United States

Background. Carotid artery dissection is a major cause of stroke in patients younger than 40 years old, accounting for up to 25% of ischemic cerebral events that lead to a long-term disability and a large economic burden. The clinical picture of this condition varies widely, thus, the disease may be easily misdiagnosed. We present a case of spontaneous left carotid artery dissection in a previously healthy 29-year-old female. Our aim is to stress on the presentation and a diagnostic approach of this condition, as prompt diagnosis and, thus, early treatment decrease the rate of a stroke and its consequences in this age population.
Case Presentation. A 29-year-old female presented to our clinic with the presumptive diagnosis of first manifestation of migraine, complaining of moderate unilateral headache, involving the left half of the head and neck, including periorbital region, exacerbated by physical activity. However, the clinical presentation of migraine was atypical, including the absence of aura, persistent character of pain, inadequate response to therapy. A decision to perform carotid artery ultrasound was made, revealing a hypoechoic thrombus, completely occluding the lumen of the left internal carotid artery, arising from posterior aspect of the left common carotid artery bifurcation. On color doppler, blood flow in the internal carotid artery was not visualized. MRA of the brain revealed dissection of the left carotid artery with the false lumen that has been completely clotted. A true lumen, 2 mm in diameter, also contained thrombus. The patient started anticoagulant therapy that lasted for 8 weeks. Our patient had a favorable outcome: symptoms were resolved and the blood flow was restored due to complete recanalization of the thrombus.
Discussion. In young adults, carotid artery dissection may occur spontaneously, thus, may be missed or misdiagnosed as other more common conditions, such as migraine, cluster or tension headache. Carotid artery dissection may cause serious complications (TIA, stroke, or one-sided blindness), so it should be included into the differential diagnosis of headache. Early diagnosis is imperative to initiate appropriate therapy that improves patients’ prognosis. More studies are needed to improve diagnostic approach and outcomes in this group of patients.


AMA Research Challenge 2022

Spontaneous carotid artery dissection presenting with migraine-like headache in a young female: a case report.

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poster

Background
Fetal tachyarrhythmia presents in 0.4%-0.6% of pregnancies. Possible causes include cardiac structural abnormalities, atrioventricular node dysfunction, and atrioventricular re-entry tachycardia (AVRT). It is believed that around 41% of fetal supraventricular tachycardia (SVT) cases will progress to hydrops fetalis, making early identification and treatment imperative. This case illustrates challenges of managing fetal SVT as well as various avenues of treatment to consider in refractory cases.
Case Presentation
This patient is a 27-year-old G4P2012 female with a history of epilepsy controlled on Lamictal. After 32 weeks of uncomplicated prenatal exams, fetal tachycardia at 220-240 bpm and fetal pericardial effusion were found incidentally. Initial treatments with sotalol for 2 days, followed by digoxin and sotalol for 4 days were unsuccessful. On day 7, cordocentesis with adenosine was attempted unsuccessfully. Finally, flecainide in combination with sotalol led to spontaneous cardioversion. On day 10, prolonged PR and QRS intervals were noted. Sotalol was discontinued and by hospital day 14 the PR interval normalized. Three weeks later the patient was readmitted for scheduled induction of labor using Pitocin, and vaginal delivery at 36 weeks and 6 days was uncomplicated. The neonate required 5 days of NICU evaluation with escalating doses of propranolol from 1mg to 5.85mg daily in order to suppress SVT recurrences. The infant was discharged on 2mg propranolol twice daily with continuous fetal heart rate monitoring. On posthospital day 7 the baby was evaluated by pediatric cardiology and was diagnosed with Wolff- Parkinson-White (WPW) syndrome. Propranolol was continued with a plan for eventual ablation.
Discussion
SVT with ventricular preexcitation, specifically AVRT, is typically the presenting arrhythmia of WPW. Clinical manifestations can be recognized in utero, though most cases are not diagnosed until early childhood. Hydrops fetalis may develop if the tachyarrhythmia persists for greater than 6-12 hours. Management strategies include pharmacologic therapy, vagal stimulation, and ablation. Many options exist within the pharmacologic treatment category alone, and various combinations may be considered. Due to the critical nature of these cases, there should be strong familiarity with treatment pathways for refractory cases to ensure patients are managed appropriately and quickly enough to avoid morbidity and


A Rare and Challenging Case of Refractory Fetal Supraventricular Tachycardia

Background:
Local anesthetics such as lidocaine and other sodium channel blockers are commonly used in obstetrics for laceration repair, epidurals, and spinal blocks. Although rare, the most common adverse effects from these medications result from drug toxicity or allergic reactions. Numerous studies in dentistry, dermatology, and emergency medicine have shown that a 1% diphenhydramine solution is comparable in anesthetic effects to lidocaine for minor laceration repairs, however no cases have been described in obstetrical literature. 

Case Presentation:
A 23-year-old gravida 1 with a reported history of an anaphylactic reaction to lidocaine and confirmatory allergy testing in Venezuela presented to Labor and Delivery for induction of labor. She strongly desired an epidural for pain control; however, our hospital was unable to offer a benzoic acid ester sodium channel blocker as an alternative to lidocaine. After fully discussing options, she agreed to an induction of labor with IV pain meds and potential use of 1% diphenhydramine local injection for laceration repair after delivery. She had a spontaneous vaginal delivery with a 2nd degree perineal laceration. The physician created a 1% diphenhydramine solution using 2mL of 5% diphenhydramine (50mg/mL) provided by the pharmacy combined with 8mL of normal saline. She then injected the solution into the vaginal tissue and perineal skin along the torn edges. The repair was done in the usual fashion. She declined any IV pain meds before or during the laceration repair and tolerated the procedure well. She reported satisfactory pain control with diphenhydramine.

Discussion
Lacerations are commonly encountered during vaginal deliveries, with 53-79% of women experiencing some form. Adequate analgesia is an essential repair component, whether via epidural or local anesthetic injection. Diphenhydramine is primarily an H1 receptor antagonist with sodium channel blocking properties similar to the amide and benzoic acid ester local anesthetics, thus giving comparable anesthesia. Although rare, lidocaine allergies pose a significant problem for obstetric patients requiring laceration repair following a delivery. Currently, there are no clear guidelines on how to proceed with a laceration repair for a patient with a true lidocaine allergy. Therefore, having an available alternative option for these patients is vital. 



Analgesia for obstetric lacerations in patients with known lidocaine allergy; local injection with 1% diphenhydramine.

Background 
Cervical cancer (CC) is one of the most frequently diagnosed antepartum gynecologic malignancies. Treatment of antepartum CC varies based on stage, gestational age at diagnosis, and patient preference for continuation or termination of pregnancy. The purpose of this case report is to demonstrate an aberrant presentation of CC and describe clinical reasoning for determining medical management. 
Case Presentation 
A 31-year-old G5P5 presented to the Emergency Department with acute profuse vaginal bleeding. Her last menstrual period was unknown, and she denied using contraception. Quantitative HCG was 8,197 mlU/mL and bedside ultrasound showed a 21w5d fetus with cardiac activity. Patient was unaware of the pregnancy. 
Initial pelvic exam showed unknown tissue that easily detached from cervix. This tissue was confirmed to be stage IIIC1 squamous cell carcinoma of the cervix. Patient desired treatment that would prioritize neonatal survival and was started on weekly chemotherapy with the intent of delaying delivery until viability. 
The patient delivered at 32w3d, and postpartum lymphatic pathology was negative for metastases. Patient had uncomplicated postpartum care and resumed chemotherapy shortly after. 
Discussion 
Medical management for antepartum CC aims to balance treatment of the patient while minimizing harm to the fetus. This patient prioritized fetal survival, but due to the advanced stage of the cancer, it was crucial for her to receive prompt oncologic treatment. Due to later gestational age, there was decreased risk for chemotherapy related fetotoxicity. Future research can attempt to determine long term side effects of second and third trimester antepartum chemotherapy. 


Cervical cancer presenting as an inevitable abortion: determining treatment for new onset cervical cancer in a previously unknown second trimester pregnancy

Uterine rupture, caused by separation of the uterine myometrium, is a rare but life-threatening complication of pregnancy that has increased in incidence over the past two decades alongside the increased rate of cesarean delivery. Uterine rupture is most often correlated with a prior cesarean delivery, prior uterine surgery, or abnormal placentation such as placenta increta, placenta percreta, or placenta accreta. It can also be associated with a short interval pregnancy of less than 6 months, prior uterine rupture, or induction of labor. A thirty-four-year-old G7P3123 African American female with a history of four prior cesarean sections and a current a viable intrauterine pregnancy at 16 weeks and 2 days by bedside ultrasound presented in critical condition to the Emergency Department at the University of Louisville with sudden onset abdominal pain following intercourse. On presentation, she was hypotensive with a blood pressure of 68/30 mmHg. Pulse was 88 beats/minute with lack of tachycardia secondarily to use of a beta blocker for chronic hypertension. Respiratory rate was 24 times per minute, and oxygen saturation by pulse oximetry (Sp02) was 100% on room air. The patient on physical exam looked pale and abdomen was distended showing clinical signs of peritonism, rebound tenderness, and guarding. She was conscious but disorientated. Early diagnosis and appropriate management are essential to decrease the high maternal and fetal morbidity and mortality rates associated with uterine rupture. High clinical suspicion and early surgical intervention with a low threshold to perform a laparotomy is fundamental to successful management. The decision for method of surgical intervention should be made on a case-by-case basis depending upon patient wishes, hemodynamic stability, as well as hospital resources for mother and a potentially premature infant. For uncontrollable, life-threatening bleeding hysterectomy should be considered. In other circumstances, decision for uterine evacuation and repair versus uterine repair alone should weigh the risk and benefits of each intervention on patient outcomes.

High Index of Clinical Suspicion for a Spontaneous Uterine Rupture in the Early Second Trimester

Protein S Deficiency in Pregnancy

Background: Herpes infections can be dormant and recurrent in patients and in the eye, can cause corneal scarring. This coupled with cataracts in patients leaves them blinded with no treatment options since the herpetic scars will reoccur if removed. 
 
Case Presentation: A 72 year old Caucasian female was referred with a Herpetic corneal scar post a prior LASIK surgery. Her best corrected vision was a distorted 20/60 with an irregular cornea. Using Excimer Laser Corneoplastique™, her cornea was brought to a measurable outcome which then allowed her to undergo accurate premium cataract surgery. 
 
Discussion: By using a two step surgical method, the patient was able to achieve emmetropia, a feat normally not possible for most herpes patients. Each problem was separately addressed: first the corneal scar secondary to herpes, and next the cataracts with a Corneoplastique technique.

Converting Irregular Herpes Cornea to Regular for Accurate Premium Cataract Surgery to 20/20

Abstract Title: Impact of Pregnancy on Idiopathic Intracranial Hypertension: A Case Series of 13 Patients
Richard Pellizzari BS, Nathan Lambert-Cheatham DO, Nathaniel Pasmanter DO, PhD, Beom Lee BS, Neysa Miller BS, Lina Nagia DO, David Kaufman DO

Background
Idiopathic Intracranial Hypertension (IIH) is a syndrome in which intracranial pressure is elevated due to unknown causes leading to papilledema and potentially permanent vision loss. Due to the association of weight gain to worsening papilledema, it has been believed that pregnancy induced weight gain could worsen IIH exacerbations. Current literature has challenged this assertion. These findings have raised questions about the impact of pregnancy in IIH and how this differs from weight gain outside the context of pregnancy. To explore these questions, our study reviews the changes in papilledema and symptoms in pregnant IIH patients at Michigan State University.

Methods
Charts were reviewed for patients who had diagnoses of IIH, had at least two clinic visits with neuro-ophthalmology, were seen during pregnancy, and did not have prior surgery for IIH. Data collected included demographics, weight/BMI, gestation, diagnostic studies (OCTs, Humphrey visual fields, head imaging, lumbar punctures), clinical tests (visual acuity, color vision, alignments, disc edema grade), and treatments. For analysis, data collected during pregnancy was compared to data from their initial IIH diagnosis, their baseline exam prior to pregnancy, and their first clinical visit after delivery.

Results
13 patients were identified who met selection criteria. 11/13 patients gained more than their previous exacerbation weight. 8/13 patients gained more weight than their goal during pregnancy. 6/13 had pharmacologic therapy for IIH discontinued during pregnancy. 3/13 patients developed a clinically significant increase in disc edema during pregnancy. 2/3 of the patients developed optic nerve atrophy and new visual field defects post pregnancy.

Conclusion
In our case series, most patients had risk factors for worsening papilledema including increased weight from initial exacerbation, weight gains over goal for pregnancy, and discontinuation of pharmacologic therapy. With these risk factors, we would expect most patients to have worsening papilledema. Despite this, only 3 patients had worsening papilledema. This finding suggest that weight gain during pregnancy likely has less impact on papilledema than traditional weight gain in IIH.

Impact of Pregnancy Weight Gain on Papilledema and Vision in IIH Patients: A Case Series


Abstract Title: MOG-Positive Optic Neuritis In A Child Presenting With Bilateral Disc Edema And Pre-Retinal Hemorrhage

Rasika Sudharshan1, Mark S. Borchert2, Melinda Y. Chang2
1USC Keck School of Medicine, 2Children’s Hospital Los Angeles, Department of Ophthalmology 

Background
Our case discusses an 8-year-old male with no relevant past medical history or trauma, who presented with two weeks of progressive bilateral vision loss that acutely worsened over 3 days. This case demonstrates typical features of optic neuritis in children, including bilaterality, severe vision loss, disc edema, and the atypical finding of extensive retinal and preretinal hemorrhages in the macula. 

Case Presentation
On presentation, his visual acuity was light perception with dilated and poorly reactive pupils. Fundus exam showed bilateral optic disc edema (with pallor) and retinal venous tortuosity without cotton wool spots. Pupil exam showed a right RAPD. His left fundus exam was also significant for preretinal and intraretinal hemorrhages. Lumbar puncture showed an elevated opening pressure (36mmH2O) with normal glucose, abnormal protein at 77, and mildly elevated pleocytosis at 12. Brain MRI showed an incidental small posterior fossa arachnoid cyst but no white matter lesions, and orbit MRI showed bilateral optic nerve enhancement consistent with optic neuritis, right greater than left. Lab tests showed a normal CBC and a MOG-Ab positive titer level of 1:100. 

After six doses of IV methylprednisolone and completion of five days of plasmapheresis, visual acuity improved to 10/200 OD and 20/100 OS. Then, after three rounds of IVIG and prednisone taper, his visual acuity improved to 20/80 and 20/30. Forty-eight days after treatment, bilateral optic disc edema and retinal hemorrhages resolved. His final examination showed bilateral optic atrophy OU and visual acuity of 20/40 OD and 20/20 OS. 

Discussion
Due to increased intracranial pressure (ICP) on lumbar puncture and intra- and preretinal hemorrhages, papilledema or leukemia were considered. Increased ICP has not been consistently reported in patients with MOG-Ab ON, and ICP was believed to be falsely elevated in this case due to excessive hypercapnia associated with sedation. Retinal hemorrhages are rare in optic neuritis, which typically occurs without disc edema in adults. In children, optic neuritis may be associated with disc edema and peripapillary hemorrhages in severe cases, especially in those with MOG-Ab positivity. This is the first case, to our knowledge, of preretinal hemorrhage in the macula associated with MOG-Ab positive optic neuritis.


MOG-Positive Optic Neuritis In A Child Presenting With Bilateral Disc Edema And Pre-Retinal Hemorrhage

Background: Pigmentary retinal dystrophy (PRD) is a group of inherited disorders involving the progressive degeneration of rod and cone photoreceptors and the retinal pigment epithelium (RPE), which can progress to pigmentary retinopathy (PR). We present a case of PRD in a female pediatric patient with mutations in the PRPH2 and PEX1 genes.

Case Presentation: A 4-year-old female patient presented to the pediatric ophthalmologist with concern for developmental delay and “feeling around for objects” despite full-time glasses wear. Comprehensive evaluation revealed visual acuities of 20/180 in the right eye and 20/270 in the left eye via Teller cards, hyperopia with astigmatism, extensive retinal pigmentation 360° in the mid-periphery, and decreased foveal reflexes that were concerning for macular edema bilaterally. Optical Coherence Tomography of the macula revealed thickened retinal segments and schisis/edema of the outer retinal segments. The patient was also diagnosed with bilateral sensorineural hearing loss. Subsequent genetic evaluation yielded pathogenic homozygous mutation for the PEX1 c.2528G>A, p. (Gly843Asp) gene and a heterozygous mutation for the PRPH2 c.806C>G, p. (Thr269Arg) gene. Treatment for severe macular edema has included topical dorzolamide and intravitreal dexamethasone injection with successful improvement in macular edema and vision.


Discussion: The PEX1 gene mutation is associated with peroxisome biogenesis disorder-Zellweger syndrome spectrum (PBD-ZSS) and resulting retinal dystrophies. Furthermore, the PRPH2 mutation may play a role in macular edema and PRD, as it is implicated in macular degeneration, choroid defects, and photoreceptor dysfunction. This case demonstrates the potential for multiple gene etiologies in a single patient with PRD and the significant resulting visual impairment. Ocular findings in historical cases of PBD-ZSS have consistently included loss of photoreceptors, RPE, and ganglion cells. Retinal pigment dispersion, optic disc pallor and hypoplasia, and narrowed retinal arterioles were also consistently observed. PBD-ZSS has less consistently been associated with cataracts, glaucoma, and corneal clouding. Treatment remains supportive, aiming to control macular edema and correct refractive errors.

Retinal Dystrophy associated with Peroxisome Biogenesis Disorder-Zellweger Syndrome Spectrum

Background: Klebsiella pneumoniae, a gram-negative anaerobic rod-shaped bacterium, is a rare cause of endogenous endophthalmitis. We describe a case of Klebsiella endophthalmitis presenting with a uniquely rapid time course and evidence of intraocular abscesses.

Case Presentation: 
A 37-year-old Hispanic male, with no known ocular history and a past medical history significant for type 2 diabetes mellitus and recent diagnosis of COVID19, was admitted to the hospital with shortness of breath, chest pain and hyperglycemia. Ophthalmology was consulted due to subjective change in vision in the right eye. On examination, his near visual acuity was 20/40 and 20/25 in the right eye and left eye, respectively. Funduscopic exam was notable for mild macular edema with exudates and scattered intraretinal hemorrhages in the right eye, which was attributed to diabetic retinopathy. 
One day after the ophthalmologic consultation, the patient acutely decompensated requiring intubation and transfer to the MICU. Blood cultures grew gram negative rods speciating to Klebsiella. Two days later, he was noted to have worsening redness in the right eye, and ophthalmology was re-consulted. Fundoscopic exam at this time was limited, but showed inferonasal white-centered hemorrhages. B-scan ultrasound demonstrated vitreous debris with a grossly attached retina. He was started on treatment for endophthalmitis with intravitreal antibiotic injections. 
Subsequent repeat examinations showed continued decreased visual acuity to light perception (LP), then no light perception in the right eye and development of a near total retinal detachment. Goals of care were discussed, and a decision was made to focus on comfort measures for the right eye, less than a month after his initial examination.

Discussion: In contrast to previously published case reports, our patient had a bedside visual acuity of 20/40 upon initial consultation which, within six days of the initial evaluation, worsened to LP. This rapid deterioration of vision is likely a function of the bacteria’s quick doubling time of 2.31 hours. Due to the rapidly progressive nature of Klebsiella endogenous endophthalmitis, we recommend to primary teams to consult ophthalmology for daily funduscopic examinations once blood cultures have confirmed the causative organism.


Next from AMA Research Challenge 2022

A Rare and Challenging Case of Refractory Fetal Supraventricular Tachycardia

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