Bronchogenic Cyst in Infants-Clinical and Histopathological Features.

A male infant, one month old, weight 4000 g, breastfed only, no pathological history, was admitted to the 2nd Pediatric Clinic, Clinical Emergency County Hospital in Craiova with fever and cough. Clinical findings when he was admitted: fever 38.7°C, perioral cyanosis, spastic cough, expiratory dyspnea, intercostal retraction, polypnea, subcrepitant rales in the right lung area. The chest x-ray revealed pneumonia aspect in the right middle perihilar region. He was administered antibiotic treatment, HHC, antithermics, with a favorable evolution. The control x-ray, when the infant was in a fair general state, with no disease symptomatology, pointed out a cystic formation at the level of the right middle lobe. The pulmonary CT revealed cavitary lesion, with a diameter of about 40mm in the right lung, and with the presence of septa to the interior and air content. The infant was urgently transferred to Marie Curie Hospital in Bucharest, where the cyst formation was removed through a surgical procedure. The anatomo-pathological examination revealed a bronchogenic cyst. The evolution was favorable after the surgical procedure.

Introduction

The bronchogenic cyst belongs to congenital non-vascular lung malformations.

It has a prevalence of 1:42,000-68,000 cases.

It accounts for 13-15% of congenital cystic lung diseases and 6% of mediastinal masses in children.

Through its evolution and complications, it can create diagnostic difficulties, especially at a young age [1].

The bronchogenic cysts are round, solitary, without communicating to the tracheobronchial tree, but they can also be multiple or multilobular [2].

Most of the congenital pulmonary malformations are represented by malign tumors, relatively rare, having an incidence of about one in 15,000 newborn babies [3].

Congenital pulmonary malformations can be cystic or solid, and they include a vast array of congenital malformations [3].

Between the third and the sixth week of the embryo period, the primitive gut divides dorsally into the esophagus and ventrally into the trachea [4].

The development disorders of the ventral part of the foregut may lead to anomalies such as: bronchogenic cysts, trachea-esophageal fistulas, and pulmonary sequestrations [5,6].

Case Report

A one-month-old male infant was admitted to the 2nd Pediatric Clinic, Clinical Emergency County Hospital in Craiova with fever and cough.

Anamnesis revealed: first born child, from a monitored pregnancy, full-term delivered via cesarean section, weight at birth 3150g, length 48cm, Apgar score 8/9; the infant presented physiologic icterus in maternity, and was discharged after 5 days, weighing 2900g.

The infant was vaccinated in maternity.

He received vitamin D3 as rickets prophylaxis. He was breastfed only.

The psychomotor evolution corresponded to his age.

He had good living conditions in a house within the urban area.

The onset of the disease was seven days before the child was admitted to the hospital with fever 38.2°C and dry cough.

A physician examined him at home and the child was initially administered antithermics and a three-day ampicillin injection treatment.

After three days without fever and antibiotics, the 38°C fever reappeared, being associated with a spastic cough which were the reasons for his admission.

When the infant was admitted, he presented fever 38.4°C, weight 4000g, discretely icteric teguments, dry cough, nose obstruction, expiratory dyspnea, polypnea (44/min.), suprasternal and intercostal retraction, moan, nasal flaring, piston-like movement of the head, oxygen saturation 92%, pulmonary stetacustic subcrepitant rales in the right lung area, rhythmic heart beats, precordial systolic murmur stage I/II, heart rate 128/bpm., with meteorism, abdominal pain, nasopharyngeal secretions which could be aspired, anterior fontanelle 3/4cm normotensive, Moro reflex present, without meningeal signs.

The laboratory findings when the infant was admitted to the 2nd Pediatric Clinic, Clinical Emergency County Hospital in Craiova revealed:

Hb=8.3g%,

L=34700/mm3,

N=54.3%,

Ly=18.9%,

M=15%,

E=3.2%,

B=8.6%,

Tr=280000/mm3,

anisocytosis ++,

ESR=70/110mm,

AST=16UI/L,

ALT=24UI/L,

urea=15mg%,

creatinine=0.49mg%,

glycaemia=73mg%,

total bilirubin=2.78mg%,

indirect bilirubin=2.69mg%,

calcemia=8.7mg%,

negative urine culture,

negative hemoculture,

electrolytes in serum:

Na=125mEq/l,

K=5mEq/l.

The echocardiogram and the cardiovascular examination were normal.

The chest x-ray revealed opacity with an alveolar character in the right middle perihilar region (Figure 1).

Figure 1

Chest x-ray in the first day: opacity with an alveolar character in the right middle perihilar region

The diagnosis of acute pneumonia was confirmed by the clinical findings, leukocytosis with neutrophilia, high levels of ESR, and chest r-ray.

He was administered Cefort i.v., Gentamicin i.v., HHC, humidified oxygen through facial mask, DNF and secretion absorption, antithermics, Espumisan.

The evolution was favorable under treatment and the patient returned to a normal state of health.

Considering the results of the first chest x-ray, in the eighth day from admission, he had a control chest x-ray which revealed right hilum transparency circumscribed on the previously mentioned opaque area, net outline (Figure 2).

Figure 2

Chest x-ray in the eighth day: right hilum transparency circumscribed on the previously mentioned opaque area, net outline

The required pediatric surgery examination recommended a control chest x-ray in two days’time.

The third x-ray revealed: the circumscribed transparency image persisted, with an increased projection surface (Figure 3).

Figure 3

Chest x-ray in the tenth day: the circumscribed transparency image persisted, with an increased projection surface

There was a fair evolution of the infant’s general state.

We proceeded to new laboratory findings:

Hb=7.9g%,

L=208000/mm3,

N=36.9%,

Ly=24.9%,

M=22.2%,

E=4.2%,

B=11.8%,

Tr=320000/mm3,

anisocytosis++,

ESR=18/35mm.

When the infant had no symptomatology, we repeated the laboratory examination, which revealed a decrease of the hemoglobin levels as compared to the values he presented when admitted, a decrease of the number of leucocytes, anemia and anisocytosis, a decrease of the ESR, most probably because of the pulmonary infection, and we also repeated the chest x-ray which revealed a pulmonary cyst aspect.

After the patient was re-examined by the pediatric surgeon and the thoracic surgeon, a pulmonary CT was urgently needed which revealed a cavitary lesion with the diameter of about 40mm, in the right lung, with thin walls, and the presence of septa to the interior and air content, without pleuropericardial displacement (Figure 4).

Figure 4

Pulmonary CT: cavitary lesion with the diameter of about 40 mm, in the right lung, with thin walls, and the presence of septa to the interior and air content

The infant was urgently transferred to Marie Curie Hospital in Bucharest, within the Pediatric Surgery Clinic, where the cyst formation was removed through a surgical procedure.

The histopathological analysis reported a right lobe pulmonary cyst-specimen of violet-greyish tissue, partially well delineated, with a firm, elastic consistency; the cystic cavity by section, greyish and whitish walls; dimensions 3.5/2/1.5cm.

The results of the histopathological examination pointed out that the fragment of the pulmonary parenchyma presented a cystic formation with a fibro-conjunctive wall covered by bronchic respiratory epithelium, flattened in places, with lympho-plasmocitary inflammatory infiltrate, with numerous PMN neutrophils, fibrinoid necrotic detritus, hematic and hyperemic extravasations.

In the adjacent pulmonary tissue, there were areas of bronchiolization and atelectatic and hyperemic areas.

The histopathological examination revealed the presence of a bronchogenic cyst (Figure 5).

Figure 5

Unilocular cyst lined by respiratory type epithelium with exulcerated / folded areas, suggestive for a bronchogenic cyst, HE staining, (left 4×, right 10×)

The subsequent evolution of the infant was favorable; however, he remained to be further monitored by our clinic.

A written informed consent was obtained from the family, regarding the publication of this material.

Discussions

The bronchogenic cyst represents a congenital non-vascular bronchopulmonary anomaly which is seldom met in the pediatric medical practice [2].

Congenital pulmonary malformations can be cystic or solid, and they include a vast array of congenital malformations: cystic adenomatoid and pulmonary malformations, bronchopulmonary sequestration, bronchogenic cyst, congenital lobar emphysema, and the segmental bronchial atresia [3,7].

The bronchogenic cyst represents the consequence of a bronchial embryogenesis anomaly, which occurs from day 20 until day 40 of intrauterine life [8,9].

Embryologically, it represents an ectopic bud which remains in its early, embryonic stage of development [10].

The bronchogenic cysts are round, solitary, without communicating to the tracheobronchial tree, but they can also be multiple or multilobular [4,10].

In our case, the cyst was solitary.

There are several classifications of the bronchogenic cysts, according to location, the best-known one belonging to Maier, who classified them into five groups: paratracheal, carinal, paraesophageal hilar, and miscellaneous [11].

Macroscopically, the cyst has a grey-bluish, white, or brownish color [12].

The histopathological examination reveals a ciliated mucosa, with mucous glands, smooth muscle fibers, cartilage [13], and sometimes an arterial system. Its content is often mucoid and viscous, rich in glycoproteins. Exceptionally, the content may be gaseous or purulent, if there is an intracystic infection [14].

Diagnostic certainty of the bronchogenic cyst can only be confirmed by means of a histopathological examination, with the presence of the respiratory epithelium [4,13].

The presence of the cartilaginous structures is characteristic only to the bronchogenic cysts, as revealed by the histopathological examination in the presented case.

The bronchogenic cysts can be revealed through a fetal echography [3].

The bronchogenic cysts are asymptomatic in 5-19% of the cases [2]. Most of the cases are symptomatic in infants and toddlers [15].

There may be a very early onset, in the first days of life, with respiratory distress, struggling signs, stridor or wheezing, accompanied by cyanosis [16].

Symptoms can be intermittent, varying according to position, and being aggravated during crying or feeding episodes. Infection, hemoptysis, paralysis of recurrent nerve, heart arrhythmia due to compression are seldom met [15].

In the presented case, symptomatology occurred when the infant was one month old because of an acute pneumonia, with respiratory distress, confirmed by the chest x-ray and the biochemical investigations: leukocytosis with neutrophils, anemia, and increased levels of the acute phase reactants.

The diagnosis of the bronchogenic cysts requires a chest x-ray which may display possible modifications and can suggest the presence of the bronchogenic cyst [17].

In infants, there appears a respiratory distress, secondary to the compression on the airways [2,15].

Other manifestations which occur are, as follows: cough, stridor, wheezing, intercostal and suprasternal retraction, exacerbated by feeding and crying [4,18].

In the presented case, the infant had the symptomatology of a bacterial pneumonia with respiratory distress, confirmed by the chest x-ray and the biochemical investigations: leukocytosis with neutrophilia, anemia, and increased levels of acute phase reactants.

In order to diagnose the bronchogenic cysts, we need a chest x-ray which can show whether there are modifications, and can suggest the bronchogenic cyst: hyperinfections, pneumonitis, atelectasis, mediastinal deviation and abnormal separation between the trachea and the esophagus [19].

In the presented case, the repeated chest x-ray, after the pneumonia treatment, under a good state of health, revealed the pulmonary cyst and was essential for establishing the diagnosis. The pneumonia overlaid the cyst which was emphasized after healing the pneumonia.

There are other useful investigations such as thorax CT and thorax NMR which help having a direct visualization of the cyst, highlighting its precise stage and reports [11].

In our presented case, a thorax CT was performed.

One can also perform bronchic endoscopy or pulmonary scintigraphy [9].

The multidisciplinary corroboration among the pediatrician, the pediatric surgeon, the thoracic surgeon, the radiologist, and the pathologist led to the recovery of this infant after the surgical procedure, in due time, without further complications.

The infant underwent a respiratory infection episode, with no further complications.

In many cases, the bronchogenic cysts do not have obvious signs and symptoms, but there is always a risk for some life-threatening complication, i.e., the compression against some neighboring organs or the penetration with spontaneous pneumothorax [18].

The risk of infection, hemorrhage, or malign transformation of such a cyst is also present [19].

The common treatment suggestion for the bronchogenic cyst is represented by surgical resection, as it happened in our presented case.

The infant had a favorable evolution and continued to be monitored by our clinic.

Conclusions

Having another chest x-ray after the end of the treatment was essential to our case for establishing the diagnosis.

The surgical procedure was performed in due time, thus leading to no further complications.

Conflict of interests

None to declare.