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Pulmonary, Critical Care, and Sleep Pearls |

A 7-Year-Old Boy With Sudden Onset of Loud Snoring FREE TO VIEW

Lourdes M. DelRosso, MD
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Division of Pulmonary Medicine, Department of Pediatrics, University of California at San Francisco, San Francisco, CA

CORRESPONDENCE TO: Lourdes M. DelRosso, MD, University of California at San Francisco Benioff Children's Hospital, 747 52nd St, Oakland, CA 94609


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(5):e133-e135. doi:10.1016/j.chest.2016.04.002
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A 7-year-old boy was referred for evaluation of loud nightly snoring. Snoring started suddenly 2 weeks prior to presentation and grew progressively worse. Currently, the parents witnessed breathing pauses and gasping at night. The parents moved the child to a recliner to be able to breathe better, but the snoring and apneas persisted. There was no sleepwalking, night terrors, or nocturnal enuresis. During the day, the boy did not endorse symptoms of excessive sleepiness. The review of systems was negative for fever, weight loss, night sweats, sore throat, dysphagia, nasal congestion, ear pain, chest pain, shortness of breath, stridor, and abdominal pain. The child did not complain of throat discomfort. The parents noticed a sudden change in the child’s voice and described it as “muffled, like speaking with cotton balls in his mouth.” His school performance did not change. He had had a viral illness 2 weeks prior to presentation that was diagnosed as viral pneumonia. He was not on medications.

Figures in this Article

The patient’s physical examination revealed a well-developed boy in no acute distress. He was afebrile. His vital signs were normal, and his height and weight were within the 50th percentile. The child appeared in no distress. He had no adenoid facies, midface hypoplasia, or retrognathia. Nares were patent, with no evidence of turbinate hypertrophy. The oropharyngeal examination is shown in Figure 1. There was no lymphadenopathy. There was no stridor or harsh inspiratory sounds. Lungs were clear to auscultation bilaterally. The remainder of the examination was normal.

Figure 1

Question: What are the next steps in the evaluation of this patient?

Answer: The patient presented with rapid onset of snoring and signs of airway obstruction. The physical examination revealed significant tonsillar asymmetry, and the clinical presentation suggested a malignant process. In this case, admission with otolaryngology consultation and tonsillectomy are recommended.

Although mild tonsillar asymmetry in the absence of other symptoms is usually benign, sudden pronounced tonsillar asymmetry may represent a serious underlying condition. Unilateral tonsillar enlargement can be commonly associated with peritonsillitis, peritonsillar abscess, and pharyngeal tumors; in the first two cases, there is usually associated fever and sore throat. Among pharyngeal tumors, lymphoma localized to the tonsils is the greatest concern in this case. Although observation is indicated for children with mild tonsillar asymmetry, children with rapid significant tonsillar asymmetry should undergo prompt tonsillectomy.

Non-Hodgkin's lymphoma (NHL) in children usually presents with unilateral tonsillar enlargement or tonsillar asymmetry. Lymphomas are the third most common childhood cancer and the most common malignancy of the head and neck in children. NHL is the most common type of lymphoma in children, and the palatine tonsils are the most common extranodal sites involved. Burkitt lymphoma is the most common subtype of NHL; it is considered one of the most aggressive malignancies and one of the fastest growing tumors (doubling time of 24-48 h). Burkitt lymphoma presents in three variants: endemic, sporadic, and HIV associated. The endemic type is usually associated with Epstein-Barr virus, and it affects children with an average age of onset of 9 years. This type is usually localized and commonly affects the Waldeyer ring (tonsils and adenoids). Symptoms depend on the location of the tumor; when localized in the tonsils, symptoms may include snoring, dysphagia, dyspnea, and potentially threatening airway obstruction. Pain is usually absent. Sporadic cases occur in older children (age 12 years) and usually affect the abdomen, medulla, or lymphatic nodules (rarely affects the neck). Only 2.9% of sporadic Burkitt lymphomas are localized to the tonsils as the initial presentation. The HIV type affects the abdomen and the bone marrow.

Diagnosis is made on the basis of histopathologic findings. Localized Burkitt lymphoma is successfully treated with chemotherapy, with survival rates of 90%.

Clinical Course

In this patient, the level of suspicion for a fast-growing malignancy was high due to the sudden onset of snoring, the quick worsening of symptoms requiring sleeping on a recliner, and the dramatic asymmetry in tonsil size. A CT scan performed on admission showed a 5.1-cm left palatine mass that extended across the midline and inferiorly past the epiglottis. The patient underwent tonsillectomy, and the snoring and voice changes resolved immediately after the surgery. Histopathologic analysis of the tonsillar tissue revealed morphologic and immunophenotypic features diagnostic of classic Burkitt lymphoma: routine hematoxylin and eosin–stained sections revealed extensive infiltration of tonsillar tissue and underlying skeletal muscle by a dense and diffuse lymphoid infiltrate composed of atypical, intermediate-sized lymphoid cells. There were frequent scattered mitotic figures and apoptotic debris among these cells, as well as scattered body macrophages that imparted a “starry sky” appearance to the infiltrate on low-power magnification.

PET scans performed postsurgically revealed edema and hypermetabolism localized to the left peritonsillar resection bed thought to be either postoperative or secondary to residual disease. The patient underwent a four-phase chemotherapy regimen consisting of a 1-week reduction phase (cyclophosphamide, vincristine, prednisone, and methotrexate), two 4-week induction phases (cyclophosphamide, vincristine, prednisone, doxorubicin, and methotrexate), and a 4-week consolidation phase (cytosine arabinoside and methotrexate). A PET scan conducted after the second phase was normal. The child ended chemotherapy 4 months ago and remains asymptomatic. He does not snore, gasp, or have breathing pauses; he also has no dysphagia or postsurgical pharyngeal weakness. The patient’s postsurgical oropharyngeal examination is shown in Figure 2.

Figure 2
Figure Jump LinkFigure 2 Postsurgical oropharyngeal examination.Grahic Jump Location

  • 1.

    Sudden onset of snoring with fast-growing asymmetric tonsils is highly suspicious of malignancy.

  • 2.

    Burkitt lymphoma can be localized to the tonsils and is the fastest growing tumor, with a doubling size of 24 to 48 h.

  • 3.

    The diagnosis and management of Burkitt lymphoma should not be delayed due to the risk of rapid tumor growth and associated complications (airway obstruction).

Financial/nonfinancial disclosure: None declared.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.


Figures

Figure Jump LinkFigure 2 Postsurgical oropharyngeal examination.Grahic Jump Location

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