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OCCIPITAL ENCEPHALOCELE IN A FETUS
AUTHOR: DR. ASHUTOSH PATEL , 1st Year Resident. CO-AUTHORS: DR. NIRMALA CHUDASAMA, DR. HARSHAD SHAH, DR. RASESH VYAS. INSTITUTE: C. U. SHAH MEDICAL COLLEGE AND HOSPITAL, SURENDRANAGAR, GUJARAT
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Clinical History: • A 26-year-old woman, primigravida, presented to our department with five months of amenorrhea for a routine antenatal check up without any previous ultrasonographic examinations. • No complaints of bleeding p/v or pain in abdomen.
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y FIGURE 1 defect in the Occipital region as seen on ultrasonography.
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FIGURE 2. Sagittal section, showing bony defect in occipital region, with herniation of intracranial contents.
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FIGURE 3: Coronal Section shows herniation of intracranial contents.
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FIGURE 4. Single largest pocket measuring 11.4 cm, with
AFI 25 suggestive of Polyhydramnios.
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On MRI : There is evidence of defect(shown by pink arrow) in the occipital bone, through which herniation of brain tissue (arrow head) and meninges can be seen.
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Discussion: • Ultrasonographic scan shows, evidence of single viable fetus with anechoic cystic lesion herniating through a defect in the occipital region. AFI was 25 suggesting polyhydramnios. • Spine appeared normal, no evidence of any spinal anomaly seen. • Rest of the scan was normal. • Kidneys & limbs were normal, which ruled out the possibility of a commonly linked syndrome i.E. Meckel- gruber syndrome (encephalocele, polycystic kidneys, polydactyly). MRI scan shows defect in the occipital bone through which there is herniation of brain tissue and meninges .
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Diagnosis: OCCIPITAL ENCEPHALOCELE
• Encephalocele is characterized by a protrusion of the brain and/or meninges through a defect in the skull that is covered with skin. There is herniation of cerebral tissue and meninges. Approximately 75% are occipital, 13% frontal and 12% occur in parietal region . Rare sites are protrusions through base of skull, orbits, nose or mouth. • It is one of the most severe neural tube defects with a prevalence estimated to be 0.8 to 5/10 000 births. • Occipital encephaloceles have been diagnosed on ultrasound from about 9 weeks. After cranial ossification at 10 weeks, the skull defect and occipital sac may be demonstrated. In 30% cases there is associated spina bifida.
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References: 1.Sandler MA, Beute GH, Madrazo BL, Hudak SF, Walter R, Haggar AH, Maywood CM. Ultrasound case of the day. Occipital meningoencephalocele. Radiographics 1986; Nov.6(6): 2. Ants Toi, The fetal head and brain, Rumack : Diagnostic ultrasound 3rd edition, 2005, Volume 2, ,37:1247. 3. Shaff MI, Blumenthal B, Coetzee M: Meningoencephalocoele: prepartum ultrasonic and fetoamniographic findings. Br.J Radiol 1977 october 50:
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