Makrosefalisi Olan Hastalarda Nörogörüntülemenin Tanısal Değeri

Amaç: Bu çalışmada makrosefali için yapılan görüntülemelerde benign eksternal hidrosefali (BEH), hidrosefali ve diğer ilişkili durumların göreceli sıklıklarını belirlemek ve nörogörüntüleme değerlendirmesinden fayda sağlayacak olan makrosefali hastaları için ek risk faktörlerini tespit etmek amaçlanmıştır. Materyal ve Metot: Merkezimizdeki 1 Ocak 2014 ile 1 Haziran 2019 tarihleri arasındaki tıbbi kayıtlar geriye dönük olarak makrosefali tanı kodu ile tarandı. 36 aylıktan büyük hastalar çıkarılarak yapılan taramada her hasta için yaş, cinsiyet, semptomlar ve klinik bulgular (nöbetler, hipotoni gibi muayenede nörolojik anormallikler), nörogörüntüleme bulguları, gelişimsel gecikme, ailede makrosefali öyküsü ve baş çevresi bilgileri elde edildi. Bulgular: Toplamda 103 hasta taramaya dahil oldu. Ortalama görüntüleme yaşı 9 ay idi. (±5,5 ay). Yirmi bir hasta kız (%20,38), seksen iki hasta ise erkek idi (%79,61). Yapılan nörogörüntelemelerden yirmi bir tanesi manyetik rezonans görüntülemesi, yirmi altısı bilgisayarlı tomografi ve altmış beş tanesi transfontanel ultrasonografi idi. Anormal nörogörüntüleme bulguları gelişimsel geriliği veya anormal nörolojik bulgusu olanlarda diğer gruplara (BEH ve normal görüntüleme) göre anlamlı olarak daha yüksek saptandı. (p=0.003 ve p<0.0001). Sonuç: Bu çalışmayla makrosefalisi olan hastalarda gelişme geriliği veya anormal nörolojik muayene bulgusu saptanmamışsa yapılan nörogörüntülemelerin tanısal katkısı olmadığı ortaya konulmuştur.

Anahtar Kelimeler:

Makrosefali, benign eksternal hidrosefali, nörogörüntüleme

Neuroimaging indications for children with macrocephaly

Objectives The goal of this study was to determine the relative frequencies of benign external hydrocephalus (BEH), hydrocephalus and other conditions in a large series of imaging studies performed for macrocephaly and identify additional risk factors for patients with macrocephaly that would most likely benefit from neuroimaging evaluation. Methods Medical records at our center were searched for the term macrocephaly retrospectively. The search extended from 1th January 2014 to 1th June 2019. Studies in patients older than 36 months of age were excluded. Information about age, gender, symptoms and clinical signs (seizures, neurologic abnormalities on exam such as hypotonia), neuroimaging findings, developmental delay, family history of macrocephaly and head circumference (HC) were collected for each patient. Results A total of 103 patients were included in the analysis. The mean age at the time of imaging was 9 months (±5.5 months). Twenty-one (20.3%) of the subjects were female and 82 (79.6%) were male. Twenty-nine of the imaging studies were magnetic resonance imaging, 26 were computed tomography and 65 were head ultrasounds. Patients with abnormal neuroimaging results had significantly higher rates of developmental delay or abnormal neurologic exam than patients with normal neuroimaging results or BEH (p=0.003 and p<0.0001). There was no significant difference between the neuroimaging results of patients with and without positive family history of macrocephaly. Conclusion This study suggests that neuroimaging for macrocephaly has almost negligible diagnostic yield unless having developmental delay or abnormal neurological examination.

Keywords:

Macrocephaly, benign external hydrocephalus, neuroimaging,

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1. Nellhaus G. Head circumference from birth to eighteen years. Practical composite international and interracial graphs. Pediatrics. 1968;41(1):106-14.2. Sampson MA, Berg AD, Huber JN, Olgun G. Necessity of Intracranial Imaging in Infants and Children With Macrocephaly. Pediatr Neurol. 2019;93:21-6.3. Zahl SM, Egge A, Helseth E, Wester K. Clinical, Radiological, and Demographic Details of Benign External Hydrocephalus: A Population-Based Study. Pediatr Neurol. 2019;96:53-7.4. Haws ME, Linscott L, Thomas C, Orscheln E, Radhakrishnan R, Kline-Fath B. A Retrospective Analysis of the Utility of Head Computed Tomography and/or Magnetic Resonance Imaging in the Management of Benign Macrocrania. J Pediatr. 2017;182:283-9 e1.5. van Wezel-Meijler G, Steggerda SJ, Leijser LM. Cranial ultrasonography in neonates: role and limitations. Semin Perinatol. 2010;34(1):28-38.6. Hamza M, Bodensteiner JB, Noorani PA, Barnes PD. Benign extracerebral fluid collections: a cause of macrocrania in infancy. Pediatr Neurol. 1987;3(4):218-21.7. Medina LS, Frawley K, Zurakowski D, Buttros D, DeGrauw AJ, Crone KR. Children with macrocrania: clinical and imaging predictors of disorders requiring surgery. AJNR Am J Neuroradiol. 2001;22(3):564-70.8. Muenchberger H, Assaad N, Joy P, Brunsdon R, Shores EA. Idiopathic macrocephaly in the infant: long-term neurological and neuropsychological outcome. Childs Nerv Syst. 2006;22(10):1242-8.9. Bosnjak V, Besenski N, Marusic-Della Marina B, Kogler A. Cranial ultrasonography in the evaluation of macrocrania in infancy. Dev Med Child Neurol. 1989;31(1):66-75.10. Laubscher B, Deonna T, Uske A, van Melle G. Primitive megalencephaly in children: natural history, medium term prognosis with special reference to external hydrocephalus. Eur J Pediatr. 1990;149(7):502-7.11. Prassopoulos P, Cavouras D, Golfinopoulos S, Nezi M. The size of the intra- and extraventricular cerebrospinal fluid compartments in children with idiopathic benign widening of the frontal subarachnoid space. Neuroradiology. 1995;37(5):418-21.12. Yew AY, Maher CO, Muraszko KM, Garton HJ. Long-term health status in benign external hydrocephalus. Pediatr Neurosurg. 2011;47(1):1-6.13. Sun M, Yuan W, Hertzler DA, Cancelliere A, Altaye M, Mangano FT. Diffusion tensor imaging findings in young children with benign external hydrocephalus differ from the normal population. Childs Nerv Syst. 2012;28(2):199-208.14. Nickel RE, Gallenstein JS. Developmental prognosis for infants with benign enlargement of the subarachnoid spaces. Dev Med Child Neurol. 1987;29(2):181-6.15. Tucker J, Choudhary AK, Piatt J. Macrocephaly in infancy: benign enlargement of the subarachnoid spaces and subdural collections. J Neurosurg Pediatr. 2016;18(1):16-20.