Limb-body wall complex in the San Ignacio Teaching Hospital at Bogota, Colombia: a case report and literature review
DOI:
https://doi.org/10.18597/rcog.162Keywords:
umbilical cord, abdominal wall anomaly, prenatal ultrasonography, genetic consultationAbstract
Introduction: umbilical cord alterations are associated with several short and long-term complications during pregnancy, some involving little chance of survival related to multiple foetal lesions. One of those anomalies is the Limb-Body Wall Complex (LBWC) which consists of lateral body wall defects and anomalies due to shortening of the limbs. Its still discussed aetiology, poor prognosis and possible recurrence make it important in ecographic diagnosis, neonatal attention and the approach during autopsy.
Objectives: reporting a case of LBWC and reviewing the literature published about its aetiology, clinical characteristics and diagnosis within the context of the complications associated with umbilical cord alterations.
Materials and methods: a clinical case of a female newborn resulting from an adolescent mother’s first pregnancy who consulted a reference and high complexity entity is presented. The newborn died a few minutes after being born, presenting a defect of the abdominal wall, scoliosis and upper left limb stump associated with a 6 cm umbilical cord. The literature published in Medline data bases was reviewed using PubMed, Ovid and Hinari.
Conclusion: LBWC is an expression of an excessively short cord and is associated with important morbidity-mortality. Thus an early diagnosis must be made and suitable genetic advice given to families.
Author Biographies
Ana Milena Gómez
Mercedes Olaya
References
Godfrey KM, Barker DJ. Fetal programming and adult health. Public Health Nutr 2001;4:611-24.
Nilsson PM, Holmäng A. Developmental origins of adult disease: an introduction. J Intern Med 2007;261:410-1.
Lahti J, Raikkonen K, Heinonen K, Pesonen AK, Kajantie E, Forsén T, et al. Body size at birth and socio-economic status in childhood: implications for Cloninger' psychobiological model of temperament at age 60. Psychiatry Res 2008;160:167-74.
Sankaran S, Kyle PM. Aetiology and pathogenesis of IUGR. Best Pract Res Clin Obstet Gynaecol 2009;23:765-77.
Moessinger AC, Blanc WA, Marone PA, Polsen DC. Umbilical cord length as an index of fetal activity: experimental study and clinical implications. Pediatr Res 1982;16:109-12.
Bolisetty S, Koh TH, Hammond S, Panaretto K, Whitehall J. Correlation of umbilical cord weight with birth weight. Arch Dis Child Fetal Neonatal Ed 2002;86:F140.
Stefos T, Sotiriadis A, Vasilios D, Tsirkas P, Korkontzelos I, Avgoustatos F, et al. Umbilical cord length and parity--the Greek experience. Eur J Obstet Gynecol Reprod Biol 2003;107:41-4.
Scher MS. Fetal and neonatal neurologic consultations: identifying brain disorders in the context of fetal-maternal-placental disease. Semin Pediatr Neurol 2001;8:55-73.
Caksen H, Atas B, Tuncer O, Odabas D, Dal H. A case of limb-body wall complex diagnosed in utero. J Emerg Med 2003;25:104-6.
Sahinoglu Z, Uludogan M, Arik H, Aydin A, Kucukbas M, Bilgic R, et al. Prenatal ultrasonographical features of limb body wall complex: a review of etiopathogenesis and a new classification. Fetal Pediatr Pathol 2007;26:135-51.
Plakkal N, John J, Jacob SE, Chithira J, Sampath S. Limb body wall complex in a still born fetus: a case report. Cases J 2008;1:86.
Tanteles GA, Suri M. Classification and aetiology of birth defects. Paediatrics and Child Health 2007;17:233-43.
Opitz JM. The developmental field concept in clinical genetics. J Pediatr 1982;101:805-9.
Van Allen MI, Curry C, Gallagher L. Limb body wall complex: I. Pathogenesis. Am J Med Genet 1987;28:529-48.
Pumberger W, Schaller A, Bernaschek G. Limbbody wall complex: a compound anomaly pattern in body-wall defects. Pediatr Surg Int 2001;17:486-90.
Aspelund G, Langer JC. Abdominal wall defects. Current Paediatrics 2006;16:192-8.
Bawa M, Samalad VM, Kanojia RP, Rao KL. Partial umbilical exstrophy with cecal patch and intact hindgut entering a cloaca: a new variant. J Pediatr Surg 2011;46:244-6.
Chen CP. Syndromes and disorders associated with omphalocele (II): OEIS complex and Pentalogy of Cantrell. Taiwan J Obstet Gynecol 2007;46:103-10.
Dillon E, Renwick M. The antenatal diagnosis and management of abdominal wall defects: the northern region experience. Clin Radiol 1995;50:855-9.
Kalousek DK. Embryonic and fetal wastage. En: Stocker JT, Dehner LP, editors. Pediatric Pathology. 2nd. ed. Philadelphia: Lippincott Williams and Wilkins; 2001. p. 55-80.
Managoli S, Chaturvedi P, Vilhekar Ky, Gagane N. Limb body wall complex. Indian Pediatr 2003;40:891-4.
Arici DS, Perçin F, Ozer H, Aslan M, Cetin A. Limb body wall defect associated with rare cardiac anomalies. Pediatr Int 2004;46:85-7.
Negishi H, yaegashi M, Kato EH, yamada H, Okuyama K, Fujimoto S. Prenatal diagnosis of limb-body wall complex. J Reprod Med 1998;43:659-64.
Patten RM, Van Allen M, Mack LA, Wilson D, Nyberg D, Hirsch J, et al. Limb-body wall complex: in utero sonographic diagnosis of a complicated fetal malformation. AJR Am J Roentgenol 1986;146:1019-24.
Frias J, Gilbert-Barness E. Disruptions. En: Gilbert-Barness E, editor. Potter's Pathology of the Fetus, Infant and Child. Second ed. Philadelphia: Elsevier; 2007.
Gordillo G, Olaya M, Zarante I. Bandas amnióticas y el proceso disruptivo-mecánico producido durante el periodo fetal. Latin American Journal of Dysmorphology 2008;1:23-5.
Halder A. Amniotic band syndrome and/or limb body wall complex: split or lump. The Application of Clinical Genetics 2010;3:7-15.
Brantberg A. Fetal and perinatal implications of anomalies in the gastrointestinal tract and the abdominal wall. Thesis for the degree of doctor medicinae. Trondheim, Norway: Norwegian University of Science and Technology; 2007.
Jones KL. Smith's recognizable patterns of human malformation. 6th ed. Philadelphia: Elsevier; 2006.
Prasun P, Behera BK, Pradhan M. Limb body wall complex. Indian J Pathol Microbiol 2008;51:255-6.
Russo R, D'Armiento M, Angrisani P, Vecchione R. Limb body wall complex: a critical review and a nosological proposal. Am J Med Genet 1993;47:893-900.
Brewer S, Williams T. Loss of AP-2alpha impacts multiple aspects of ventral body wall development and closure. Dev Biol 2004;267:399-417.
Levy R, Lacombe D, Rougier Y, Camus E. Limb body wall complex and amniotic band sequence in sibs. Am J Med Genet A 2007;143A:2682-7.
Luehr B, Lipsett J, Quinlivan JA. Limb-body wall complex: a case series. J Matern Fetal Neonatal Med 2002;12:132-7.
Viscarello RR, Ferguson DD, Nores J, Hobbins JC. Limb-body wall complex associated with cocaine abuse: further evidence of cocaine’s teratogenicity. Obstet Gynecol 1992;80:523-6.
Chen CP, Tsai FJ, Chen CY, Lin HH, Wang W. Limb-body wall complex with craniofacial defects after ovarian stimulation. Taiwan J Obstet Gynecol 2008;47:474-5.
Chen CP, Lee MS, Tsai FJ, Huang MC, Chern SR, Wang W. Limb-body wall complex in one fetus of a dizygotic twin pregnancy conceived by egg donation, in vitro fertilization and embryo transfer: prenatal diagnosis and literature review. Taiwan J Obstet Gynecol 2009;48:446-50.
Duddaiwar VA, Geeta V. Limb body wall complex: antenatal sonographic diagnosis. Eur Ultrasound 1997;5:3.
Kamata S, Ishikawa S, Usui N, Kitayama Y, Sawai T, Okuyama H, et al. Prenatal diagnosis of abdominal wall defects and their prognosis. J Pediatr Surg 1996;31:267-71.
Roberts N, Bhide A. Ultrasound prenatal diagnosis of structural abnormalities. Obstet Gynaecol Reprod Med 2007;19:1-8.
Fried AM, Woodring JH, Shier RW, Falace PB. Omphalocele in limb/body wall deficiency syndrome: atypical sonographic appearance. J Clin Ultrasound 1982;10:400-2.
Emanuel PG, Garcia GI, Angtuaco TL. Prenatal detection of anterior abdominal wall defects with US. Radiographics 1995;15:517-30.
Sohaey R, Woodward P, Zwiebel WJ. Fetal gastrointestinal anomalies. Semin Ultrasound CT MR 1996;17:51-65.
Servaes S, Victoria T, Lovrenski J, Epelman M. Contemporary pediatric gynecologic imaging. Semin Ultrasound CT MR 2010;31:116-40.
Chen CP. Prenatal sonographic diagnosis of limb-body wall complex with craniofacial defects. Ultrasound Obstet Gynecol 2003;22:101.
Colpaert C, Bogers J, Hertveldt K, Loquet P, Dumon J, Willems P. Limb-body wall complex: 4 new cases illustrating the importance of examining placenta and umbilical cord. Pathol Res Pract 2000;196:783-90.
How to Cite
Downloads
Downloads
Published
Issue
Section
License
Copyright (c) 2011 Revista Colombiana de Obstetricia y Ginecología
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
| Article metrics | |
|---|---|
| Abstract views | |
| Galley vies | |
| PDF Views | |
| HTML views | |
| Other views | |
