Manajemen Anestesi untuk Tindakan Vp-Shunt pada Bayi Sindrom Crouzon dengan Hidrosefalus
DOI:
https://doi.org/10.24244/jni.vol8i1.211Keywords:
hidrosefalus, sindrom crouzon, VP-shunt, crouzon syndrome, hydrocephalusAbstract
Sindrom Crouzon adalah sindrom dominan autosom yang ditandai dengan trias yaitu deformitas tengkorak, anomali wajah, dan eksoftalmus. Sindrom Crouzon memiliki prevalensi 1: 60.000 kelahiran. Sindrom ini disebabkan sinostosis dini sutura koronal dan sagital yang mengakibatkan dismorfisme wajah. Pada anestesi pasien dengan sindrom Crouzon, harus dilakukan langkah-langkah untuk antisipasi dan persiapan penanganan jalan napas yang sulit. Pada kasus ini, seorang bayi 5 bulan dengan sindrom Crouzon datang dengan keluhan kepala membesar sejak 2 bulan sebelum masuk rumah sakit. Dari CT-scan didapatkan hidrosefalus, dan dilakukan tindakan VP-shunt. Telah diantisipasi adanya kesulitan intubasi, maka dilakukan persiapan alat-alat termasuk bougie anak. Bougie tersebut kemudian ternyata sangat berguna saat dilakukan intubasi ulang karena ketidaksesuaian ukuran tube endotrakeal. Operasi VP-shunt berjalan dengan baik, pasca operasi dan anestesi pasien sadar baik, respirasi dan hemodinamik stabil dan kemudian pindah ke ruang rawat biasa. Dalam penanganan pasien ini, antisipasi, peralatan difficult airway yang lengkap, rencana alternatif, serta pendekatan multidisiplin sangat diperlukan.
Anaesthetic Management for VP-Shunt in Baby Crouzon syndrome with Hydrocephalus
Crouzon syndrome is an autosomal dominant syndrome characterized by triad of skull deformity, facial anomalies, and exophthalmos. Crouzon syndrome has a prevalence of 1: 60,000 births. This syndrome is characterized by early synostosis of coronal and sagittal sutures which leads to facial dysmorphism. In Crouzon syndrome, steps must be taken to anticipate and prepare for difficult airway handling.In this case, a 5-month-old baby with Crouzon syndrome presents with an enlarged head complaint 2 months before being hospitalized. CT scan showed hydrocephalus was, and VP-shunt action was planned. It is anticipated that there will be difficulty in intubation, so preparations for tools including child bougie were made. Bougie then turned out to be very useful because it had to be re-intubated because of the incompatibility of the size of the endotracheal tube. VP-shunt surgery works well, surgery and anesthesia were uneventful, then patient moved to the ward. Anticipation, complete difficult airway equipment and alternative plans, as well as a multidisciplinary approach are needed in handling these patients.
Downloads
References
Ahmed I, Afzal A. Diagnosis and evaluation of crouzon syndrome.J Coll Phys Surg Pak 2009;19(5):318–20.
Cohen MM Jr. Kreiborg S. Birth prevalence studies of the crouzonsyndrome: comparison of direct and indirect methods. Clin Genet.1992;41(1):1–5.
Rutland P, Pulleyn LJ, Reardon W. Identical mutations in the FGFR2 gene cause both pfeiffer and crouzon syndrome phenotypes. NatGenet. 1995;9(2):173–76.
Wilkie AO, Slaney SF, Oldridge M. Apert syndrome results fromlocalized mutations of FGFR2 and is allelic with crouzon syndrome.Nat Genet. 1995;9(2):165–72.
Bowling EL, Burstein FD. Crouzon syndrome. Optometry.2006;77(5):217–22.
Cinalli G, Sainte-Rose C, Kollar EM, Zerah M, Brunelle F, Chumas P, et al. Hydrocephalus and craniosynostosis. J Neurosurg 1998; 88:209–214.
Sainte-Rose C, LaCombe J, Pierre-Kahn A, Renier D, Hirsch JF. Intracranialvenous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 1984;60:727–736.
Nienaber J. Anaesthesia for ventriculoperitoneal shunts. Southern African Journal of Anesth Analg, 2011;17:1, 73–5.
Roche J, Frawley G, Heggie A. Difficult tracheal intubation induced by maxillary distraction devices in craniosynostosis syndromes. Paed Anesth 2002; 12: 227–34.
Kumar A, Goel N, Sinha C, Singh A. Anestheticimplications in a child with crouzon syndrome. Anesth Essays Res2017;11:246–7.
Hlongwa P. Early orthodontic management of Crouzon syndrome: Acase report. J Maxillofac Oral Surg 2009;8:74–6.
Hughes C, Thomas K, Johnson D, Das S. Anesthesia for surgery related to craniosynostosis: A review. Part 2. Paediatr Anaesth 2013;23:22–7.
Sirotnak J, Brodsky L, Pizzuto M. Airway obstruction in the Crouzonsyndrome: case report and review of the literature. Int J PediatrOtorhinolaryngol 1995; 31:235–46.
