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Summarize the treatment options for a patient with Paget Schroetter syndrome. Explain modalities to improve care coordination among interprofessional team members in order to improve outcomes for patients affected by Paget Schroetter syndrome. Access free multiple choice questions on this topic. The description of this entity dates back to when Sir James Paget reported a case of spontaneous thrombosis of the subclavian vein in a patient.

In , von Schroetter hypothesized that the condition was a consequence of injury to the vein from repetitive musculoskeletal motion. The subclavian vein travels in the proximity of the clavicle, first rib, anterior scalene, and subclavius muscles.

PSS is related to the compression and subsequent thrombosis of the subclavian vein due to these structures. PSS is more commonly seen in younger patients in their 20s and 30s with a male to female ratio of Often these patients will not have any other significant medical comorbidities. The incidence ranges from 1 to 2 per , individuals per year in various studies. It is more commonly encountered on the right side. Often affected individuals with PSS will be athletes, specifically, those engaged in the repetitive over-the-head motion of their arms, including baseball or softball pitchers, swimmers, rowers, or weightlifters.

Repetitive strain from vigorous physical activity and compression of the subclavian vein from adjacent anatomic structures leads to venous injury and subsequent thrombosis. This impedes the venous return from the affected extremity leading to edema. Over time, the body forms collaterals to bypass the venous obstruction. In the chronic phase, the vein becomes fibrotic. Surrounding inflammatory changes from the thrombosis lead to scar tissue formation.

Patients may present with upper extremity swelling and pain. The presentation of these symptoms can be acute, subacute, or chronic. For the onset of symptoms in PSS, a history of an antecedent strenuous exercise can usually be elicited from the patients.

On examination, the upper extremity will demonstrate edema with an enlarged girth of the extremity compared to the contralateral side. The upper extremity may be tense and cyanotic. A convenient non-invasive first test is ultrasonography of the upper extremities. This will demonstrate thrombus in the deep venous system. Acute thrombus will be non-compressible on ultrasound. A vein with chronic thrombus will have an irregular and more collapsed appearance with the development of collaterals on ultrasound.

Ultrasonography will not allow for complete demonstration of the central veins. Computed tomography CT venography and magnetic resonance MR venography can demonstrate thrombus in the venous system as well. Contrast venography is a more invasive test that can demonstrate patency of the central venous system. Laboratory workup includes a thrombophilia panel protein C and S levels, antithrombin levels, prothrombin gene mutation analysis, factor V Leiden mutation testing, among others and D-dimer levels.

D-dimer levels will be elevated in a patient with deep venous thrombosis; however, this elevation can also be seen in other infectious, inflammatory, and physiologic conditions. If the patients have any symptoms concerning for a pulmonary embolism, a CT angiogram CTA thorax with pulmonary embolism protocol should be obtained.

Alternatively, a ventilation-perfusion study can diagnose pulmonary embolism as well, but CTA thorax remains the study of choice. PSS is a deep venous thrombosis, and, as such, the first step in the management is to initiate anticoagulation therapy, typically, via an intravenous IV heparin drip. Patients are given an initial bolus of intravenous IV heparin and then kept on a maintenance IV heparin drip. The dose of the bolus and maintenance heparin drip is weight-based.

The affected extremity should be elevated to mitigate edema. If the patient presents within 2 weeks of the onset of symptoms, it is reasonable to consider a venogram and catheter-directed thrombolysis to reduce the clot burden.

Under fluoroscopic guidance, a lysis catheter is placed to drip a thrombolytic agent such as alteplase for 24 to 48 hours. Alternatively, a pharmacomechanical thrombectomy system can be used.

When using alteplase, the fibrinogen levels should be monitored closely to direct the dosing and duration of the catheter-directed lysis. When using pharmacomechanical thrombectomy, one should be mindful of the risk of acute kidney injury associated with hemolysis. If the presentation is more than 2 to 4 weeks from the onset of symptoms, heparinization alone may be initiated without thrombolytic therapy as the success of thrombolytic therapy is low beyond that window of time.

Another option for treatment is mechanical catheter-directed thrombectomy. This must be done early in the course of the disease. Decompression of the thoracic outlet is the definitive management of PSS. The surgical approach can either be transaxillary or supra, infra, or para-clavicular.

The timing of decompression after thrombolytic therapy is widely debated. The role of long-term anticoagulation in these patients after adequate decompression remains unclear. Some clinicians perform a venogram, often with intravascular ultrasound, 2 weeks postoperatively and decide about the duration of anticoagulation at that time. In this setting, venoplasty can be used for any residual stenosis since decompression has been achieved surgically.

She denied trauma or fever. Creatinine kinase and thyroid stimulating hormone were unremarkable. Ultrasound of lower extremities showed no evidence of deep vein thrombosis, and X-ray of the right leg was unremarkable. Magnetic resonance imaging MRI right leg showed nonspecific myositis noted throughout the calf with areas of nonenhancement and soft tissue edema.

The Patient was diagnosed with mild diabetic ketoacidosis and diabetic myonecrosis of the right calf. DKA was treated per protocol, low-dose aspirin was started, and symtomatic treatment was given with pain control for diabetic myonecrosis.

Patient improved with no further complications. Spontaneous diabetic myonecrosis is a rare complication of diabetic mellitus. The pathogenesis is uncertain. Patients usually present with swelling and pain. The most common affected area is the front of the thigh, followed by the back of thigh or calf. Awareness of the syndrome will frequently suggest the diagnosis and laboratory and imaging studies can be used to exclude other diagnoses.

Interestingly, creatinine kinase is normal in many patients. MRI may show high intensity in the involved muscle on T2-weighted sequences as well as subcutaneous edema and subfascial fluid. MRI with contrast is the diagnostic tool of choice which can distinguish nonenhancing infarcted muscle from surrounding inflammation or edema. Muscle biopsy is not necessary; it is indicated only when the diagnosis remains in doubt or when infection cannot be excluded by other investigative techniques.

Treatment includes rest, analgesia, low-dose aspirin, and optimal glycemic control. Complications including compartment syndrome and secondary infections are reported in some patients. Spontaneous diabetic myonecrosis is a rare, debilitating complication of diabetes. The treatments are symptomatic treatment with analgesia, aspirin and optimize glycemic control.

Without a high clinical suspicion for this condition, patients may be at risk for life- and limb-threatening progression to compartment syndrome and superimposed infection.

It is characterized by non-thrombocytopenic palpable purpura, abdominal pain, arthritis, and renal involvement. Pathologically, it can be considered a form of immune complex-mediated leukocytoclastic vasculitis LCV involving the skin and other organs. This is an year-old gentleman with CKD stage IV, coronary artery disease, status post stent placement, who presented withupper extremities pain, mild swelling, and a subsequent development of palpable rash that spread on the lower extremities while being nonpainful and with no itch.

He had mild proteinuria and microscopic hematuria notable in his urinalysis. Further deterioration of his kidney function was noted with normal complement levels and ANCA panel. TEE ruled out infective endocarditis vegetations. The patient developed NSTEMI during his hospital stay prompting a percutaneous coronary intervention of the right coronary and left circumflex arteries. The rash and joint pain improved completely while his residual kidney function remained stable.

There was no immediate improvement of kidney function after the plasmapheresis course of 5 days. The diagnosis can be easily missed.

Accordingly, a high degree of suspicion and attention to noninvasive laboratory work culminating into kidney biopsy with immuno-fluorescence studies is mandatory to establish the diagnosis. Adults with HSP carry a different prognosis, and the development of hematuria may be a harbinger for more serious complications such as nephritic or nephrotic syndrome. Malignancy is common in adult-onset HSP and imaging should be done to exclude this possibility.

Physicians should be aware of the possibility of HSP in patients who present with vasculitic rash and kidney disease.

Normal complement level can distinguish between IgA nephropathy and vasculitis like granulomatosis with angiitis and lupus erythematosus. Early diagnosis of HSP with kidney biopsy may improve the outcome. He had tachycardia, tachypnea, and fever with an erythematous, tender wound on left index finger.

He had lacerated his finger 2 weeks prior with a pocket knife while cleaning dirt under his fingernail and failed cephalexin and clindamycin oral therapies prior to admission. Intravenous broad spectrum vancomycin and piperacillin-tazobactam were initiated and subsequently blood cultures grew Pasteurella multocida. He lived on a farm with a cat. In view of the implanted cardiac device, fever and positive blood cultures, infective endocarditis was a concern. However, transthoracic echocardiogram did not reveal any vegetations.

XR and bone scan imaging of his finger also ruled out osteomyelitis. During his four days in the hospital, his condition and laboratory results improved and he was asymptomatic. Follow up blood cultures were negative and patient was discharged home on oral amoxicillin clavulanate for another week duration.

Pasteurella multocida is a commensal gram-negative bacteria seen in the mouths of many domesticated animals that is the most common cause of cellulitis after an animal bite. While it typically presents as soft tissue infections, it can more rarely cause meningitis, endocarditis, and bacteremia. It seems that contamination of the self-inflicted subungual minor wounds with cat saliva is what caused Pateurella multocida bacteremia.

The patient also presented with splinter hemorrhages. This physical exam finding is caused by rupture of capillaries underneath the nail plate. Blood attaches to the nail plate and moves distally as the nail grows, creating the characteristic linear pattern. While the association between splinter hemorrhages and endocarditis is often emphasized in medical training, they are present in other pathologies as well and in and of themselves do not constitute a criteria for endocarditis.

They most commonly appear due to trauma, but are also seen in endocarditis, vasculitis, medications, and renal failure due to platelet dysfunction from buildup of uremic toxins as was likely the case in this patient.

The patient was unlikely to have endocarditis since he became asymptomatic in 2—3 days and tested negative in repeat blood culture. Although a transesophageal echocardiogram would have been beneficial in definitively ruling it out infective endocarditis, a transthoracic echo of good quality is adequate especially in persons with prompt clinical improvement.

Although it primarily attacks the respiratory system, the extrapulmonary targets of the virus include the gastrointestinal tract and hepatobiliary system, among others. A year-old male with a past medical history of coronary artery disease, hypertension, 0.

He denied shortness of breath, cough, fever, or chills. Physical examination was significant for epigastric tenderness. Normal troponin. CXR on admission showed mild patchy bibasilar infiltrates suggestive of developing pneumonia. Right upper quadrant abdominal ultrasound showed no gallstones or other abnormalities within the pancreatobiliary system. CT abdomen and chest with and without contrast showed evidence of acute pancreatitis and no other abnormalities.

There is a limited but growing amount of literature supporting the diagnosis of SARS-CoV-2 induced viral pancreatitis. We worked up common causes of pancreatitis, and we do believe the patient presented with SARS-CoV-2 induced pancreatitis. ACE2 is most abundantly expressed in the pulmonary sytem, but is also expressed in pancreatic cells as well as other cells in the gastrointestinal tract.

This could explain a correlation between the virus and pancreatitis and other gastrointestinal symptoms. Although there is growing evidence of COVID-induced pancreatitis, the causal relationship is still debated between the two presentations.

Achondrogenesis Type II is an autosomal dominant lethal collagen disorder characterized by prominent forehead, recessed chin, small chest and ribs with resulting lung hypoplasia, enlarged abdomen, shortened limbs, and decreased ossification of the pelvis and spine due to pathologic variants of the COL2A1 gene.

The gene encodes for the pro-alpha1 chain, a key component of type II collagen which is necessary in developing the framework for embryologic skeletal development. The phenotype of achondrogenesis is variable, and that variation remains poorly understood. Infant and mother presented after a detailed sonographic exam showed micromelia and polyhydramnios concerning for skeletal dysplasia. An amniocentesis was performed at 22 weeks gestation age wga was notable for a heterozygous missense mutation on the COL2A1 gene concerning for a collagenopathy.

The infant was born at Upon delivery infant was noted to have high palate, protuberant abdomen, rhizomelic shortening of all extremities, sacral dysgenesis, and small thoracic cavity. He required intubation and mechanical ventilation immediately following delivery due to respiratory failure. He has failed multiple attempts at extubation and has remained ventilator dependent since birth. He was initially considered to have a perinatally lethal disease phenotype due to upper airway obstruction and severe lung hypoplasia, however, after 6 months he may be considered as a tracheostomy candidate due to improved amount of lung tissue and continued survival.

The clinical and radiographic phenotype of achondrogenesis are widely varied and may place a significant burden on a family as the definition of perinatal lethality remains challenging. The definition of lethality could be altered as a phenotype progresses with age.

The number of skeletal dysplasias is growing rapidly due to technological advancements in genetic testing. A detailed description of this patient in the medical literature will aid the ongoing differentiation of the collagenopathy types. Skeletal dysplasias may be inherited via autosomal dominant, autosomal recessive, X-linked dominant, X-linked recessive, and Y-linked fashions.

Mutation can occur de novo or inherited. Early prenatal diagnosis helps to guide genetic and palliative discussions with families to determine postnatal treatment options and recurrence risk.

Congenital dislocation of the spine CDS is a rare spinal malformation due to defective embryogenesis of the spine and spinal cord at a single level, that results in abrupt angulation of the spinal cord. We report a rare case of congenital displacement of the spine in a neonate which was missed at first admission.

An 8-day old Exweek old female infant was admitted to our neonatal intensive care unit due to concerns about a bump in the back. The infant was born to a year-old female via spontaneous vaginal delivery at an outside hospital. The prenatal course was unremarkable, all prenatal ultrasounds were reported as normal, and the mother was not on any medications during pregnancy except prenatal vitamins.

She was discharged from the delivery hospital after routine care. On follow-up exam with the primary care pediatrician, she was found to have a lump on the lower back. Spinal imaging including ultrasonography and magnetic resonance imaging were done which revealed posterior dislocation of L3 vertebral body relative to L2 with spinal cord compression.

She was admitted to the neonatal intensive care unit after neurosurgery consultation. On physical exam, the infant was noted to have bilateral talipes valgus deformity with an otherwise unremarkable neurological exam. On spinal exam, she had midline bony swelling step-up defect at L3 level with no other neurocutaneous findings.

Chromosomal microarray and whole-exome sequencing were normal. Posterior dislocation of L3 vertebral body relative to L2 with spinal cord compression. Although rare, congenital dislocation of the spine when diagnosed needs an immediate stabilization of the spine. Our case stresses the importance of a thorough spinal examination for appropriate diagnosis. Human herpesvirus-6 HHV-6 is a beta herpesvirus found ubiquitously. However, establishing HHV-6 as a cause of meningitis or encephalitis may be challenging as a polymerase chain reaction PCR test alone does not imply causality.

Examination revealed a clinically stable neonate. There was no CSF pleocytosis. After consult with infectious disease specialist, baby received IV Ganciclovir and repeat Biofire meningitis panel at 7 days continued to remain positive for HHV A decision was made to continue IV Ganciclovir for total of 21 days.

As the positive HHV6 was a serendipitous finding and the literature review showed possibility of lifelong latent infection the decision was made to discontinue Ganciclovir.

Neonate was closely monitored as she was in the NICU with feeding issues and her counts recovered. Baby remained asymptomatic and had an uneventful hospital course. A unique feature of HHV-6 is establishment of lifelong latency by integration into human chromosomes.

If such integration occurs in a germ cell, it can be vertically transmitted to the fetus through chromosomal integration ciHHV With the advent of newer diagnostic tests like Biofire meningitis panel, there has been an increasing rate of detection of HHV-6 virus, but majority are not associated with encephalitis. A positive test may either be due to chromosomal integration or subclinical reactivation of latent virus. Physicians caring for patients who test positive for HHV-6 on the Biofire panel should approach therapeutic decision making using all available information to minimize significant morbidity due to unnecessary use of antiviral agents, and emotional and financial burden associated with unwanted hospital stay.

Disseminated Herpes Simplex Virus HSV is a feared neonatal infection typically presenting after the first week of life with sepsis-like features and encephalopathy. Congenitally acquired HSV infection represents a rare, serious variety of HSV in the neonatal period, providing a unique diagnostic challenge with significant morbidity and mortality.

A female infant was delivered at Physical exam at 1 hour of life demonstrated erosive lesions of the knee, foot, and cheek. Dermatology was consulted and favored infectious source of lesions, so a sepsis evaluation including HSV, VZV, and CMV studies was performed and ampicillin, gentamicin, acyclovir, and amphotericin B were started.

Given high concern for HSV vs. Laboratory evaluation revealed transaminitis, thrombocytosis, and CSF pleocytosis with elevated protein. The remainder of infectious studies were negative. Serial HUS showed initially normal findings that progressively worsened to feature bihemispheric cystic encephalomalacia, periventricular leukomalacia with ex vacuo dilation of lateral and third ventricles. She developed central diabetes insipidus and was started on desmopressin.

Ocular involvement subsequently included retinal necrosis and diffuse retinal hemorrhage. She developed severe myoclonic jerks in the absence of electrographic correlate on EEG.

Levetiracetam and phenobarbital alleviated jerks, although she developed progressive hypotonia as neurologic status continued to deteriorate. Considering persistently positive HSV studies, foscarnet was added to acyclovir. However, at 3 weeks of life, she was intubated for apnea and respiratory failure, and given clinical trajectory and devastating prognosis, mother asked to compassionately withdraw support and allow natural death on day of life This case of congenital, disseminated HSV is particularly unique in that it occurred in a premature infant of 29 weeks gestation and had significantly elevated copy numbers in the blood and CSF as well as skin lesions, indicating likely longstanding infection at the time of delivery.

This disorder often results from a GATA1 mutation, causing uncontrolled proliferation of blast cells, with spontaneous regression in most cases.

Complications include thrombocytopenia, hepatosplenomegaly, pericardial and pleural effusions, and rarely tumor lysis syndrome TLS. We present the case of a late pre-term male infant, who at delivery had phenotypic features consistent with Trisomy 21, hepatosplenomegaly, and respiratory distress, prompting transfer to our facility.

Echocardiography revealed an atrial septal defect and pulmonary hypertension. The patient received supportive care with mechanical ventilation, intravenous hydration, Allopurinol, Rasburicase, packed red blood cells, cryoprecipitate, and fresh frozen plasma. A double volume exchange transfusion was also performed. Despite aggressive management, he remained hypotensive with metabolic acidosis, hyperkalemia, and multi-organ failure succumbing to his disease, on day 2 of life, prior to chemotherapy.

Most patients with TMD do not need cytotoxic drugs, as the process spontaneously resolves. However, symptomatic babies with high blast counts and organ dysfunction benefit from early interventions, including exchange transfusion and cytotoxic agents. TLS is a complication of TMD which usually results from the rapid degradation of malignant cells after initiation of chemotherapy. Our case indicates that TLS can occur before the initiation of chemotherapy and can be fatal.

Respiratory distress is a common clinical presentation seen in neonates. Common causes of respiratory distress in term neonates include, but are not limited to, transient tachypnea of newborn, infections, aspiration, or cardiac etiologies. We present the rare case of a term infant who presented with an unusual cause of respiratory distress in newborn. Our patient was born at an outside facility to a year-old female who had poor prenatal care.

Infant was delivered at 37 weeks gestation via emergent C-Section due to non-reassuring fetal heart rates. He was noted to have an obvious chest wall deformity with a concavity to the right anterior chest and slight lateral curvature of thoracic vertebra. He was unable to maintain saturations on room air and had persistent tachypnea so was placed on supplemental oxygen and transferred to NICU.

Chest X-ray obtained at birth revealed multiple mid upper thoracic developmental vertebral body anomalies, diminished left thoracic volume and multiple bilateral rib fusion abnormalities. Blood work obtained was unremarkable with no concern for sepsis, metabolic conditions, or hematological issues. He failed multiple attempts to wean off oxygen and would drop his saturations mostly when feeding. He was therefore transferred to our facility for higher level of care at two weeks of life.

On arrival to our NICU, Pulmonology was consulted, and further workup was obtained per their recommendations including a CT Chest which showed the known thoracic abnormality but no parenchymal or intrapulmonary processes. Modified Barium Swallow Study was negative for aspiration. Echocardiogram showed a Patent Foramen Ovale but was otherwise normal. Cranial Ultrasound was normal and Abdominal Ultrasound showed a right pelvic kidney. Genetics was consulted, and genetic tests were obtained including chromosomal microarray which was normal and the working diagnosis was possible variant of Skeletal Dysplasia.

Since all other causes of respiratory distress were ruled out, his continual oxygen requirement was attributed to restrictive lung disease secondary to congenital chest wall deformity.

He was unable to be weaned to room air and continued to be tachypneic on nasal cannula. He was eventually weaned off oxygen and at his last follow up at 9 months of age, he had been doing well on room air. He was referred to Pediatric Orthopedics and is scheduled for Expansion Thoracoplasty when he turns 1 year old. Restrictive Lung Disease secondary to chest wall deformities can be a cause of respiratory distress in newborns.

High flow nasal cannula is a newer means of home oxygen delivery and its use in this population is still evolving. A newborn female was delivered via elective C-section at 34 weeks due to maternal preeclampsia, growth restriction and a suspected right lung mass.

Two doses of prenatal steroids were given to mother prior to delivery. The chest mass was noticed on a prenatal ultrasound which was followed by a fetal MRI. However, the MRI did not reveal an abnormal chest mass but it was somewhat limited by motion of the fetus.

Infant developed respiratory distress shortly after delivery and required CPAP for poor color and perfusion. Stat x-ray confirmed immature lungs with right lower chest mass. She was admittedto NICU and was promptly intubated due to increasing oxygen requirement. Due to persistent respiratory distress and the size of the mass, decision was made to perform right upper lobectomy to release the compression. During the surgery, the patient was noticed to have total of 6 lobes of lung on the right side and 3 lobes looks abnormal.

The abnormal lobes were intimately attached to the normal lobes. The patient had normal appearing upper, middle and lower lobes once the abnormal tissue was removed. No aberrant vessel from any of the abnormal lobes to the aorta was seen. From the middle abnormal lung a frozen section biopsy was taken which showed cystic appearance close to the pleural surface up to 1.

Thoracotomy and resection of triple lobectomy was successfully performed on the 3-day old female and two chest tube was placed on the right side. Patient was remained to be on Jet Ventilation with chest tube to suction after surgery.

She was then extubated and weaned to NIPPV one week later and both chest tubes were removed 2 weeks after surgery. Extra-lobar sequestration was confirmed presenting as three abnormal lobes, with histologic feature of CPAM type 2, but no aberrant vessel from any of the abnormal lobes to a systolic artery was seen. A male infant was born at 28 weeks gestation to a G2P2 mother due to non-reassuring fetal status. Pregnancy was complicated by intractable seizures requiring induced coma, suspected chorioamnionitis, and insulin-dependent diabetes mellitus.

Mother had epilepsy requiring multiple anti-epileptics. At delivery, the baby was limp, edematous, and apneic requiring mechanical ventilation. He received surfactant for respiratory distress syndrome, dopamine for hypotension and antibiotics for presumed sepsis. On the third day of life, he was noted to have a systolic ejection murmur with widened pulse pressures. Echocardiogram showed multiple non-obstructive echogenic masses scattered over the myocardium of the ventricles, septum, and papillary muscles suggestive of rhabdomyomas.

Due to maternal epilepsy and suspected cardiac rhabdomyomas, the diagnosis of familial tuberous sclerosis complex TSC was considered. Neither mother nor infant had cutaneous findings. Mother had unremarkable head imaging. His cranial ultrasound showed nodular intraparenchymal echogenicity in bilateral subcortical frontal lobes suspicious for subcortical tubers and left lateral ventricle prominence with ependymal nodularity suspicious for a subependymal hamartoma.

Renal ultrasound and ophthalmologic exam were unremarkable. TSC is a genetic disorder featuring the growth of benign tumors in multiple organ systems. Disease severity is variable and specific physical manifestations appear at progressive ages. Often, mutations in TSC2 are associated with a more severe clinical course and developmental delay.

Cardiac rhabdomyomas may lead to arrhythmias or outflow obstruction. Seizures typically present in infancy and can be difficult to control. Major criteria include hypomelatonic macules, angiobriomas, ungual fibromas, shagreen patch, retinal hamartomas, cortical tubers, subependymal nodules, subependymal giant cell astrocytoma, cardiac rhabdomyomas, lymphagioleiomyomatosis or angiomyolipmas.

Minor criteria include confetti skin lesions, dental enamel pits, intraoral fibromas, retinal achromatic patch, multiple renal cysts, nonrenal harmatomas and sclerotic bone lesions. The baby was diagnosed with TSC given his clinical findings.

He was discharged at term without cutaneous findings or evidence of seizures. He will be followed by cardiology, genetics, ophthalmology, and the premature developmental follow-up clinic. Congenital ectropion is an eversion of eyelid, where the skin of the lid is folded on itself, and conjunctival surface is exposed externally.

The incidence is higher in black infants, Trisomy 21 syndrome, and in infants born with collodion skin disease. An early term female newborn was born to a 25 year old G3P2 mother of African ethnicity by vaginal delivery. Mother had adequate prenatal care with negative infectious prenatal labs.

Mother had spontaneous rupture of membranes lasting for 14 hours and a healthy-looking female baby was delivered without any complications. The underlying pupils or sclerae could not be visualized, and red reflex could not be performed. The eyeballs were palpated in the socket. Edematous upper palpebral conjunctiva was separated using eyelid retractors and underlying pupils and conjunctivae were noted to be normal in appearance.

Condition was diagnosed as bilateral congenital ectropion with severe chemosis. Hypertonic saline compresses over the eyelids were given for edema. On day of life 4, there was complete resolution of ectropion and normally appearing pupils and conjunctivae were noted. Congenital ectropion is rarely reported in literature.

Venous stasis, birth trauma and various abnormalities of muscles and ligaments of orbit including hypotonia of orbicularis muscle, vertical shortening of anterior lamella or vertical elongation of posterior lamella, failure of fusion of orbital septum with levator aponeurosis have all been proposed as possible mechanisms of congenital eversion.

Initially, eversion occurs only when the infant squeezes the eyelids shut during crying and resolve when the infant is calm. As edema increases, the eversion persists and may be difficult to reposition.

The chemosed conjunctiva protects the underlying cornea and therefore, corneal complications are uncommon. Management strategies include both conservative and surgical approaches. Surgical treatment includes tarsorrhaphy, fornix sutures and full thickness skin grafts to the upper eye lid.

The condition resolves without any sequalae with prompt management, but delay in treatment can lead to complications including amblyopia, corneal ulcers, and permanent blindness.

Congenital eyelid eversion is a rare condition. A favorable clinical outcome may be expected with prompt diagnosis and intervention of this condition. Knowledge of this condition with early intervention by clinicians may help in the prevention of complications that arise from poorly treated cases. Cystic hygroma was noted on the fetal ultrasound at 14 weeks.

On physical exam at birth, she had pterygium colli and facial dysmorphism hypertelorism, downslanting palpebral fissures, posteriorly rotated low set ears. She was admitted to the neonatal intensive care unit NICU due to respiratory distress. The patient was initially started on Ampicillin and Gentamicin for suspected sepsis.

She had seizure-like activity on day two of life. Gentamicin was discontinued and a total of day course with Ampicillin and Ceftazidime was completed. Blood and CSF cultures were negative. EEG showed no seizure activity. Cranial ultrasound revealed hypoplastic corpus callosum, paucity of gyri, mild colpocephaly, cerebellar vermis hypoplasia, and wide extra-axial space.

RASopathy panel identified a pathogenic variant c. She also had splenomegaly. WBC count decreased and thrombocytopenia resolved after starting the treatment with 6-MP. JMML should be suspected in any patient with NS who has persistent leukocytosis with monocytosis and thrombocytopenia without evidence of an infectious process. A year-old sexually active female, presented with diffuse abdominal pain and distension.

The symptoms were waxing and waning for a year. She denied recent travel. Family history of a breast canacer was reported. On examination: Patient was vitally stable but appeared uncomfortable. Abdomen was markedly distended, dull to percussion with positive fluid thrill but bowel sounds were appreciable. Pelvic exam was negative for cervical tenderness or discharge. CRP 5. Liver enzymes, LDH and uric acid were normal. CBC unremarkable. CT scan abdomen-pelvis showed severe ascites with mesenteric omental caking.

Working diagnosis was peritoneal carcinomatosis vs tuberculous peritonitis. TB workup was negative. Laparoscopy showed pelvic inflammation around the uterus with abscess and benign lymph nodes. Pelvic inflammatory disease PID was presumed as the cause of ascites. Patient was started on intravenous antibiotics. Symptoms resolved and the patient was discharged on Doxycycline and Metronidazole. Although the majority of females with CT infection are asymptomatic, CT known to cause urethritis, salpingitis, and cervicitis.

Ascites was rarely reported as the only presenting symptom of CT. Exudative ascetic fluid and predominance of lymphocytes were noted in all reported CT cases with ascites. Hospitalization with parenteral antibiotic for 1—2 days is recommended for severe PID. Clindamycin or metronidazole should be used when tubo-ovarian abscess present. CT abdomen-pelvis: White arrow pointing towards fluid collection in the abdominal cavity.

Appropriate testing should be pursued early in the workup of these patients. Transverse myelitis is the segmental inflammation of the spinal cord with motor and sensory abnormalities at and below the level of the lesion.

Often, the etiology is unknown but may be attributed to autoimmune conditions or viruses. A 5-year-old male with a history of asthma presented for vomiting and altered mental status.

The patient was noted to be altered, lethargic, and in respiratory distress. Intubation was performed. History also revealed that he had slurred speech, ataxia, and a fall with trauma to forehead 1 day prior to arrival. Dexamethasone was started. When sedation was weaned in hopes of extubation, patient was noted to be alert, but not moving extremities and had minimal gag and cough reflex.

MRI of Brain and Spine were conducted and revealed findings suggestive of long segment transverse myelitis involving C2 to C3. Further, patient underwent ten sessions of plasmapheresis.

Repeat MRI was consistent with previous. Physical and occupational therapy initiated at the onset of illness in hopes of achieving musculoskeletal improvement. Patient had some minimal musculoskeletal improvement, however, given his condition, decision was made for patient to undergo placement of gastrostomy and tracheostomy tubes.

Patient was weaned off of sedatives and withdrawal was treated with a clonidine taper. Once stabilized, patient was transferred to neurological inpatient rehabilitation center. Pathophysiology is multifactorial, including direct invasion of the CNS, vascular insufficiency, immune dysregulation and autoimmunity. Imaging is paramount in the diagnosis of transverse myelitis.

Treatments are emerging and may include steroids, immunoglobulin, plasmapheresis, and monoclonal antibodies. Information is emerging and evolving daily. Cases of transverse myelitis in COVID have been reported in few adult patients and minimal pediatric patients. Practitioners should keep transverse myelitis on their list of differentials for neurological complications of SARS-CoV-2 infections and initiate aggressive treatment with a multidisciplinary approach.

This is a case of partial retinal artery occlusion in an adolescent. She denied any trauma, redness, excessive tearing, discharge, floaters, vomiting, fever or headaches. Her family history was unknown as patient was an adopted child. Her initial external ocular, including intraocular pressure, and remainder of the neurologic exams were normal. Retinal edema along the superotemporal arcade on the right was noted during a dilated fundoscopic examination by Ophthalmology, concerning for branched retinal artery occlusion BRAO.

Transthoracic echocardiogram showed trivial tricuspid and mitral valves insufficiency, but was otherwise normal with an intact interventricular septum, no atrial septal defect, and no thrombus. She was discharged home on daily Aspirin with close outpatient follow-up with Ophthalmology and Neurology. The central retinal artery is a branch of the ophthalmic artery, which itself is a branch of the internal carotid artery. The following authors declared potential conflicts of interest: Nathan A. Sports Health.

Nathan A. Geoffrey S. Van Thiel. Wendell M. George A. Bernard R. Bach, Jr. Author information Copyright and License information Disclaimer. Abstract Paget-Schroetter is a rare diagnosis in the general population; however, it is more common in younger, physically active individuals. Keywords: Paget-Schroetter syndrome, effort thrombosis, upper extremity, sports medicine.

Open in a separate window. Figure 1. Diagnosis Presentation Patients with effort thrombosis usually present after the subclavian vein has completely thrombosed with a bluish hue to the arm, discoloration, asymmetric muscle bulk or increased postworkout swelling that does not dissipate, heaviness, and pain in the affected upper extremity Figure 2.

Figure 2. Table 1. Differential diagnosis of Paget-Schroetter. Neurologic thoracic outlet syndrome Arterial thoracic outlet syndrome Malignant tumors of the head, neck, or arm Pancoast tumor Complex regional pain syndrome Brachial neuritis Cervical nerve root compression Quadrilateral space syndrome Peripheral nerve compression. Physical Examination The asymmetric swelling is typically obvious through visual inspection.

Imaging The diagnostic study of choice is duplex ultrasonography. Figure 3. Operative Treatment Catheter thrombolysis with subsequent first rib resection has emerged as the treatment of choice for PSS. Conclusion PSS is rare. Footnotes The following authors declared potential conflicts of interest: Nathan A. References 1. Conventional versus thrombolytic therapy in spontaneous effort axillary-subclavian vein thrombosis. Am J Surg. Intermittent subclavian vein obstruction without thrombosis.

J Trauma. Adson W. Surgical treatment for symptoms produced by cervical rib and scalenus anticus muscle. Surg Gynecol Obstet. Effort-related axillosubclavian vein thrombosis: a new theory of pathogenesis and a plea for direct surgical intervention. Axillary and subclavian venous thrombosis: prognosis and treatment. Arch Intern Med. Butsch JL. Subclavian thrombosis following hockey injuries. Am J Sports Med. The importance of thrombophilia in the treatment of Paget-Schroetter syndrome.

Ann Vasc Surg. Sonographic evaluation of upper extremity deep venous thrombosis. J Ultrasound Med. Effort thrombosis in the elite throwing athlete. Green R, Rosen R. The management of axillo-subclavianvenous thrombosis in the setting of thoracic outlet syndrome. In: Gloviczki P, ed. Handbook of Venous Disorders. Primary upper-extremity deep vein thrombosis: high prevalence of thrombophilic defects.



Southern Medical Research Conference | Journal of Investigative Medicine – Citation, DOI & article data

Paget-Schroetter is a rare diagnosis in the general population; however, it is more common in younger, physically active individuals. Patients with chronic kidney disease (CKD) and non-st-elevation myocardial infarction (NSTEMI) are a significant clinical challenge. Agreement between.


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