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\begin{document}

\title{Congenital Lobar Emphysema: A diagnostic dilemma with co-existent
Congenital Heart Defects~~~~}



\author[1]{Dr. Vishal V. Bhende}%
\author[1]{Dr. Jigar P. Thacker}%
\author[1]{Dr.Tanishq S. Sharma}%
\affil[1]{Affiliation not available}%


\vspace{-1em}



  
  \date{May 17, 2024}


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\sloppy


Congenital Lobar Emphysema: A diagnostic dilemma with co-existent
Congenital Heart Defects

Short running title: Congenital Lobar Emphysema with Congenital Heart
Defect

\textbf{Authors:}

Jigar P. Thacker\textsuperscript{1}, Vishal V.
Bhende\textsuperscript{2}, Tanishq S. Sharma\textsuperscript{2}

\textsuperscript{1}Associate Professor, Department of Pediatrics,
Pramukhswami Medical College, Bhaikaka University, Shree Krishna
Hospital, Bhaikaka University, Gokal Nagar,Karamsad,Anand,Gujarat-388
325,India.

E mail -- thackerjigar@yahoo.co.in

\textsuperscript{2}Department of Pediatric Cardiac Surgery, Bhanubhai
and Madhuben Patel Cardiac Centre, Shree Krishna Hospital, Bhaikaka
University, Gokal Nagar,Karamsad,Anand,Gujarat-388 325,India.

E mail -- drvishalbhende@gmail.com

\textbf{Corresponding Author:} Vishal V. Bhende Department of Pediatric
Cardiac Surgery, Bhanubhai and Madhuben Patel Cardiac Centre, Shree
Krishna Hospital, Bhaikaka University, Karamsad,Anand,Gujarat-388 325,
India

E mail: drvishalbhende@gmail.com; Phone:+91 98453 83405

\textbf{Author Contributions:}

\textbf{Data Curation:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Formal Analysis:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Funding Acquisition:} Jigar P. Thacker, Vishal V. Bhende,

Tanishq S. Sharma

\textbf{Investigation:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Methodology:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Project Administration:} Jigar P. Thacker, Vishal V. Bhende

Tanishq S. Sharma

\textbf{Resources:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Software:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Supervision:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Validation:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Visualization:} Jigar P. Thacker, Vishal V. Bhende

\textbf{Writing-Original Draft Preparation:} Jigar P. Thacker, Vishal V.
Bhende

\textbf{Writing -- Review \& Editing:} Jigar P. Thacker, Vishal V.
Bhende



\textbf{Acknowledgments}

Nil

\textbf{Funding statement:}

All authors have declared that no financial support was received from
any organization for the submitted work.

\par\null

\textbf{Conflict of interest statement:}

All authors have declared that they have no financial relationships at
present or within the previous three years with any organizations that
might have an interest in the submitted work.~All authors have declared
that there are no other relationships or activities that could appear to
have influenced the submitted work.

\textbf{Transparency statement:}

The corresponding author confirms that manuscript is an honest,
accurate, and transparent account of the study being reported; that no
important aspects of the study have been omitted; and that any
discrepancies from the study as planned have been explained.



\textbf{Data availability statement :}

The data that support the findings of this study are openly available
in{[}Authorea{]} at, reference number {[}reference number{]}.

\textbf{Informed consent statement :}

Statement of patient informed consent for all the participants involved
in the study was waived off as the data collected was retrospective and
based on observational inputs. After perusal, Institutional Ethics
Committee -2 (IEC-2), approved the project vide letter
IEC/BU/2022/Cr.04/125/2023 dated 26.04.2023.



\textbf{Abstract}

Congenital Lobar Emphysema (CLE) is a rare and life-threatening
congenital lung anomaly that often poses a diagnostic dilemma. The
problem can be further augmented with co-existent Congenital Heart
Defect (CHD) with the left to right shunts. The clinical presentation of
congenital heart defects in infantile life is quite similar to the CLE,
and CHD being more common gets detected early. The presence of
underlying CLE may only be suspected after the repair of the CHD. In our
case, a 2-month-old patient presented with respiratory distress, and on
evaluation large Ventricular Septal Defect (VSD) and Patent Ductus
Arteriosus (PDA) were detected. After the successful repair of the
cardiac defects, the infant continued to have cough, respiratory
distress, and failure to thrive. Then on radiological re-analysis CLE
was suspected on X-ray, and confirmed by Computed Tomography (CT). CLE
can get detected on an X-ray chest, but it is often confusing and
misleading at an early stage.

\textbf{Key Words:}

Lobectomy, Left to Right shunt, CT scan lung, Right middle lung lobe,
Ventricular Septal Defect, Patent Ductus Arteriosus, Respiratory
distress, Failure to thrive



\textbf{Introduction}

Congenital Lobar Emphysema (CLE) is a rare and life-threatening anomaly
of the lung characterized by hyperinflation of usually one or sometimes
more of the lung lobes. It sincidence rate is around 1 in the 20,000 to
30,000 live births with 3:1 male preponderance\textsuperscript{1,2}. The
hyperinflated lobe compresses the surrounding normal lung tissue causing
atelectasis leading to ventilation-perfusion mismatch and hypoxia. It is
usually manifested in early infancy with respiratory
distress\textsuperscript{3,4}. The most common site of lobe involvement
is the left upper lobe (43\%), followed by the right middle lobe(32\%)
and right upper lobe (21\%).Congenital Heart Defects (CHD) can present
in around 14 -- 20\% of cases of CLE, and Ventricular Septal Defect
(VSD) is the most commonly associated anomaly\textsuperscript{1,4}. The
clinical presentation of CLE and most CHDs are similar and overlapping
in the infantile age, and CHD being far more common gets detected
early.Often the diagnosis of CLE may get overlooked in presence of the
CHD\textsuperscript{5,6}. We encountered a case where the presence of
CLE was established after the corrective repair of the large
perimembranous VSD with moderate Patent Ductus Arteriosus (PDA).



\textbf{Case Presentation}

A 1 month 24 days old male child presented with cough, tachypnea, and
minimal chest retraction for a short duration. The child was afebrile
with fair activity and sucking. The child's weight and height on
admission were 3.5 kgs. and 52 cms. respectively, and both of the
parameters were \textless{} 3\textsuperscript{rd} centile. His birth
weight was 2.6 kgs., suggesting poor weight gain. The child was on mixed
feed (breastfeeding + cow's milk) from 1 month of age. The X-ray chest
was reported as right upper lobe pneumonia. (Figure 1)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

\textbf{Figure 1} X-ray chest Postero-anterior (PA) view at 2 months of
age reported as right upper zone pneumonia.

The child was kept on low-flow oxygen and an empirical antibiotic was
started. The septic screen was unremarkable and the blood culture was
sterile. A faint murmur was appreciated at the left parasternal region
with loud S2.2D Echocardiography revealed large perimembranous VSD with
moderate PDA and severe Pulmonary Artery Hypertension (PAH).
Anti-failure medications (Furosemide and spironolactone) were started,
and definitive repair was planned after sufficient weight gain. Oxygen
was weaned off and child was discharged after 7 days.

On regular subsequent follow-ups, the child persisted to have cough and
tachypnea, as well growth failure was also reported. Thus, definite
cardiac surgery was performed at 3 months and 7 days of age.The baby
underwent Glutaraldehyde treated Pericardial Patch Closure of
Ventricular Septal Defect with Patent Ductus Arteriosus Ligation. The
surgical procedure was uneventful.

Even after the definitive cardiac surgery, the child continued to have
cough, tachypnea, occasional retraction, and growth failure. Repeated
evaluations ruled out the presence of infection or congestive cardiac
failure (CCF). Repeated Echocardiography affirms the success of the
cardiac repair. Then after, the child had lost follow-up for almost 3
months.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image2/image2}

\end{center}
\end{figure}

The follow-up was resumed at around 7 months and 15 days of age with the
persistence of the above complaints. He had several admissions at
another healthcare facility. He continued to grow poorly with a weight
of 5.1 kgs. and a length of 62 cms. On repeat X-ray chest evaluation,
hyperlucency of the right middle lobe and crowding of both the right
upper and lower lobe was noted. (Figure 2)

\textbf{Figure 2} X-ray chest Postero-anterior (PA) view showing clear
hyperluceny of the right middle lobe with crowding of right upper and
lower lobe with mediastinal shift. Sternal wires from the previous heart
surgery can be made out.

CT scan confirmed the presence of Congenital Lobar Emphysema involving
the right middle lobe. (Figure 3,4)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image3/image3}

\end{center}
\end{figure}

\textbf{Figure 3} Pre-operative Coronal reformatted High-resolution
Computed Tomography (HRCT) scan shows congenital lobar emphysema of
right middle lobe.

(Image Credits: Dr. Viral B. Patel)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image4/image4}

\end{center}
\end{figure}

\textbf{Figure 4} Axial view HRCT lung image conforming the presence of
congenital lobar emphysema of right middle lobe.

(Image Credits: Dr. Viral B. Patel)

The lobectomy was planned after weighing the benefit-risk of surgical
resection with a thorough literature review. Right middle lobe resection
was performed at 8 months and 9 days of age. The baby was placed in the
left lateral position, chest cavity entered through the right 4th
intercostal space. Dissection was done by Reverse Chamberlain Method
from the periphery towards the hilum all around the fissure.The right
middle lobe vein was identified and ligated proximally and branches were
individually tied. The right middle lobe artery entering the right
middle lobe was identified and branches were separated. The brisk nature
of bleeding was controlled by hemostasis using electro-cautery. A
bronchial clamp was applied after the right middle lobe bronchus was
slinged by the rubber tubes. Right middle lobe bronchus divided proximal
to the bronchial clamp. Single lung anesthesia was commenced. The
divided bronchial stump is closed with multiple interrupted vertical
mattress 5/0 polypropylene sutures. Stump integrity is checked
underwater with sustained ventilation. A minor leak was noted from the
lung parenchymal surface and no leak was noted from the bronchial stump.
The right upper lobe and right lower lobe surfaces adhered together with
two interrupted 5/0\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image5/image5}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image6/image6}

\end{center}
\end{figure}

polypropylene pledgetted sutures. The specimen was sent for
histopathological examination (Figure 5, a \& b)

\textbf{Figure 5} -- (a) Intra-operative image showing expanded right
middle lobe, (b) Excised right middle lobe of lung

which was consistent with the radiological diagnosis.The clinical course
was stable after the surgery. The child recovered fast during the
postoperative period. The child remained asymptomatic and the tachypnea
and retraction disappeared. X-ray \& High-resolution CT lungs showed
good expansion of the right sided lung with both the upper and lower
lobe expanded well and filling the gap of middle lobe resection at 5
years of age. (Figure 6,7)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image7/image7}

\end{center}
\end{figure}

\textbf{Figure 6} Post-operative X-ray chest showing good expansion of
right sided lung with filling of the gap of the right middle lobe
resection with right upper and lower lobes in the immediate
post-operative period.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image8/image8}

\end{center}
\end{figure}

\textbf{Figure 7} Post-operative X-ray chest showing good expansion of
right sided lung with filling of the gap of the right middle lobe
resection with right upper and lower lobes demonstrated @ 5 years of age
on follow-up.

Weight gain was observed during the subsequent follow-up. HRCT done on
follow-up at 5 years of age showed normally aerated lungs (Figure 8,9)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image9/image9}

\end{center}
\end{figure}

\textbf{Figure 8 Post-operative Coronal CT Image show normal lung
parenchyma}

(Image Credits: Dr. Viral B. Patel)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image10/image10}

\end{center}
\end{figure}

\textbf{Figure 9 Post-operative HRCT Axial Image showing normally
aerated lungs}

(Image Credits: Dr. Viral B. Patel)



\textbf{Discussion}

The association of congenital heart defects and congenital lobar
emphysema is well known, and the large left to right shunts are commonly
associated with CHD. Though, Tetralogy of Fallot (TOF) and other right
to left shunts are seen sometimes\textsuperscript{6}. Cough, tachypnea,
and respiratory distress in early infancy are the usual clinical
presentation in both conditions; CLE and left to right shunts. It is
quite possible in clinical practice, that CHD being much more commoner
is suspected and diagnosed first. Then, the entire clinical
constellation is attributed to the CHD and the presence of CLE is only
detected after correction of the CHD. Similar cases have been reported
in the past\textsuperscript{6,7}.

The etiology of CLE remains unknown in almost half of the cases. Absent
or dysplastic bronchial cartilage is evident in one-fourth of the cases.
Internal obstruction or external compression of the lobar bronchus due
to various causes is postulated in the remaining cases. These defects
cause ball valve effect and air trapping during the expiration with
progressive hyperinflation of the affected lobe. The hyperinflated lobe
may compress the surrounding normal lobes. In extreme cases, the
hyperinflated lobe can herniate to the surrounding lobes as well as to
the opposite thoracic cavity with tracheal and mediastinal shifts. This
reduces the respiratory reserve and causes ventilation/perfusion
mismatch with resultant hypoxia\textsuperscript{1,3}.

In the embryo, the development of the bronchial cartilage occurs during
4 to 6\textsuperscript{th} intrauterine weeks. This time coincides with
an important developmental stage of cardiac chamber septation. Thus, the
defect during this stage can affect both the heart and
lungs\textsuperscript{1}. Many a time, CLE is secondary to bronchial
compression due to aberrant or dilated pulmonary vessels owing to
pulmonary hypertension in presence of a large left to right shunt. In
this case, CLE may get self-resolved on correction of underlying
CHD\textsuperscript{5,6}.

CLE continues to pose a diagnostic dilemma. The first diagnostic
modality is the X-ray chest. The affected lung lobe is hyperlucent with
atelectasis of the adjacent lung lobes due to compression. As well, a
mediastinal shift can be seen. Though, all the features are not evident
on X-ray at an early age. X-ray findings are often misreported as
pneumonia owing to the crowding of the surrounding lobes, and the
affected lobe hyperlucency may get reported as a pneumothorax. Computed
Tomography (CT scan) of the lung is confirmatory and delineates
anatomical details. Bronchoscopy may aid the diagnosis when the internal
bronchial obstruction is suspected\textsuperscript{1-4}.

Treatment of CLE is the surgical resection of the affected lobe.
Conservative management is proposed for older children having no or
minimal symptoms.But the infants with persistent distress are the ideal
candidates for the surgery. Surgery is by far well tolerated with low
mortality and favorable outcomes\textsuperscript{2,8}. In the case of
concomitant CLE and CHD, there are diverging views in the management as
to what to repair first, or a combined lung and cardiac repair would be
the ideal approach. An individualized approach has been suggested. Many
a time, CLE gets corrected with the relaxation of the vascular
compression on the repair of the large left to right shunt and reduction
of pulmonary hypertension\textsuperscript{5,9}.

In our case, the child presented with respiratory distress. During the
stay, clinical suspicion leads us to perform 2D Echocardiography which
revealed a large VSD with moderate PDA with PAH. The presence of a
congenital defect of the lung was overshadowed by the presence of a
large heart defect. The symptoms were obviously contributed to the heart
defect.The X-ray finding was interpreted as pneumonia initially. We may
see in Figure 1 (two months of age) that there is no obvious
hyperlucency of the right middle lobe, rather haziness is evident in the
right upper lobe. In contrast, later X-ray\textbf{,} Figure 2 (seven
months of age) clearly demonstrated a large hyperlucent area in the
right middle lobe with surrounding lobe crowding and mediastinal
shift.In retrospect, we could make out the faint hyperlucency in Figure
1, which was initially missed out.We believe that the X-ray chest being
a basic investigation is often given less attention, particularly when
the other advent diagnostic modality has suggested alternative
diagnosis.

Congenital lobar emphysema is a diagnostic challenge, and the confusion
can be further augmented in the presence of an obvious large congenital
heart defect. A close look over the chest X-ray may give the lead, but
the CT scan is a confirmatory investigation. Regular follow-up and a
high index of suspicion are necessary for early diagnosis. Lobectomy can
lead to dramatic improvement and is well tolerated.



\textbf{Conclusion}

Congenital lobar emphysema is a diagnostic challenge, and the confusion
can be further augmented in the presence of an obvious large congenital
heart defect. A close look over the chest X-ray may give the lead, but
the CT scan is a confirmatory investigation. Regular follow-up and a
high index of suspicion are necessary for early diagnosis. Lobectomy can
lead to dramatic improvement and is well tolerated.



\textbf{Key Clinical Message}

A clinician should think beyond pneumonia and left-to-right shunts when
young children persisted to have respiratory distress after their
correction, and congenital lung anomalies including congenital lobar
emphysema can be the possibility. X-ray chest at an early age though not
confirmatory but can give a clue and should be assessed carefully, and
the CT scan can confirm the diagnosis.

I am the submitting author and corresponding author. I have provided
ORCID IDs of all the co-authors including me.

\begin{itemize}
\tightlist
\item
  \textbf{Jigar P. Thacker https://orcid.org/0000-0001-5213-704X}
\item
  \textbf{Vishal V. Bhende https://orcid.org/0000-0003-3014-4315}
\item
  \textbf{Tanishq S. Sharma https://orcid.org/0000-0002-7135-8160}
\end{itemize}



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