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Supratentorial Multiloculated Arachnoid Cystic Malformation in an Infant
Pediatric Neurology 48 (2013) 481e482
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Images in Child Neurology
Prenatal Diagnosis and Management of a Huge Infratentorial/Supratentorial Multiloculated Arachnoid Cystic Malformation in an Infant Doniel Drazin MD a, *, J. Gordon McComb MD b a b
Department of Neurological Surgery, Cedars-Sinai Medical Center, Los Angeles, California Division of Neurosurgery, Children’s Hospital of Los Angeles, Department of Neurological Surgery, USC Keck School of Medicine, Los Angeles, California
A 23-year-old woman had a normal fetal ultrasound at 16 weeks’ gestation. A second ultrasound at 25 weeks revealed a cystic brain malformation in the fetus. A fetal magnetic resonance image (MRI) at 28 weeks showed the presence of a huge infratentorial/supratentorial multiloculated arachnoid cystic malformation and hydrocephalus (Fig 1A) [1]. Although the prognosis was highly uncertain, the possibility of blindness, endocrine abnormalities, and severe developmental delay was discussed with the family. At 38 weeks, an infant boy was delivered via elective cesarian section with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. Other than macrocephaly with full fontanelles, the physical examination was normal and without evidence of diabetes insipidus. A postdelivery MRI found the cystic malformation to have enlarged when compared with the fetal study (Fig 1B). A craniotomy was performed to fenestrate this multiloculated cystic malformation [2]. Histology of the submitted tissue was arachnoid. No problems were encountered postoperatively, and the infant was discharged home where growth and development progressed normally and the head circumference diminished by 50%. At 6 months an MRI (Fig 1C) showed an increase in size of the third ventricle and both lateral ventricles when compared with the MRI performed just after the fenestration. As continued enlargement was expected, a second operative procedure was performed consisting of an endoscopic cyst fenestration and third ventriculostomy. At 9 months of age, an MRI (Fig 1D) showed a decrease in the size of the cyst and lateral ventricles. At 1 year of age, his cognitive function appears mildly delayed. His vision is grossly normal; ocular motility is full, conjugate, and free of nystagmus. Tone and strength are normal and without focal deficit. The fetal MRI alerted the family to the presence of this huge multiloculated arachnoid cystic malformation that could be associated with severe neurologic and * Communications should be addressed to: Dr. Drazin; Department of Neurosurgery; Cedars-Sinai Medical Center; 8700 Beverly Boulevard, Los Angeles, CA 90048. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2013.02.016
endocrinologic abnormalities. These cysts frequently enlarge, as was evident with this infant who has done remarkably well considering the magnitude of the cystic malformation.
Figure 1. (A) Antenatal sagittal magnetic resonance image (MRI) at 28 weeks’ gestation demonstrated a large infratentorial/supratentorial multiloculated cystic malformation displacing the brainstem superiorly and posteriorly. (B) Postnatal T2-weighted sagittal MRI showed this suprasellar-prepontine midline cyst extending into the posterior fossa to have enlarged when compared with the fetal study. (C) Follow-up T1-weighted sagittal MRI at 6 months of age showed the third ventricular cyst and lateral ventricles had increased in size compared with the immediate postoperative MRI following the initial fenestration procedure (not shown). (D) Follow-up T2-weighted sagittal MRI at 9 months of age showed a decrease in the size of the cyst and ventricles following endoscopic cyst fenestration and third ventriculostomy.
482
D. Drazin, J.G. McComb / Pediatric Neurology 48 (2013) 481e482
References [1] Gedikbasi A, Palabiyik F, Oztarhan A, et al. Prenatal diagnosis of a suprasellar arachnoid cyst with 2- and 3-dimensional sonography and fetal magnetic resonance imaging: Difficulties in management
and review of the literature. J Ultrasound Med 2010;29(10): 1487e93. [2] Fewel ME, Levy ML, McComb JG. Surgical treatment of 95 children with 102 intracranial arachnoid cysts. Pediatr Neurosurg 1996;25: 165e73.
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Images in Child Neurology
Prenatal Diagnosis and Management of a Huge Infratentorial/Supratentorial Multiloculated Arachnoid Cystic Malformation in an Infant Doniel Drazin MD a, *, J. Gordon McComb MD b a b
Department of Neurological Surgery, Cedars-Sinai Medical Center, Los Angeles, California Division of Neurosurgery, Children’s Hospital of Los Angeles, Department of Neurological Surgery, USC Keck School of Medicine, Los Angeles, California
A 23-year-old woman had a normal fetal ultrasound at 16 weeks’ gestation. A second ultrasound at 25 weeks revealed a cystic brain malformation in the fetus. A fetal magnetic resonance image (MRI) at 28 weeks showed the presence of a huge infratentorial/supratentorial multiloculated arachnoid cystic malformation and hydrocephalus (Fig 1A) [1]. Although the prognosis was highly uncertain, the possibility of blindness, endocrine abnormalities, and severe developmental delay was discussed with the family. At 38 weeks, an infant boy was delivered via elective cesarian section with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. Other than macrocephaly with full fontanelles, the physical examination was normal and without evidence of diabetes insipidus. A postdelivery MRI found the cystic malformation to have enlarged when compared with the fetal study (Fig 1B). A craniotomy was performed to fenestrate this multiloculated cystic malformation [2]. Histology of the submitted tissue was arachnoid. No problems were encountered postoperatively, and the infant was discharged home where growth and development progressed normally and the head circumference diminished by 50%. At 6 months an MRI (Fig 1C) showed an increase in size of the third ventricle and both lateral ventricles when compared with the MRI performed just after the fenestration. As continued enlargement was expected, a second operative procedure was performed consisting of an endoscopic cyst fenestration and third ventriculostomy. At 9 months of age, an MRI (Fig 1D) showed a decrease in the size of the cyst and lateral ventricles. At 1 year of age, his cognitive function appears mildly delayed. His vision is grossly normal; ocular motility is full, conjugate, and free of nystagmus. Tone and strength are normal and without focal deficit. The fetal MRI alerted the family to the presence of this huge multiloculated arachnoid cystic malformation that could be associated with severe neurologic and * Communications should be addressed to: Dr. Drazin; Department of Neurosurgery; Cedars-Sinai Medical Center; 8700 Beverly Boulevard, Los Angeles, CA 90048. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2013.02.016
endocrinologic abnormalities. These cysts frequently enlarge, as was evident with this infant who has done remarkably well considering the magnitude of the cystic malformation.
Figure 1. (A) Antenatal sagittal magnetic resonance image (MRI) at 28 weeks’ gestation demonstrated a large infratentorial/supratentorial multiloculated cystic malformation displacing the brainstem superiorly and posteriorly. (B) Postnatal T2-weighted sagittal MRI showed this suprasellar-prepontine midline cyst extending into the posterior fossa to have enlarged when compared with the fetal study. (C) Follow-up T1-weighted sagittal MRI at 6 months of age showed the third ventricular cyst and lateral ventricles had increased in size compared with the immediate postoperative MRI following the initial fenestration procedure (not shown). (D) Follow-up T2-weighted sagittal MRI at 9 months of age showed a decrease in the size of the cyst and ventricles following endoscopic cyst fenestration and third ventriculostomy.
482
D. Drazin, J.G. McComb / Pediatric Neurology 48 (2013) 481e482
References [1] Gedikbasi A, Palabiyik F, Oztarhan A, et al. Prenatal diagnosis of a suprasellar arachnoid cyst with 2- and 3-dimensional sonography and fetal magnetic resonance imaging: Difficulties in management
and review of the literature. J Ultrasound Med 2010;29(10): 1487e93. [2] Fewel ME, Levy ML, McComb JG. Surgical treatment of 95 children with 102 intracranial arachnoid cysts. Pediatr Neurosurg 1996;25: 165e73.