Congenital Lung Malformations in Neonates: A 9‐Year Tunisian Cohort Highlighting Diagnostic Challenges and Outcomes in a Resource‐Limited Setting

ABSTRACT

Background

Congenital lung malformations (CLMs) represent a broad spectrum of developmental anomalies with highly variable prenatal and postnatal presentations and outcomes. Their postnatal management remains non‐standardized. This study aimed to describe the spectrum of bronchopulmonary malformations diagnosed in the neonatal period, assess the contribution of antenatal and postnatal imaging, and characterize their clinical course and management in a resource‐limited setting.

Methods

We conducted a retrospective descriptive study in the Intensive Care Unit of the Maternity and Neonatology Centre of Tunis, over a 9‐year period. All newborns diagnosed with CLMs were included. Data were collected from obstetric, neonatal, and pediatric surgery records.

Results

Twenty‐three newborns were included (prevalence: 0.23 per 1000 live births; M/F ratio: 0.91). The mean gestational age was 37.5 ± 2.9 weeks, and mean birth weight 2901 ± 833 g. Antenatal diagnosis was achieved in 65% of cases ($n=15/23$) at a mean gestational age of 24.4 ± 4.5 weeks, following documented prenatal ultrasound screening in 74% of cases ($n=17/23$). Respiratory distress occurred in 78% of newborns. The most frequent diagnoses were congenital pulmonary airway malformation (57%), pulmonary sequestration (22%), pulmonary hypoplasia (13%), and tracheal atresia (9%). Conservative management was used in 65% of cases, while surgery was required in four cases. In‐hospital mortality was 34.8% and was significantly associated with respiratory distress (p = 0.04), delivery room resuscitation, comorbidities (p = 0.03), and associated malformations (p = 0.03).

Conclusions

While symptomatic CLMs in resource‐limited settings carry significant early mortality due to system‐level constraints, active conservative surveillance was associated with favorable outcomes in selected asymptomatic patients in this cohort when supported by structured parental education.

1. Introduction

Congenital lung malformations (CLMs) represent a heterogeneous spectrum of developmental anomalies affecting the tracheobronchial tree and pulmonary parenchyma. They classically include congenital pulmonary airway malformations (CPAM), pulmonary sequestrations (PS), congenital lobar emphysema, bronchial atresia, laryngeal or tracheal atresia or stenosis, as well as pulmonary and lobar agenesis, aplasia, or hypoplasia [1]. These lesions are rare and frequently associated with other malformations.

Advances in antenatal imaging have increased the prenatal detection of CLMs. Ultrasound remains the first‐line examination, while fetal magnetic resonance imaging (MRI) serves as a second‐line modality to clarify lesion topography, cystic versus solid components, and estimated residual lung volume [1]. Multiple studies report that fetal MRI improves diagnostic accuracy by approximately 20–30% and is particularly useful for perinatal decision‐making, including intensive monitoring and delivery planning [2, 3].

Clinical presentations range from life‐threatening lesions in the neonatal period to asymptomatic malformations detected later in infancy. Prognosis depends not only on lesion size and location but also on associated complications such as hydrops fetalis, pulmonary hypoplasia, or polyhydramnios, which may lead to preterm delivery [3].

Postnatal management is not standardized and requires a multidisciplinary approach. While surgical excision is often indicated for localized lesions, conservative follow‐up may be appropriate for small, asymptomatic malformations [4, 5].

In Tunisia, as in many resource‐limited settings, epidemiological and clinical data regarding neonatal CLMs remain scarce. The primary aim of this study was to describe the epidemiological, clinical, and radiological spectrum of CLMs in a resource‐limited setting. Secondary aims included assessing the complementary diagnostic roles of antenatal and postnatal imaging and exploring clinical factors associated with neonatal mortality.

2. Methods

2.1. Study Design and Setting

We conducted a single‐center, retrospective, descriptive study with exploratory analytical components over a 9‐year period (January 1, 2015, to December 31, 2023) in the Neonatal Intensive Care Unit (NICU) at the Maternity and Neonatology Center of Tunis (MNCT). The MNCT is the largest tertiary maternity hospital in Tunis and serves as a national referral center for high‐risk pregnancies and complex prenatal diagnoses.

2.2. Participants and Eligibility Criteria

All newborns diagnosed with CLMs within the first 28 days of life and hospitalized in our department during the study period were included. Neonates with a gestational age < 26 weeks and/or birth weight < 700 g for whom a collegial decision of non‐resuscitation was made, as well as cases with prenatal diagnosis (PND) leading to medical termination of pregnancy, were excluded.

A subset of patients in this cohort has been previously reported in a separate publication in the same journal; however, the present study includes the full neonatal cohort with additional analyses and outcomes not previously reported.

2.3. Antenatal Screening Context in Tunisia

While Tunisian national guidelines recommend three routine ultrasounds—including a second‐trimester (T2) anomaly scan—for all pregnancies, real‐world coverage remains limited. Out‐of‐pocket financial constraints, limited awareness of fetal risks, and regional disparities prevent many women from obtaining T2 scans. Particularly in rural areas, antenatal care is frequently managed solely by midwives without access to routine ultrasound, while advanced fetal MRI remains restricted to specialized tertiary referral centers.

2.4. Postnatal Diagnostic and Institutional Protocols

Neonates with suspected CLMs are systematically admitted for initial evaluation, baseline day‐1 chest radiography, and bedside ultrasound. Contrast‐enhanced chest (computed tomography) CT remains the diagnostic gold standard, but lacking an on‐site scanner, inpatient CT is selectively reserved for symptomatic neonates, ambiguous imaging findings, or surgical candidates.

To avoid prolonged hospitalization solely to await imaging availability amid severe NICU overcrowding (~ 300% capacity), stable asymptomatic infants—including those with suspected prenatal lesion regression—are discharged following initial evaluation. They are monitored via joint outpatient clinics with pediatric surgery, backed by structured parental red‐flag education, with definitive contrast‐enhanced CT scheduled at 1 month of age or beyond, depending on appointment availability and technical resource constraints.

2.5. Management Decision Strategy

Treatment strategy for CLMs (conservative management vs. surgical resection) is determined by a multidisciplinary team involving neonatologists and pediatric surgeons. Emergency or early surgical resection is indicated for neonates with life‐threatening respiratory compromise, massive compressive lesions causing severe mediastinal shift, or persistent pneumothorax/vascular stealing. Conservative, watchful waiting is adopted for hemodynamically stable, asymptomatic, or mildly symptomatic neonates. In our setting, this conservative approach is further justified by the high surgical and anesthetic risks in neonates, the lack of dedicated pediatric thoracic surgical units operating on an elective asymptomatic basis, and logistical/socioeconomic barriers to long‐term surgical scheduling.

2.6. Data Collection

Data were systematically collected from obstetric charts, NICU hospitalization records, outpatient follow‐up notes, and surgical/pathological reports from the Pediatric Surgery Departments of Bechir Hamza Children's Hospital and Habib Thameur Hospital in Tunis. Collected parameters included maternal demographics, prenatal diagnostic details (gestational age at diagnosis, imaging features), neonatal characteristics (gestational age at birth, birth weight, delivery room resuscitation, Apgar scores), post‐birth clinical course (respiratory support, complications), radiological findings, surgical details/histopathology, and post‐discharge long‐term outcomes (recurrent respiratory symptoms, imaging follow‐up).

2.7. Statistical Analysis

Statistical analyses were performed using Microsoft Excel. Continuous variables were assessed for normality and expressed as means ± standard deviations (SD) or medians with interquartile ranges (IQR), as appropriate. Categorical variables were summarized using absolute counts and percentages. Point estimates for prevalence and overall mortality are presented with 95% confidence intervals (95% CI). Bivariate comparisons between survivors and non‐survivors were conducted using the Chi‐square test or Fisher's exact test for categorical variables, and the Student's t‐test or Mann‐Whitney U test for continuous variables. Statistical significance was set at a two‐tailed p‐value < 0.05. Given the small cohort size, identified mortality‐related factors are presented as exploratory observations rather than definitive predictive models.

2.8. Ethical Considerations

This study was conducted in accordance with the principles of the Declaration of Helsinki. Ethical approval was obtained from the Ethics Committee of the MNCT (approval No. 17/2024). Parents of all participants were informed about the study objectives, procedures, potential risks, and benefits. Written informed consent was obtained for all patients included in the study.

3. Results

3.1. Prevalence and Types of CLMs

Between January 1, 2015, and December 31, 2023, 23 cases of CLMs were diagnosed among 27,019 total neonatal admissions to the NICU, corresponding to a hospital admission rate of 0.85 per 1000 admissions (95% CI: 0.54–1.28). Based on the 100,805 total live births delivered at our institution during this 9‐year period, the institutional birth prevalence was 0.23 per 1000 live births (95% CI: 0.14–0.35). The malformations included CPAM ($n=13$, 57%), PS ($n=5$, 22%), pulmonary hypoplasia ($n=3$, 13%), and tracheal atresia ($n=2$, 9%). Excluding tracheal atresia, lesions were left‐sided in 52% of cases. All CPAM cases presented radiologically as single‐lobe, unilateral lesions.

3.2. Prenatal Imaging

Antenatal ultrasound screening was documented in 74% of cases ($n=17/23$). Among these 17 examinations, observed sonographic features included multi‐cystic echogenic masses ($n=7),$localized hyperechoic lung parenchyma ($n=5$), a well‐circumscribed echogenic mass with systemic arterial supply ($n=2$), a large cystic mass causing mediastinal displacement ($n=1),\mathit{an}$d normal lung appearance ($n=2$).

Based on initial ultrasound findings, a specific diagnostic hypothesis was formulated in 14 cases—suspecting CPAM ($n=10$), bronchopulmonary sequestration ($n=2$), congenital lobar emphysema ($n=1$), and tracheal atresia ($n=1$)—while no preliminary hypothesis was established in three cases. Overall, prenatal diagnosis was successfully achieved in 65% of the total cohort ($n=15/23$) at a mean gestational age of 24.4 ± 4.5 weeks (range: 18–34 weeks). Based on the Adzick radiological criteria, the 13 CPAM cases were classified as macrocystic (n = 7; cysts $\ge 5\text{mm}$) or microcystic (n = 6; solid/hyper‐echoic appearance with microcysts $<5\text{mm}$).

Fetal MRI was performed in seven cases at a mean gestational age of 26.0 ± 2.8 weeks. MRI successfully delineated lesion topography and confirmed pulmonary hypoplasia in three cases, CPAM in two, and PS in two (located in the left lower lobe and right basal lobe, respectively). Associated antenatal complications observed across the cohort included polyhydramnios in two fetuses, hydrops fetalis in one, and secondary dextrocardia in another. Quantitative prenatal risk stratification using the CPAM volume ratio (CVR) was documented in two cases, yielding values of 0.50 and 1.76; the latter exceeded the established high‐risk threshold of 1.6 associated with fetal hydrops.

Apparent antenatal regression of CPAM lesions on serial sonographic monitoring was noted in two cases, including one with apparent complete resolution.

3.3. Neonatal Characteristics

The male‐to‐female ratio was 0.9. Mean gestational age at birth was 37.5 ± 2.9 weeks (31–41), with five preterm births. Cesarean delivery occurred in 70% of cases. Delivery was indicated due to acute fetal distress on cardiotocography in seven cases, a scarred uterus during labor in five cases, transverse fetal presentation in two cases, and cephalopelvic disproportion in two cases. Apgar score < 7 at 5 min was observed in four neonates. Delivery room resuscitation was required in six cases, including intubation in three. Mean birth weight was 2901 ± 833 g, with four small‐for‐gestational‐age neonates.

Respiratory distress was observed in 78% of neonates ($n=1$8). In most cases, this distress was driven by underlying respiratory distress syndrome, neonatal infectious alveolitis, or persistent pulmonary hypertension of the newborn (PPHN). Non‐invasive support with nasal continuous positive airway pressure was required in three cases, while invasive mechanical ventilation was necessary in eight. The mean duration of invasive ventilation was 15.7 ± 12.8 days (range 1–25), and the total duration of respiratory support, including non‐invasive ventilation, averaged 5.6 ± 7.3 days (range 1–25). The mean maximum inspired oxygen fraction was 76.3 ± 38.2% (range 21%–100%).

Complications included one case of left‐sided pneumothorax requiring chest tube insertion, and three cases of PPHN managed with nitric oxide therapy for a mean of 4.5 days; sildenafil was used in one patient with severe pulmonary hypertension.

Associated malformations were identified in three patients: a horseshoe kidney, trisomy 18, and a right middle cerebral artery arteriovenous malformation.

The overall distribution of the 23 cases, stratified by PND status, clinical presentation at birth, and final pathological subtype, is summarized in Figure 1.

Figure 1.

Flowchart of the study population. Patient distribution stratified by prenatal diagnosis status, clinical presentation at birth (symptomatic vs. asymptomatic), and definitive pathological subtype. CPAM, Congenital Pulmonary Airway Malformation; N, Number of cases. *Note: Numbers reflect counts within each clinical subgroup. [Color figure can be viewed at wileyonlinelibrary.com]

3.4. Postnatal Diagnosis and Management

In accordance with our institutional diagnostic algorithm, baseline chest X‐rays were obtained on the first day of life for all 23 neonates (100%), with observed radiological abnormalities summarized in Table 1.

Table 1.

Chest X‐ray Findings According to Type of the Congenital Lung Malformation.

Chest X‐ray findings	CPAM (n = 13)	PS (n = 5)	Tracheal atresia (n = 2)	Pulmonary hypoplasia (n = 3)	Overall (n = 23)
White lung	0 (0%)	0 (0%)	2 (100%)	1 (33.3%)	3 (13.0%)
Hyperlucency of the lung fields	0 (0%)	0 (0%)	0 (0%)	1 (33.3%)	1 (4.3%)
Basal alveolar opacity	0 (0%)	5 (100%)	0 (0%)	0 (0%)	5 (21.7%)
Alveolar syndrome of the lung fields	2 (15.4%)	0 (0%)	0 (0%)	0 (0%)	2 (8.7%)
Localized cystic images	4 (30.8%)	0 (0%)	0 (0%)	0 (0%)	4 (17.4%)
Multiple cystic chambers	8 (61.5%)	0 (0%)	0 (0%)	0 (0%)	8 (34.8%)
Normal chest X‐ray	1 (7.7%)	0 (0%)	0 (0%)	0 (0%)	1 (4.3%)
Pneumothorax	0 (0%)	0 (0%)	0 (0%)	1 (33.3%)	1 (4.3%)

Note: Data are expressed as absolute numbers and percentages $\mathit{n}(\%)$. Some patients had more than one radiological finding; therefore, the sum of individual findings may exceed the total number of patients.

Abbreviations: CPAM, Congenital pulmonary airway malformation; L, Left; PS, Pulmonary sequestrations; R, Right.

Bedside chest ultrasound was performed as a preliminary diagnostic tool in 57% of cases (n = 13/23) at a mean age of 10.6 ± 18.7 days, serving as an initial bedside screening examination while awaiting cross‐sectional imaging or when off‐site CT transfer was delayed.

Inpatient chest CT was performed in eight neonates (35%) at a mean age of 26.2 ± 21.9 days. The remaining 15 neonates did not undergo inpatient CT due to specific clinical and logistical factors:

• −Asymptomatic and stable neonates (n = 5): Inpatient CT was deliberately deferred to scheduled outpatient follow‐up at 1 month of age due to high NICU turnover and off‐site transfer constraints.
• −Symptomatic or unstable neonates (n = 10): Inpatient CT could not be safely performed due to severe clinical instability (respiratory, hemodynamic, or neurological compromise rendering off‐site inter‐hospital transport unsafe), early neonatal death prior to imaging, or temporary unavailability of the external CT scanner platform.

Among the eight patients who underwent inpatient CT, cross‐sectional imaging confirmed or rectified the diagnosis in all cases, including cystic lesions consistent with CPAM in four patients (Figure 2), PS in three, and right pulmonary agenesis in one. In the latter case, prenatal imaging had erroneously suggested a macrocystic CPAM, but postnatal radiography and CT definitively confirmed right pulmonary agenesis with total mediastinal displacement (Figure 3). Additionally, Type III tracheal atresia was diagnosed post‐mortem in one patient through radiopaque opacification of the tracheobronchial tree.

Figure 2.

Representative postnatal radiological evaluation of a Congenital Pulmonary Airway Malformation. (A) Anteroposterior chest radiograph demonstrating multiple air‐filled, thin‐walled cystic lesions occupying the right upper and middle lung fields (yellow arrows), causing a mild mass effect with displacement of the mediastinum to the contralateral side (asterisk). (B) Corresponding postnatal transthoracic ultrasound showing a well‐defined, multicystic pulmonary lesion characterized by multiple anechoic cystic chambers separated by thin hyperechoic septa (yellow arrowheads). [Color figure can be viewed at wileyonlinelibrary.com]

Figure 3.

Postnatal diagnostic correction of right pulmonary agenesis initially suspected prenatally as macrocystic. (A) Anteroposterior chest radiograph showing complete opacification (“white lung”) of the right hemithorax (yellow arrow), severe ipsilateral mediastinal shift to the right side (asterisk), and compensatory hyperinflation of the contralateral left lung (white arrow). (B) Coronal contrast‐enhanced chest CT scan confirming the complete absence of the right pulmonary parenchyma, right main bronchus, and right pulmonary artery (asterisk), with total mediastinal displacement into the right hemithorax and compensatory expansion of the left lung. [Color figure can be viewed at wileyonlinelibrary.com]

Surgical intervention was indicated in four cases. One infant successfully underwent left lower lobectomy for CPAM at 9 months of age, with an uncomplicated postoperative course and histopathological analysis confirming a Stocker Type II CPAM. Resection of a PS was performed in one neonate due to acute respiratory compromise. Two neonates with massive CPAM died from refractory hypoxemia preoperatively before surgical intervention could be completed. The mean duration of NICU stay for the entire cohort was 7.2 ± 10.6 days (range 1–31 days).

3.5. Mortality and Associated Factors

The in‐hospital mortality rate was 34.8% ($n=8\mathit{;}95\%\text{CI}:16.4-57.3\%$), with causes including refractory hypoxemia (5), heart failure (1), and severe bronchiolitis with secondary infection (2). Mean age at death was 18.6 ± 27.1 days. The detailed clinical presentation, associated comorbidities, and specific context of death for each fatal case are summarized in Table 2.

Table 2.

Detailed Clinical Characteristics, Comorbidities, and Context of In‐Hospital Mortalities.

Case	Type of pulmonary malformation	PND/Gestational age (weeks)	Gestational age at birth	Birth weight (grams)	Clinical presentation	Comorbidities	Age at death (days) and cause
1	CPAM	Yes (22)	30	3500	Hydrops fetalis	Prematurity	1; Heart failure
2	Right pulmonary agenesis	Yes (34)*	35	2000	NRD	Prematurity	60; Severe bronchiolitis
3	CPAM	No	38	2800	NRD	EOS, PAH	18; Refractory hypoxemia
4	CPAM	No	29	1020	NRD	Prematurity, NEC, DIC	63; Severe bronchiolitis
5	Pulmonary hypoplasia	No	37	2995	Perinatal asphyxia	Pneumothorax	3; Refractory hypoxemia
6	Tracheal atresia	No	34	1900	NRD, cyanosis	Prematurity	1; Refractory hypoxemia
7	Pulmonary hypoplasia	No	29	1250	Perinatal asphyxia	Prematurity	2; Refractory hypoxemia
8	Tracheal atresia	Yes (Trisomy 18)	35	1500	Perinatal asphyxia	—	1; Refractory hypoxemia

Abbreviations: CPAM, Congenital Pulmonary Airway Malformation; DIC, Disseminated Intravascular Coagulation; EOS, Early‐Onset Sepsis; NEC, Necrotizing Enterocolitis; NRD, Neonatal Respiratory Distress; PAH, Pulmonary Arterial Hypertension; PND, Prenatal Diagnosis; T18, Trisomy 18;

*Prenatally suspected as CPAM.

Statistical analysis identified neonatal respiratory distress ($p=0.04$), delivery room resuscitation ($p=0.01$), associated comorbidities ($p=0.03$), and additional congenital anomalies ($p=0.03$) as exploratory factors associated with early mortality. Given the small cohort size, these associations should be interpreted cautiously as exploratory observations.

3.6. Long‐Term Outcomes for Survivors

Long‐term outpatient follow‐up was maintained for the 15 surviving infants—comprising 10 CPAM (66.7%) and 5 PS cases (33.3%)—for a mean duration of 34.6 ± 19.8 months (range: 6–60 months).

Overall post‐discharge respiratory morbidity occurred in 26.7% of survivors (n = 4/15), including recurrent wheezing (13.3%, n = 2/15), community‐acquired pneumonia (6.7%, n = 1/15), and bronchial asthma (6.7%, n = 1/15).

‐CPAM subgroup outcomes (n = 10 survivors): One operated patient remained completely asymptomatic post‐lobectomy. Nine conservatively managed infants achieved excellent outcomes without secondary complications over a mean follow‐up of 4 years. Notably, in one conservatively managed infant with a normal baseline newborn X‐ray, a protocolized follow‐up CT at 6 months confirmed complete spontaneous anatomical resolution.

‐PS subgroup outcomes (n = 5 survivors): Three patients treated interventionally—comprising two surgical resections (including one previously mentioned who was operated during the acute neonatal period due to severe respiratory compromise) and one endovascular arterial embolization—recovered without sequelae. In the two conservatively managed PS patients, follow‐up CT at 2 years of age demonstrated complete spontaneous disappearance of both the sequestration parenchyma and the systemic feeding artery.

4. Discussion

Our study provides a comprehensive evaluation of the clinical spectrum, diagnostic trajectory, therapeutic management, and long‐term outcomes of CLMs in a tertiary care setting within a low‐to‐middle‐income country (LMIC). While our findings regarding epidemiological distribution and clinical presentation broadly align with international literature, they reveal critical divergences in postnatal diagnostic precision, in‐hospital mortality, and management paradigms tailored to resource‐constrained environments.

4.1. Principal Findings and Epidemiological Context

The observed incidence of CLMs in our neonatal cohort (0.23 per 1000 live births) is consistent with historical epidemiological estimates. However, contemporary multicenter registries in high‐income settings report higher detection rates (up to 1 in 7200 to 1 in 3000 live births) [6], largely driven by the widespread implementation of high‐resolution routine prenatal ultrasound screening and advanced fetal MRI. In agreement with established global registries, CPAM remains the predominant histological subtype in our series, followed PS [7, 8].

4.2. Diagnostic Discrepancies and the Pitfall of Antenatal “Regression”

A critical contribution of this study is the characterization of diagnostic discrepancies between prenatal ultrasound and postnatal cross‐sectional imaging. Although prenatal ultrasound diagnostic accuracy exceeds 85% in specialized fetal centers, our findings underscore its operator‐ and technology‐dependent limitations—most starkly illustrated by the prenatal misclassification of right pulmonary agenesis as macrocystic CPAM [9].

Furthermore, our series underscores the pitfall of apparent antenatal lesion “regression.” Among two CPAM cases with apparent prenatal sonographic resolution, postnatal CT revealed contrasting outcomes: one represented an optical artifact from microcystic tissue becoming isoechoic to expanding lung parenchyma, whereas the other represented true, CT‐confirmed spontaneous involution at 6 months.

Although spontaneous regression is reported in the literature [10, 11], confirming true anatomical resolution requires structured longitudinal imaging—specifically a protocolized CT scan at or after 6 months of age. Crucially, apparent prenatal regression does not rule out persistent tissue dysgenesis or the risk of postnatal respiratory distress [10].

4.3. Pathophysiology of Mortality and Resource Constraints

Our in‐hospital mortality rate was 34.8% (n = 8/23), standing in stark contrast to the > 90–95% survival reported in high‐resource centers [12, 13]. Early mortality was heavily weighted by complex or lethal phenotypes—accounting for five of the eight deaths (pulmonary hypoplasia, tracheal atresia, or pulmonary agenesis) and was significantly associated with respiratory distress, delivery room resuscitation, comorbidities, and structural anomalies. As a tertiary national referral maternity center, our institution naturally receives the most severe and complex prenatal cases, directly influencing cohort mortality.

Neonatal respiratory distress was predominant, driven not only by mass effect but also coexisting respiratory distress syndrome, infectious alveolitis, or PPHN. In massive macrocystic CPAMs (>70% thoracic volume), severe bilateral hypoplasia and PPHN caused fixed right‐to‐left shunting and profound ventilation‐perfusion mismatch. While pharmacotherapy (inhaled nitric oxide, sildenafil, surfactant) was utilized, medical management alone could not overcome mechanical compression. Lacking extracorporeal membrane oxygenation or bedside thoracotomy, transport of critically unstable neonates carried a prohibitive risk of cardiac arrest, resulting in two preoperative deaths. Ultimately, early mortality in resource‐limited settings reflects system‐level infrastructure constraints rather than surgical failure [14, 15].

4.4. Management Paradigms: Active Surveillance Versus Surgical Intervention

The optimal management of asymptomatic congenital lung malformations remains one of the most vigorously contested topics in pediatric surgery [16]. While many high‐resource centers advocate for routine prophylactic resection during early infancy to eliminate the long‐term risks of secondary infection and late malignant transformation, our findings support a more nuanced, risk‐stratified strategy tailored to local healthcare infrastructure [17].

In resource‐constrained environments, the risk‐benefit equation for elective intervention is fundamentally altered. The theoretical benefits of prophylactic surgery must be weighed against the tangible morbidity of major thoracic procedures. Crucially, implementing conservative surveillance in a low‐to‐middle‐income countries context introduces specific operational hazards, most notably geographic barriers, loss to follow‐up, and delayed emergency presentation. Conservative management cannot be treated as passive neglect; it requires a structured, proactive safety framework.

Broadly, the long‐term prognosis for children with CLMs is highly favorable, with most patients enjoying excellent functional outcomes, unimpaired exercise tolerance, and normal physical development due to early compensatory lung growth [18, 19]. Over a mean follow‐up of 34.6 months, our cohort exhibited an expected and manageable spectrum of mild respiratory symptoms, predominantly transient wheezing and asthma‐like episodes. However, while this duration provides reassuring data on early‐to‐mid‐term respiratory stability, it remains inherently insufficient to evaluate very long‐term complications, including late malignant transformation or adult functional reserve.

Although some mid‐term studies report subclinical spirometric abnormalities in up to half of resected patients, the clinical significance of these findings remains limited, as most affected children remain entirely asymptomatic in daily activities [20, 21].

5. Limitations

This study has several limitations inherent to its design and setting. First, its retrospective, single‐center design may limit the generalizability of findings to highly resourced tertiary fetal centers. Second, the small overall sample size ($n=23$) restricted statistical power, precluding multivariable logistic regression modeling to adjust for confounding variables when evaluating mortality associated factors. Third, standardized antenatal prognostic parameters, specifically the CVR, were documented in only two fetuses due to historical limitations in routine fetal ultrasound reporting. Finally, our mean outpatient follow‐up of 34.6 months, while sufficient to characterize mid‐term respiratory symptoms, does not allow for conclusions regarding late childhood or adult complications, such as long‐term pulmonary function impairment or late neoplastic transformation.

6. Conclusions and Implications for Practice

While CLMs carry an excellent intrinsic prognosis in high‐resource environments, severe symptomatic presentations combined with structural healthcare constraints drive substantial early mortality in LMICs. Our experience indicates that early demise in low‐resource settings stems primarily from critical care and transport limitations rather than surgical technique. When supported by protocolized CT evaluation and robust parental safety nets, active conservative surveillance was associated with favorable outcomes in selected asymptomatic neonates in this cohort, offering a viable alternative to routine early surgery in resource‐constrained settings. To build on these findings, prospective multicenter registries across diverse global settings are needed to validate context‐specific care algorithms and track long‐term cardiopulmonary trajectories into adulthood.

Author Contributions

Hajer Chourou: conceptualization, formal analysis, visualization, methodology, writing – original draft. Rim Ben Aziza: formal analysis, visualization, writing – review and editing, conceptualization, software. Wafa Belhadj Ammar: formal analysis. Cyrine Saadi: data curation. Maryem Marzouki: data curation. Youssef Hellal: supervision, project administration, validation. Said Jlidi: supervision, project administration, validation. Samia Kacem: project administration, supervision, validation. Bilel Jerbi: writing – original draft, methodology, software, data curation. All authors read and approved the final version of the manuscript.

Funding

The authors have nothing to report.

Ethics Statement

This study was conducted in accordance with the principles of the Declaration of Helsinki. Ethical approval was obtained from the Ethics Committee of the Maternity and Neonatology Center of Tunis (approval No. 17/2024). Parents of all participants were informed about the study objectives, procedures, potential risks, and benefits.

Consent

Informed consent was obtained from all the participants’ parents included in the study.

Conflicts of Interest

The authors declare no conflicts of interest.

Generative AI Statement

The authors declare that generative AI was used in the creation of this manuscript. During the preparation of this work the authors used Gemini to improve language and readability. After using this tool, the authors reviewed and edited the content as needed and took full responsibility for the content of the published article.

Data Availability Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.