Dr Terrentia DrSajith Kesavan (intensivist and paediatric Pulmonology)DrC Jayakumar
Four months old male child k/c/o tetralogy of fallot and persistent pneumonia preeented with high grade fever spikes, cough with increased work of breathing and grunting with few episodes of hemoptysis.
Child was detected to have Tetrology of Fallot at 5 months anti natal scan
Postnatal ECHO done showed :TOF,VSD ,single umblical artery.
USG abdomen showed prominent renal pelvis. NSG was Normal
At 4 months of age child was admitted with similar complaints. Child was diagnosed with b/l pneumonia and was started on IV antibiotics, nebulizations and other supportive care. Serum immunoglobulin profile and Basic lymphocyte subset analysis done was normal. In view of persistent cough Broncho scopy was done and that showed hemorrhagic secretions from RUL post segment and left lower lobe which was washed. Aspergillus galactomannan test were found to be positive and was started on anti-fungals. Child improved symptomatically and was hence discharged.
DIFFERENTIAL DIAGNOSIS:-
1.Bronchopulmonary sequestration
2.Congenital pulmonary airway malformations
3.Bronchogenic cyst
4. Congenital diaphragmatic hernia
On examination-
Child was sick looking and tachypneic with Intercostal and subcostal retractions
Vitals- Temp:98.4F F, PR- 168bpm, RR- 68cpm, BP- 87/60mmhg, SpO2:86% on room air, CRT <2s
PICCLE normal
Anthropometry:Weight: 4.24kg (b/w -3SD), Height: 58cm(btw -2SD and -3SD), Head Circumference: 40cm(b/w 0 and -1SD).
No Facial Dysmorphism, No Neurocutaneous markers
Systemic Examination:
RS: Lower chest retractions present, decreased air entry on right side with scattered crepitations +
CVS: S1 S2 +,pan systolic murmur of grade 4/6 present,morein lower left sternal border
GIT : Soft,non tender, No organomegaly , Bowel sounds heard
CNS: Conscious, alert,PEARL. No focal neurological deficits. Normal tone . power 4/5 in all limbs.
No meningeal or cerebellar signs.
Child was initially managed in the ward but In view of respiratory distress in form of tachypnea and increased work of breathing child was shifted to PICU. Since airway was not maintained child was immediately intubated.
Figure 1-CXR done showed right lower lobe consolidation.
Initial labs in showed elevated counts(TC-11.39K/uL,Hb-9.6g/dl,Plt-193K/uL) and elevated inflammatory markers(CRP-59.81mg/L).RVP was positive for Human metapneumovirus. Child was started on IV antibiotics, nebulzations and other supportive measures. Child had persistent ET bleeds in view of which CT Pulmonary angiogram(CTPA) was done.
Figure 2- CTPA showed Persisting right lower lobe consolidation, with interval mildly improved aeration, persisting, slightly larger, air filled cavities within, Aberrant/ anomalous systemic arterial supply to this lung arising from celiac axis . Findings were suspicious for an underlying congenital malformation .Scattered segmental bilateral consolidations and Mediastinal adenopathy was also present.
Aorta gram and coiling of the collaterals was done on 18/9/24. Blood transfusions were given as required targeting Hb levels more than 8. Antibiotics were titrated according to cultures as per the sensitivity index.
NBT-DHR and S.Immunoglobulin levels to rule out primary immunodeficiency were normal. Extended lymphocyte subset analysis was done and showed decrease in Absolute NK cell (CD56+/CD16+) count, Absolute Naive CD4-CD8+ T cell count and increase in Absolute Terminal Differentiated CD4+CD8- T cell count, Absolute CD27- Terminal Differentiated CD4-CD8+ T cell count as per the age-wise reference values. As ET bleeds persisted Rightlower Lobectomy of the sequestrated lung with collaterals from systemic circulation. Was done
Biopsy of lung tissue showed Possibility of bronchopulmonary foregut malformation with pancreatic tissue.
There was no further ET bleed after procedure. Child improved symptomatically. O2 requirement on ventilator had reduced and child was slowly extubated to HFNC. CRP and other inflammatory markers were downtrending and IV antibiotics were stopped. Child was slowly weaned off to low flow and was tolerating well. Since child improved symptomatically and maintained saturation well with no further episodes of desaturation or respiratory distress andchild was shifted out to ward.
Definition and types – Bronchopulmonary sequestration (BPS) is a rare congenital abnormality of the lower respiratory tract. It consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its arterial blood supply from the systemic circulation. The connections to the tracheobronchial tree and systemic artery distinguishes BPS from congenital pulmonary airway malformation (CPAM).
•An intralobar sequestration (ILS) is located within a normal lobe and lacks its own visceral pleura. This type often has aberrant connections to bronchi, lung parenchyma, or the gastrointestinal tract and often presents with recurrent infections.
•An extralobar sequestration (ELS) is located outside the normal lung and has its own visceral pleura. Infectious complications are rare, except in ELS with connections to the gastrointestinal tract or intrapulmonary structures, which is unusual.
●Clinical presentation – The clinical presentation of BPS is variable and depends on the type, size, and location of the lesion. Many cases are initially detected prenatal ultrasound; most of these regress during gestation, while others progress and hydrops may develop. The affected newborn is usually asymptomatic but sometimes presents with respiratory distress. Some cases (usually ILS) present with recurrent pneumonia during infancy or childhood.
●Postnatal evaluation – All cases of BPS or other congenital abnormalities of the lower airway should be further evaluated with postnatal imaging. This includes cases that regressed or appeared to resolve in utero because few lesions resolve completely and advanced imaging is more sensitive than prenatal ultrasound for detecting small lesions. (
•After birth, the first step is a plain chest radiograph. On a chest radiograph, sequestrations typically appear as a uniformly dense mass within the thoracic cavity or pulmonary parenchyma. Recurrent infection can lead to cystic areas within the mass, and there may be air-fluid levels if the lesion communicates with a bronchus.
•The second step is advanced thoracic imaging, the timing of which depends on the patient’s characteristics, as outlined in the algorithm. This is to confirm the diagnosis, including identifying the aberrant artery that distinguishes BPS, and to help with surgical planning.
●Management
•Symptomatic – Infants with BPS that is causing any respiratory symptoms are treated with surgical excision; surgery is curative and is associated with minimal morbidity. The procedure is performed urgently in newborns with significant respiratory distress. Surgical resection is typically performed electively in older children who present with infection.
•Asymptomatic, high risk – For asymptomatic patients of any age with characteristics that suggest a high risk for developing complications (large lesions occupying >20 percent of the hemithorax, bilateral or multifocal cysts, pneumothorax, or a family history of pleuropulmonaryblastoma-associated conditions ), we suggest surgical resection rather than observation .
•Asymptomatic, low risk – For asymptomatic patients without these high-risk characteristics, either elective surgical resection or conservative management with observation are reasonable options and practice varies
Take over mesage:-In the absence of other significant congenital anomalies, the prognosis for children with BPS is generally very good. For symptomatic infants who undergo emergency surgery, at least 20 percent have postoperative complications, which include air leak, infection, or effusion. For asymptomatic infants who undergo elective surgery, approximately 10 percent have postoperative complications. Long-term pulmonary outcomes for both of these groups are generally good and appear to depend upon the extent of the lung resection. The remaining lung parenchyma undergoes compensatory growth and development