Abstract
Introduction
Subcutaneous tumors with extra limbs are very rare, and they are considered either as fetus in fetu or fetiform teratoma.
Case report
We report here the case of a 6-day-old presenting a mass extending at the level of the occipital bone. This mass is developed in the extracranial region and contains two forelimbs including hands with digits. CT shows that the squamous part of the occipital bone is involved with several defects through which a part of the cerebellum herniates. The boy was operated on and the tumor was removed. The herniated region of the cerebellum has also been removed. After surgery, the boy develops normally.
Conclusion
This type of tumor is extremely rare and is only the second case that has been reported at this exact location. This could be the so-called céphalomélie described by Isidore Geoffroy Saint-Hilaire in a duck in his famous Treatise of Teratology (1836). The cause of this malformation is still a matter for debate.
Similar content being viewed by others
References
Chen C-P, Chern S-R, Liu F-F, Jan S-W, Lee H-C, Sheu J-C, Lee T, Wang T-Y (1997) Prenatal diagnosis, pathology, and genetic study of fetus in fetu. Prenat Diagn 17:13–21
Gangopadhyay AN, Srivastava A, Srivastava P, Gupta DK, Sharma SP, Kumar V (2010) Twin fetus-in-fetu in a child: a case report and review of the literature. J Med Case Rep 4:96. doi:10.1186/1752-1947-4-96
Geoffroy Saint-Hilaire I (1836) Traité de tératologie. Tome Troisième. J-B Baillière, Paris, pp. 262–290
Geoffroy Saint-Hilaire I (1837) Traité de tératologie. Atlas. Table générale des matières. J-B Baillière, Paris
Hing A, Corteville J, Foglia RP, Bliss DP, Donis-Keller H, Dowton SB (1993) Fetus in fetu: molecular analysis of a fetiform mass. Am J Med Genet 47:333–341
Ji Y, Chen S, Zhong L, Jiang K, Jin S, Kong F, Wang Q, Li C, Xiang B (2014) Fetus in fetu: two case reports and literature review. BMC Pediatr 14:88 doi:1186/1471-2431-14-88
Krishna A, Chandna S, Mishra NK, Gupta AK, Upadhyaya P (1989) Accessory limb associated with spina bifida. J Pediatr Surg 24:604–606
Kuno N, Kadomatsu K, Nakamura M, Miwa-Fukuchi T, Hirabayashi N, Ishizuka T (2004) Mature ovarian cystic teratoma with a highly differentiated homunculus: a case report. Birth Defects Res (Part A) 70:40–46
Mohta A, Khurana N (2011) Fetus-in-fetu or well-differentiated teratoma—a continued controversy. Indian J Surg 73:372–374
Montalvo N, Redrobán L, Espín VH (2014) Incomplete duplication of a lower extremity (polymelia) a case report. J Med Case Rep 8:184. doi:10.1186/1752-1947-8-184
Pessel C, Fratto V, Laifer-Narin S, Simpson LL, Nhan-Chang C-L (2014) Going out on a limb for a difficult diagnosis: a case report. Clin Imaging 38:63–66
Ratan SK, Rattan KN, Magu S, Rohilla S, Purwar P, Mathur SK (2004) Thoracolumbar rachipagus parasite. Pediatr Surg Int 20:298–300
Sanoussi S, Rachid S, Sani CM, Mahamane B, Addo G (2010) Rachipagus: a report of two cases—thoracic and lumbar. J Surg Tech Case Rep 2:27–29
Sood N, Kamboj M, Chaabra M (2013) Sacrococcygeal fetiform teratoma Altman type 1 : a rare case report in a 11 year old girl. Indian J Surg 75(Suppl 1):S359–S361
Tahilyani NDA, Singh KM (1976) Occipital teratoma bearing digits and nails. Br J Surg 63:493
Verma S, Khanna M, Tripathi VN, Yadav NC (2013) Occurrence of polymyelia in a female child. J Clin Imaging Sci 3:18. doi:10.4103/2156-7514.111235 Print 2013
Weiss JR, Burgess JR, Kaplan KJ (2006) Fetiform teratoma (Homunculus). Arch Pathol Lab Med 130:1552–1556
Conflict of interest
The authors report no conflict of interest in relation to this publication.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kelani, A.B., Moumouni, H., Younsa, H. et al. A case of cephalomelia discovered in a baby born in Niger. Childs Nerv Syst 32, 205–208 (2016). https://doi.org/10.1007/s00381-015-2831-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-015-2831-2