Keywords Klippel–Feil syndrome - airway management - tracheostomy - intracranial hemorrhages
Introduction
Klippel–Feil syndrome (KFS) is a rare skeletal disorder characterized by abnormal
fusion of two or more cervical vertebrae. Securing the airway in such patients is
challenging and a thorough examination overcomes it. Although awake fiberoptic intubation
is considered ideal for difficult airway (DA), the use of video laryngoscopes in patients
with adequate mouth opening may offer a superior glottis exposure.[1 ] Long-term intensive care unit (ICU) stay, delayed weaning from the ventilator, increased
incidence of reintubation, and difficult tracheostomies are the other challenges in
patients with deviated anatomy. We report a case of hemorrhagic stroke in a patient
with KFS scheduled for emergency decompressive craniotomy and challenges faced in
the perioperative period.
Case Report
A short-statured (height 122 cm; weight 46 kg; body mass index 30.9 kg/m2 ), 43-year-old female with no known medical comorbidities was presented with a history
of sudden onset unresponsiveness followed by altered sensorium. She was a diagnosed
case of KFS and had undergone neck contracture release at 1 year of age. Her family
history was unremarkable. Her Glasgow Coma Scale (GCS) was E2 V3 M4 and pupils were bilaterally equal and reactive to light. Cardiovascular and respiratory
system examinations were unremarkable. Vitals were stable but there was airway obstruction
with tongue fall, requiring emergency endotracheal intubation. Airway examination
revealed a short-webbed neck, mouth opening of 2 finger-breadths, Mallampati grade
4, macroglossia, and complete restriction of movement at the cervical spine. Trachea
was deviated to the right and the tracheal rings were not well appreciated ([Fig. 1A, B ]).
Fig. 1 (A , B ) Anteroposterior (AP) and lateral profile. (C ) Chest radiograph. (D ) Computed tomography (CT) brain showing left external capsular bleed with mass effect.
Chest radiograph showed scoliosis of the thoracic spine and tracheal shift to the
right ([Fig. 1C ]). Noncontrast computerized tomography (CT) of the brain revealed a left external
capsular bleed with midline shift and mass effect ([Fig. 1D ]). The patient was planned for emergency decompressive craniotomy and hematoma evacuation.
Four percent lignocaine 100 mg as nebulization was administered 30 minutes before
surgery.
In the operating room, she was placed in a propped-up position, and an oropharyngeal
airway of size 2 was inserted to prevent tongue fall. After administering injection
fentanyl 50 µg and injection esmolol 25mg, laryngoscopy was performed with Karl Storz
C-MAC video laryngoscope size 3 blade, and the trachea was intubated with styleted
7.0 mm ID flexometallic cuffed endotracheal tube and secured at 15.5 cm after confirming
position. Anesthesia was induced with propofol titrated to response, vecuronium 6 mg,
and fentanyl 80 µg. The intraoperative period was uneventful with the brain being
lax postevacuation of hematoma. Postoperatively, as we decided to electively ventilate,
the flexometallic tube was exchanged with a 7.5-mm ID Portex cuffed endotracheal tube.
She was shifted to neurointensive care for postoperative ventilation and further management.
Antiedema and antiepileptic measures were continued. Over 10 days, neurologically
she improved with a GCS of E4 Vt M5 and mild hemiparesis of the right upper and lower limbs. After spontaneous breathing
trials, she was extubated but had to be reintubated within 3 days of extubation as
she could not maintain oxygen saturation with noninvasive ventilation (NIV). Anticipating
prolonged ventilation, the patient was planned for tracheostomy. Bedside ultrasonography
(USG) of the neck uncovered a deep-seated trachea which was confirmed on CT thorax
(8 cm from skin). It also revealed a fused atlantoaxial joint with a complex segmentation
anomaly of the cervical spine ([Fig. 2A ]). The possibility of sternotomy during tracheostomy was suggested by the otorhinolaryngologist.
Specialized extra-long tracheostomy tubes with adjustable flanges known as Extra TracheoFlex
tubes by RUSCH (Georgia, United States), sizing from 7.5 to 9.5 mm ID with a length
of 15.5 cm ([Fig. 2B ]) was arranged. As it was deemed high risk, the patient's relatives did not consent
for tracheostomy.
Fig. 2 (A ) Computed tomography (CT) thorax—trachea at 8 cm skin depth. (B ) Extra TracheoFlex tube with adjustable flange by RUSCH.
We continued mechanical ventilation with graded intermittent spontaneous breathing
trials for another 10 days along with adequate enteral nutrition, physiotherapy, and
measures to prevent bedsore and deep vein thrombosis. With improved neurological status
and pulmonary function, she was extubated onto preemptive NIV and finally to oxygen
supplementation via facemask. She was discharged and advised to continue physiotherapy,
antiepileptics, and neuroprotective medications.
Discussion
KFS is a congenital skeletal disorder occurring due to abnormal segmentation of the
cervical somites during organogenesis. It was first described by Maurice Klippel and
Andre Feil in 1912.[2 ] The clinical triad of short neck, decreased cervical mobility, and low posterior
hairline is seen only in 40 to 50% of cases.[3 ] Cases are mostly sporadic, few due to autosomal dominant or autosomal recessive
inheritance.[4 ]
Awake fiberoptic intubation is considered the gold standard for the management of
the airway in patients with KFS. Shah et al reported awake fiberoptic intubation in
a KFS parturient with type 1 Arnold-Chiari malformation undergoing elective caesarean
delivery. Conscious sedation was achieved using dexmedetomidine infusion until successful
tracheal intubation.[5 ] Stallmer et al described a series of successful airway management in 10 patients
with KFS, four of them with laryngeal mask airway placement and six patients underwent
endotracheal intubation of which five were accomplished with direct laryngoscopy while
only one patient required fiberoptic intubation.[3 ] The dynamic nature of the airway has been described by Pavani and Krishna, where
the airway was managed by videolaryngoscopy and direct laryngoscopy in the same KFS
patient under different surgical contexts.[6 ] In our patient with a low GCS and compromised airway, sedation was avoided and awake
intubation was achieved with the aid of a videolaryngoscope.
Anthropometric studies using chest radiograph, neck USG, and CT anticipates unconventional
extended-length tracheostomy tubes with adjustable flanges when skin-trachea distance
was > 4.4 cm.[7 ]
[8 ]
[9 ] Ahuja et al reported the use of endotracheal tube in a difficult tracheostomy scenario
arising secondary to surgical emphysema.[10 ] It can be tried in such dire emergencies when specialized tracheostomy tubes are
unavailable. But navigating and securing the tube would be a cumbersome process.
There is a lack of literature about the management of such patients postoperatively.
Weaning and extubation attempts should be gradual and graded with spontaneous breathing
trials and cuff leak tests onto preemptive NIV or high-flow nasal cannula. Attempting
early tracheostomy and weaning off the ventilator decreases their ICU stay, prompting
early mobilization and rehabilitation thereby reducing the incidence of hospital-acquired
infections and other complications.
Conclusion
Even though awake fiberoptic intubation is considered the gold standard for the management
of DA in cases of KFS, other options could also be explored. The use of imaging tools
like plain radiographs, USG, CT, and magnetic resonance imaging of the neck and chest
can help in the planning and execution of intubation. Depending on the neurological
status of the patient, one can anticipate prolonged mechanical ventilation and ICU
care. The use of unconventional extra-long tubes for tracheostomy can be of help in
early weaning and discharge.
