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Neuroscience in Anesthesiology and Perioperative Medicine: Case Report

Intracranial Placement of a Nasotracheal Tube in a Patient with Goldenhar Syndrome Associated with Cribriform Plate Agenesis

Allen, Frederick MD*; Riopelle, James MD; Sinha, Ashish MD, PhD*

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doi: 10.1213/ANE.0b013e3181fca689

Orotracheal intubation is the most common method of securing the airway for most routine surgical cases requiring intubation, but nasotracheal intubation is a viable alternative, and preferred in some instances. Studies suggest that patients who are going to remain mechanically ventilated for a prolonged period of time should be nasally intubated because this method of securing the airway more readily facilitates prolonged ventilator weaning.1 Nasotracheal intubation is favored in patients having oral surgical procedures because it permits excellent access to all intraoral structures while reducing risk of inadvertent displacement of an orotracheal tube. Nasotracheal intubation also permits intraoperative maxillomandibular fixation after surgery for mandibular fracture. In neurosurgical cases in which immediate extubation is not anticipated, nasotracheal intubation should be considered because it is associated with less coughing during emergence, avoiding resulting increases in intracranial pressure.2 Nasotracheal intubation is also frequently used in patients with severely limited oral opening, in whom direct visualization of the larynx using a standard laryngoscope is challenging. We report a case of inadvertent intracranial placement of a nasotracheal tube in a patient with undiagnosed congenital absence of the bony cribriform plate undergoing an oral surgical procedure.


A 32-year-old blind, severely mentally challenged female with cleft lip, cleft palate, and dental caries presented for oral examination under anesthesia and possible multiple dental extractions. The patient described in this report had been abandoned by her natural family at a young age and was, at the time of examination, a ward of the state. Given her severe mental impairment and agitation, a thorough preoperative examination was impossible. Minimal health information was obtained by review of previous medical records and a discussion with the accompanying guardian. Physical assessment demonstrated a severely agitated, poorly nourished, moderately developed female. She was noted to have bilateral anophthalmia, preauricular skin tags, maldevelopment of her external auditory meatus, and a collapsed nasal bridge.

Because of combativeness, the patient was initially sedated using IM ketamine. Subsequently, IV access was obtained, and propofol administered. After confirmation of adequate ventilation, a nondepolarizing neuromuscular blocker was given. The left nostril was serially dilated with lubricated size 28F, 32F, and 34F nasal trumpets. Subsequently, the anesthesiologist attempted blind nasotracheal intubation using a standard 7.0 nasotracheal tube. No significant resistance was encountered. However, during the attempt, a significant volume of brownish fluid was noted to appear in the nasotracheal tube. Operating under the assumption of gastric regurgitation, the oropharynx was suctioned, but no additional fluid was noted. The anesthesiologist then proceeded to leave the nasally placed tube in situ and perform direct laryngoscopy. A full view of the vocal cords was obtained without any evidence of gastric regurgitation. Of note, the physician did not readily observe the nasally placed endotracheal tube in the posterior pharynx. The decision was then made to place an orotracheal tube, which was accomplished without difficulty and mechanical ventilation subsequently initiated. Unsure of the location of the nasotracheal tube, the tube was left in place and an intraoperative cross table skull film ordered. While waiting for the radiology technician to perform the film, the oral surgical procedure was completed without incident and was even completed before the arrival of the radiologic equipment.

The skull radiograph obtained in the operating room demonstrated that the endotracheal tube had traversed a large postnasal cystic cavity and the tip appeared in the cranial vault. An immediate intraoperative otolaryngology consultation was obtained and removal of the tube was recommended. Subsequent to removal, free-flowing cerebrospinal fluid was noted to be leaking from the nares. The nasal cavity was packed with gel-foam and the still-anesthetized patient was taken for an urgent computed tomographic scan. The images demonstrated complete congenital absence of the midline skull base and a nasopharyngeal fluid level, along with associated hemorrhage extending from the nasopharynx to the prepontine cistern (Fig. 1). After the imaging study had been obtained, the young woman was transferred to the intensive care unit where her condition deteriorated. Over the following several days, she developed severe meningitis and diabetes insipidus. She had a ventriculoperitoneal shunt placed but never regained consciousness. Later in her hospital course, a percutaneous endoscopic gastrostomy tube was placed for feeding. This tube migrated into an intraperitoneal position necessitating an emergent laparotomy. When finally discharged to a long-term care facility, on the first day after discharge, she suffered a poorly described “respiratory event” and died.

Figure 1
Figure 1:
Computed tomographic scan of the brain after removal of the nasotracheal tube demonstrating a fluid level in the nasopharynx and absence of the midline skull base structures including the posterior wall of the sphenoid sinus and planum sphenoidale. There is continuous heterogeneous hemorrhage extending from the nasopharynx to the prepontine cistern.


Given the paucity of medical information available to the anesthesiologist preoperatively, we are only able to retrospectively analyze the case. Most likely, the patient in question had a variant of Goldenhar syndrome, also described as “oculo-auriculo-vertebral spectrum.” This rare condition is characterized by a wide-ranging constellation of symptoms that can vary significantly among individual patients.3 Most cases are unilateral, but up to one-third may display bilateral disease. Despite the variable presentation, most abnormalities involve facial soft tissue and bony development.4 Other typical symptoms can include macrostomia, cleft lip and cleft palate, irregularity of the teeth, anotia, microtia, blind ending, or absent external ears, maldevelopment of the middle and inner ear with associated deafness, preauricular tags, wide-ranging ocular manifestations, and potentially mild to severe mental retardation.5 The etiology of observed cases is primarily sporadic, but both autosomal recessive and autosomal dominant inheritance have been observed.6

Although there was no documentation that our patient had previously received a diagnosis consistent with Goldenhar syndrome, the constellation of symptoms found makes it highly likely that she had this disorder. In particular, the preauricular skin tags, deafness, mental retardation, dental abnormalities, blindness, cleft lip, and cleft palate are all consistent with this condition. Although not readily apparent on external examination, the findings on computed tomographic scan demonstrated absence of the bony cribriform plate. This craniofacial abnormality is also consistent with Goldenhar syndrome and the absence of an intact skull base was a primary cause in the intracranial placement of her breathing tube.

Nasotracheal, like orotracheal, intubation can lead to problems including esophageal intubation, but trauma is more likely. Important complications of nasotracheal intubation include epistaxis and turbinectomy.7,8 Rarely, attempting nasotracheal intubation in a patient with recent basilar skull trauma can result in the devastating complication of intracranial placement of a nasally inserted tracheal tube.9 There is a higher likelihood of inadvertent intracranial nasogastric tube placement in cases involving skull base fractures secondary to the smaller caliber and increased stiffness of nasogastric tubes, with >40 cases having been reported.10,11 Similar occurrences have also been reported from attempted nasotracheal intubation in patients who have undergone transphenoidal surgery.12 Because of the cases reported above, disruption of the skull base, whether resulting from trauma or a previous surgical procedure, serves as a contraindication to nasotracheal intubation or nasogastric tube placement.13 A retrospective analysis of inadvertent intracranial nasogastric and nasotracheal cases has shown that in 71% of cases the tube enters the cranial vault through a defect in the cribriform plate. Our case highlights the need for vigilance in the placement of nasogastric and nasotracheal tubes also in patients with congenital abnormalities or malformations of craniofacial development. These patients may, as the patient reported in this case report, have bony defects that increase the risk of intracranial misplacement of nasally introduced tubes. Unfortunately, this is a devastating and frequently fatal complication. Consequences of intracranial placement are associated with a mortality of >50% and frequent litigation.14 The congenital absence of the midline skull base put this patient in a high-risk category for inadvertent intracranial nasotracheal placement. Based on our experience in this case and the medical information currently available, we believe it would be advisable to obtain imaging studies in patients with known or suspected craniofacial abnormalities to confirm intact bony architecture of the skull base before any nonemergent procedure for which nasogastric placement or nasotracheal intubation is planned.


We would like to express our gratitude to Bryan Pukenas, MD, a neuroradiologist at the Hospital of the University of Pennsylvania, for his assistance.


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All authors helped design and conduct the study, analyze the data, and write the manuscript. All authors approved the final manuscript.

© 2011 International Anesthesia Research Society