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Open-book pelvic fractures are an uncommon orthopedic emergency that requires prompt recognition and treatment. A 37-year-old male was involved in high-energy trauma, resulting in an open-book pelvic fracture with bilateral sacroiliac joint diastasis, bilateral superior and inferior pubic rami fractures, a comminuted sacral fracture, and a traumatic hernia. On presentation, he was hemodynamically unstable, with bruising in the right hemipelvis. Acute treatment included a cervical collar, transfusion protocol, central venous access, and pelvic binder. Trauma and orthopedic services were consulted to manage the patient with an interdisciplinary approach. The patient initially underwent external fixation with concomitant exploratory laparotomy. Definitive treatment concluded with colorectal anastomosis, diverting loop ileostomy creation, abdominal closure, open-reduction internal fixation (ORIF) of the pelvis, and removal and reapplication of external fixation.

AMA Research Challenge 2024 

Multidisciplinary Approach to Managing Open-Book Pelvic Fractures in Trauma Care

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Background:
Cactus thorn injuries are an uncommon cause of inflammatory joint pathologies that are often difficult to identify and treat. Patients present with diverse symptoms and findings, requiring a high degree of clinical suspicion for diagnosis. Curative treatment is primarily surgical, including synovectomy, irrigation and debridement, and foreign body removal. In this case, we describe a patient presenting to a Southwest pediatric hospital with a cactus thorn physeal injury resulting in limb deformity.

Case Presentation:
An otherwise healthy 12-year-old active boy presented with knee pain, stiffness, and uneven gait secondary to 2 cm leg length discrepancy (LLD) and genu valgum three years after sustaining a cactus thorn injury to the lateral aspect of his right knee. He had a history of three prior irrigation and debridements at the time of injury and received several months of antibiotics, but no foreign bodies were found and cultures were negative. Advanced imaging showed early degenerative changes that will likely ultimately require a total knee arthroplasty. At our institution, he underwent right knee manipulation under anesthesia, arthroscopic loose body removal, and synovectomy to improve range of motion, right distal medial femoral hemiepiphysiodesis to prevent worsening of genu valgum, and left femur epiphysiodesis to prevent worsening of LLD. Right distal femur lateral opening wedge osteotomy was later performed to fully correct the valgus deformity. His knee extension improved, but persistent flexion limited to 70 degrees caused physical limitations. CT scan and dynamic ultrasound demonstrated a known posterior osteophyte as a mechanical block and a thorn embedded in the posterior knee soft tissues. Five years after initial injury, he underwent an open procedure with osteophyte excision and thorn removal with ultrasound assistance. Knee range of motion improved to 0-95 degrees which was maintained at his most recent follow-up. He now has normal coronal alignment of his bilateral lower extremities with return to all activities despite his persistent LLD. 

Discussion:
This case is the first to describe a partial physeal closure and resulting limb deformity due to an intra-articular cactus thorn injury. This is significant given most documented thorn injuries describe temporary disability that resolves after definitive surgical treatment, whereas this patient suffered permanent joint damage. To date, he has undergone six operative procedures and will likely require future total knee arthroplasty. While this is an uncommon result of a thorn injury, this case emphasizes the importance of identifying and removing intra-articular bodies to minimize subsequent joint damage.

Pediatric Leg Length Discrepancy and Genu Valgum from Cactus Thorn Physeal Injury: A Case Report

Title : proximal femoral focal deficiency A case report

Background 
Proximal Femoral Focal Deficiency (PFFD) is an extremely rare condition, with an incidence of 1-2 per 100,000 births. It is characterized by dysgenesis and hypoplasia of varying segments of the proximal femur. The severity of the defect ranges from femoral shortening with varus deformity to complete absence of both the acetabulum and proximal femur.

Case Presentation
A 7-month-old male infant, born to consanguineous parents, presented with limb discrepancy, noting a shorter left thigh compared to the right since birth. On examination, the left thigh was shorter and had fewer skin folds compared to the right. The Galeazzi sign was positive, with the left knee lower than the right. No other skeletal or systemic anomalies were observed. Although developmental dysplasia of the hip (DDH) was initially suspected, the short thigh length was unusual. Radiographs of the pelvis and thighs confirmed the diagnosis, revealing partial absence of the proximal end of the left femur shaft with the femoral head present. CT or MRI was not performed. Antenatal scans at 36 weeks showed a breech presentation with a short left femur (35 mm, corresponding to 21 weeks and 2 days) and a right femur (65 mm, corresponding to 34 weeks and 5 days).

Discussion
Both DDH and PFFD can cause limb shortening. DDH is characterized by limited hip abduction and asymmetrical thigh folds, with possible femoral head dislocation. PFFD typically results in more significant and persistent shortening, which can extend to the mid-tibia or knee of the unaffected limb, or even involve near-total absence of the femur. The affected hip in PFFD is usually flexed, abducted, and externally rotated.
Etiological factors like diabetes, drug exposure, viral infections, and trauma, noted in the literature, were absent in this case. PFFD may present as isolated, with associated facial anomalies, or affect multiple limb segments. Management is individualized and may involve prosthesis, pelvic-femoral osteotomies, Van Nes rotationplasty, femoral derotation and valgus osteotomy, or limb lengthening.This case is Type C as per Aitken classification.

Proximal Femoral Focal Deficiency : A Case Report

Background 
Hand-foot-mouth disease (HFMD) is a viral illness that typically presents in children under the age of five with fevers and characteristic papulovesicular lesions of the hands, feet, and oral mucosa. With the rise of coxsackievirus-A6 and atypical Hand-foot-mouth disease (HFMD) presentations with ear lesions, providers need to consider a broad differential for external ear lesions that includes HFMD.
Case Presentation
A 3-year-old previously healthy male patient presents to his primary care clinic with a worsening rash. Three days ago, he developed fever, congestion, and rhinorrhea, and two days later, he developed a rash. The rash initially started on his hands, feet, and perioral region, but has since developed ear lesions. He has known sick contacts with fever and rash only on their hands and feet. On exam, he is afebrile. Skin exam is notable for pruritic, crusting, erythematous papulovesicular eruption concentrated on his palms, soles, and perioral regions. He has multiple, similarly-appearing lesions on the auricles of both ears, but no lesions within the helix or external canal. Oral exam is notable for erythematous macules on the soft palate, posterior oropharynx, and lower inner lip with normal gingiva and tongue.

Discussion
With the patient’s ear lesions, causes such as vascular, inflammatory, neoplastic, and traumatic causes were considered. However, because of his associated symptoms, an infection was more likely. Infections such as CV-A6, Rocky Mountain Spotted Fever, impetigo, Herpes Simplex Virus, Varicella Zoster Virus, and Monkeypox are all potential causes of papulovesicular ear lesions. Given the patient’s age and exposure to known sick contacts with typical HFMD presentation, CV-A6 was the most likely. In recent years CV-A6 has been associated with outbreaks of more severe disease and atypical HFMD worldwide – including China, Spain, Taiwan, the United States, and the UK.

External ear lesions are a rare atypical presentation of HFMD. When patients have no other symptoms, clinicians must consider the broad differential for papulovesicular ear lesions. 


Atypical Hand-Foot-Mouth Disease - The Need to Consider the Various Causes of Pediatric Papulovesicular Ear Lesions ​

Background
The life cycle of Plasmodium parasites, the parasites responsible for the transmission of malaria, depends on the parasites’ ability to invade red blood cells. Mutations that alter the structure of red blood cells, including the mutation that causes sickle cell disease (Hb-SS), interfere with the life cycle of Plasmodium parasites and provide protection against malaria. Patients with Hb-SS rarely develop severe anemia that requires blood transfusions before 6 months of age.
Case Presentation
A female infant with Hb-SS discovered on routine newborn screening began needing monthly transfusions to maintain hemoglobin levels above 7 g/dl at the age of 6 weeks of life. Due to the severity of her anemia, hemolytic anemia gene sequencing was performed and revealed the presence of hereditary elliptocytosis and heterozygosity for G6PD deficiency. Splenectomy, performed at 2 years of age due to splenic sequestration, resulted in decreased frequency of transfusions, highlighting the role of hereditary elliptocytosis in her presentation.
Discussion
Hereditary elliptocytosis modifies the severity of Hb-SS; coexistence of hereditary elliptocytosis with Hb-SS can result in severe, early-onset anemia. Infants with Hb-SS and severe anemia need genetic testing to investigate for other causes of anemia.

Hereditary Elliptocytosis as a Modifier of Sickle Cell Disease Severity

Background
Blisters in children are typically attributed to common causes such as impetigo, herpes zoster, burns, sunburn, friction, contact dermatitis, bug bites, and thumb-sucking. However, less common causes like suction blisters from pacifiers are seldom reported. This case highlights a rare presentation of a suction blister on the thumb caused by a pacifier, emphasizing the need for vigilant monitoring of pacifier use in young children.

Case Presentation
A 21-month-old male with no significant past medical history presented with a suction blister on the left thumb. The blister was discovered after the child woke up crying in the crib, with the pacifier suctioned over the thumb. The thumb exhibited a circumferential pale appearance from the proximal interphalangeal joint to the distal thumb, with an intact, oval bulla filled with clear fluid on the volar thumb pad. The surrounding skin turned erythematous after removing the pacifier. The child was treated with ibuprofen for perceived pain and brought to the clinic. Physical examination confirmed the blister as approximately 8 mm in diameter, with no signs of infection or complications. The blister was managed conservatively. Parents were advised to keep the area clean, avoid puncturing the blister, and follow anticipatory guidance for care. The parents chose to discontinue pacifier use.

Discussion
Pacifiers, while beneficial for soothing young children, can pose risks such as suction blisters from prolonged negative pressure. This case report of a suction blister on the thumb caused by a pacifier highlights the importance of considering uncommon causes of blisters in pediatric patients and underscores the need for careful monitoring of pacifier use. Educating parents on the potential risks and proper usage of pacifiers is crucial to prevent similar injuries. Recommendations include ensuring pacifiers are appropriately sized, made of safe materials, and not left with children unsupervised for extended periods. Improved education and product design may help mitigate these risks.


Pacifiers: Blissful or Blister? Suction Blister on the Thumb

Background: NMDA receptor encephalitis is a rare autoimmune disorder characterized by the production of antibodies against the N-methyl-D-aspartate (NMDA) receptors in the brain. It often presents with a variety of neurological and psychiatric symptoms, including seizures, speech impairment, and behavioral changes. 

Patient: We present the case of a 4-year-old girl initially diagnosed with focal epilepsy of unknown etiology, who exhibited speech regression and behavioral changes. Despite treatment with antiepileptic medication, her symptoms persisted and worsened over time. 

Results: Further evaluation, including imaging studies and EEG monitoring, did not reveal any significant abnormalities. However, autoimmune encephalitis was suspected due to the atypical presentation and lack of response to conventional treatments. Serum testing confirmed the presence of NMDA receptor antibodies. The patient was subsequently treated with high-dose steroids for 5 days, which led to a remarkable improvement in her condition. Within a week of discharge, she began speaking in full sentences again. 

Conclusion: This case highlights the importance of considering autoimmune encephalitis in children presenting with atypical seizure manifestations and behavioral changes. Prompt recognition and treatment with immunomodulatory therapy, such as high-dose steroids, can result in significant clinical improvement and better outcomes for these patients.

Pediatric NMDA Receptor Encephalitis: a Case of Speech Regression and Behavioral Changes

Background
Pericardial effusions have a variety of causes ranging from malignancy and trauma to lymphatic obstruction1. Infection is the most common cause of pericardial effusions in children, with serous effusions being the most common type2. Here we report a case of a post-viral hemorrhagic pericardial effusion with tamponade physiology in a pediatric patient with no history of trauma.

Case Presentation
A 12-year-old female with no past medical history presented to the pediatric emergency department with a two-week history of worsening shortness of breath on exertion. Two weeks prior, she had experienced a viral illness with fevers up to 102ºF and inspiratory chest pain. She tested negative for flu, strep throat, and COVID; she was given azithromycin, which initially improved her symptoms. About one week after her initial presentation, she developed shortness of breath, non-productive cough on exertion, and some congestion. At that time, she was given amoxicillin/clavulanic acid at an after-hours clinic, which her mother supplemented with home prednisolone and an albuterol inhaler. None of these improved her symptoms. She was then discovered to have cardiomegaly on an outside chest x-ray, which prompted a trip to the pediatric emergency department. On presentation to the emergency department, the patient had normal vital signs. In the emergency department, a bedside echocardiogram showed a large pericardial effusion with tamponade physiology, right ventricle diastolic collapse, and >30% mitral valve inflow variation. Cardiology was consulted and the patient was taken to the PICU for urgent pericardiocentesis. A pericardiocentesis and drain placement was performed at bedside in the PICU and drained 1200cc of hemorrhagic fluid, with cultures obtained and showing no growth. Hematology/Oncology was consulted due to concern for malignancy; however, CT and tumor marker labs showed no evidence of malignancy. Rheumatology was also consulted to rule out an autoimmune process, with autoimmune markers returning normal. The patient was given furosemide for the effusion, and repeat echocardiograms showed no accumulation, prompting the drain to be removed. The patient was discharged with ibuprofen and a close cardiology follow-up. Since then, the patient had a normal chest x-ray two weeks after admission, and an echo one month after admission showed no significant residual pericardial effusion, valvular dysfunction, and normal systolic motion.

Discussion
Pericardial effusions are most often serous but can sometimes be hemorrhagic. Hemorrhagic pericardial effusions are usually due to trauma but should not be excluded as a possibility when dealing with viral illnesses or other causes. It is also important to rule out other pathological processes, such as malignancy and autoimmune conditions, when working up hemorrhagic effusions. It is also important to gather a thorough history from patients, including potential trauma or prior infections. Life threatening cardiac tamponade occurs when a patient develops an obstructive shock due to buildup of fluid in the pericardium. Cardiac tamponade is diagnosed with echocardiogram showing right ventricle diastolic collapse. Definitive treatment is pericardiocentesis, which removes pericardial fluid and relieves the obstructive shock.

Serious but Not Serous: a case of infectious, non-traumatic hemorrhagic effusion in pediatric cardiac tamponade

Background
VACTERL Association is a group of birth defects that can present as a combination of Vertebral, Anal, Cardiac, Tracheal, Esophageal, Renal and Limb abnormalities. It is a diagnosis of exclusion requiring at least three of these features to be present. There is no true explanation for VACTERL from either a causation or embryo-pathogenesis standpoint, but risk factors include gene mutations, chromosomal anomalies and maternal diabetes. VACTERL occurs in 1 out of 10,000 to 40,000 births, though it is likely underdiagnosed due to its variability in presentation.

Case Presentation
Patient is a 4-month-old male with history of VACTERL association status-post tracheoesophageal fistula ligation, esophageal atresia repair and G-Tube dependency, presenting for follow-up after 118-day NICU stay for prematurity and fetal anomaly workup. Hospital course was complicated by respiratory distress, sepsis rule out, cardiac resuscitation, pneumothorax, and hemidiaphragmatic paralysis. Patient was born at 35w6d to a refugee mother from Venezuela via spontaneous vaginal delivery. Pregnancy was complicated by polyhydramnios, abnormal prenatal ultrasound and late prenatal care. Delivery was complicated by premature prolonged rupture of membranes. Patient currently presents with global developmental delays and requires follow-up with surgery, gastroenterology, pulmonology, nephrology, cardiology, genetics, ENT and ophthalmology.

Discussion
Early signs of VACTERL association included (1) multicystic left kidney seen on prenatal ultrasound, and (2) polyhydramnios and lack of gastric bubble indicating tracheoesophageal fistula (TEF) or imperforate anus. While not present in this patient, a single umbilical artery also frequently accompanies VACTERL. Since VACTERL is a diagnosis of exclusion, the differential included Fanconi anemia, CHARGE syndrome, DiGeorge syndrome and Alagille syndrome, all of which share features with VACTERL.
From “VACTERL,” the patient presented with: (V) supernumerary ribs, S1 defect and rib hypoplasia; (C) patent foramen ovale, atrial septal defect and patent ductus arteriosus; (TE) TEF Type C, and (R) left multicystic kidney. As outlined in a new Pediatric Research (2020) guideline, VACTERL phenotypes fall under four subtypes—STRICT-VACTERL, VACTERL-LIKE, VACTERL-PLUS and NO-VACTERL—based on major and minor criteria. The patient was classified as STRICT-VACTERL.
Treatment required careful coordination of care from the neonatology team, and despite surgical interventions, long-term care from various specialists throughout childhood will likely be necessary. Prognosis for VACTERL is generally good, though chronic health issues into adulthood are common. While early identification of anomalies through antenatal care is key for early coordination of care and birth plan, further research is needed to identify and correct modifiable causes for future prevention.

The 118 Day NICU Voyage of a VACTERL Baby from Venezuela

Abstract Title: Under Pressure: Brain Abscess and Meningitis Complicated by Orbital and Ethmoidal Encephaloceles-A Pediatric Case Report

Alisa Liberman, BS1, Timur Raghib, MD2
1Midwestern University Arizona College of Osteopathic Medicine, Glendale, AZ; 2Phoenix Children’s Hospital, Phoenix, Arizona

Background
An encephalocele is a herniated sac containing meninges and brain tissue that protrudes out of a skull defect. The majority of primary encephaloceles are congenital. Secondary encephaloceles can arise due to trauma, tumors, surgery, and chronic sinusitis. Literature review of infectious pathology associated with encephaloceles yielded documented cases of different type of encephaloceles associated with meningitis and/or empyema in both adult and pediatric populations. Here we present, to our knowledge, the first documented case of brain abscess and
meningitis complicated by concurrent orbital and ethmoidal encephaloceles in the pediatric population.

Case Presentation
A 10-year-old male with a history of headaches presented to an emergency department with fever, altered mental status, swelling near his right eye and mid-forehead, vomiting, and worsening headaches. One year prior, the patient suffered unspecified skull fractures after a motor vehicle accident and subsequently developed headaches. One week prior to presentation his headaches increased in intensity and frequency. In the emergency department, CT of the head showed pansinusitis, and lumbar puncture showed a leukocyte count 12694, glucose 7, and
protein 307. The patient was started on Vancomycin and Ceftriaxone. He was transferred to another hospital, where repeat CT and MRI showed a right inferior frontal lobe abscess with intraventricular extension, right anterior cranial fossa bony defect with an encephalocele protruding into the right superior orbit and a small component extending into right ethmoid, in addition to pansinusitis. The patient underwent endoscopic sinus surgery, cerebral abscess drainage, and external ventricular drain catheter placement. CSF and brain abscess cultures were positive for Streptococcus Pneumoniae and Staphylococcus Aureus/Staphylococcus Epidermis, respectively. The patient was treated with culture-sensitive antibiotics and transitioned to Ceftriaxone and Metronidazole for an additional six weeks.

Discussion
This case describes an uncommon presentation of brain abscess and meningitis complicated by worsening orbital and ethmoidal encephaloceles. In our patient, we propose that the encephaloceles initially developed secondary to the patient’s traumatic skull fractures and expanded due to increased intracranial pressure and mass effect from the frontal brain abscess. From this case we learn that even before obtaining imaging, it may be important for clinicians to keep infectious intracranial pathology complicated by encephaloceles on the differential in a patient with a history of skull trauma presenting with fever, altered mental status, forehead and
eye swelling, and worsening headaches. Additionally, while clinicians may be cautious in repeating CT/MRI given the radiation/cost, our case illustrates that it may be necessary to prevent diagnostic delay.

Under Pressure: Brain Abscess and Meningitis Complicated by Orbital and Ethmoidal Encephaloceles-A Pediatric Case Report

Epstein-Barr Virus (EBV), a member of the Herpesviridae family, is globally prevalent. While most children infected are asymptomatic, around 25% develop infectious mononucleosis (IM) characterized by fever, sore throat, and lymphadenopathy. IM can lead to multisystem involvement, including rare pulmonary manifestations like pneumonia. In literature, pulmonary nodules are exceedingly rare in immunocompetent children. Here we discuss a unique case of nodular lung findings in a patient with IM to highlight the diverse clinical manifestations of EBV infection.

An 11-year-old male previously healthy male presented to the emergency room (ER) with one week of fever, neck pain and sore throat. In the ER, vitals were unremarkable. Physical examination revealed a non-fluctuant, right larger than left submandibular lymphadenopathy with mild tenderness to palpation. CT soft tissue and neck with IV contrast revealed bilateral cervical and supraclavicular lymphadenopathy. CT chest noted multiple small scattered noncalcified pulmonary nodules. Clinical symptoms work-up was significant for WBC 15.4 TH/cmm, CRP 3.00 mg/dL, LDH mildly elevated at 394 U/L, and uric acid 6.0 mg/dL. Histoplasmosis and Bartonella antibody panels were negative. ENT was consulted and he was started on 0.15 mg/kg of Decadron for four doses to minimize airway swelling and IV Unasyn for infectious coverage. EBV serologies were also obtained showing IgG positive, IgM positive, Nuclear Ag IgG negative, and Early Ag IgG positive, indicating the patient’s lymphadenitis and suspicious apical pulmonary nodules were secondary to infectious mononucleosis. The patient’s family was notified of the diagnosis and strict return precautions and counseling were given.

Pulmonary manifestations in EBV-related illnesses are relatively uncommon, but can present as pneumonia. In immunosuppressed patients, EBV can cause lymphoproliferative disorders (PTLD) or lymphoma which can present as pulmonary nodules. In literature, pulmonary nodules rarely occur in an immunocompetent child. Pulmonary nodules could be part of the clinical presentation of IM in otherwise healthy children and in an appropriate setting, a close follow-up with complete resolution is adequate to rule out malignancy. Our patient was managed conservatively with close monitoring and regular follow- up. The decision on follow up imaging should be individualized based on clinical signs and symptoms and imaging characteristics. The discussed case emphasizes the importance of understanding and recognizing the variable presentations of EBV even in the most unlikely of patients.

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Multidisciplinary Approach to Managing Open-Book Pelvic Fractures in Trauma Care

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Pediatric Leg Length Discrepancy and Genu Valgum from Cactus Thorn Physeal Injury: A Case Report

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