00)01388-6. PMID: 10921692
Repair of thoracoabdominal ectopia cordis with myocutaneous flaps and intraoperative tissue expansion.
Hochberg J, Ardenghy MF, Gustafson RA, Murray GF
Plast Reconstr Surg 1995 Jan;95(1):148-51. doi: 10.1097/00006534-199501000-00026. PMID: 7809230

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