Pulmonary Sequestration and Intrathoracic Kidney: CT Diagnosis of a Rare Congenital Malformation with Bochdalek Hernia

 



When a Trauma CT Reveals an Entirely Different Story

A 22-year-old woman was evaluated after falling from a scooter. Because the clinical question was trauma, the initial purpose of contrast-enhanced CT was straightforward: identify fractures, hemorrhage, solid-organ injury, vascular injury, or other acute traumatic abnormalities.

No significant traumatic injury was identified.

Yet the CT examination revealed something much more unusual.

Within the left lower lobe was an abnormal segment of lung supplied by three systemic feeding arteries arising from the thoracic aorta. The venous drainage was unusual, involving the hemiazygos system. At the same time, the left kidney was not in its expected retroperitoneal position. Instead, it was located within the thoracic cavity through a left posterolateral diaphragmatic defect, accompanied by part of the left adrenal gland, the gastric fundus, and the spleen.

The final imaging constellation was:

Intralobar pulmonary sequestration + intrathoracic kidney + left Bochdalek hernia + complex systemic and renal vascular anatomy.

The case is valuable because none of these abnormalities should be interpreted in isolation.

The diagnostic answer emerges only when the radiologist connects four anatomical questions:

Where is the abnormal tissue?
Where does its blood supply come from?
Where are the organs located?
What happened to the diaphragm?

That approach transforms a routine trauma CT into a detailed map of congenital anatomy.



1. The Case at a Glance

The patient was a 22-year-old woman who underwent chest, abdominal, and pelvic CT after a scooter accident. The examination did not demonstrate significant traumatic injury, but it revealed several congenital abnormalities.

The major CT findings were:

  • Left lower-lobe intralobar pulmonary sequestration
  • Three systemic feeding arteries arising from the thoracic aorta
  • Abnormal venous drainage toward the hemiazygos system
  • Left posterolateral Bochdalek hernia
  • Left intrathoracic kidney
  • Two accessory renal arteries
  • Partial herniation of the left adrenal gland
  • Herniation of the gastric fundus
  • Herniation of the spleen
  • No significant acute traumatic injury

The combination is exceptionally instructive because pulmonary sequestration and intrathoracic kidney are individually uncommon, while their coexistence with a Bochdalek hernia creates a much more complex developmental anatomy.

The central lesson is simple:

Do not stop reading a trauma CT when the trauma is negative.

A normal trauma study can still contain clinically important congenital, vascular, oncologic, or incidental findings.


2. Figure-by-Figure CT Interpretation

Figure 1. Frontal Scout View

Figure 1. Frontal scout view demonstrating an abnormal left diaphragmatic configuration and providing the initial anatomical clue to the congenital thoracoabdominal abnormality.

The scout image does not establish the diagnosis of pulmonary sequestration or intrathoracic kidney by itself. Its value is more subtle.

It provides the first anatomical overview.

The radiologist should examine:

  • The position and contour of the left hemidiaphragm
  • The distribution of abdominal gas
  • The lower thoracic soft-tissue contours
  • The cardiomediastinal silhouette
  • Any apparent displacement of abdominal organs

In this patient, the scout image serves as an anatomical warning sign. A structure that normally belongs below the diaphragm may appear unexpectedly high, prompting careful evaluation of the subsequent cross-sectional images.

The important principle is that the scout image should not be regarded merely as a technical positioning image. It can provide the first clue that the anatomy is not normal.


Figure 2. Axial Contrast-Enhanced Arterial-Phase CT

Figure 2. Axial arterial-phase CT demonstrating three systemic feeding arteries arising from the thoracic aorta and entering the abnormal left lower-lobe pulmonary tissue, a key diagnostic feature of pulmonary sequestration.

This is arguably the most diagnostically important image in the case.

The defining anatomical abnormality in pulmonary sequestration is not simply an abnormal mass-like pulmonary opacity.

It is the combination of:

abnormal pulmonary tissue + absent normal bronchial communication + systemic arterial supply.

The most important question is therefore:

Is there a systemic artery entering the abnormal basal pulmonary tissue?

In this patient, three feeding arteries arise from the thoracic aorta.

That finding dramatically changes the differential diagnosis.

A lower-lobe opacity may initially suggest:

  • Pneumonia
  • Pulmonary abscess
  • Atelectasis
  • Bronchiectasis
  • Congenital pulmonary airway malformation
  • Lung neoplasm

But once a systemic feeding artery is demonstrated, pulmonary sequestration becomes a leading diagnosis.

Modern multidetector CT angiography is particularly valuable because it simultaneously demonstrates the lung abnormality and the vascular anatomy required for treatment planning.

The presence of three arteries is particularly important. A common interpretive error is to identify one anomalous artery and stop searching.

This case demonstrates why that approach is unsafe.

The radiologist should actively search for:

  1. Number of feeding arteries
  2. Arterial origins
  3. Vessel diameter
  4. Course
  5. Entry point into the sequestered tissue
  6. Relationship to the aorta and diaphragm
  7. Venous drainage
  8. Pulmonary arterial connections

The CT therefore functions not merely as a diagnostic examination but as a preoperative vascular roadmap.


Figure 3. Axial Contrast-Enhanced Arterial-Phase CT

Figure 3. Axial arterial-phase CT demonstrating two accessory renal arteries arising superior to the main left renal artery and supplying the ectopic intrathoracic left kidney.

The next diagnostic challenge is the kidney.

The left kidney is not in its expected retroperitoneal location. However, identifying an ectopic kidney is only the beginning.

The vascular anatomy is equally important.

Two accessory renal arteries are demonstrated in addition to the principal renal artery.

This matters because an ectopic kidney may have a variable vascular supply. Unlike the typical kidney, whose renal artery commonly originates from the abdominal aorta near the level of the renal hilum, an ectopic kidney may receive arteries at unusual levels.

Consequently, before any surgical procedure, the following must be documented:

  • Main renal artery
  • Accessory renal arteries
  • Arterial origins
  • Renal veins
  • Ureteral course
  • Renal parenchymal enhancement
  • Collecting-system anatomy

Missing an accessory renal artery can have direct surgical consequences.

This is why CT angiography of an intrathoracic kidney is not merely an anatomical curiosity. It is a surgical safety study.


Figure 4. Coronal Contrast-Enhanced Arterial-Phase CT

Figure 4. Coronal arterial-phase CT demonstrating the left intrathoracic kidney, its venous drainage, and the abnormal relationship between the kidney and the diaphragm.

Coronal reconstruction is particularly powerful in this case.

Axial images show the anatomy slice by slice. Coronal images provide a much clearer understanding of the superior-inferior relationship between:

  • Kidney
  • Diaphragm
  • Thoracic cavity
  • Abdominal cavity
  • Herniated organs

The coronal plane also allows the radiologist to follow the renal vessels more naturally along their longitudinal course.

For complex congenital anatomy, multiplanar interpretation should therefore include:

Axial + Coronal + Sagittal + MPR/3D reconstruction.

Three-dimensional volume rendering can be particularly useful when multiple anomalous arteries are present, and surgery or vascular intervention is being considered.


Figure 5. Coronal Contrast-Enhanced Portal-Venous Phase CT

Figure 5. Coronal portal-venous-phase CT demonstrating the left kidney within the thoracic cavity through a left Bochdalek hernia, with associated herniation of abdominal structures.

The key question in this image is:

Why is the kidney in the chest?

The answer is not simply “renal ectopia.”

The kidney has passed through a congenital posterolateral diaphragmatic defect.

The associated contents include:

  • Left kidney
  • Part of the left adrenal gland
  • Gastric fundus
  • Spleen

This establishes the anatomical relationship between intrathoracic kidney and Bochdalek hernia.

When evaluating an ectopic kidney in the chest, the radiologist should systematically document:

  1. Kidney location
  2. Kidney size
  3. Parenchymal enhancement
  4. Collecting system
  5. Renal arteries
  6. Renal veins
  7. Ureter
  8. Diaphragmatic defect
  9. Other herniated organs

The distinction between congenital and traumatic displacement is particularly important in this case because the patient presented after a fall.


Figure 6. Sagittal Contrast-Enhanced Portal-Venous Phase CT

Figure 6. Sagittal portal-venous-phase CT demonstrating the left intrathoracic kidney and its direct anatomical relationship with the posterolateral diaphragmatic defect.

Sagittal reconstruction provides the final spatial confirmation.

It demonstrates that this is not simply a “high-riding kidney.” The kidney is genuinely located within the thoracic cavity.

This distinction is clinically important.

A high-positioned abdominal kidney, diaphragmatic eventration, congenital thoracic kidney, Bochdalek hernia, and traumatic diaphragmatic rupture can produce overlapping appearances.

The sagittal image helps establish the actual anatomical pathway.


3. What Is Pulmonary Sequestration?

Pulmonary sequestration, also called bronchopulmonary sequestration, is a congenital malformation consisting of abnormal pulmonary tissue that lacks normal communication with the tracheobronchial tree and receives systemic arterial blood supply.

It is best understood as a developmental abnormality of the lung and its vascular connections.

Two major forms are recognized:

Intralobar Pulmonary Sequestration

In intralobar sequestration (ILS), the abnormal lung tissue is incorporated within an existing pulmonary lobe and shares the visceral pleural covering.

This is the type identified in this patient.

Extralobar Pulmonary Sequestration

In extralobar sequestration (ELS), the abnormal tissue has its own visceral pleural investment and is anatomically separate from the adjacent normal lung.

The venous drainage pattern can help distinguish the two. Intralobar sequestration generally drains through pulmonary veins, whereas extralobar sequestration more commonly drains into systemic veins.

The present case is unusual because the venous drainage is described toward the hemiazygos system, emphasizing why actual vascular anatomy should take priority over assumptions based on textbook patterns.


4. Pathophysiology of Pulmonary Sequestration

The exact embryological mechanism remains incompletely established.

One widely accepted concept is that an abnormal accessory lung bud develops during embryogenesis. This tissue becomes separated from the normal bronchial tree and develops an independent vascular supply.

The most clinically important abnormality is systemic arterialization.

Normal pulmonary circulation is a low-pressure vascular system. By contrast, systemic arteries operate at substantially higher pressure.

When systemic arterial blood enters abnormal pulmonary tissue, several consequences may occur:

  • Recurrent infection
  • Chronic inflammation
  • Hemoptysis
  • Pulmonary vascular remodeling
  • Left-to-left shunting
  • Increased cardiac workload
  • Rare pulmonary hypertension or heart failure

The high-pressure systemic arterial supply is therefore not merely an anatomical curiosity.

It explains why pulmonary sequestration can become clinically significant even when the patient has relatively mild respiratory symptoms.


5. Epidemiology

Pulmonary sequestration is uncommon and represents a small proportion of congenital pulmonary malformations.

It is most frequently identified in the lower lobes, with a recognized predominance of left-sided lesions.

The literature indicates that systemic arterial supply is the defining imaging feature, while the number and origin of feeding arteries can vary considerably. In a major imaging review, most aberrant arteries arose from the descending thoracic aorta, although other origins have also been described, and multiple supplying arteries occurred in a minority of cases.

The present patient fits the classic lower-lobe distribution but demonstrates an unusual vascular complexity with three feeding arteries.

Pulmonary sequestration may be diagnosed:

  • During childhood
  • During investigation of recurrent pneumonia
  • During evaluation of hemoptysis
  • During prenatal imaging
  • During CT performed for unrelated disease
  • During trauma imaging

The increasing use of multidetector CT has made incidental adult diagnosis increasingly possible.


6. Clinical Presentation

The clinical spectrum is broad.

Some patients remain completely asymptomatic.

Others present with:

Recurrent pneumonia

Repeated infection in the same lower-lobe region should raise suspicion for an underlying congenital pulmonary abnormality.

Chronic cough

Persistent cough may result from recurrent infection or chronic inflammatory changes.

Hemoptysis

Hemoptysis is particularly important because abnormal systemic arterial pressure can produce significant bleeding.

Dyspnea

Large lesions, infection, vascular shunting, or associated congenital anomalies may contribute to respiratory symptoms.

Incidental detection

The present case belongs to the most educational category: incidental diagnosis during trauma CT.

The patient did not undergo CT because of pulmonary symptoms.

The abnormality was discovered because the entire thoracoabdominal anatomy was reviewed.


7. Imaging Features of Pulmonary Sequestration

A practical CT approach is to evaluate four major domains.

7.1 Location

Look for an abnormality, particularly in the lower lobe.

7.2 Parenchymal appearance

The lesion may appear as:

  • Consolidation
  • Solid mass-like tissue
  • Cystic abnormality
  • Mixed solid-cystic tissue
  • Abnormal hyperlucent or emphysematous region

7.3 Systemic arterial supply

This is the most important diagnostic feature.

The radiologist should trace the artery back to its origin.

7.4 Venous drainage

Determine whether drainage is through:

  • Pulmonary veins
  • Azygos system
  • Hemiazygos system
  • Other systemic veins

CT angiography is particularly effective because it can demonstrate both the parenchymal lesion and its arterial and venous anatomy in one examination.


8. A Critical Modern Differential: Anomalous Systemic Arterial Supply

A particularly important modern distinction is between true intralobar pulmonary sequestration and anomalous systemic arterial supply to the basal segment of otherwise normally connected lung.

These entities can look similar because both may demonstrate systemic arterialization.

However, the bronchial and pulmonary arterial anatomy differs.

Recent work has emphasized this distinction, and a 2026 comparative study specifically evaluated intralobar pulmonary sequestration versus anomalous systemic arterial supply using clinical characteristics and three-dimensional imaging.

Therefore, a systemic feeding artery should not automatically be equated with pulmonary sequestration.

The radiologist should ask:

Is the bronchial anatomy abnormal, or is this normal lung receiving an anomalous systemic artery?

This distinction may influence surgical strategy.


9. Intrathoracic Kidney

An intrathoracic kidney is an exceptionally rare form of renal ectopia in which the kidney is located above the diaphragm.

It can occur as an isolated congenital anomaly or in association with congenital diaphragmatic defects.

The present case is particularly important because the ectopic kidney is associated with a left Bochdalek hernia.

The kidney is therefore not simply “high.”

It has a defined anatomical pathway through a diaphragmatic defect.


10. Pathophysiology of Intrathoracic Kidney

During embryogenesis, the kidneys initially develop in the pelvic region and subsequently ascend toward their final retroperitoneal position.

Abnormal renal ascent, abnormal diaphragmatic development, or congenital diaphragmatic herniation can result in thoracic renal ectopia.

The differential mechanism becomes particularly important after trauma.

An intrathoracic kidney can theoretically result from:

  • Congenital thoracic renal ectopia
  • Congenital diaphragmatic hernia
  • Diaphragmatic eventration
  • Traumatic diaphragmatic rupture
  • Acquired displacement

A published review of traumatic intrathoracic kidney specifically emphasizes the importance of distinguishing congenital ectopia, diaphragmatic herniation, eventration, and traumatic rupture.

This distinction is essential in trauma radiology.

A kidney found in the chest after a fall does not automatically mean that trauma caused the kidney to move.

The radiologist must examine the morphology of the diaphragm, chronicity indicators, associated congenital anatomy, and vascular configuration.


11. Epidemiology and Clinical Presentation of Intrathoracic Kidney

Intrathoracic kidney is extremely rare.

Published case series have documented predominantly left-sided examples as well as bilateral cases and associations with other congenital abnormalities. Most patients are asymptomatic or have symptoms unrelated to the ectopic kidney.

Possible clinical problems include:

  • Hydronephrosis
  • Urinary obstruction
  • Recurrent urinary infection
  • Hematuria
  • Renal dysfunction
  • Associated diaphragmatic abnormalities

However, the presence of a thoracic kidney does not automatically mean that treatment is required.

A recent review of functional congenital intrathoracic kidney emphasizes that preserved renal function is possible and that management should be individualized according to renal function, symptoms, and associated abnormalities.


12. Bochdalek Hernia

A Bochdalek hernia is a congenital posterolateral diaphragmatic defect through which abdominal structures can enter the thoracic cavity.

Although classically recognized in neonates, adult Bochdalek hernia can remain clinically silent and be discovered incidentally.

A systematic review of adult cases found that left-sided defects predominated and that gastrointestinal and pulmonary symptoms were common among symptomatic patients.

In this patient, the defect contains:

  • Left kidney
  • Part of the left adrenal gland
  • Gastric fundus
  • Spleen

This pattern is highly informative because the presence of multiple abdominal organs above the diaphragm strongly supports a congenital diaphragmatic defect rather than a simple positional variant.


13. Differential Diagnosis

Pulmonary Differential

Pneumonia

A persistent lower-lobe opacity may mimic pulmonary sequestration.

Lung abscess

Cavitation can create a misleading appearance.

Bronchiectasis

Recurrent infections may produce chronic lower-lobe abnormalities.

Lung tumor

A focal mass in an adult should raise oncologic considerations.

However, a systemic feeding artery from the aorta dramatically shifts the differential toward a congenital vascular-pulmonary anomaly.

Congenital pulmonary airway malformation

CPAM may overlap radiologically with pulmonary sequestration, and hybrid lesions can occur. Modern congenital lung-malformation reviews emphasize that multiple congenital abnormalities may coexist and that CT findings can overlap.


Diaphragmatic Differential

Diaphragmatic eventration

The diaphragm may appear elevated but remains intact.

Traumatic diaphragmatic rupture

This is particularly important after blunt trauma.

Bochdalek hernia

A true posterolateral defect with herniation of abdominal contents supports congenital Bochdalek hernia.

Posterior mediastinal mass

An intrathoracic kidney can mimic a posterior mediastinal mass if its vascular and collecting-system anatomy is not recognized.


14. Diagnosis: A Structured CT Strategy

A practical diagnostic sequence for this case is:

Step 1: Identify the abnormal lung tissue

Locate the lesion and characterize its morphology.

Step 2: Search for systemic arterial supply

Trace every abnormal vessel back to its origin.

Step 3: Count the feeding arteries

Never assume there is only one.

Step 4: Evaluate venous drainage

Determine whether the drainage is pulmonary or systemic.

Step 5: Search for associated congenital abnormalities

Examine the diaphragm, mediastinum, abdomen, and genitourinary tract.

Step 6: Locate both kidneys

If one kidney is absent from its expected location, actively search the thorax.

Step 7: Map renal vasculature

Identify main and accessory renal arteries and renal veins.

Step 8: Evaluate the diaphragm

Determine whether there is a congenital defect, eventration, or traumatic rupture.

Step 9: Perform multiplanar and 3D reconstruction

Use axial, coronal, sagittal, MPR, and—when appropriate—volume-rendered images.

This structured strategy follows the anatomical logic demonstrated by the present case.


15. Treatment

Pulmonary Sequestration

Management depends on symptoms, lesion anatomy, systemic arterial supply, venous drainage, associated abnormalities, and procedural risk.

Potential approaches include:

  • Surgical resection
  • Lobectomy
  • Sequesterectomy in selected lesions
  • Endovascular embolization
  • Selected surveillance strategies in carefully chosen asymptomatic patients

Surgical resection remains a well-established approach for symptomatic pulmonary sequestration, while endovascular techniques have also been used in selected situations.

The three feeding arteries in this patient are particularly important for operative planning.

If one vessel is missed, residual systemic perfusion may remain.


Intrathoracic Kidney

A functionally normal asymptomatic congenital intrathoracic kidney does not necessarily require repositioning.

Treatment becomes more relevant when there is:

  • Renal dysfunction
  • Urinary obstruction
  • Hydronephrosis
  • Recurrent infection
  • Symptomatic hernia
  • Organ compression
  • Other clinically significant complications

Published cases of Bochdalek hernia with functionally normal ectopic kidneys demonstrate that the kidney can sometimes be safely left in its thoracic position while repairing the diaphragmatic defect.

The decision should therefore be individualized rather than based solely on the kidney's unusual location.


16. Prognosis

The prognosis of pulmonary sequestration is generally favorable when appropriately recognized and managed.

Potential complications of untreated symptomatic disease include:

  • Recurrent infection
  • Hemoptysis
  • Chronic inflammation
  • Cardiopulmonary effects from systemic shunting
  • Rare severe vascular complications

For intrathoracic kidney, preserved renal function is compatible with an excellent long-term outcome in many patients.

The long-term prognosis depends more on:

  • Renal function
  • Urinary drainage
  • Infection
  • Vascular anatomy
  • Associated congenital anomalies

than on the thoracic position alone.

Adult Bochdalek hernia has a more variable prognosis because acute complications such as obstruction, strangulation, or respiratory compromise can occur. In the adult systematic review, surgical management was common, particularly when symptoms or complications were present.


17. How Artificial Intelligence Could Change Diagnosis

Artificial intelligence is becoming increasingly important in CT interpretation, but an important distinction must be made.

There is currently no sufficiently validated, disease-specific AI system that should independently diagnose pulmonary sequestration, intrathoracic kidney, and Bochdalek hernia as a complete syndrome.

Therefore, it would be inappropriate to claim that a commercial AI algorithm can currently replace expert interpretation of this rare combination.

However, modern medical AI can support the diagnostic workflow in several highly relevant ways.

17.1 Automated Thoracic Segmentation

Deep-learning algorithms can segment:

  • Lungs
  • Aorta
  • Pulmonary arteries
  • Pulmonary veins
  • Kidneys
  • Diaphragm
  • Abdominal organs

This creates an anatomical framework within which unusual relationships can be detected.

17.2 Vessel Tracking

One of the most promising applications is automated vascular-tree analysis.

An AI system could trace an artery from the thoracic aorta and identify whether it enters an abnormal basal pulmonary segment.

For this case, such a system could potentially generate:

Aorta → Feeding artery 1 → Sequestered lung

Aorta → Feeding artery 2 → Sequestered lung

Aorta → Feeding artery 3 → Sequestered lung

The key benefit would be reducing the risk of overlooking a second or third feeding vessel.



17.3 Automated Kidney Localization

An AI system can potentially compare expected and observed organ locations.

If the left renal fossa is empty while a kidney-like structure is detected above the diaphragm, an automated alert could be generated:

“Left kidney not identified in expected location—evaluate thorax for ectopic kidney.”

This is conceptually different from a simple disease classifier. It is an anatomical anomaly detection system.

17.4 Multiplanar 3D Reconstruction

AI-assisted segmentation could automatically generate a 3D vascular roadmap showing:

  • Feeding arteries
  • Renal arteries
  • Renal veins
  • Aorta
  • Diaphragmatic defect
  • Herniated organs

Such a model could become particularly useful for surgical planning.

Recent work has also highlighted the clinical value of 3D imaging in differentiating intralobar pulmonary sequestration from anomalous systemic arterial supply to the basal segment.



17.5 Explainable AI

For rare congenital disease, an AI system should not simply output:

“Pulmonary sequestration: 94%.”

A clinically useful system should show why.

For example:

Detected abnormal basal lung tissue

Detected systemic artery from descending thoracic aorta

Three arterial branches identified

Normal pulmonary arterial connection not demonstrated

Left kidney absent from renal fossa

Renal tissue detected superior to diaphragm

Posterolateral diaphragmatic defect detected

This type of explainable output would be much more clinically meaningful.


18. AI and Treatment Planning

The most realistic near-term role of AI may not be autonomous diagnosis.

It may be preoperative anatomical intelligence.

For this patient, an AI-assisted planning system could automatically produce a surgical map containing:

Pulmonary system

  • Location of sequestration
  • Volume of abnormal tissue
  • Three systemic feeding arteries
  • Vessel diameters
  • Arterial origins
  • Venous drainage

Renal system

  • Main renal artery
  • Two accessory renal arteries
  • Renal vein
  • Ureter
  • Renal parenchymal volume

Diaphragm

  • Defect size
  • Defect location
  • Relationship to aorta
  • Herniated organs

This would allow surgeons, interventional radiologists, and radiologists to work from the same three-dimensional anatomical model.

The emerging paradigm is therefore not:

AI replaces the radiologist.

It is:

AI reconstructs complex anatomy; the radiologist validates it; the multidisciplinary team uses it for clinical decision-making.


19. Imaging Checklist for Daily Practice

Domain

What to Check

Lung

Location and morphology of abnormal tissue

Systemic artery

Origin and course

Feeding arteries

Number and diameter

Venous drainage

Pulmonary or systemic

Pulmonary artery

Normal connection present or absent

Diaphragm

Defect, eventration, or rupture

Kidney

Position, size, enhancement

Renal arteries

Main and accessory arteries

Renal veins

Number and drainage

Ureter

Course and continuity

Hernia contents

Kidney, adrenal, stomach, spleen, bowel

Trauma

Fracture, hematoma, organ injury

3D reconstruction

Preoperative vascular roadmap

This structured approach is consistent with the central lesson of the case: anatomy should be interpreted as a connected system rather than as isolated abnormalities.


20. Seven Things Every Radiologist Should Remember

1. A lower-lobe mass is not always a tumor.

Look for a systemic feeding artery.

2. A systemic artery can be the diagnostic key.

Trace it to its origin.

3. Never assume there is only one feeding artery.

This patient had three.

4. If a kidney is missing from the renal fossa, search the thorax.

Do not stop at the abdomen.

5. Always inspect the renal vessels.

Ectopic kidneys frequently have variant vascular anatomy.

6. After trauma, do not automatically label an intrathoracic kidney as traumatic.

Congenital diaphragmatic hernia can explain the finding.

7. Do not interpret complex congenital anatomy on axial images alone.

Use axial, coronal, sagittal, MPR, and 3D reconstruction.


21. Why This Case Matters Beyond a Rare Diagnosis

The most important finding in this patient is not any single abnormality.

It is the relationship among the abnormalities.

The lung abnormality is connected to the systemic arterial circulation.

The kidney is connected to the diaphragm.

The diaphragm is connected to the thoracoabdominal transition.

The renal position is associated with abnormal renal vessels.

The hernia contains several abdominal organs.

In other words, the CT tells an embryological story.

This is one of the most important skills in modern radiology:

Recognize anatomical relationships rather than simply naming individual abnormalities.

A radiologist who sees only “left lower-lobe opacity” may miss pulmonary sequestration.

A radiologist who sees only “left kidney absent” may miss an intrathoracic kidney.

A radiologist who sees only “elevated left hemidiaphragm” may miss a Bochdalek hernia.

But a radiologist who connects:

abnormal lung → systemic artery → abnormal kidney position → diaphragmatic defect → herniated abdominal organs

can recognize the entire congenital syndrome.


Quiz

Question 1

A 22-year-old woman undergoes CT after a scooter accident. The CT demonstrates abnormal tissue in the left lower lobe supplied by three arteries arising from the thoracic aorta.

What is the most likely diagnosis?

A. Pneumonia
B. Pulmonary embolism
C. Pulmonary sequestration
D. Bronchiectasis
E. Lung cancer

Correct answer: C. Pulmonary sequestration

Explanation

The defining imaging clue is systemic arterial supply to abnormal pulmonary tissue. The presence of three feeding arteries arising from the thoracic aorta strongly supports pulmonary sequestration.


Question 2

CT demonstrates that the left kidney is located within the thoracic cavity through a posterolateral diaphragmatic defect. The gastric fundus and spleen are also partially herniated.

What is the most appropriate diagnosis?

A. Renal tumor
B. Renal dislocation
C. Intrathoracic kidney associated with Bochdalek hernia
D. Pyelonephritis
E. Horseshoe kidney

Correct answer: C. Intrathoracic kidney associated with Bochdalek hernia

Explanation

A posterolateral diaphragmatic defect with transdiaphragmatic herniation of abdominal organs is characteristic of Bochdalek hernia. In this case, the left kidney is one of the herniated structures.


Question 3

What is the most important preoperative CT angiography information in a patient with suspected pulmonary sequestration?

A. Gallbladder position
B. Spleen size
C. Systemic feeding arteries and venous drainage
D. Colon length
E. Ureteral diameter alone

Correct answer: C. Systemic feeding arteries and venous drainage

Explanation

The anomalous systemic arterial supply is central to diagnosis and treatment planning. Importantly, more than one feeding artery may exist, as demonstrated by the three arteries in this case.


Final Take-Home Message



Recommended Reading

[1] P. Abbey, C. J. Das, G. S. Pangtey, A. Seith, R. Dutta, and A. Kumar, “Imaging in bronchopulmonary sequestration,” Journal of Medical Imaging and Radiation Oncology, vol. 53, no. 1, pp. 22–31, 2009. DOI: 10.1111/j.1754-9485.2009.02033.x.

[2] N. Bolca, U. Topal, and S. Bayram, “Bronchopulmonary sequestration: radiologic findings,” European Journal of Radiology, vol. 52, no. 2, pp. 185–191, 2004. DOI: 10.1016/j.ejrad.2004.03.005.

[3] R. E. Felker and I. L. Tonkin, “Imaging of pulmonary sequestration,” American Journal of Roentgenology, vol. 154, no. 2, pp. 241–249, 1990. DOI: 10.2214/ajr.154.2.2105007.

[4] D. Lim and R. Kostin, “Intralobar pulmonary sequestration associated with Bochdalek hernia: first reported case in an adult male and literature review,” Journal of Surgical Case Reports, 2018, rjy211. DOI: 10.1093/jscr/rjy211.

[5] I. Katsaros et al., “Bochdalek hernias in the adult population: a systematic review of the literature,” ANZ Journal of Surgery, vol. 92, no. 9, pp. 2037–2042, 2022. DOI: 10.1111/ans.17651.

[6] M. Sarac et al., “Bochdalek hernia and intrathoracic ectopic kidney: Presentation of two case reports and review of the literature,” Nigerian Journal of Clinical Practice, vol. 21, no. 5, pp. 681–686, 2018. DOI: 10.4103/njcp.njcp_217_17.

[7] S. Sabharwal, B. Young, S. Sabharwal, et al., “A review of literature of a functional, congenital intrathoracic kidney,” Journal of Cardiothoracic Surgery, vol. 20, p. 20, 2025. DOI: 10.1186/s13019-024-03306-5.

[8] F. Halis et al., “Intrathoracic Kidney after Blunt Abdominal Trauma: A Case Report and Review of the Literature,” Case Reports in Urology, 2015, Article 682649. DOI: 10.1155/2015/682649.

[9] S. M. Donat and P. E. Donat, “Intrathoracic kidney: a case report with a review of the world literature,” Journal of Urology, vol. 140, no. 1, pp. 131–133, 1988. DOI: 10.1016/S0022-5347(17)41506-0.

[10] N. D. Hawass et al., “Intrathoracic kidneys: report of 6 cases and a review of the literature,” European Urology, vol. 14, no. 1, pp. 83–87, 1988. DOI: 10.1159/000472905.

[11] M. S. Hermelijn et al., “A clinical guideline for structured assessment of CT-imaging in congenital lung abnormalities,” 2021. The proposed structured framework emphasizes location/extent, airway, lesion, vascularization, and surrounding tissue.

[12] M. Sugino et al., “Clinical differences between intralobar pulmonary sequestration and anomalous systemic arterial supply to the basal segment of the lung,” European Respiratory Journal, vol. 66, suppl. 69, PA989, 2025. DOI: 10.1183/13993003.congress-2025.PA989.

[13] “Intralobar pulmonary sequestration and anomalous systemic arterial supply to the basal segment of the lung: A comparative study of clinical features and 3D imaging,” Respiratory Investigation, vol. 64, no. 4, 101447, 2026. DOI: 10.1016/j.resinv.2026.101447.

[14] S. M. Doi and A. Sakamoto, “Thoracic kidney,” New England Journal of Medicine, vol. 361, e35, 2009. DOI: 10.1056/NEJMicm0807840.

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