Case Reports Eyelid Swelling, an Unusual Presentation of Pneumomediastinum Richard Y. Hwang, M.D., Ph.D., Kasra A. Rezaei, M.D, and Mark R. Melson, M.D. Abstract: An 11-year-old girl with no significant medical or ocular history presented to the emergency department with asymmetrical eyelid swelling for 2 days. She was noted to have eyelid crepitus on examination. The patient reported a prolonged episode of crying followed by chest pain and dyspnea that resolved prior to her presentation. Both face and chest CT showed extensive air tracking in the subcutaneous tissues of the neck and face and pneumomediastinum without pneumothorax. The patient was discharged home without further intervention. On follow-up 1 to 2 weeks later, she had complete resolution of her symptoms. While pneumomediastinum is extremely rare in the pediatric population, it can be considered in the differential diagnoses of eyelid swelling and crepitus in the appropriate context. To this author’s knowledge, this is the first pediatric case of pneumomediastinum with an initial presentation of eyelid swelling.

OD and 20 in the OS. Her anterior and posterior examinations were unremarkable. CT imaging of the face revealed extensive subcutaneous emphysema (Fig. 2A) involving the face, eyelid (Fig. 2B), and neck with retrobulbar air. This prompted further evaluation of the source of the subcutaneous air, and chest CT showed extensive pneumomediastinum (Fig. 2C,D) without pneumothorax with air tracking in the subcutaneous tissues of the neck and right lateral chest wall. An esophagram demonstrated an intact esophagus. The patient was discharged home later that day and followed up in ophthalmology clinic 1 to 2 weeks later. Her facial swelling and crepitus were completely resolved, and extraocular movements were full without diplopia.

DISCUSSION The most common underlying condition of spontaneous pneumomediastinum is asthma with up to 35% of cases associated with asthma or other pulmonary diseases.3,9 Causes of spontaneous pneumomediastinum include infectious processes such as bronchitis or viral pneumonia and valsalva maneuvers, such as coughing, screaming, or deep breathing.10,11 Other

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pontaneous pneumomediastinum is defined as free air or gas contained within the medastinum that is not associated with surgery, procedural intervention, foreign body ingestion, or mechanical ventilation.1–6 It is rare, with an estimated incidence of 1 in 14,000.7,8 The case presented here is that of spontaneous pneumomediastinum with an initial presentation of asymmetrical eyelid swelling.

CASE REPORT An 11-year-old girl with no medical history, including that of asthma or recent fever, presented with 1 day of eyelid swelling. On examination, she was noted to have right greater than left periorbital and facial swelling with crepitus. The patient reported crying for several hours the day before because of an incident at school. She subsequently developed chest pain, dyspnea, and facial swelling. She was seen at an urgent care clinic and noted to have oxygen saturation in the low 90s. She was initially treated with Benadryl and steroids for concern of an allergic reaction, though no inciting allergen was identified. The patient’s chest pain and dyspnea eventually resolved and her oxygen saturation rose to high 90s. However, her facial swelling persisted the following day, at which time a local ophthalmologist noted significant right eyelid swelling and crepitus associated with impaired supraduction, for which the patient was instructed to go to the emergency department (ED) for further reevaluation. In the ED, the patient’s examination was again notable for nonerythematous right greater than left periorbital swelling with crepitus, most notably in her right eyelid (Fig. 1). She had impaired OD supraduction of −2 with mild diplopia in upgaze. The remainder of her examination was normal, with visual acuity of 20/20 in each eye and intraocular pressures of 17 in the Vanderbilt Eye Institute, Nashville, Tennessee, U.S.A. Accepted for publication June 17, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Mark R. Melson, M.D., Vanderbilt Eye Institute, 2311 Pierce Avenue, Nashville, TN 37232. E-mail: [email protected] DOI: 10.1097/IOP.0b013e3182a22f0b

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FIG. 1.  A, Photograph of the patient’s face on initial presentation reveals mild facial swelling and significant right greater than left upper eyelid swelling. B, Close up of the patient’s right upper eyelid is noted to have significant swelling without erythema. Examination revealed crepitus.

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FIG. 2.  A, Coronal image of noncontrasted face CT shows air tracking in subcutaneous neck tissues (arrow). B, Axial image of noncontrasted face CT shows air tracking in subcutaneous facial tissues, including the right upper eyelid (arrow). C, Axial image of noncontrasted chest CT demonstrates air tracking up the neck (arrow). D, Axial image of noncontrasted chest CT reveals air in the mediastinum (arrow).

causes include diabetic ketoacidosis, esophageal rupture,12 and foreign body inhalation.13 It is thought that alveolar rupture in the pulmonary interstitium followed by dissection of gas toward the hilum and mediastinum with tracking of free air into the subcutaneous space of the upper chest and neck is responsible for pneumomediastinum. A less common cause can occur when air escapes the respiratory tract, gastrointestinal tract, or intrathoracic airways.14 Clinical presentation typically includes chest pain, neck pain, dyspnea, and subcutaneous emphysema. Exam findings may include Hamman’s sign, which is precordial crepitus auscultated during systole. The primary differential diagnoses are esophageal perforation, pneumothroax, and pericarditis. Diagnosis is confirmed by chest radiologic studies. Management includes treating the underlying cause if one is identified, rest with avoidance of valsalva and other aggravating factors, analgesics, and clinical monitoring. Predisposing factors should be identified so that they can be managed to minimize recurrence. Typically, management can be performed in an outpatient setting unless significant hypoxia, respiratory distress, or esophageal perforation is present. Complications can include laryngeal compression, pseudotamponade, mediastinitis in the setting of esophageal perforation, and tension pneumomediastinum or pneumothorax.7

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In the case of the patient, the cause of her pneumomediastinum most likely arose from alveolar rupture from a prolonged period of valsalva-like maneuvers related to her severe and excessive crying. She did not have any complications that precluded outpatient management. No other predisposing factors were identified, and the patient was advised to be more cognizant of symptoms during times of strong emotion or stress. To this author’s knowledge, this is the first pediatric case of pneumomediastinum with an initial presentation of eyelid swelling.

REFERENCES 1. Chang MY, Chang ML, Wu CT. Esophageal perforation caused by fish vertebra ingestion in a seven-month-old infant demanded surgical intervention: a case report. World J Gastroenterol 2006;12:7213–5. 2. Findlay CA, Morrissey S, Paton JY. Subcutaneous emphysema secondary to foreign-body aspiration. Pediatr Pulmonol 2003;36:81–2. 3. Marathe US, Tran LP. Pediatric neck trauma causing massive subcutaneous emphysema. J Trauma 2006;61:440–3. 4. Okada T, Sasaki F, Todo S. Perforation of the piriform recessus by a swallowed glass splinter presenting as pneumomediastinum in a child. Pediatr Surg Int 2004;20:643–5.

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5. Otgün I, Fakioğlu E, Arda IS, et al. Subcutaneous emphysema and pneumomediastinum due to foreign body aspiration. Eur J Pediatr Surg 2008;18:129–30. 6. Peña MT, Aujla PK, Choi SS, et al. Acute airway distress from endotracheal intubation injury in the pediatric aerodigestive tract. Otolaryngol Head Neck Surg 2004;130:575–8. 7. Chalumeau M, Le Clainche L, Sayeg N, et al. Spontaneous pneumomediastinum in children. Pediatr Pulmonol 2001;31:67–75. 8. Yellin A, Gapany-Gapanavicius M, Lieberman Y. Spontaneous pneumomediastinum: is it a rare cause of chest pain? Thorax 1983;38:383–5. 9. Cay A, Imamoğlu M, Sarihan H, et al. Tracheobronchial rupture due to blunt trauma in children: report of two cases. Eur J Pediatr Surg 2002;12:419–22. 10. Chiu CY, Wong KS, Yao TC, et al. Asthmatic versus non-asthmatic spontaneous pneumomediastinum in children. Asian Pac J Allergy Immunol 2005;23:19–22. 11. Lee CY, Wu CC, Lin CY. Etiologies of spontaneous pneumo mediastinum in children of different ages. Pediatr Neonatol 2009;50:190–5. 12. Engum SA, Grosfeld JL, West KW, et al. Improved survival in children with esophageal perforation. Arch Surg 1996;131:604–10; discussion 611. 13. Burton EM, Riggs W Jr, Kaufman RA, et al. Pneumomediastinum caused by foreign body aspiration in children. Pediatr Radiol 1989;20:45–7. 14. Mason RJ, Broaddus C, Martin T, et al. Murray and Nadel’s Textbook of Respiratory Medicine. 5th ed. Philadelphia, PA: Saunders, An Imprint of Elsevier Inc, 2010.

Recurrent Cholesterol Granuloma of the Orbit Yoon Jeon Kim, M.D., and Ho-Seok Sa, M.D. Abstract: A 40-year-old woman presented with a 1-year history of left proptosis and hypoglobus. Eight years ago, she had undergone the surgical removal of left orbital cholesterol granuloma by Krönlein lateral orbitotomy. MRI showed a lesion of high T1-signal intensity and heterogeneous T2signal intensity, which did not demonstrate gadolinium enhancement. The lesion was excised via a sub-brow skin incision, and careful drilling and curettage of the affected bony surface of the superolateral orbit were also performed. The diagnosis of orbital cholesterol granuloma was made by histopathologic examination. There was no evidence of tumor recurrence after 2 years of follow up. The authors present a rare case of recurrent cholesterol granuloma of the orbit and its successful surgical treatment with complete excision of the tumor and the surrounding bony base.

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holesterol granuloma of the orbit is an uncommon, bonedestroying lesion characterized by a granulomatous reaction to cholesterol crystals and associated fibrosis.1 Once it is surgically removed, recurrence is known to be extremely rare owing to the absence of epithelial elements.2 As far as is Department of Ophthalmology, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea Accepted for publication June 24, 2013. Presented, in part, as a poster at the 27th annual meeting of The Asia Pacific Academy of Ophthalmology, Pusan, Korea, 2012. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Ho-Seok Sa, M.D., Department of Ophthalmology, Asan Medical Center, University of Ulsan, College of Medicine, 388-1 Pungnap-2-dong, Songpa-gu, Seoul 138-736, Korea. E-mail: [email protected] DOI: 10.1097/IOP.0b013e3182a22f26

Case Reports

known, there were only 5 reported cases of recurrent cholesterol granuloma of the orbit.1–5 Another case of rare, recurrent cholesterol granuloma is reported and the treatment considerations discussed. Prior to the study, approval from the Institutional Review Board at Asan Medical Center was obtained.

CASE REPORT A 40-year-old woman presented with a 1-year history of left proptosis and hypoglobus, which seemed to have progressed recently. She had a medical history of left orbital mass removal by Krönlein lateral orbitotomy at an outside hospital 8 years ago. Her medical records revealed that the mass had been diagnosed as cholesterol granuloma and was treated by excision without bony tissue removal. On clinical examination, her OS was 3 mm inferiorly displaced compared with the OD, and Hertel exophthalmometry revealed 3 mm of left proptosis. Her other ophthalmologic examination was unremarkable. MRI demonstrated a 2.5 × 2.3 × 1.4-cm-sized, extraconal mass displacing the globe and orbital contents inferomedially (Fig. 1). It showed high signal intensity on T1-weighted images and heterogeneous signal intensity on T2-weighted images. It did not demonstrate gadolinium enhancement. The patient underwent an excisional biopsy via a subbrow skin incision. A cystic mass was detected, which contained dark-brown fluid and yellow granular material. Accompanied by the release of thick fluid, the cystic wall was completely removed without violating the periorbita. Careful drilling and curettage of the affected bony surface of the superolateral orbit were also performed. Histopathologic examination revealed chronic granulomatous inflammation, consisting of numerous cholesterol clefts, blood breakdown products, fibrosis, and bony fragments, which was consistent with cholesterol granuloma (Fig. 2). The patient’s preoperative proptosis and hypoglobus completely resolved postoperatively, and the patient showed a successful clinical course without recurrence for 2 years following the surgery.

DISCUSSION Cholesterol granuloma of the orbit is a rare disease, which should be differentiated from other osteolytic lesions in the superolateral orbit, such as epidermoid cyst, cholesteatoma, chondroma, mucocele, and lacrimal gland tumors.1–6 MRI has facilitated the preoperative diagnosis of cholesterol granuloma because of its typical findings. On T1- and T2-weighted images, it is seen as a nonenhancing lesion with high signal intensity due to its high lipid cholesterol content, and it does not have sclerotic margins.1–3 In the present case, however, the lesion showed heterogeneous signal intensity on T2-weighted images, and it is possibly due to its greater blood component. Pathologic examination is confirmative for the diagnosis of cholesterol granuloma, and the lack of an epithelial lining is the most important feature for arriving at a differential diagnosis.1,2 While some authors have argued that complete extirpation of the adjacent bone is not necessary, there is general agreement that surgical excision of the mass and curettage of the bony base is a virtually curative treatment and that subsequent recurrence is truly rare. There were only 5 cases of recurrent orbital cholesterol granulomas.1–5 However, the rate of recurrent cholesterol granuloma of the petrous apex, the common area in the head and neck region, was reported in up to approximately 30% of the study patients.6 This could be because exposing a tumor of the petrous apex is more difficult than that of the orbit, which emphasizes the importance of the

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Eyelid swelling, an unusual presentation of pneumomediastinum.

An 11-year-old girl with no significant medical or ocular history presented to the emergency department with asymmetrical eyelid swelling for 2 days. ...
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