|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 430-431
Anesthetic course of a patient with pineal gland cyst and osteogenesis imperfecta: A rare experience
Bashir Ahmad Dar1, Iqra Nazir2, Zulfiqar Ali1, Altaf Kirmani3
1 Department of Anesthesiology, Division of Neuroanesthesiology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
2 Department of Anesthesiology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
3 Department of Neurosurgery, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||29-Jul-2015|
Department of Anesthesiology, Division of Neuroanesthesiology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar - 190 011, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Dar BA, Nazir I, Ali Z, Kirmani A. Anesthetic course of a patient with pineal gland cyst and osteogenesis imperfecta: A rare experience. J Anaesthesiol Clin Pharmacol 2015;31:430-1
|How to cite this URL:|
Dar BA, Nazir I, Ali Z, Kirmani A. Anesthetic course of a patient with pineal gland cyst and osteogenesis imperfecta: A rare experience. J Anaesthesiol Clin Pharmacol [serial online] 2015 [cited 2020 Sep 20];31:430-1. Available from: http://www.joacp.org/text.asp?2015/31/3/430/161748
Osteogenesis imperfecta (OI) is an inherited connective tissues disorder of type I collagen formation characterized by brittle bones, hypermobile limbs, kyphoscoliosis, pectus carinatum, short neck, with difficult airway and risk of odonto-axial dislocation during laryngoscopy and intubation. ,, We report anesthetic management of a baby with OI with pineal gland cyst.
A 2½-year-old male child, known case of OI, with magnetic resonance imaging (MRI) documented cyst of the pineal gland with tri-ventricular hydrocephalus [Figure 1] was scheduled for cyst excision. He had suffered multiple fractures of upper and lower limb bones [Figure 2] and was being treated conservatively. Hemogram and serum biochemistry values were within normal range. Chest X-ray revealed thoracic cage deformity with crowding of ribs. MRI findings revealed large cystic area 60 mm × 40 mm × 31 mm in the region of pineal gland causing mass effect on 3 rd ventricle with dilatation of lateral ventricles. The patient was carefully placed in supine position. After attaching routine monitors, an intravenous line was secured. After preoxygenation anesthesia was induced with propofol and fentanyl. Airway was secured by endotracheal tube of 4 mm internal diameter after manual in-line stabilization of the neck. Arterial line was secured in right radial artery. Central venous line was placed in right internal jugular vein with the help of ultrasound. Anesthesia was maintained using intermittent IV vecuronium and N 2 O in O 2 (1:1) inhalation with variable concentration of sevoflurane. Meticulously patient was kept prone, and pressure areas were padded. A suboccipital craniectomy was done with total excision of the cyst. At the end of surgery, patient was carefully repositioned to supine. Neuromuscular blockade was reversed using neostigmine and glycopyrrolate. After an uneventful extubation, patient was shifted to Intensive Care Unit without any evidence of iatrogenic trauma.
|Figure 1: Magnetic resonance Imaging of brain axial cut showing a huge arachnoid cyst in the pineal region|
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Our case was diagnosed as type IV (A) OI characterized by fragile bone, short stature, and barrel-shaped rib cage. Karabiyik et al.  used total intravenous anesthesia along with intubating laryngeal mask airway for nephrolithotomy and ureterolithotomy. Malde and Jagtap  used general anesthesia in case of OI for hysterectomy. These authors reported fracture shaft of the femur while transferring the patient to the recovery room. Increased intra-operative bleeding may occur in OI  due to deficiency of factor VIII and deficient platelet aggregation. Due precautions were taken in the form of availability of adequate blood products. There was no significant blood loss during surgery in our case. Intra-operative hyperthermia is more common in patients with anticholinergic medications and inhalational versus intravenous anesthesia.  In our case, we used sevoflurane as inhalational agent and no hyperthermia was noticed.
We would like to emphasize that in case of OI careful preoperative assessment of the patient is mandatory so as to provide safe anesthesia. When general anesthesia is considered, attention is required for airway management and positioning of the patient. Recognition of potential complications of this disorder is important for anesthesiologist to prevent iatrogenic trauma.
| References|| |
Colvin MP, Wilkinson K. Hazards and Complications of Anaesthesia. Edinburgh: Churchhill Livingstone Inc.; 1993. p. 535-60.
Karabiyik L, Parpucu M, Kurtipek O. Total intravenous anaesthesia and the use of an intubating laryngeal mask in a patient with osteogenesis imperfecta. Acta Anaesthesiol Scand 2002;46:618-9.
Malde AD, Jagtap SR, Pantvaidya SH, Kenkare JS. Osteogenesi imperfecta: Anaesthetic management of a patient for abdominal hysterectomy (a case report). Indian J Anaesth 1993;41:203-6.
Edge G, Okafor B, Fennelly ME, Ransford AO. An unusual manifestation of bleeding diathesis in a patient with osteogenesis imperfecta. Eur J Anaesthesiol 1997;14:215-9.
Libman RH. Anesthetic considerations for the patient with osteogenesis imperfecta. Clin Orthop Relat Res 1981;159:123-5.
[Figure 1], [Figure 2]