A nine year old male with no past medical history presents to the emergency department accompanied by his mother, describing a two day history of acute on chronic right hip pain. The patient states that while bathing two days ago, he slipped on a bar of soap onto his right side. Subsequent to the fall, the patient has been having difficulty walking secondary to right hip pain, and the mother reports that the patient has developed a noticeable right sided limp. Additionally, the mother states that the patient has had occasional right hip pain for the past year. He has been able to participate in all school physical activities, albeit with some difficulty.
What's the diagnosis?
Aneurysmal bone cysts (ABCs) are benign bone lesions that predominantly develop in the pediatric population and form to create blood-filled cavities lined with fibroblasts, giant cells and trabecular bone (Figure 1). They are commonly found in the first two decades of life, and can present in any skeletal location, although there is a propensity for their development in the metaphysis of long bones. Their origin is thought to arise as either a primary neoplasm from a gain-of-function translocation causing rapid growth and expansion of lesions, or secondarily from other benign bone tumors [i].
ABCs universally present with pain and swelling at the affected site. Plain film radiography is obtained to identify the localized destruction and dilation of affected bone. Plain films characteristically demonstrate a radiolucent lesion surrounded by a thin layer of bony cortex (Figure 2); because plain films do not fully demonstrate the extent of invasion into nearby structures, or tissue characteristics and adjacent edema, cross-sectional imaging is also obtained [i].
Computed tomography is less frequently used as an imaging adjunct in the pediatric population due to radiation exposure; however when compared to magnetic resonance imaging (MRI), it is better able to evaluate cortical changes and diagnose pathologic fractures [ii].
Combined with plain radiography, MRI provides the highest positive predictive value and interobserver agreement in diagnosing ABCs [iii]. Features characteristically apparent on MRI include trabecular loculations, adjacent soft tissue edema, and fluid-fluid levels comprised of cellular blood products layering under serous components (Figure 3). However, fluid-fluid levels may also be present in malignant neoplasms, namely telangiectatic osteosarcomas, thereby necessitating a histologic analysis to achieve a definitive diagnosis [iv].
The standard treatment of ABCs is surgical: curettage with or without bone grafting depending on the size and position of the resulting void. Adjuvant therapies developed to decrease recurrence include augmentation of curettage with a high speed burr, argon beam coagulation, addition of phenol, cryosurgery, cementing, radiotherapy, arterial embolization and sclerotherapy. An emerging technique is “curopsy,” a less aggressive surgical technique involving percutaneous biopsy to obtain lining for diagnostic evaluation that by itself is hypothesized to disrupt the internal lesion architecture and induce healing [i]. Curopsy may be combined with an experimental treatment, instillation of doxycycline, which has anti-neoplastic properties and has been shown to further reduce recurrence [v].
The patient was admitted for interventional radiology guided percutaneous biopsy of the lesion, with instillation of intra-lesional doxycycline. The biopsy specimen was reviewed by pathology, and deemed insufficient for definitive diagnosis although aneurysmal bone cyst was suspected. Post-procedure, the patient was monitored with no complications, and discharged with pain control and follow-up.
Resident Reviewer: Dr. Ross
Faculty Reviewer: Dr. Tubbs
[i] Park HY, Yang SK, Sheppard WL, et al. Current management of aneurysmal bone cysts. Curr Rev Musculoskelt Med. 2016;9:435-44.
[ii] Wyers MR. Evaluation of pediatric bone lesions. Pediatr Radiol. 2010;40:468-73.
[iii] Mahnken AH, Nolte-Ernsting CCA, Wildberger JE, et al. Aneurysmal bone cyst: value of MR imaging and conventional radiography. Eur Radiol. 2003;13:1118–24.
[iv] Biermann JS. Common benign lesions of bone in children and adolescents. J Pediatr Orthop. 2002;22:268-73.
[v] Shiels WE 2nd, Beebe AC, Mayerson JL. Percutaneous Doxycycline Treatment of Juxtaphyseal Aneurysmal Bone Cysts. J Pediatr Orthop. 2016;36(2):205-12.
WELCOME BACK TO ANOTHER CLINICAL IMAGE FROM THE CASE FILES OF THE BROWN EM RESIDENCY!
HPI: A 27 year-old male with no significant past medical history presents to the ED with chest pain. The patient states about 30 minutes prior to arrival he was eating a meatball sub and got the feeling “like something was stuck in my chest”. He drank water in an attempt to push the food into his stomach but it did not help. He then tried to self induce vomiting multiple times when he developed acute chest pain. Over the course of several minutes his chest pain worsened and was now associated with shortness of breath, nausea and dry heaving, dysphagia, and odynophagia. He also feels as though his voice sounds hoarse. He denies dizziness, syncope, palpitations, or abdominal pain. He’s been otherwise well leading up to this.
Vitals: T: 99.4 BP: 138/68 P: 86 R: 16 SpO2: 100% on room air
Physical examination: Patient found sitting up in the stretcher and appears extremely uncomfortable. He is cool and clammy. He has diminished breath sounds bilaterally. He is noted to have right facial and neck swelling as well as underlying crepitus. Heart sounds are regular rate and rhythm without murmur, rub, or gallop. He has no abdominal tenderness, guarding, rebound, or distention. No other pertinent exam findings.
Chest X-ray imaging was obtained:
What does the chest x-ray show and what is the presumptive diagnosis?
In the chest x-ray above we can see air tracking along the mediastinum (red arrows), as well as extensive subcutaneous emphysema tracking into the neck (green arrows).
The patient’s respiratory distress worsened and he became hypoxic requiring oxygen by NRB. Repeat chest x-ray demonstrated a left sided pneumothorax, as identified by a pleural line (arrows).
Some quick facts about Boerhaave’s Syndrome:
- Full thickness perforation of the esophagus after a sudden increase in intraesophageal pressure, typically in the setting of forceful emesis.
- Foreign body ingestion and food impaction may also result in perforation either directly or indirectly (forceful vomiting).
- Blunt or penetrating neck trauma can also cause perforation, as well as instrumentation (endoscopy).
- Most perforations are left sided and distal, although proximal perforations are more commonly seen with instrumentation.
- Classic presentation is sudden onset, severe chest pain following forceful emesis. It often radiates into the back, abdomen, neck, and shoulders.
- Patients are typically ill appearing, diaphoretic, dyspneic, and/or tachycardic.
- On examination patients may have subcutaneous crepitus appreciated in the chest and neck. Hamman’s crunch, an audible crepitus appreciated on heart auscultation, is sometimes heard in the setting of pneumomediastinum.
- Chest x-ray imaging may reveal pneumomediastinum, pneumoperitoneum, pneumothorax, subcutaneous air, or pleural effusions (typically left sided), although a normal x-ray does not rule out the diagnosis as mediastinal emphysema takes time to develop.
- Patients can develop mediastinitis, pneumonitis, or peritonitis from the leakage of esophageal contents, which can rapidly develop into septic shock.
- ED management includes resuscitation in the setting of septic shock, administration of broad spectrum antibiotics (consider anti-fungals as well), and surgical consultation.
- Delay in diagnosis and treatment > 24 hours is associated with an increased rate in mortality.
- Definitive management ranges from conservative for smaller tears with a more indolent clinical course, to surgical management for more severe perforations.
Pneumomediastinum versus Pneumopericardium: Does the distinction matter?
Of course! The main importance is that pneumopericardium can develop tension physiology, whereas as pneumomediastinum typically does not. With pneumomediastinum, in addition to air tracking along the mediastinum, you can also see a pleural edge along the upper heart border. In this case, the pleural reflection is very thin, versus pneumopericardium in which there is a much thicker edge given the thickness of the pericardium. In pneumopericardium the air is confined to the pericardial space, where in pneumomediastinum it often decompresses into the subcutaneous tissue.
Our patient underwent a left sided chest tube insertion for management of his pneumothorax followed by barium swallow to assess the severity of his perforation. He was found to have a small, focal, contained perforation in the distal esophagus.
He received broad spectrum antibiotics and was admitted to the cardiothoracic surgery service where he underwent successful conservative management.
Faculty Reviewers: Dr. Alyson McGregor and Dr. Robert Tubbs
1: Gorrochategui, M., et. al. Pneumothorax. Radiopaedia. 2017 <https://radiopaedia.org/articles/pneumothorax>.
2: Gorrochategui, M., et. al. Pneumomediastinum. Radiopaedia. 2017. <https://radiopaedia.org/articles/pneumomediastinum>.
3: Raymond, D., Jones, C. Surgical Management of Esophageal Perforation. UptoDate. 2017.
4: Tintinalli, et. al. Emergency Medicine. 8th Edition. 2016. 328; 511-512.
WELCOME BACK TO ANOTHER CLINICAL IMAGE FROM THE CASE FILES OF THE BROWN EM RESIDENCY!
HPI/ROS: 49-year-old female with a history of hypothyroidism and asthma presents to the ED with right eye swelling and pain. She reports that four days ago she initially developed a severe right-sided headache, which progressed to right eye swelling, redness and pain with movement. She was seen at an urgent care center and diagnosed with conjunctivitis and treated with topical antibiotics[am1] . Today, she awoke with a new rash on her scalp as well as chills, nausea, and watery discharge from the eye. She denies visual changes or fevers.
Vital Signs: T: 98.6, HR: 91, BP: 123/73, R: 16, SpO2: 99% on room air
Visual Acuity: R 20/25 L 20/25
Physical Examination: The patient is alert and oriented. Normocephalic, atraumatic head. Tympanic membranes are clear. Oropharnyx clear and moist. Cranial nerves II-XII are intact. Pupils are 4 mm and reactive bilaterally. Extra-ocular movements are intact. Peripheral vision is intact. Patient accommodates appropriately. Neck is supple. Lungs are clear to auscultation. Heart is regular rate and rhythm without murmurs, rubs, or gallops. Abdomen is soft, non-tender, non-distended. A rash is appreciated above the right eye with some associated mild peri-orbital swelling (see image 1). There is conjunctival injection. Slit lamp examination is performed as well (see image 2). No other pertinent exam findings.
What’s the diagnosis?
Here are some quick facts:
- Herpetic Zoster Ophthalmicus (HZO) is a vision threatening condition secondary to Varicella Zoster Virus (VZV) reactivation, “shingles”, within the trigeminal ganglion, specifically the first division (V1).
- Up to one-half of all patients with VZV V1 reactivation experience direct ocular involvement.
- Typical prodromal symptoms include headache, malaise, fever, pain and photophobia in the affected eye and surrounding dermatome.
- Upon eruption of vesicular lesions within the trigeminal dermatome, patients will likely experience hyperemic conjunctivitis, blurred vision, and/or lid droop. The rash typically does not cross the midline.
- Two thirds of patients will develop corneal involvement (keratitis), which can either manifest as punctate (our patient) or dendritic lesions on slit lamp examination.
- The anterior chamber can show cells and flare if deeper structures are affected (iritis).
- Lesions on the nose are fairly specific for HZO due to involvement of the nasociliary branch of the trigmeninal nerve, which also innervates the eye.
- Early diagnosis is critical and management involves oral anti-retrovirals and adjunctive topical steroid drops to reduce the inflammatory response. Associated conjunctivitis can be treated with topical erythromycin ointment. Pain reduction can be achieved with topical cycloplegic agents.
- If the patient is immunocompromised or systemically ill, consider admission with IV acyclovir.
- Prompt ophthalmological follow up is warranted as well.
This patient was discharged home on oral acyclovir and topical steroid drops. She had follow up with ophthalmology the following day.
Dr. Alyson McGregor
Albrecht, Mary. Clinical Manifestations of Varicella-Zoster Virus Infection: Herpes Zoster. UptoDate. 2017.
Tintinalli, et. al. Emergency Medicine. 8th Edition. 2016. 1061-1062.
The contents of this case were deliberately altered to protect the identity of the patient. All content in this report are for educational purposes only. The patient consented to the use of these images.