Acta Neurochirurgica

, Volume 149, Issue 10, pp 1071–1075

A girl with spina bifida, an extra leg, and ectopic intestinal loops – a “foetus in foetu” or a whim of the neural crest?

  • G. Lende
  • W. Wendemu
  • S. Mørk
  • K. Wester
Case Report

Summary

This article describes a girl with an extra leg attached to her lower back, combined with a spina bifida and a myelomeningocele. Despite lacking sensory or motor functions, the leg grew proportionately with the rest of the body. The bony structures were almost normal. A cross section showed fat tissue with some centrally situated blood vessels, nerve bundles, and muscular fragments. Proximally, an isolated colon loop was found. The extra leg and intestine respected the dorsal fascia, without connection with the peritoneal or retroperitoneal compartments. The finding is discussed with reference to existing hypotheses for limb formation.

Keywords: Foetus in foetu; maldevelopment; neural crest; parasitic twin; teratoma. 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Agarwal, P, Wylie, JN, Galceran, J, Arkhitko, O, Li, C, Deng, C, Grosschedl, R, Bruneau, BG 2003Tbx5 is essential for forelimb bud initiation following patterning of the limb field in the mouse embryoDevelopment130623633PubMedCrossRefGoogle Scholar
  2. al Baghdadi, R 1992Fetus in fetu in the liver: case report and review of the literatureJ Pediatr Surg2714911492PubMedCrossRefGoogle Scholar
  3. Banks, J 1789An account of a monster of the human species, in two letters; one from Baron Reichel to Sir Joseph Banks, Bart. and the other from Mr. Andersson to Baron Reichel. Communicated by Sir Joseph Bank, BartP. R. S. Transactions of the Royal Society of London79157160CrossRefGoogle Scholar
  4. Beaudoin, S, Gouizi, G, Mezzine, S, Wann, AR, Barbet, P 2004Mediastinal fetus in fetu. Case report and embryological discussionFetal Diagn Ther19453455PubMedCrossRefGoogle Scholar
  5. Boulet, AM, Moon, AM, Arenkiel, BR, Capecchi, MR 2004The roles of Fgf4 and Fgf8 in limb bud initiation and outgrowthDev Biol273361372PubMedCrossRefGoogle Scholar
  6. Brand, A, Alves, MC, Saraiva, C, Loio, P, Goulao, J, Malta, J, Palminha, JM, Martins, M 2004Fetus in fetu – diagnostic criteria and differential diagnosis – a case report and literature reviewJ Pediatr Surg39616618PubMedCrossRefGoogle Scholar
  7. Chua, JHY, Chui, CH, Sai Prasad, TR, Jacobsen, AS, Meenakshi, A, Hwang, WS 2005Fetus-in-fetu in the pelvis: report of a case and literature reviewAnn Acad Med Singapore34646649PubMedGoogle Scholar
  8. Crossley, PH, Minowada, G, MacArthur, CA, Martin, GR 1996Roles for FGF8 in the induction, initiation, and maintenance of chick limb developmentCell84127136PubMedCrossRefGoogle Scholar
  9. Delgado Luengo, WN, Hernandez, L, Rodriguez, M, Valbuena Pirela, I, Gonzalez Ferrer, S, Estrada Corona, P, Chacon Fonseca, I, Delgado Luengo, J, Morales-Machin, A, Borjas Fuentes, L, Caridad Martinez Basalo, M, Chacin, J 2004Human disorganization complex, as a polytopic blastogenesis defect: a new caseAm J Med Genet A125181185PubMedCrossRefGoogle Scholar
  10. Donnai, D, Winter, RM 1989Disorganisation: a model for ‘early amnion rupture’?J Med Genet26421425PubMedCrossRefGoogle Scholar
  11. Faris, JC, Crowe, JE 1975The split notochord syndromeJ Pediatr Surg10467472PubMedCrossRefGoogle Scholar
  12. Federici, S, Prestipino, M, Domenichelli, V, Antonellini, C, Sciutti, R, Domini, R 2001Fetus in fetu: report of an additional, well-developed casePediatr Surg Int17483485PubMedCrossRefGoogle Scholar
  13. Ferrari, D, Harrington, A, Dealy, CN, Kosher, RA 1999Dlx-5 in limb initiation in the chick embryoDev Dyn2161015PubMedCrossRefGoogle Scholar
  14. Fowler, CL 1998Intraabdominal leg: unique variant of split notochord syndromeJ Pediatr Surg33522524PubMedCrossRefGoogle Scholar
  15. Grant, P, Pearn, JH 1969Fetus-in-fetuMed J Aust110161019PubMedGoogle Scholar
  16. Hoeffel, CC, Nguyen, KQ, Phan, HT, Truong, NH, Nguyen, TS, Tran, TT, Fornes, P 2000Fetus in fetu: a case report and literature reviewPediatrics10513351344PubMedCrossRefGoogle Scholar
  17. Hoffman, CH, Dietrich, RB, Pais, MJ, Demos, DS, Pribram, HF 1993The split notochord syndrome with dorsal enteric fistulaAJNR Am J Neuroradiol14622627PubMedGoogle Scholar
  18. Husain, AN, Muraskas, J, Lambert, G, Dado, D, Lynch, J 1989Parasitic conjoined twins with omphalocele and tetralogy of FallotPediatr Pathol9321328PubMedGoogle Scholar
  19. Keen, WW, Coplin, WM 1906Sacrococcygeal tumor (teratoma) with an opening through the sacrum, and a sinus passing through this opening and communicating with the rectum, the sinus resembling a bronchusSurg Obstet Gynecol3661671Google Scholar
  20. Kim, OH, Shinn, KS 1993Postnatal growth of fetus-in-fetuPediatr Radiol23411412PubMedCrossRefGoogle Scholar
  21. Kumar, AN, Chandak, GR, Rajasekhar, A, Reddy, NC, Singh, L 1999Fetus-in-fetu: a case report with molecular analysisJ Pediatr Surg34641644PubMedCrossRefGoogle Scholar
  22. Lappin, TR, Grier, DG, Thompson, A, Halliday, HL 2006HOX genes: seductive science, mysterious mechanismsUlster Med J752331PubMedGoogle Scholar
  23. Larsen, WJ 1997Human embryology, 2nd edn. Developments of the limbsChurchillLivingstone311344Google Scholar
  24. Magnus, KG, Millar, AJ, Sinclair-Smith, CC, Rode, H 1999Intrahepatic fetus-in-fetu: a case report and review of the literatureJ Pediatr Surg3418611864PubMedCrossRefGoogle Scholar
  25. Matsuoka, T, Ahlberg, PE, Kessaris, N, Iannarelli, P, Dennehy, U, Richardson, WD, McMahon, AP, Koentges, G 2005Neural crest origins of the neck and shoulderNature436347355PubMedCrossRefGoogle Scholar
  26. Meckel JF (1815) De duplicitate monstrosa commentarius. Halle Berlin E Librariis Orphanotrophei: 1–98Google Scholar
  27. Moorthy, K, Rao, PP, Deshpande, AA, Thakur, RK, Supe, AN 1997Fetus in fetu or a retroperitoneal teratoma – a controversy revisited. A case report and review of literatureIndian J Cancer34179181PubMedGoogle Scholar
  28. Sekine, K, Ohuchi, H, Fujiwara, M, Yamasaki, M, Yoshizawa, T, Sato, T, Yagishita, N, Matsui, D, Koga, Y, Itoh, N, Kato, S 1999Fgf10 is essential for limb and lung formationNat Genet21138141PubMedCrossRefGoogle Scholar
  29. Stephens, TD, Siebert, JR, Graham, JM,Jr, Beckwith, JB 1982Parasitic conjoined twins, two cases, and their relation to limb morphogenesisTeratology26115121PubMedCrossRefGoogle Scholar
  30. Stevenson RE (2006) Limbs. In: Stevenson RE, Hall JG (eds.) Human Malformations and Related Anomalies, pp 835–933Google Scholar
  31. Takeuchi, JK, Koshiba-Takeuchi, K, Suzuki, T, Kamimura, M, Ogura, K, Ogura, T 2003Tbx5 and Tbx4 trigger limb initiation through activation of the Wnt/Fgf signaling cascadeDevelopment13027292739PubMedCrossRefGoogle Scholar
  32. Thakral, CL, Maji, DC, Sajwani, MJ 1998Fetus-in-fetu: a case report and review of the literatureJ Pediatr Surg3314321434PubMedCrossRefGoogle Scholar
  33. Willis, RA 1935The structure of teratomataJ Pathology Bacteriology40136CrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • G. Lende
    • 1
  • W. Wendemu
    • 2
  • S. Mørk
    • 3
  • K. Wester
    • 4
    • 5
  1. 1.Myungsung Christian Medical CentreAddis AbabaEthiopia
  2. 2.CURE INTERNATIONAL and Myungsung Christian Medical CentreAddis AbabaEthiopia
  3. 3.Department of PathologyThe Gade Institute, Haukeland University HospitalBergenNorway
  4. 4.Section for Neurosurgery, Institute of Surgical SciencesUniversity of BergenBergenNorway
  5. 5.Department of NeurosurgeryHaukeland University HospitalBergenNorway

Personalised recommendations