An Achondroplasic Case with Foramen Magnum Stenosis, Hydrocephaly, Cortical Atrophy, Respiratory Failure and Sympathetic Dysfunction

Background: Achondroplasia is a relatively frequent genetic disorder that may lead to limb weakness, motormental retardation, hydrocephaly, and respiratory disorders. In this pathology, foramen magnum stenosis and accompanying disorders like respiratory depression is well documented. Case Presentation: A 2.5 year-old child with the diagnosis of achondroplasia admitted to our clinic with severe respiratory depression, limb weakness, and motor mental retardation as well as sympathetic dysfunction. In radiologic evaluations, severe foramen magnum stenosis was detected. The patient was operated and posterior fossa decompression was accomplished to prevent compression of respiratory centers and neurons. Conclusion: This case is unique with the narrowest foramen magnum reported up to date and the sympathetic dysfunction which is not reported as a complication in achondroplasic patients. The authors review the relevant literature, focusing on the indications for cervicomedullary decompression in infants with achondroplasia

Methemoglobinemia due to prilocaine administration

Sünnet sırasında ağrı çekilmesini önlemek amacı ile sıklıkla prilokain kullanılarak dorsal penil sinir bloğu yerel anestezi yöntemi olarak uygulanmaktadır. Prilokainin küçük bebek ve yenidoğanlarda methemoglobinemiye yol açtığı bilinmektedir. Burada, sünnet sonrası aniden gelişen ani ve belirgin morarma yakınması ile yoğun bakım birimine sevk edilen ve prilokane bağlı methemoglobinemi geliştiği saptanan 56 günlük erkek olgu sunulmuştur. Hastaya yerel anestezi uygulaması için iki defa 1 mg/kg dozdan prilokain uygulandığı saptanmıştır. Başvurusunda hastanın siyanoz dışında bulgusu yoktu. Kan methemoglobin düzeyi %16,2 saptandı ve tedavi ile tam iyileşme sağlandı. Fetal hemoglobinin “ferrik” duruma daha yatkın olması ve methemoglobinemiyi önlemeye yardımcı olan sitokrom b5 enzim düzeyinin hayatın ilk aylarında düşük bulunması nedeniyle altı aydan küçük çocuklarda yerel anestezik madde olarak prilokain yerine diğer yerel anestezik maddeler kullanılması gerektiği üzerinde durulmuştur.Prilocaine is the one of the most utilized agents during dorsal penile block in order to prevent pain during circumcision. It is known that prilocaine may lead to methemoglobinemia in neonates and infants. Here we present a 56 days old male patient with prilocaine induced methemoglobinemia who had been referred to intensive care unit for his frank and instant cyanosis after circumcision. Detailed history taking revealed that he needed 1 mg/kg prilocaine administration twice for adequate anesthesia. Patient had frank cyanosis at administration with no other sign. During admission rate of methemoglobin was 16,2% in the blood of the patient and conservative measures lead to complete resolution of all symptoms. Since infants below six months of age are particularly prone to methemoglobinemia due to their low cytochrome b5 levels and fetal hemoglobine is closer to “ferric” state than adult hemoglobine, it is emphasized in this paper that other alternative anesthetic agents should be utilized instead of prilocaine for regional anesthesia in infants under six months age