Download Open Reduction And Internal Fixation of Left Radius in Autosomal

Open Reduction And Internal Fixation of Left Radius in Autosomal Recessive
Osteogenesis Imperfecta Type VII
Michael Pence, DO1, Caroline Wright MD2
1Department of Anesthesiology, Walter Reed National Military Medical Center, Bethesda, MD
2Department of Anesthesiology, Children’s National Medical Center, Washington DC
The Patient
6 year old female with past medical history significant
for osteogenesis imperfecta type VII with homozygous
mutation of cartilage associated protein (CRTAP) gene,
restrictive lung disease, developmental delay, GERD,
scoliosis, and G-tube in place to supplement oral feeds.
The patient had undergone multiple procedures
previously for rod placement and hardware removal.
She had a history of PICU admission with BiPAP
support s/p 8 hour rod placement in the past.
Post-Operative
The patient was taken to the PACU after being extubated while deeply anesthetized to minimize
the possibility of subsequent fracture in the setting of restraining the patient upon emergence.
She maintained spontaneous ventilation and did not require additional airway support
postoperatively as a oxygen saturation >93% was maintained. Her pain was well controlled
post-operatively with one additional dose of 5mcg fentanyll in addition to oxycodone 1mg every 6
hours as needed and naproxen 50mg every 12 hours as needed.
Pre-Operative
The patient was to undergo an ORIF of the left radius
after a fall with a subsequent radial fracture. She was
anxious during initial evaluation. She was given a 6mg
dose of midazolam (0.6mg/kg) in pre-op 15 minutes prior
to transport to OR. Despite premedication, the patient
was still anxious upon separation.
Intra-Operative
She was immediately connected to pulse-oximetry upon
entering the operating room and an inhalational induction
was performed with 8% sevoflurane, 70% nitrous oxide,
and 30% oxygen. A blood pressure cuff was applied to
the patient's right arm with neonatal settings to limit the
maximum inflation pressure. The cuff was cycled every
15 minutes to minimize the risk of fracture after the
patient was anesthetized. A 24g peripheral IV was placed
without the use of a tourniquet in the patient's left foot.
She was given 3 separate 10mg doses of propofol
(1mg/kg) prior to laryngoscopy, which was performed
gently with minimal cervical extension. Extra foam
padding was applied underneath the patient during the
procedure. Anesthesia was maintained with sevoflurane
and the patient was given a total dose of 5mcg fentanyl
throughout the case for pain control.
Discussion
This case illustrates the clinical challenges associated
with perioperative care in a patient with osteogenesis
imperfecta undergoing surgery. Extra precaution must be
taken to prevent further fractures in the perioperative
setting. This especially holds true for patients with severe
forms of the disorder like the patient listed in this report.
Short stature and cervical instability present additional
issues that must be considered and can make airway
management and intubation particularly difficult. Two
classic associations with osteogenesis imperfecta type
VII are coxa vara and rhizomelia which were both present
in the above patient1. This genotype demonstrates a
recessive inheritance pattern compared to types I-VI
which demonstrate dominant inheritance and is only seen
in 5-10% of cases2. In addition, pulmonary function must
be taken into consideration based upon the degree of
scoliosis present and chest wall deformities which can
have a significant impact on perioperative management
and ventilation strategies. Osteogenesis imperfecta was
once considered a risk factor for malignant hyperthermia
however; central core disease is the only musculoskeletal
disorder that appears to have a true association with
malignant hyperthermia.
References
Figure 1. (a) shortening of the humeri (rhizomelia). (b) bilateral coxa vara. Bowing
deformity of the lower extremities is evident1.
1.Ward, L.m, F. Rauch, R. Travers, G. Chabot, E.m
Azouz, L. Lalic, P.j Roughley, and F.h Glorieux.
"Osteogenesis Imperfecta Type VII: An Autosomal
Recessive Form of Brittle Bone Disease." Bone 31.1
(2002): 12-18. Web.
2.Osteogenesis Imperfecta Foundation." Osteogenesis
Imperfecta Foundation | OIF.org. Osteogenesis
Imperfecta Foundation, n.d. Web. 16 Jan. 2017.
*Views expressed in this case report are the authors’ and do not reflect the official policy or position of the Department of the Army, Navy, Department of Defense, or the U.S Government.