A Child With a Brief History of Coughing Up Blood.

CASE PRESENTATION A 6-year-old boy presented to our institution with a history of 3 episodes of coughing up blood, approximately 1 month previously. The blood appeared fresh and was about a spoonful on each occasion. There was no history of fever, rapid breathing, chronic or recurrent cough, weight loss, trauma or suspected inhalation of a foreign body. There was no bleeding from any other site, including the gums, cheek mucosa, nasal cavity or mouth. There were no skin lesions suggesting petechiae, purpura or bleeding from other sites. There was no history of redness of the eyes, joint swelling, oral ulcers, blood in the urine or exposure to tuberculosis. The child had visited a local physician when the episodes occurred. Physical examination was reportedly normal. Laboratory investigations ruled out thrombocytopenia and coagulation abnormalities. A chest radiograph showed right upper lobe consolidation (Fig. 1). Contrast enhanced computed tomography (CECT) showed a focal area of consolidation within the anterior segment of the right upper lobe, measuring 43 × 34 mm in size. There was obliteration of the segmental bronchus and adjacent segmental atelectasis (Fig. 2). The pretracheal and right hilar region nodes were enlarged. The radiology report suggested an infectious etiology.FIGURE 1.: Chest radiograph showing an opacity in the right upper zone (red arrow) at initial presentation.FIGURE 2.: Chest CECT chest images. The mediastinal window (A–C) showed heterogenous fluid attenuation within the lesion (pink arrow), pretracheal lymphadenopathy (orange arrow), and right hilar lymphadenopathy (red arrow). The lung window (D) showed focal segmental consolidation in the right lung upper lobe (white arrow). The coronal image (E) showed obliteration of the segmental bronchus (yellow arrow).Abdominal ultrasonography showed a few enlarged mesenteric lymph nodes, the largest measuring 17 × 8 mm. Mycobacterium tuberculosis (MTB) was not detected in the smear of gastric lavage specimens. He was prescribed a week of oral co-amoxyclav (amoxycillin-clavulanate), and remained asymptomatic. However, a repeat chest radiograph after 2 weeks showed persistence of the right upper lobe consolidation (see Figure, Supplemental Digital Content 1, https://links.lww.com/INF/E979). Therefore, he was referred to our institution for further evaluation. Detailed history did not reveal anything new, except that his father had sustained an intracranial hemorrhage in a road traffic accident about 4 years previously. The child had attained age-appropriate developmental milestones and vaccinations as per the Indian National Immunization Schedule. Physical examination showed heart rate of 96 beats/minute, respiratory rate of 24 breaths/min, blood pressure of 90/60 mm Hg, temperature of 37 °C, and oxygen saturation of 98% in ambient air. His weight was 18.2 kg (−0.74 z-score), height was 121 cm (0.64 z-score) and body mass index was 12.43 (−1.79 z-score). There was no pallor, icterus, clubbing, lymphadenopathy, elevated jugular venous pressure, petechiae, purpura or ecchymoses. Careful nasal, oral and pharyngeal examination did not reveal any evidence of bleeding. The chest wall was symmetrical, the trachea was centrally positioned and the apex beat was visible and palpable in the fifth intercostal space medial to the mid-clavicular line. Vocal fremitus and vocal resonance were reduced, along with a dull percussion noted in the right supramammary area. Breath sounds were diminished in the same area. There was no other abnormality. Cardiovascular, abdominal and neurological system examinations were normal. The initial clinical possibilities considered were endobronchial tuberculosis, airway foreign body and an endobronchial mass. Investigations showed hemoglobin of 11.7 g/dL, total leukocyte count of 11,570/mm3 (64% polymorphs, 29% lymphocytes, 6% monocytes, 1% eosinophils), platelet count of 4,24,000/mm3 and normal red cell indices. Prothrombin time was 11.1 seconds, activated partial thromboplastin time was 30.5 seconds, prothrombin index was 100 and the international normalized ratio was 0.96. Liver and renal function, as well as serum electrolyte levels were normal. Fasting gastric lavage specimens did not show evidence of acid-fast organisms on Ziehl-Neelsen staining, and Xpert MTB RIF assay was negative. The specimens did not show hemosiderin-laden macrophages. Flexible fiberoptic bronchoscopy was performed under conscious sedation. The upper airway, larynx, trachea and carina were normal. The airways of the left lung, and the right middle and lower lobes were normal. The apical division of the right upper lobe showed a yellow, hard mass covered in mucus, obstructing the lumen completely (see Figure, Supplemental Digital Content 2A https://links.lww.com/INF/E979). The appearance resembled a corn kernel. The family confirmed that the child often ate corn off the cob, but denied any recollection of aspiration. Analysis of the mucus and bronchoalveolar lavage (BAL) fluid showed 115 cells/mm3, with 67% lymphocytes, and 33% polymorphs. In contrast, BAL from the left lung showed 14 cells/mm3 with 98% lymphocytes, and 2% polymorphs. BAL fluid Gram staining and bacterial culture did not show any organism in either lung. Ziehl-Neelsen staining for acid-fast bacilli and Xpert MTB RIF assay did not detect M. tuberculosis in either lung. BAL fluid fungal smear from both lungs did not show any fungal elements. Suspecting foreign body aspiration, the child underwent rigid bronchoscopy under general anesthesia on the same day, but the lesion was located beyond the view of the scope; hence, it could not be visualized. Therefore, flexible fiberoptic bronchoscopy was repeated, wherein the lesion appeared to be ivory colored (see Figure, Supplemental Digital Content 2B, https://links.lww.com/INF/E979). Analysis of brush scrapings and a biopsy from the lesion confirmed the diagnosis. Denouement The biopsy specimen and endobronchial scrapings were sent for cytopathology which showed acellular hyalinized laminated membranes of hydatid cyst, on Periodic Acid Schiff staining (see Figure, Supplemental Digital Content 3, https://links.lww.com/INF/E980). There were no hooklets. Polymerase chain reaction on suctioned fluid did not identify hydatid elements. Hydatid serology was reported positive with a titer of 1:1600. Abdominal ultrasonography did not identify hepatic hydatid cysts. The child underwent elective surgical excision of the lesion. Intraoperative findings revealed a collapsed right lung upper lobe (RUL) by a cystic structure, which was carefully transected from the surrounding lung parenchyma. The surgeon noted a “contained rupture” of the cyst where the contents of the cyst remain within the confines of the peri-cyst, and not a “communicating rupture.” The lung around the hydatid was consolidated. On gross examination, the cyst was pearly white, measuring 8 cm in diameter, and had a thickness of 0.1–0.2 cm (see Figure, Supplemental Digital Content 4, https://links.lww.com/INF/E980). No daughter cyst was identified. Histopathological examination showed acellular laminated pale eosinophilic membrane with germinal membrane, surrounded by eosinophilic rich infiltrate; however, no brood capsule or scolices were identified on hematoxylin and eosin staining (see Figure, Supplemental Digital Content 5, https://links.lww.com/INF/E980). The final diagnosis was pulmonary hydatid disease. On follow-up, 8 weeks after excision, the child was asymptomatic and the chest radiograph was normal. Cystic hydatid disease is caused by the larval cysts of Echinococcus granulosus, while Echinococcus multilocularis causes alveolar echinococcosis.1 The life cycle of E. granulosus involves two hosts. The definitive hosts (such as dogs or wolves) harbor the mature parasite in the small intestine, and excrete helminth eggs. The intermediate host (cattle, sheep, horses) get infected by eating contaminated grass. The eggs are hatched in the small intestine, and pass to the liver through the portal circulation, or the lungs through the systemic circulation. They develop into hydatid cysts in the viscera of the intermediate host. Human beings are accidental intermediate hosts, and get infected by consuming food or water contaminated with the parasite eggs, or by contact with infected dogs. Humans cannot transmit the disease and are dead ends for the parasite. About 40% to 80% cases with cystic hydatid present with a single cyst involving a single organ system.2 The liver is most often affected, followed by the lung.2 However, solitary pulmonary hydatid cyst (as in the index case) is not uncommon. The clinical presentation depends upon the site, size, intactness and associated complications such as rupture or infection. Small, unruptured lung hydatid cysts could remain asymptomatic for years and may be incidentally detected on chest radiograph. Growing cysts may create symptoms or signs of airway compression, or erosion of adjacent structures. In such situations, there may be cough (with or without sputum), breathlessness, hemoptysis or chest pain. Cyst rupture and escape of contents into the airway may be associated with fever, coughing up white membranous material, and even hypersensitivity reactions like urticaria, wheezing and rarely fatal anaphylaxis.3 A diagnosis of lung hydatid can be suspected (though not confirmed) on the basis of clinical presentation (especially in endemic regions or a history of exposure to sheep or dogs), characteristic radiological lesions and serology.3 On radiology, uncomplicated cysts usually appear as round or oval, homogenous opacities, with sharp borders, encircled by lung tissue. Complicated cysts occasionally have pathognomonic radiological signs like cumbos sign, serpent sign and water lily sign.4,5 On CECT chest, simple hydatid cysts appear as homogenous opacities with liquid attenuation, with peripheral enhancement of the rim. Complicated cysts may present with air bubble sign, or air fluid level within the cyst. Pleural rupture of a hydatid cyst may present with pleural effusion or hydropneumothorax.6 Serology for antihydatid IgG antibodies can be helpful, but is not confirmatory. Furthermore, while it is positive in about 90% of liver cysts, only 50% of lung hydatid cysts have positive serology.7 False-positive reactions have been observed with other helminthic infections, cancer and immune disorders. False-negative reports are reported with intact, calcified or nonviable cysts and also in children, and pregnant women. Thus, a definitive diagnosis of hydatid cystic hydatid disease can be confirmed by surgical excision and histopathologic examination or polymerase chain reaction confirmation of the excised material. There are a few case reports of incidental detection of hydatid cysts during bronchoscopy. The procedure is usually avoided for fear of rupturing the cyst (which can result in fatal anaphylaxis), and seeding unaffected parts of the lungs.8–10 On bronchoscopy, hydatid cyst membranes appear whitish-yellow, and may be covered with mucus as in the index case. This can make it difficult to differentiate it from other childhood endobronchial lesions such as mucus plugs, endobronchial tuberculosis or airway foreign body.11 However, our institution has developed experience and expertise in confirming pulmonary hydatid cyst in children, using flexible fiberoptic bronchoscopy under conscious sedation. Cysts invading the airways are often directly visualized, and/or analysis of (cautiously obtained) BAL or membrane fragments confirms the diagnosis.12,13 This is a significant advancement in the management of pulmonary hydatid disease, as it clinches the diagnosis even before surgical excision and histopathologic analysis. This experience enabled us to suspect a hydatid cyst (communicating with an airway) in the index case, despite the absence of characteristic symptoms, lack of radiological suspicion and anticipation of an airway foreign body. However, bronchoscopy and/or biopsy should be performed cautiously as there can be a risk of cyst rupture, spillage of contents with the risk of anaphylaxis, seeding of unaffected parts of the lungs and airway obstruction ruptured membranes. Pulmonary hydatid cysts can be treated surgically or medically. Surgery is the treatment of choice for large cysts, those likely to rupture, infected cysts, those exerting mass effect and cysts located close to vital structures. Surgical techniques include resection, enucleation (removal of the cyst with intact germinal epithelium but leaving the peri-cyst), pericystectomy, cystostomy (aspiration of the cyst fluid and removal of the germinal epithelium) or capitonnage (closure of cyst cavity by folding and suturing the peri-cyst walls). The risk of intraoperative spillage of cyst contents can be reduced by using 20% hypertonic saline or povidone iodine solution. When cysts occupy more than 50% of a lung lobe, pulmonary lobectomy may be required. It may also be considered for cysts associated with excessive suppuration, hemorrhage, fibrosis or bronchiectasis. Medical therapy with albendazole or mebendazole is indicated for small cysts (especially if they are multiple), recurrent cysts despite therapy, multiorgan disease or contraindications to surgery. The optimal duration of medical therapy is not clearly defined. Uneventful, unexpected FOB-guided complete extraction of lung hydatid has also been described.14 This case highlights an unusual cause of hemoptysis in a child, where the diagnosis could be clinched on the basis of prior experience and expertise of the treating physicians.