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DOI: 10.4103/0970-0358.41120
Progressive skin necrosis of a huge occipital encephalocele
Publication History
Publication Date:
15 January 2020 (online)
ABSTRACT
Objects: Progressive skin necrosis of giant occipital encephalocoele is an extremely rare complication found in neonates. infection and ulceration of the necrosed skin may lead to meningitis or sepsis. we present here a neonate with giant occipital encephalocoele showing progressive necrosis during the first day of his life. Methods: a newborn baby was found to have a huge mass in the occipital region, which was covered by normal pink-purplish skin. during the last hours of the first day of his life, the sac started becoming ulcerated accompanied with a rapid color change in the skin, gradually turning darker and then black. the neonate was taken up for urgent excision and repair of the encephalocele. two years after the operation, he appears to be well-developed without any neurological problems.Conclusion: necrosis may have resulted from arterial or venous compromise caused by torsion of the pedicle during delivery or after birth. the high pressure inside the sac associated with the thin skin of the encephalocoele may be another predisposing factor. in view of the risk of ulceration and subsequent infection, urgent surgery of the necrotizing encephalocele is suggested.
- 1 Lorber J, Schofield JK. The prognosis of occipital encephalocele. Z Kinderchir Grenzgeb 1979; 28: 347-51
- 2 Walia B, Bhargava P, Sandhu K. Giant occipital encephalocele. MJAFI 2005; 61: 293-4
- 3 Chapman PH, Swearingen B, Caviness VS. Sub torcular occipital encephaloceles: Anatomical consideration relevant to operative management. J Neurosurg 1989; 71: 375-81
- 4 Simpson DA, David DJ, White J. Cephaloceles: Treatment, outcome and antenatal diagnosis. Neurosurgery 1984; 15: 14-21
- 5 Chatterjee MS, Bondoc B, Adhate A. Prenatal diagnosis of occipital encephalocele. Am J Obstet Gynecol 1985; 153: 646-7
- 6 Mayer U, Klinger M, Rott HD. A rare form of optical, choroidal and retinal dysplasia combined with an occipital encephalocele. Graefes Arch Clin Exp Ophthalmol 1982; 219: 72-5
- 7 Sertie AL, Quimby M, Moreira ES, Murray J, Zatz M, Antonarakis SE. A gene which causes severe ocular alterations and occipital encephalocele. (Knobloch syndrome) is mapped to 21q22.3. Hum Mol Genet 1996; 5: 843-7
- 8 Lin HJ, Cornford ME, Hu B, Rutgers JK, Beall MH, Lachman RS. Occipital encephalocele and MURCS association: Case report and review of central nervous system anomalies in MURCS patients. Am J Med Genet 1996; 61: 59-62
- 9 Morioka T, Nishio S, Muraishi M, Hisada K, Mihara F, Nakayama H. Occipital encephalocele and epilepsy. No To Shinkei 2000; 52: 511-6
- 10 Martinez-Lage JF, Poza M, Ramos J, Almagro MJ, Sempere M, Mestre J. Occipital encephalocele associated with a dermoid cyst. J Child Neurol 1992; 7: 427-30
- 11 Chowdhary UM, Ibrahim AW, Ammar AS, Dawodu AH. Tecto-cerebellar dysraphia with occipital encephalocele. Surg Neurol 1989; 31: 310-4
- 12 Sinha A, Ojha S, Mahajan JK. Gangrene of an occipital encephalocele. Indian J Pediatr 2005; 72: 451
- 13 Caviness Jr VS, Evarard P. Occipital encephalocele: A pathologic and anatomic analysis. Acta Neuropathol 1975; 32: 245-55
- 14 Hoving E, Blaser S, Kelly E, Rutka JT. Anatomical and embryological considerations in the repair of a large vertex cephalocele. J Neurosurg 1999; 90: 537-41
- 15 Bozinov O, Tirakotai W, Sure U, Bertalanffy H. Surgical closure and reconstruction of a large occipital encephalocele without parenchymal excision. Childs Nerv Syst 2005; 21: 144-7