A Rescue Approach to Difficult Airway in Fahr Syndrome: Ultrasound-Guided Interscalene Brachial Plexus Block

Abstract

Fahr Syndrome is a neurodegenerative disease characterized by bilateral intracerebral calcifications. Its symptoms are associated with extensive calcification of the nervous system and vessels, and hypocalcemia. In addition to cardiac problems, difficult airway management can be encountered, and in this case, regional anesthesia techniques can be a safe option. Interscalene brachial plexus block is frequently used in shoulder and upper extremity surgeries because it is easy to apply, has a high success rate and provides postoperative analgesia. In this case report, we aimed to share our successful anesthesia management with interscalene brachial plexus block in a patient with Fahr syndrome who is planned to undergo upper extremity surgery. No complications were encountered during the perioperative period. We think that peripheral nerve blocks can be applied as an alternative anesthetic method in patients with Fahr syndrome.

Keywords

• Fahr Syndrome
• Regional Anesthesia
• Interscalene Brachial Plexus Block

Fahr Sendromunda Zor Havayoluna Karşı Kurtarıcı Bir Yaklaşım: Ultrason Eşliğinde İnterskalen Brakial Pleksus Bloğu

Abstract

Fahr Sendromu, bilateral intraserebral kalsifikasyonlarla karakterize nörodejeneratif bir hastalıktır. Semptomları, sinir sistemi ve damarların yaygın kalsifikasyonu ve hipokalsemi ile ilişkilidir. Kardiyak problemlerin yanı sıra zor havayolu yönetimi ile de karşılaşılabilir ve bu durumda rejyonel anestezi teknikleri güvenli bir seçenek olabilir. İnterskalen brakial pleksus bloğu, uygulanması kolay, başarı oranı yüksek olduğundan ve postoperatif analjezi sağladığından omuz ve üst ekstremite cerrahilerinde sıklıkla kullanılmaktadır. Bu makalede, Fahr sendromlu üst ekstremite cerrahisi planlanan bir olguda uygulanan interskalen brakial pleksus bloğu ile başarılı anestezi yönetimimizi paylaşmayı amaçladık. Perioperatif dönemde herhangi bir komplikasyonla karşılaşılmadı. Fahr sendromlu olgularda, periferik sinir bloklarının alternatif anestezi tekniği olarak uygulanabileceğini düşünmekteyiz.

Keywords

• Fahr Sendromu
• Rejyonel Anestezi
• İnterskalen Brakial Pleksus Bloğu

References

1. Hoque MA, Siddiqui MR, Arafat Y, Khan SU, Rahman KM, et al. Fahr’s disease: a very rare cause of epilepsy. Mymensingh Med J. 2010;19: 127-129.
2. Batla A, Tai XY, Schottlaender L, et al. Deconstructing Fahr’s disease/ syndrome of brain calcification in the era of new genes. Parkinsonism Relat Disord 2017;37:1–0. DOI: 10.1016/j.parkreldis.2016.12.024.
3. Westenberger A, Klein C. The genetics of primary familial brain calcifications. Curr Neu-rol Neurosci Rep. 2014;14:490. DOI: 10.1007/s11910-014-0490-4.
4. Park S, Jee DL, Kim H. General anesthesia for patient with Fahr’s syndrome: A case report. Medicine (Baltimore). 2019;98(17):e15390. DOI: 10.1097/MD.0000000000015390.
5. Baum VC, O’Flaherty JE. Anesthesia for Genetic, Metabolic, and Dysmorphic Syndromes of Childhood. 2nd edition, Philadelphia: Lippincott Williams & Wilkins; 2007. p. 129.
6. Kıvrak E. Fahr sendromu olan hastada anestezi yönetimi: Olgu sunumu. DEÜ Tıp Fakül-tesi Dergisi. 2018;32(2):141-143. DOI: 10.5505/deutfd.2018.25633.
7. Kilicaslan A, Gok F, Korucu I H , Ozkan A, Yılmaz R. Anatomical variations detected dur-ing ultrasoundguided interscalene brachial plexus block and clinical implications. Marmara Med J. 2020;33(3):141-145, DOI:10.5472/marumj.816311.
8. Okçelik S, Tasdemir S, Öztürk E, Soydan H. Subinguinal Varicocelectomy under Spinal Anesthesia in a Fahr’s Syndrome Patient with Non-Obstructıve Azoospermia. Andrology (Los Angel). 2016; 5:163. DOI:10.4172/2167-0250.1000163.

9. El Boukhrissi F, Zoulati G, En-nafaa I, et al. Fahr syndrome secondary to primary hypopar-athyroidism: about a case. Pan Afr Med J. 2017;26:2. DOI:10.11604/pamj.2017.26.2.10689.
10. Eom TH, Kim YH, Kim JM. Recurrent seizures, mental retardation and extensive brain calcinosis related to delayed diagnosis of hypoparathy- roidism in an adolescent boy. J Clin Neu-rosci. 2015;22:894–6. DOI:10.1016/j.jocn.2014.11.020.
11. Policepatil SM, Caplan RH, Dolan M. Hypocalcemic myopathy secondary to hypoparathyroidism. WMJ. 2012;111(4):173-175. PMID: 22970532.
12. Yang CS, Lo CP, Wu MC. Ischemic stroke in a young patient with Fahr’s disease: a case report. BMC Neurol. 2016;16:33. DOI: 10.1186/s12883-016-0557-8.
13. Sgulo FG, di Nuzzo G, de Notaris M, et al. Cerebrovascular disorders and Fahr’s disease: report of two cases and literature review. J Clin Neurosci 2018;50:163–4. DOI: 10.1016/j.jocn.2018.01.002.
14. Carden E, Ori A. Applying cervical spine anatomy to interscalene brachial plexus blocks. Pain Physician. 2005; 8:357-361. DOI: 10.36076/ppj.2005/8/357.
15. Heath PJ, Brownlie GS, Herrick MJ. Latency of brachial plexus block. The effect on onset time of warming local anaes thet i c solut ions . Anaes thes ia. 1990;45:297-301. DOI :10.1111/j.1365‑2044.1990.tb14736.x.