The new england journal of medicine Case Records of the Massachusetts General Hospital Founded by Richard C. Cabot Eric S. Rosenberg, M.D., Editor Nancy Lee Harris, M.D., Editor Jo-Anne O. Shepard, M.D., Associate Editor Alice M. Cort, M.D., Associate Editor Sally H. Ebeling, Assistant Editor Emily K. McDonald, Assistant Editor Case 25-2015: An 8-Year-Old Girl with a Chest-Wall Mass and a Pleural Effusion Samuel M. Moskowitz, M.D., Randheer Shailam, M.D., and Eugene J. Mark, M.D. Presentation of Case Dr. Jessica M. Rosenthal (Pediatrics): An 8-year-old girl who had required long-term From the Departments of Pediatrics tracheostomy because of airway stenosis caused by an injury in infancy was admitted (S.M.M.), Radiology (R.S.), and Patholo- gy (E.J.M.), Massachusetts General Hos- to this hospital because of a chest-wall mass and a large pleural effusion. pital; and the Departments of Pediatrics The patient was born in China. At 7 months of age, she underwent emergency (S.M.M.), Radiology (R.S.), and Pathology tracheostomy after a caustic injury from either ingestion or inhalation. At 2.5 years (E.J.M.), Harvard Medical School — both in Boston. of age, she was adopted by an American family and moved to the United States. Her adoptive parents noted that she had stridor and snoring, and her pediatrician N Engl J Med 2015;373:657-67. DOI: 10.1056/NEJMcpc1400836 referred her to an otolaryngologist at the Massachusetts Eye and Ear Infirmary (MEEI) Copyright © 2015 Massachusetts Medical Society. at 2 years 10 months of age. On examination, the nasopharynx was completely ob- structed; there was fusion of the posterior tongue and hard palate and of the hard palate and epiglottis, with a narrow opening in the oropharynx. The supraglottic pas- sage was normal beyond the stenosis; the subglottic airway was not evaluated. A tracheostomy was performed, and the left nasal choanal obstruction was opened with a laser. Repeat bronchoscopy revealed that the larynx and subglottic trachea were normal. On two occasions, esophagogastroduodenoscopy revealed no evidence of gastroesophageal reflux. The tracheostomy tube was removed when the patient was 5.5 years of age; there was a residual tracheal–cutaneous fistula. When she was 7.5 years of age (7 months before this admission), another trache- ostomy was performed because of worsening oropharyngeal stenosis that pre- cluded intubation and because of concern about possible obstruction. The patient had been in her usual state of health until approximately 6.5 weeks before this admission; during a 10-day period, her mother noted recurrent blood- tinged sputum in the tracheostomy tube, and an episode of bleeding at the trache- ostomy site occurred at school. The patient was seen in the emergency department of the MEEI, where a flexible tracheoscopy, which was performed at the bedside after removal of the tracheostomy tube, revealed no exposed blood vessels or granulation tissue in the trachea or stoma. A chest radiograph showed consolida- tion in the right lower lobe, which was thought to be due to aspiration, pneumo- nia, or atelectasis. Gram’s staining of the sputum showed abundant neutrophils, abundant gram-positive cocci in pairs and a few chains, abundant gram-negative diplococci, a few gram-positive rods, and a few thin gram-negative rods. Culture n engl j med 373;7 nejm.org August 13, 2015 657 The New England Journal of Medicine Downloaded from nejm.org at The University Of Illinois on March 10, 2017. For personal use only. No other uses without permission. Copyright © 2015 Massachusetts Medical Society. All rights reserved. The new england journal of medicine of the sputum from the tracheostomy tube grew and siblings; she was physically active, did well in methicillin-susceptible Staphylococcus aureus, Morax- school, and was able to breathe, speak, and eat ella catarrhalis, Haemophilus influenzae, and Strepto- normally, with the tracheostomy tube capped while coccus pneumoniae. The patient remained afebrile, she was awake. She lived with two dogs and a cat, and no antibiotic agents were administered. She and the mother smoked outside the home; she was admitted overnight for observation and dis- had not recently traveled or had contact with ill charged home the next day (5 weeks before this persons. admission). She had one more episode of blood- On examination, the temperature was 37.0°C, tinged sputum thereafter. the blood pressure 102/68 mm Hg, the pulse During the 2 weeks before this admission, the 135 beats per minute, the respiratory rate 16 to oxygen saturation, which was obtained during 30 breaths per minute, and the oxygen saturation continuous overnight monitoring for sleep apnea, 95% while the patient was breathing ambient air. fell to approximately 85% (from the usual level The oropharynx was clear, without erythema or of approximately 95%) for prolonged periods. focal lesions. The neck was supple, and the tra- Examination at school revealed decreased breath cheostomy site was clean and dry, with an under- sounds at the base of the right lung. One day be- lying small cavity. Lymphadenopathy (with nodes fore this admission, while the patient was being measuring up to 1 cm in diameter) in the right bathed, her mother noticed that her chest was anterior cervical chain and a mildly tender right asymmetric, with the right nipple appearing lower axillary lymph node (approximately 1 cm in di- than the left, and that blood vessels were prom- ameter) were present. There were decreased inent on the right side of the chest. breath sounds at the base of the right lung. At the pediatrician’s office the next day, the There was a bulging area (4 cm by 7 cm) on the patient described a recent episode of discomfort right lateral chest wall, which was tender on in her chest that occurred while she was being palpation and had no fluctuance or overlying skin hugged. There was no history of respiratory dis- changes. The hematocrit, red-cell indexes, and tress, fevers, or cough. On examination, she was anion gap were normal, as were blood levels of tearful and hesitant. The temperature was 36.1°C, calcium, magnesium, glucose, albumin, amylase, and a tracheostomy tube was in place. The right lipase, uric acid, and lactic dehydrogenase and lateral chest wall was more prominent than the results of renal- and liver-function tests; other left, had no crepitus, and was tender to touch. test results are shown in Table 1. Breath sounds were normal on the left side and Dr. Randheer Shailam: A frontal chest radiograph diminished on the right. The right upper quad- that had been obtained 7 months before this rant of the abdomen was diffusely tender, and the admission showed a tracheostomy tube, clear remainder of the examination was normal. The lungs, and no pleural effusion. Frontal, lateral, patient was referred to another hospital for chest and bilateral decubitus chest radiographs that radiography, which revealed a large pleural effu- were obtained on the day of admission (Fig. 1) sion on the right side, with underlying pneumo- showed dense air-space opacification in the right nia. The patient was referred to the emergency lower lung zone, obscuring the right hemidia- department of this hospital. phragm and right cardiac border, as well as a large The patient had sleep apnea and a history of pleural effusion on the right side that tracked dental caries that necessitated the extraction of along the lateral chest wall, with evidence of locu- several deciduous teeth between 5 and 7 years lation. The left lung and pleural space were clear. of age. Immunizations, including the 7-valent Computed tomography (CT) of the chest, per- pneumococcal conjugate vaccine (PCV7), were formed on the same day after the administration current; she had reportedly received the bacille of contrast material (Fig. 2), revealed asymmetry Calmette–Guérin (BCG) vaccine at 1 month of of the chest wall that was due to multiple hetero- age. A purified protein derivative (PPD) skin test geneous soft-tissue masses and thickening of the was negative on her arrival in the United States. soft tissue of the right chest wall. Consolidation She took no medications and had no known aller- was present in the right lower lobe and portions gies. She lived at home with her adoptive parents of the right middle lobe. Areas of low attenua- 658 n engl j med 373;7 nejm.org August 13, 2015 The New England Journal of Medicine Downloaded from nejm.org at The University Of Illinois on March 10, 2017. For personal use only. No other uses without permission. Copyright © 2015 Massachusetts Medical Society. All rights reserved. Case Records of the Massachusetts General Hospital tion in the right lower lobe were suggestive of de- Table 1. Laboratory Data.* creased perfusion and possible necrosis. A large loculated pleural effusion, irregularly shaped pa- Reference Range, On Admission, renchymal nodules in the right upper lobe, promi- Variable Age-Adjusted† This Hospital nent enhancing ipsilateral axillary lymph nodes, Hemoglobin (g/dl) 11.5–15.5 11.2 and subtle periosteal elevation of the medial as- White-cell count (per mm3) 4500–13,500 11,500 pect of the right eighth rib were also present. Dr. Rosenthal: The patient was admitted to the Differential count (%) pediatric intensive care unit. The temperature rose Neutrophils 33–59 73 to 38.7°C, and acetaminophen was administered, Lymphocytes 33–50 20 with improvement. On the second hospital day, a diagnostic pro- Monocytes 4–11 5 cedure was performed. Eosinophils 0–8 2 Platelet count (per mm3) 150,000–450,000 891,000 Differential Diagnosis Sodium (mmol/liter) 135–145 134 Dr. Samuel M.