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case_images: Chest XR showing opacification of the entire left pulmonary field with a retractile effect on adjacent structures.
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case_images: SpO2 levels between 88-92%, leading to the initiation of non-invasive ventilation via BCPAP (flow 7 L/min, PEEP 7.5 cmH2O, FiO2 25-45%), which achieved SpO2 levels of 90-94%. Serial chest X-rays revealed heterogeneous opacification of the entire left pulmonary field with a retractile effect on adjacent structures (Figure 1).
case_images: TTE at admission showed severe aortic coarctation and PDA (persistent ductus arteriosus) with right to left shunt.
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case_images: A large patent ductus arteriosus was noted, measuring 4 mm in diameter at the pulmonary end and 2 mm at the aortic extremity, with bidirectional shunting (Figure 2).
case_images: Chest CT angiography. (A) LSA: Left subclavian artery, proximal stenosis; CoAo: aortic coarctation; PDA: persistent ductus arteriosus; DT: desmoid tumor; (B) desmoid tumor (DT) compressing the left pulmonary artery and invasion of the left main bronchus which cannot be visualized; (C) 3D CT reconstruction.
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case_images: Additionally, the left lung was hypoplastic, and the tumor mass invaded the left main bronchus, causing severe obstruction (Figure 3).
case_images: (A) Postoperative TTE showing the postoperative result of aortic coarctation repair and proximal left subclavian artery stenosis; (B) chest CT angiography showing the result of aortic coarctation repair; (C) postoperative 3D CT reconstruction; LSA: left subclavian artery.
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case_images: The abdominal aorta was pulsatile, with a maximum velocity of 0.6 m/s at the diaphragmatic level (and a peak velocity of 8 m/s at the celiac trunk), and persistent arterial flow was observed during diastole (Figure 4A). Another chest CT scan was obtained, which showed a residual mediastinal tumor mass encasing the left pulmonary hilum, severe stenosis/collapse of the left main bronchus, severe stenosis of the left pulmonary artery, severe stenosis of the left subclavian artery, and hypoplasia of the left lung (Figure 4B).
case_images: Microscopic examination of the lesion reveals a spindle cell tumor characterized by an interlacing fascicular arrangement of elongated myofibroblastic cells. The tumor cells exhibit elongated, vesicular nuclei with mild pleomorphism and eosinophilic cytoplasm. A prominent perivascular pattern is evident, with tumor cells concentrically arranged around thin-walled capillaries. The stroma is variably collagenous, with focal myxoid areas providing a contrasting background to the more cellular zones. Scattered mitotic figures are observed but remain within a benign range. No significant necrosis or atypical features are noted in this field, and the findings are consistent with infantile myofibromatosis.
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case_images: The anatomopathological examination raised suspicion of a desmoid tumor, with the final diagnosis being infantile myofibromatosis (Figure 5).
case_images: Chest CT comparison at 12 months vs. preoperative; (A, arrow) preoperative severe proximal left bronchus stenosis; (B, arrow) moderate left main bronchus proximal stenosis at 12 months; (C, arrow) preoperative severe proximal left pulmonary artery stenosis; (D, arrow) moderate proximal left pulmonary artery stenosis at 12 months; (E, circle) preoperative superior mediastinal tumor mass; (F, circle) no tumor mass can be seen at the level of superior mediastinum at 12 months.
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case_images: Compared with the postoperative CT scan (Figure 6), there was no residual mediastinal tumor mass encasing the left pulmonary hilum, as was the case immediately after surgery.
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