Anesthetic management of a rare case of craniosynostosis
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0975-2870.157087
Craniosynostosis is a condition of premature intrauterine fusion of sutures causing abnormal skull shape, neurological abnormalities and increased intracranial pressure. It can occur as an isolated condition or may be part of a syndrome. The key to treating an infant with craniosynostosis is early diagnosis and accurate treatment as untreated craniosynostosis can affect mental development and visual abnormalities which are linked to raised intracranial pressure. In this case, we describe the anesthetic management of a 13-month-old baby, a case of nonsyndromic craniosynostosis involving sagittal, metopic and coronal sutures posted for strip craniectomies. Keywords: Craniosynostosis, hydrocephalus, massive blood loss, scaphocephaly, strip craniectomy, venous air embolism
Craniosynostosis is a condition in which one or more of the six fibrous sutures prematurely fuse by ossification. Embryologically, cranial vault starts to ossify at the 8 th week after conception. [1] Fusion involves one of the five major sutures namely: Sagittal (common), metopic, coronal, lambdoid, and squamosal (rare). [2] The type of craniosynostosis depends on whether a single or multiple sutures are fused; each with a unique presentation of the skullcap and facial bones. The most common type is the sagittal craniosynostosis with a prevalence of 1 in 4,200 live births. Majority of the babies with craniosynostosis are nonsyndromic or may occur along with syndromes such as apert, crouzon, carpenter, pfifer, with other symptoms including delayed brain growth and trunk deformities. [3] Premature fusion of cranial sutures is responsible for the lack of growth perpendicular to fused sutures with a compensatory overgrowth in the normal areas as stated by the Virchows law. This law helps in predicting the pattern of skull deformity. In this case, premature sagittal suture closure resulted in restricted brain growth in a perpendicular plane and so the head remained narrow. There is also a prominent ridge over the fused sagittal suture. Compensatory growth occurred forwards at the coronal suture and also at the lambdoid suture resulting in a prominent forehead or frontal bossing and a prominent occiput. When seen from the side, the shape of the head looks like a boat and hence the Greek term "scaphocephaly" literally meaning boat head. [4] The management of craniosynostosis is multidisciplinary and the anesthetic challenges continue to be management of prolonged anesthesia, massive blood loss, venous air embolism, disseminated intravascular coagulation positional injury and hypothermia in small children, further complicated by syndrome specific issues, if any.
A male baby aged 13 months weighing 6 kg with sagittal, metopic and coronal craniosynostosis was admitted for strip craniectomies. The mother gave a history of premature delivery by lower segment caesarean section at 6 months, as she complained of bleeding pemphigus vulgaris. The baby weighed 1.5 kg at birth and was kept in Neonatal Intensive Care Unit. There were delayed milestones with neck holding at 5 months, inability to sit or stand with support and absent dentition. The baby had scaphocephaly that is a narrow elongated head due to sagittal suture craniosynostosis. There was also a prominent ridge along the sagittal suture. Baby also had a prominent forehead and protruding eyes that is brachycephaly due to affection of both coronal sutures. The forehead was vertically recessed with retrusion of the supraorbital ridge. [5] There was no history of visual abnormalities, lethargy, persistent vomiting or seizures. On physical examination, child was conscious, comfortable, recognizing mother. Pulse rate -110/min regular, Respiratory rate -20/min, SpO 2 on room air -98%. Chest was clear and cardiovascular examination was normal [Figure 1] and [Figure 2].
Investigations Routine hemogram, urine, chest X-ray, two-dimensional echo were all normal. Computed tomography (CT) scan brain revealed findings suggestive of mild hydrocephalus (non-communicating). Visualized bones of skull and calvarium show scalloping and thinning (luckenschadel skull). Magnetic resonance imaging brain showed craniosynostosis (brachycephaly, scaphocephaly), marked thinning of corpus callosum with paucity of white matter and delayed myelination. Anesthetic management The patient was kept nil by mouth for 6 h and was premedicated with oral triclofos sodium (pedichloryl) 70 mg/kg, 45 min before surgery and then shifted to operation theater (OT). Pulse oximeter, electrocardiogram, precordial stethoscope and noninvasive blood pressure monitors were attached. Packed cell volume was kept ready in OT before induction. Inhalational induction done with 6% sevoflurane and oxygen using Jackson Rees (JR) circuit. Then a 24 g intravenous (IV) line secured over the dorsum of the left hand. IV drugs glycopyrrolate 0.004 mg/kg, fentanyl 2 mcg/kg, and vecuronium bromide 0.1 mg/kg given. Intermittent positive pressure ventilation (IPPV) with 100% oxygen was given for 3 min. Laryngoscopy with Macintosh blade no.1 and oro-tracheal intubation with 4 mm uncuffed flexometallic tube done. Bilateral equal air entry checked. Tube fixed with dynaplast after applying benzoin. Maintenance was with oxygen, nitrous oxide and sevoflurane with manual IPPV via JR circuit. To maintain normothermia, baby was covered with aluminum foil. Under all aseptic precautions a femoral line was secured using a 5 French arrow double lumen catheter into the right femoral vein. Urinary catheterization was done with 8 mm Foley's catheter. All vital parameters were stable intraoperatively [Figure 3].
Surgical procedure Multiple strip craniectomies were done starting with metopic suture and extending posteriorly over sagittal suture line and bilaterally over coronal sutures. During metopic craniectomy, Packed cell volume (PCV) blood was given at the rate of 1 ml/m and 30 ml was given to replace the on-going loss as the allowable blood loss was 46 ml. During Sagittal craniectomy, there was profuse oozing that was controlled with surgiseal and gel foam. Simultaneously, bolus transfusion of 40 ml PCV was given, and hemostasis was achieved. 10 ml sodium bicarbonate and 5 ml calcium gluconate were given IV slowly. Surgery lasted for 3.5 h, blood loss was 80 ml, intraoperative fluid maintenance was with Ringer lactate 250 ml and urine output measured was 25 ml. Reversal was done with neostigmine 0.05 mg/kg and glycopyrrolate 0.008 mg/kg and was extubated. Postoperatively child was awake, alert and was shifted to the pediatric intensive care unit for monitoring. Paracetamol 15 mg/kg IV was given 8 hourly for post-operative analgesia [Figure 4].
Craniosynostosis is a complex condition that is most likely caused by multiple is influencing factors. It is the current standard of care to consider early surgical intervention for most forms of craniosynostosis. The various types of surgeries that can be done for craniosynostosis include reconstructive surgeries as done in our case and also endoscopic approach. The goals of Anesthesia include preventing blood loss, hypothermia and venous air embolism. Massive blood loss from the scalp and bony edges along with the rapidity with which it may occur can lead to unstable hemodynamics which we were able to combat by securing a large bore IV access and the timely infusion of blood and fluids. Hypothermia that occurs due to exposure of large surface area and infusion of cold fluids was taken care by adequately covering the baby in aluminum foil and administering warm fluids. Venous air embolism is another dreaded complication which may occur due to the reverse trendelenberg position. In this patient, we secured the airway using a flexometallic tube to prevent kinking during various head movements by the surgeon, and extra precautions were taken to fix the tube to prevent accidental extubation.
The uniqueness of reporting this case is that most patients belong to the young age group, combined with the high likelihood of significant blood loss, hypothermia and venous air embolism make these children some of the most challenging cases for an anesthesiologist. With proper preoperative planning and careful attention to intravascular volume status intraoperatively, patient outcome was excellent.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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