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Continuous Non-Invasive Hemodynamic Monitoring in an Infant with Tetra-Amelia
1. Marissa G. Vadi, MD, MPH
Title: Assistant Professor of Anesthesiology
Affiliation: Department of Anesthesiology; Loma Linda University School of Medicine;
Loma Linda, CA, U.S.A.
Email: [email protected]
Conflicts of Interest: None
2. Elizabeth A. Ghazal, MD
Title: Assistant Professor of Anesthesiology
Affiliation: Department of Anesthesiology; Loma Linda University School of Medicine;
Loma Linda, CA, U.S.A.
Email: [email protected]
Conflicts of Interest: None
3. Mathew R. Malkin, MD
Title: Assistant Professor of Anesthesiology
Affiliation: Department of Anesthesiology; Loma Linda University School of Medicine;
Loma Linda, CA, U.S.A.
Email: [email protected]
Conflicts of Interest: None
4. Abisola Ayodeji, MD
Title: Pediatric Anesthesiology Fellow
Affiliation: Department of Anesthesiology; Loma Linda University School of Medicine;
Loma Linda, CA, U.S.A
Email: [email protected]
Conflicts of Interest: None
5. Richard L. Applegate II, MD
Title: Professor of Anesthesiology
Affiliation: Department of Anesthesiology and Pain Medicine; University of California
Davis, CA, U.S.A.
Email: [email protected]
Conflicts of Interest: None
Manuscript (All Manuscript Text Pages in MS Word format,
including Title Page, References and Figure Legends)
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Name of Department(s) and Institution(s): Department of Anesthesiology, Loma Linda
University School of Medicine, Loma Linda, CA, U.S.A. and Department of Anesthesiology and
Pain Medicine; University of California Davis, CA, U.S.A.
Short Title: Hemodynamic Monitoring in Tetra-Amelia
Corresponding Author:
Marissa G. Vadi, MD, MPH
Department of Anesthesiology
Loma Linda University School of Medicine
11234 Anderson Street, Room 2532
Loma Linda, CA 92354
Phone: 909-558-4475
Fax: 909-558-4143
Email: [email protected]
Word Counts: Abstract - 74; Introduction - 82; Case Description - 454; Discussion – 565
Key Words: case report; tetra-amelia; hemodynamic monitoring; pediatric anesthesia
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Abstract
Tetra-amelia syndrome is a congenital disorder associated with near or complete absence
of all four limbs. Non-invasive hemodynamic monitoring may be difficult or impossible in such
patients. We describe the use of a finger cuff blood pressure system for continuous non-invasive
blood pressure monitoring in an infant with near-complete tetra-amelia undergoing laparoscopic
gastrostomy tube placement. This case suggests the potential use of such a blood pressure
monitoring system for other patients with small extremities.
Number of words: 74
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Introduction
Tetra-amelia syndrome is a rare congenital disorder characterized by the absence of all
four limbs with other associated craniofacial, cardiopulmonary, neurologic, or urogenital
anomalies. The absence of limbs may render non-invasive blood pressure (NIBP) measurement
with traditional blood pressure cuffs impossible. We report a case of continuous non-invasive
hemodynamic monitoring in an infant with tetra-amelia using a finger cuff blood pressure
measurement system (ClearSight
TM
System, Edwards, Irvine, CA, USA). The patient's mother
reviewed the case report and provided written consent for publication.
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Case Description
An 11-week-old, 4.2 kg infant with near complete tetra-amelia, sacral dysgenesis, and
congenital micrognathia, hospitalized since birth at 39 weeks gestation in the Neonatal Intensive
Care Unit (NICU) of our tertiary care hospital, was scheduled for laparoscopic gastrostomy tube
placement secondary to poor nippling and failure to thrive. Prior surgical procedures included
mandibular distractor placement and insertion of a single lumen tunneled venous catheter at age
3 weeks, at which time fiberoptic intubation was performed with difficulty. The infant's bilateral
upper extremities and right lower extremity were completely absent, with only a rudimentary left
lower extremity appendage present (Figure 1). Hemodynamic monitoring was previously
achieved via umbilical artery catheterization, which had been discontinued due to thrombotic
risk several weeks prior to the day of gastrostomy surgery. Subsequent attempts at NIBP
monitoring were unsuccessful, as standard neonatal blood pressure cuffs did not fit the patient's
left lower extremity appendage. Pulse oximetry measurements were obtained from the distal
appendage (Figure 1).
A multidisciplinary discussion was held between pediatric anesthesiology, neonatology,
and pediatric surgery to determine the safest plan for intra- and post-operative hemodynamic
monitoring. Full mandibular distraction had not yet been achieved, and there was a concern that
airway management attempts might be protracted. This concern heightened the desire to have
reliable hemodynamic measurements. The patient was deemed a poor candidate for invasive
arterial pressure monitoring after ultrasound examination revealed an absent right femoral artery,
with underdeveloped left femoral and bilateral axillary arteries. Cerebral near-infrared
spectroscopy (NIRS) was considered as a means to monitor trends in cerebral blood flow, but an
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appropriate sized sensor for this patient to allow use of this technology was not readily available
in our operating room.
The circumference of the patient's left lower extremity appendage was noted to be similar
to that of an adult index finger. A size small finger cuff (ClearSight™ System, Edwards
Lifesciences, Irvine, CA, USA), used in adults for non-invasive measurement of finger arterial
pressure, was placed on the left lower extremity appendage pre-operatively while the infant was
still in the NICU (Figure 2) and a reconstructed arterial pulse pressure waveform was obtained
(Figure 3). The patient, with the finger cuff blood pressure measurement system in place, was
then taken to the operating room for gastrostomy tube placement. After induction of general
anesthesia, spontaneous ventilation was maintained and a 3.5 uncuffed endotracheal tube was
placed with moderate difficulty via video laryngoscope-assisted flexible fiberoptic intubation
after 10 minutes of airway management. The anesthetic course was otherwise unremarkable.
Continuous non-invasive blood pressure measurement was provided by the finger cuff system
during the 45-minute surgical procedure and for 2 hours post-operatively. There was no
evidence of vascular compromise or other injury to the left lower extremity appendage after cuff
discontinuation.
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Discussion
Patients with severe limb abnormalities such as tetra-amelia present unique challenges to
the anesthesiologist, as standard monitor placement is often problematic. Invasive blood
pressure monitoring may be employed in such cases, but frequently target arteries are absent,
aberrant, or underdeveloped. Superficial temporal artery cannulation via surgical cut-down
under ketamine sedation has been described for hemodynamic monitoring during spinal fusion in
a teenager with tetra-amelia.
1
However, this procedure would be difficult to accomplish in a
young, uncooperative child without sedation or general anesthesia and thus was not attempted in
our infant patient with a suspected difficult airway.
Few reports outline alternative strategies for non-invasive blood pressure monitoring in
patients with significant limb deformities. Temporary penile blood pressure monitoring was
used in a 3-year-old boy with severely shortened limbs unable to accomodate a standard blood
pressure cuff.
2
Only intermittent cuff inflation was allowed and periodic penile inspection was
required to rule out penile ischemia. After a 2-hour period, a femoral arterial catheter was placed
with good correlation between the blood pressure cuff and arterial catheter. In the case we report,
this would not have been a feasible option based on the patient’s size.
General anesthetics have been performed in tetra-amelia patients with hemodynamic
monitoring via carotid arterial Doppler
3
and transesophageal Doppler.
4
However, changes in
Doppler sounds or pattern only allow for an approximation of blood flow trends, not adequacy of
blood pressure, and the ability to detect subtle variations in sound intensity may vary by type of
equipment and by operator. The use of NIRS to measure cerebral and somatic oxygentation
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trends in a patient with tera-amelia was described in a case report.
1
The authors hypothesized that
a sustained drop in oxygenation is related to ischemic events that could be due to hypotension or
blood loss. The primary use of NIRS in anesthetized infants and children has been to monitor
cerebral oxygenation in children undergoing cardiac surgery but not as a sole monitor of
perfusion adequacy.
The system we employed uses a pneumatic finger cuff and infrared technology to detect
pulsations that appear to correlate to blood pressure readings. This technology shows promising
results in pediatric studies but has yet to be validated in the neonatal population.
5
Although this
technology may only monitor trends in patients such as ours, a provider may be able to use this
to guide management in short cases where the use of traditional monitoring devices may not be
feasible due to small extremity size. The minimum cuff size available and the need for adequate
arterial pulsatility limit use in very small infants. Each cuff can only be used once, so cost of
system usage increases if more than on cuff is required per anesthestic. Long term use should be
avoided in small infants as there is an increased risk of limb edema and ischemia distal to the
cuff position.
5
In conclusion, routine monitoring during anesthesia in patients with small or malformed
extremities can pose a significant challenge to anesthesia providers. This case illustrates that use
of a continuous noninvasive finger blood pressure system may allow successful blood pressure
monitoring in infants with these conditions, and may be an alternative to continuous invasive
blood pressure monitoring in short procedures without significant blood loss. Such systems
1
Anesthetic Management of a Patient with Tetra-Amelia Syndrome. Available at:
https://www.webmedcentral.com/wmcpdf/Article_WMC00592.pdf. Accessed 3/7/16.
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could potentially replace arterial cannulation in children who do not have shock or hemorrhage
conditions. Further studies are required to validate this technology in the pediatric population.
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Figure Legends
Figure 1: Frontal View of Patient
The patient was born with complete absence of the bilateral upper extremities and right lower
extremity. Only a rudimentary left lower extremity appendage was present. Mandibular
distractors were previously placed for correction of severe micrognathia and remained in situ at
the time of presentation for laparoscopic gastrostomy tube placement.
Figure 2: Finger Cuff Positioning
A size small finger cuff (ClearSight™ System, Edwards Lifesciences, Irvine, CA, USA) was
placed on the distal portion of the patient’s left lower extremity appendage. Pulse oximetry
measurements were obtained from the proximal portion of the left lower extremity appendage.
Figure 3: Arterial Pulse Pressure Waveform
The reconstructed arterial pulse pressure waveform obtained from the left lower extremity
appendage finger cuff is displayed above. This waveform was monitored throughout the
intraoperative and postoperative period.
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References
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monitoring and anesthetic management for spinal fusion in an amelic patient. Journal of
pediatric orthopedics 1982;2:299-301.
2. Gurnaney H, Ganesh A, Storm PB. Penile blood pressure monitoring for a pediatric
patient with hypomelia. Anesth Analg 2010;111:1328.
3. Tallmeister A, Sheehan MM, Pelton DA. Dental anaesthesia for a child with complete
amelia. Can Anaesth Soc J 1986;33:484-7.
4. Mukhtar K, Jayaseelan S, Allsop E. Anaesthesia and orphan disease: phocomelia--a
lesson from the past. Eur J Anaesthesiol 2012;29:353-4.
5. Garnier RP, van der Spoel AG, Sibarani-Ponsen R, Markhorst DG, Boer C. Level of
agreement between Nexfin non-invasive arterial pressure with invasive arterial pressure
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Figure 1 Click here to download Figure Figure 1.tiff
Figure 2 Click here to download Figure Figure 2.tiff
Figure 3 Click here to download Figure Figure 3.tiff