Anaesthetic Management of a Patient with Pseudo-TORCH Syndrome

Abstract

Background: Pseudo-TORCH syndrome is a rare, chronic disorder that is characterised by dimorphic features such as microcephaly, intracranial calcification, seizures, mental retardation, hepatosplenomegaly and coagulation disorders. Case Report: We present the anaesthetic management of a forty day-old boy with Pseudo-TORCH syndrome during magnetic resonance imaging. Microcephaly, growth failure, high palate and bilateral rales in the lungs were detected in pre-anaesthetic physical examination. The peripheral oxygen saturation was 88-89% in room-air and was 95% in a hood with 5 L/min oxygen. We planned general anaesthesia to ensure immobility during magnetic resonance imaging. After standard monitoring, general anaesthesia was induced with 8% sevoflurane in 100% O2. After an adequate depth of anaesthesia was reached, we inserted a supraglottic airway device to avoid intubation without the use of a muscle relaxant. Conclusion: In patients with Pseudo-TORCH syndrome, the perioperative anaesthetic risk was increased. We believe that using a supraglottic airway device to secure the airway is less invasive than intubation, and can be performed without the need of muscle relaxants. Turkish Anahtar Kelimeler: Psödo-TORCH Sendromu, Anestezi, Supraglottik Hava Yolu Gereci Arkaplan: Psödo-TORCH sendromu; mikrosefali, intrakraniyal kalsifikasyon, nöbetler, mental retardasyon, hepatosplenomegali ve koagülasyon bozuklukları gibi dimorfik özellikler ile karakterize nadir kronik bir hastalıktır. Manyetik rezonans görüntüleme esnasında Kırk günlük Psödo-TORCH sendromlu erkek bebekteki anestezi yönetimimizi sunmaktayız. Olgu Sunumu: Anestezi öncesi yapılan fizik muayenesinde mikrosefali, gelişme geriliği, yüksek damak ve akciğerleri dinlemekle bilateral ral tespit edildi. Oda havasında periferik oksijen saturasyonu % 88-89 ve hood eşliğinde 5 lt/dk oksijen ile % 95 idi. Manyetik rezonans görüntüleme esnasında hareketsizliği sağlamak için genel anestezi planlandı. Standart monitorizasyon sonrası, % 100 oksijen ve % 8 sevofluran ile anestezi indüksiyonu yapıldı. Yeterli anestezi derinliği sağlandıktan sonra, kas gevşetici ajan kullanılmaksızın entübasyondan kaçınmak için supraglottik hava yolu gereci kullanıldı. Sonuç: Psödo-TORCH sendromlu hastalarda perioperatif anestezi riski artmaktadır. Entübasyona göre daha az invaziv olan supraglottik hava yolu gereçlerinin kas gevşetici ihtiyacı olmaksızın hava yolu güvenliğini sağlayabileceğini düşünüyoruz.

Keywords

• Pseudo-TORCH syndrome, anaesthesia, supraglottic airway device

Anaesthetic Management of a Patient with Pseudo-TORCH Syndrome

Öz

Kaynakça

1. 1. Abdel-Salam GHM, Zaki MS, Saleem SN, Gaber KR. Microcephaly, malformation of brain development and intracranial calcification in sibs: pseudo-TORCH or a new syndrome. Am J Med Genet Part 2008;146:2929-36. [CrossRef]
2. 2. Aicardi J, Goutières F. A progressive familial encephalopathy in infancy, with calcification of the basal ganglia and chronic cerebrospinal fluid lymphocytosis. Ann Neurol 1984;15:49-54. [CrossRef]
3. 3. Knoblauch H, Tennstedt C, Brueck W, Hammer H, Vulliamy T, Dokal I, et al. Two brothers with findings resembling congenital intrauterine infection-like syndrome (Pseudo-TORCH Syndrome). Am J Med Genet Part 2003;120:261-5. [CrossRef]
4. 4. Cauldwell C. Anesthesia risks associated with pediatric imaging. Pediatr Radiol 2011;41: 949-50. [CrossRef]
5. 5. Trevisanuto D, Grazzina N, Ferrarese P, Micaglio M, Verghese C, Zanardo V. Laryngeal mask airway used as a delivery conduit for the administration of surfactant to preterm infants with respiratory distress syndrome. Biol Neonate 2005;87:217-20. [CrossRef]
6. 6. Gaitini LA, Vaida SJ, Somri M, Yanovski B, Ben-David B, Hagberg CA. A randomized controlled trial comparing the ProSeal laryngeal mask airway with the laryngeal tube suction in mechanically ventilated patients. Anesthesiology 2004;101:316-20. [CrossRef]
7. 7. Brain AI, Verghese C, Strube PJ. The LMA ‚ProSeal‘-a laryngeal mask with an oesophageal vent. Br J Anaesth 2000;84:650-4. [CrossRef]
8. 8. Yu SH, Beirne OR. Laryngeal mask airways have a lower risk of airway complications compared with endotracheal intubation: a systematic review. J Oral Maxillofac Surg 2010;68:2359-76. [CrossRef]