the general population.
Failure of closure of the left anterior cardinal vein during but culture and gram stains were negative. Palindromic arthritis was considered
cardiac embryonic development results in this anomaly. PLSVC usually drains
into the right atrium via an enlarged coronary sinus. Although often of no clinical
consequence, the condition can result in challenges when attempting to place per-
manent pacemaker/defibrillator leads or central venous lines for therapeutic pur-
poses and hemodynamic monitoring. The transthoracic echocardiogram (TTE)
with agitated saline study is frequently used as a safe and non-invasive technique to
diagnose PLSVC. We report a case of PLSVC, incidentally discovered in a patient
who underwent a routine TTE and was subsequently confirmed during TTE with
agitated saline study, highlighting the role of echocardiography in the diagnosis
of this anomaly. A 76-year-old female underwent a transthoracic echocardiogram
(TTE) for evaluation of cardiac function and was found to have an echo-lucent
structure, crossing in the region of the left atrium, suggestive of a dilated coro-
nary sinus. The presence of PLSVC was suspected and the patient was referred for
TTE with agitated saline contrast study. Initially, the agitated saline was injected
through a vein in the right upper extremity, and the bubbles were visualized in
the right atrium and right ventricle, but not in the coronary sinus. Subsequently,
the injection of agitated saline through a vein in the left upper extremity showed
bubbles, first entering the dilated coronary sinus followed by the right atrium and
right ventricle, which was consistent with the presence of PLSVC. In 80%-90% of
cases, PLSVC coexists with right SVC. In most of these cases, the PLSVC drains
into the right atrium via coronary sinus, resulting in no hemodynamic conse-
quence. PLSVC is typically asymptomatic, although it can accompany other heart
defects including the atrial septal defect, bicuspid aortic valve, and coarctation
of the aorta. Its presence is usually discovered during central venous catheter-
ization, cardiac device implantation or incidentally during routine echocardio-
graphic examination. Although rare, serious complications such as arrhythmia,
cardiogenic shock, cardiac tamponade, and coronary sinus thrombosis have been
reported when pacemaker leads or catheters have been inserted via PLSVC. In
our patient, due to the presence of dilated coronary sinus noted during routine
TTE, a PLSVC was suspected. It was confirmed by doing TTE with agitated saline
study. Imaging modalities such as computed tomography, magnetic resonance
venography, transesophageal echocardiography and transthoracic echocardiogram
with agitated saline study have been used to diagnose venous anomalies. Among
these, TTE with bubble study is a relatively safe and low cost procedure that does
not involve radiation, injection of contrast, or invasive maneuvers. Most patients
who undergo PPM implantation would undergo TTE at some point before the
procedure, and if the diagnosis of PLSVC is confirmed it would allow for better
preparation when elective procedure is planned. It should be noted, however, that
not all patients with PLSVC have such prominent echocardiographic findings.
Pre-procedural diagnosis of PLSVC, when made, could help to prevent hemody-
namic complications, technical difficulties, decrease procedural time, and avoid
subjecting the patient to the possibility of a second incision when inserting the
catheter based devices. As we described in our case, the TTE with bubble study
could safely be used in the diagnosis of PSLVC if the suspicion is high, especially
in cases of a dilated coronary sinus visualized on TTE.
1352
Rare case of histoplasmosis presenting
with recurrent sepsis and hemophagocytic
lymphohistiocytosis
Lokendra Thakur1, Vivek Iyer2; 1Mayo Foundation, Rochester, MN, 2Mayo
Clinic, Rochester, MN
Introduction: A 41 y/o male presented with recurrent high grade fever for 6 weeks
along with generalized weakness and fatigue. He had been recently hospitalized
at an outside institution and treated with IV antibiotics including Vancomycin,
Meropenem and Azithromycin for 5 days. His blood cultures,TEE and imaging
studies were negative. Review of systems was positive for fever and fatigue. His
past medical history was significant for recurrent joint pain, chronic corticosteroid
therapy for asthma skin and soft tissue infection, and morbid obesity. He was
hypotensive (84/47 mmHg), slightly tachycardic (102 bpm), with a normal respi-
ratory rate and oxygen saturation on room air. He looked anxious,fatigued with
dry mucous membranes and cushingoid. Pertinent positive findings on physical
examination were the presence of a soft tissue infection in his left leg, buttock and
left elbow area, mild edema in the lower extremities. He had tenderness over the
left gluteal region and dystrophic toe nails. He was mentating well and cranial
nerves were intact, muscle strength was equal bilaterally. Systemic examination
of heart, lung and abdomen were unremarkable. Initial labs on arrival included
blood and urine cultures which were all negative. Chemistry profile showed hypo-
natremia and an elevated Creatinine at 1.7 mg/dl. Initial imaging studies included
a CT abdomen and pelvis which showed evidence of left gluteal cellulitis and
abscess. I-111 WBC scan showed a multifocal soft tissue infection in the medial
left upper thigh. HIV, Hepatitis, EBV, CMV, HHV6 PCR tests were all negative
Bone marrow was performed on day 8 and showed erythrophagocytosis. Liver
biopsy showed features of hemophagocytosis and narrow-based budding yeasts
within the sinusoids, consistent with Histoplasma species. The initial goal was to
identify the source of sepsis. Left thigh cellulitis was considered as a possible source
Crit Care Med 2013 • Volume 41 • Number 12 (Suppl.)
because of episodic and chronic joint pain but a connective tissue and vasculitis
panel was again negative. Triglycerides, ESR, CRP, and SPEP were negative too.
Adult onset Still's disease, Whipple's disease and hemophagocytic lymphohistiocy-
tosis were then considered due to development of anemia and pancytopenia with
a high serum ferritin of 37630 ng/ml. Bone-marrow and liver biopsies confirmed
hemophagocytosis and left thigh fluid eventually grew Histoplasma, leading to
the diagnosis of secondary HLH. Hemophagocytic lymphohistiocytosis (HLH)
is a multisystem illness characterized by histologic features of hemophagocytosis,
due to a dysregulated immune system that fails to de-activate. It is rapidly fatal in
the absence of prompt diagnosis and treatment. HLH is clinically characterized by
hepatosplenomegaly, cytopenias, and prolonged fevers which are often hectic and
persistent. The clinical presentation can mimic a sepsis syndrome, and co-existent
infection can initially mask the immune dysregulation thus leading to delays in
diagnosis. Disseminated Histoplasmosis as a cause of hemophagocytic syndrome is
a very rare syndrome that has been described in only a handful of cases in the liter-
ature. Histoplasmosis is the most prevalent endemic mycosis in the United States.
Most infections are asymptomatic or self-limited, and around one in 2000 acute
infections results in severe and progressive dissemination. This, usually occurs in
immunodeficient hosts (HIV, malignancy, chronic corticosteroid and immune
modulator therapies). Management of HLH is challenging due to limited data on
whether to treat patients with only antifungals or in combination with immune
suppressive medications. Cases of secondary HLH are increasingly being reported
because of incresing awareness among ICU physicians about the disease process
and improved diagnostic technique. Further studies are needed to establish diag-
nostic and therapeutic guidelines for management of secondary HLH.
1353
Two cases of fixed, dilated pupils in children
receiving isoflurane for status asthmaticus
Cara Lasley1, Jarrod Knudson2, Michael Dallman1, Jennifer Hong3; 1University
of Mississippi Medical Center, Jackson, MS, 2University of Mississippi Medical
Center, Jackson, MS, 3Univerisity of Mississippi Medical Center, Jackson, MS
Introduction: Isoflurane is conventionally used for general anesthesia, but also
has a well-documented off-label use as a bronchodilator for the treatment of
severe, refractory status asthmaticus. Several cases have been described in the
medical literature in which patients receiving prolonged isoflurane therapy for
asthma have experienced untoward neurologic side effects. To our knowledge,
there are no documented cases of bilateral fixed and dilated pupils secondary to
isoflurane therapy in critically-ill children. We report this condition in two asth-
matic patients who received isoflurane. Case One: An 8 year-old male with past
history of asthma was admitted to the pediatric intensive care unit in respiratory
distress secondary to status asthmaticus. The exacerbation was refractory to sup-
plemental oxygen, continuous albuterol, intravenous (IV) steroids, subcutaneous
epinephrine, magnesium sulfate and terbutaline infusion. He ultimately required
intubation and mechanical ventilation. Isoflurane (1.8% exhaled concentration)
was initiated via an anesthesia ventilator. Approximately 12 hours after initiation
of isoflurane, the patient was found to have bilateral dilated (5-6 mm) and non-
reactive pupils. A non-contrast head CT revealed no acute intracranial pathology.
Case Two: A 7 year-old male with past history of asthma presented in severe
respiratory distress with hypoxemia. His initial treatment included subcutane-
ous epinephrine, nebulized albuterol, IV steroids and terbutaline infusion. He
eventually required intubation, and was emergently placed on isoflurane (1.8%
exhaled concentration) via an anesthesia ventilator. The patient was noted to have
bilateral dilated (7-8 mm) and non-reactive pupils within 2 hours of isoflurane
initiation. As in case one, a non-contrast head CT revealed no acute intracranial
pathology. Discussion: In both cases, the children developed pupillary changes
(fixed and dilated pupils) during the initial hours of isoflurane therapy, which
resolved within 24 hours of isoflurane discontinuation. The extent to which iso-
flurane contributed to these findings is unclear. While it is difficult to implicate a
single causative agent underlying the pupillary changes observed in both cases, it
is likely that isoflurane was a contributing factor. Further investigation is needed
to determine the effects of isoflurane administration on pupillary activity.
1354
Propofol Infusion Syndrome: A Critical Care
Catastrophe
Bikram Sharma1,2, Shraddha Goyal3, Amit Sharma4, Dragos Manta5; 1MBBS,
Syracuse, NY, 2SUNY Upstate Medical University, Syracuse, NY, 3MD,SUNY
Upstate medical university, Syracuse, NY, 4State Universit of New York Upstate
Hospital, Syracuse, NY, 5State University of New York Upstate Medical Univer-
sity, Syracuse, NY
Introduction: Propofol is a commonly used sedative agent in ICU due to its
rapid onset of action and recovery. One of its rare but lethal complication is
propofol infusion syndrome (PRIS) which is characterized by severe metabolic
acidosis, rhabdomyolysis, acute renal failure, refractory arrhythmias, myocardial
failure, hepatic dysfunction and hyperlipidemia. Case Presentation: 35-year-old
Caucasian obese male (BMI 35) was admitted to the surgical ICU following exci-
sion of 4th rib due to fibrous dysplasia. Intra-operative course was complicated
due to self- extubation and reintubation with increasing requirement of mainte-
nance sedation. Maintenance propofol was used at a continuous infusion of 120-
160mcg/kg/min. He received propofol for 71 hours. Pertinent laboratory data
revealed creatinine 1.4, total bilirubin 1.3, AST 1006, ALT 907, CPK 12671,
triglyceride 289, serum bicarbonate 18. Arterial blood gas showed pH. He was
hypotensive with new onset refractory atrial fibrillation. These abnormalities can
be seen with sepsis, but elevated CPK could not be explained by infection alone.
There was no evidence of infection. Propofol was stopped with the concern of
PRIS. Continuous infusion of fentanyl and midazolam was used for sedation.
Metabolic acidosis, rhabdomyolysis, liver failure gradually improved with sup-
portive care. He had very slow recovery and was successfully extubated after 15
days of mechanical ventilation. He was discharged to acute rehabilitation facility.
Discussion: Incidence of PRIS is reported to be 1.1%. Though very rare, PRIS
usually runs a catastrophic course with mortality >66%. The exact mechanism of
PRIS is unknown but risk factors include young age, critical illness, obesity, pro-
longed use (>48h), large doses of propofol (>83mcg/kg/min), concomitant use
of catecholamines and steroids. Mainstay of management is discontinuation of
propofol and supportive care. Our patient had most of these risk factors including
obesity, use of large dose with prolonged use. Conclusion: Risk factor stratifica-
tion before initiation, judicious use of propofol, early recognition of the warning
signs and prompt discontinuation of propofol on earliest suspicion of PRIS will
help to prevent this lethal complication.
1355
Cerebral Resuscitation in a Patient With Coma
and Severe Diabetic Ketoacidosis
Penelope Sandiford1, Almas Syed1, Girish Deshpande2; 1Children's Hospital
of Illinois, The University of Illinois College of Medicine at Peoria, Peoria, IL,
2
Children's Hospital of Illinois, The University Of Illinois College Of Medicine-
Peoria, Peoria, IL
Introduction: Cerebral edema is a serious complication of Diabetic Ketoacidosis
(DKA). It develops independently of treatment with intravenous fluids (IVF)
and insulin. Clinically significant cerebral edema complicates only 1% of pedi-
atric DKA but is associated with a very high mortality rate (60 to 90%). Of the
survivors, 15 to 26% have permanent neurologic impairment. Cerebral edema in
DKA (CEDKA) therefore needs to be recognized early and treated aggressively.
We present the case of a 9 year old male with new onset severe DKA presenting
to the emergency room with a Glasgow Coma Score of 7 and fixed dilated pupils.
He was hypothermic (31 degrees C) and hypotensive. The pH, PaCO2 and bicar-
bonate levels were 6.82, 15 torr and 2.9 mmol/L respectively. His urea nitrogen
and creatinine were 44mg.dL and 1.70 mg/dL respectively. The glucose level was
greater than 500 mg/dL. His family was informed of his risk for a poor outcome
including survival with neurologic impairment or brain death. He was sedated,
paralyzed and intubated. Mannitol was given to prevent further reduction in his
cerebral compliance post sedation. He was hyperventilated and the head of his
bed was elevated to 30 degrees. The goal for hyperventilation was a PCO2 of 16
torr, determined using Winter's formula. He was re-warmed only to 35 degrees
C. The mean arterial pressure was maintained with an epinephrine infusion.
Insulin was started at 0.1U/kg/hr. He was given a normal saline (NS) bolus and
IVFs were continued at 1.5 times his maintenance rate. The insulin infusion was
further titrated to 0.14 units/kg/hr to promote reversal of his catabolic state and
a reduction of his ketoacid levels. The serum osmolality was maintained with 3%
NS as his urea nitrogen and glucose levels normalized. Computerized tomogra-
phy of his head post cerebral resuscitation was normal. He regained his baseline
neurologic function and was extubated on day 2 of admission. Prompt recogni-
tion of CEDKA with aggressive cerebral resuscitation can result in neurologic
recovery and a favorable outcome
1356
A Case of Streptococcal Toxic Shock Syndrome
Meghan Rane1, Emerald Banas2, Aakash Aggarwal1, Arman Khorasani-zadeh2,
Badal Kalamkar3; 1SUNY Upstate Medical University, Syracuse, NY, 2N/A, Syra-
cuse, NY, 3SUNY Upstate, Syracuse, NY
Introduction: Streptococcal toxic-shock syndrome (STSS) carries a high mortal-
ity of 39%(1,2) and is defined by a criteria of isolation of Group A Streptococ-
cus (GAS) from a sterile site with hypotension as well as clinical or laboratory
evidence of two or more of the following: renal impairment, coagulopathy, liver
abnormalities, acute respiratory distress syndrome, extensive tissue necrosis or
erythematous rash(3). Almost half the sources of infection are not found. The
mechanism of action is similar to that of Staphylococcal toxic syndrome via exo-
toxin (super antigens) triggering the immune similar to that of an allergic reac-
tion leading widespread vessel dilation and shock with multi-organ failure. A 54
year old male with no significant past medical history comes to the emergency
room with complaints of sudden fatigue, nausea, vomiting, fever and shortness
of breath for the last few hours. In the emergency room, he was noted to be
hypotensive despite adequate fluid resuscitation and tachypnic, which ultimately
required intubation. His physical exam revealed a temperature over 102°F, requir-
ing mechanical ventilation with 100% FiO2 with an oxygen saturation of 90%,
80/50 mmHg and a heart rate of 120 beats per minute. Skin was warm to touch
without any evidence of rash, awake on the ventilator able to follow commands,
clear breath sounds, tachycardic without evidence of murmur, soft abdomen with
no evidence of guarding or rigidity. No bowel sounds were heard. Radiological
pan scan was negative. Notable lab investigations yielded leukopenia, lactic acid
of 9mg/dl, coagulopathy, deranged hepatic profile and renal dysfunction. Blood
cultures yielded Streptococcus pyogenes; other cultures including lumbar, spu-
tum and urine culture were negative. Empiric antibiotics including a beta-lactam
had been started on arrival and the patient continued to deteriorate. When the
blood cultures returned clindamycin was added to supplement penicillin therapy.
Patient stabilized over one week and was transferred out of the intensive care
unit. Streptococcal toxic shock syndrome is distinctly different from Staphylo-
coccal toxic shock syndrome, besides different bacterial genus, in that it is always
associated with a desquamating rash as the prevalent feature and essential for
diagnosis. Rash is not required for STSS. It is essential to remember that both
Streptococcal and Staphylococcal toxic shock syndrome carry a high mortality
rate and that suspicion clinically should warrant a thorough check for source
(foreign body, radiology, cultures) as well as antibiotic therapy with beta lactams
as well as clindamycin. Studies report that a high concentration of GAS leads to
a loss of beta lactam binding sites, thus decreasing its efficacy(4). This highlights
the importance of adding clindamycin, as it is not affected by the concentration
of GAS, suppresses the exotoxin production, increases host phagocytosis and has
been shown retrospectively to be associated with better outcomes(5). References:
1 Darenberg J, Luca-Harari B, Jasir A, et al. Molecular and clinical characteristics
of invasive group A streptococcal infection in Sweden. Clin Infect Dis 2007;
45:450. 2 O'Loughlin RE, Roberson A, Cieslak PR, et al. The epidemiology
of invasive group A streptococcal infection and potential vaccine implications:
United States, 2000-2004. Clin Infect Dis 2007; 45:853. 3 Dennis L. Stevens,
D. Streptococcal Toxic-Shock Syndrome: Spectrum of Disease, Pathogenesis,
and New Concepts in Treatment http://wwwnc.cdc.gov/eid/article/1/3/pdfs/95-
0301.pdf 4 Stevens DL, Yan S, Bryant AE. Penicillin-binding protein expression
at different growth stages determines penicillin efficacy in vitro and in vivo: an
explanation for the inoculum effect. J Infect Dis 1993; 167:1401. 5 Zimbelman
J, Palmer A, Todd J. Improved outcome of clindamycin compared with beta-
lactam antibiotic treatment for invasive Streptococcus pyogenes infection. Pediatr
Infect Dis J 1999; 18:1096.
1357
Single Cannula Venovenous Extracorporeal
Membrane Oxygenation After Total Artificial
Heart Placement
Joseph Miessau1, Michael Baram2, Harrison Pitcher2, john entwistle2, Qiong
Yang2, Hitoshi Hirose2, Nicholas Cavarocchi2; 1Thomas Jefferson University Hos-
pital, Philadelphia, PA, 2Thomas Jefferson University, Philadelphia, PA
Introduction: Percutaneous instrumentation on a patient with the total artificial
heart (TAH) is challenging due to the presence of a mechanical valve in the tricus-
pid position. Any upper extremity wiring requires extreme caution to avoid violat-
ing the mechanical valve. We report a case of unique utilization of a single bi-caval
Avalon cannula (Maquet, Rastatt, Germany) for venovenous (VV) extracorporeal
membrane oxygenation (ECMO) in a patient on TAH. A 22-year-old female with
a dilated cardiomyopathy developed cardiogenic shock complicated by acute renal,
liver, and respiratory failure. She was placed on venoarterial (VA) ECMO for bridge
to decision. During VA ECMO support, her condition was stabilized with continu-
ous venovenous hemodialysis (CVVHD), molecular adsorbents recirculation sys-
tem, and ARDSnet ventilator protocol. The patient failed to wean from VA ECMO
due to severe biventricular failure and we placed the TAH (SynCardia, Tucson, AZ).
During the TAH implantation, the patient developed severe respiratory failure, pre-
sumably from significant alveolar leak secondary to massive fluid and blood resus-
citation. The patient was placed on VV ECMO for oxygenation and ventilation.
A single bi-caval Avalon cannula was placed in the right internal jugular (IJ) vein
percutaneously and the position was confirmed by intraoperative transesophageal
echocardiography and palpation within the operative field. The cannula tip was fixed
in the inferior vena cava using a vessel loop to prevent migration into the TAH.
VV ECMO flow was ~4 L/min and improved oxygenation/ventilation remarkably
without further intervention. CVVHD was initiated in the ICU for fluid control.
After 12 days weaning VV ECMO, the IJ cannula was removed. However, the
patient quickly developed a significant respiratory acidosis; thus, another Avalon
cannula was placed in the iliac vein via femoral access for CO2 clearance only. Our
Crit Care Med 2013 • Volume 41 • Number 12 (Suppl.)