Late-presenting BDH was first published by Kirkland in 1959. It is a rare disease. While the incidence is 2.6-45% in all diaphragmatic hernias [1,3]. In our study, all diaphragmatic hernias were found to be 41%.
The literature only contains a small number of studies on late-presenting BDH. A 2005 study of 70 patients with BDH from a study group of 30 centers was published. Forty-six patients were assessed in a different study. However, the studies did not make any comparisons. In contrast to these studies, we evaluated 46 patients for our study and compared right and left BDH. Additionally, comparisons were made based on age groups and surgical approaches.
In a multicenter study by Kitano et al. involving 50 males (65%) and 27 females (35%). The mean age at diagnosis was 372 days (31 months) (Between 32 days and 15 years) [5]
In our study, 26 (57%) were male and 20 (43%) were female. The mean age at diagnosis was 14 months (30 days -14 years). The age at diagnosis was younger than in the literature. Because since a similar study was conducted in our clinic, the diaphragmatic hernia is being investigated in patients with symptoms in this way. Therefore, it is diagnosed earlier than in the literature.
In the study of Kitano et al., 69% of the patients were on the left, while 27% were on the right [5]. In our study, 85% were on the left, while 15% were on the right. When compared to the previous research, the incidence of this condition was found to be higher on the left side in our study.
Patients with late-presenting BDH may have acute or chronic symptoms, but some are asymptomatic. It is understood that the dependent compression of the intra-abdominal organs and the abnormal positioning of the GIS are the circumstances causing the symptoms.
In the Kitano et al study, 43% of the patients reported respiratory symptoms, 33% reported GIS symptoms, 13% reported both symptoms, and 11% reported no symptoms. As a result of the liver blocking the passage of the thoracic to the GIS, GIS symptoms are less common in patients with right late-presenting BDH [5]. In our study, respiratory symptoms were reported at a rate of 67%, GIS symptoms at a rate of 20%, 7% of participants were asymptomatic, and 4% reported both symptoms simultaneously.
Although clinical findings are variable, Chest X-rays in children with late-presenting CDH are the most important imaging modality. If there are GIS symptoms, plain abdominal radiography is the first examination to be requested later. Baglaj et al studied 107 (49%) of 218 children who were diagnosed with the first chest X-ray [2]. In addition, 50% of the patients in the literature required additional radiological evaluation to make the final decision [2,5]. In our study, 47% of our patients were diagnosed with chest X-ray alone, while 53 % of them had additional CT and/or Up-Low GIS series examinations performed.
Additionally, even though PA is a common diagnostic technique in late-present BDH, the literature initially misdiagnosed more than 25% of these cases. Pneumonia and pneumothorax misdiagnoses were the most frequent [2, 7, 8, 9, 10].
In our study, 30% of the patients had pneumonia, effusion, pneumothorax, and congenital cystic adenomatoid malformation (CCAM)-like images on chest X-ray, consistent with the literature, and the diagnosis was made using CT and/or Up-Low GIS series
The stomach gas on the chest X-ray radiograph may be confused for pneumothorax in patients with late-presenting BDH, leading to the mistaken placement of a chest tube. Therefore, the insertion of a nasogastric tube before radiological diagnosis may reduce these misdiagnoses [10,11]. In our study, 22% (10/46) of patients had stomach gas in the thorax. For this reason, We insert nasogastric tubes prior to the film's shooting.
Chest X-ray is frequently used in the diagnosis of BDH. Differential diagnosis from other diseases cannot be made with Chest X-ray in every patients so advanced imaging techniques like CT and/or Up- Low GIS series are needed [12].
Today, a CT scan is the best imaging method in patients with the right BDH. It is more useful in the differential diagnosis of intrathoracic masses or suspicious findings than Chest X-ray [13]. In our study, CT examination was performed in 33% of patients.
Studies utilizing barium or contrast often involve patients with diaphragmatic anomalies. At a rate of 32%, it has been done in the literatüre [2]. Up-Low GIS series were taken in 20 % of our study, which is less than in the literature. Using the Up-Low GIS series, the stomach, small intestine, or colon can be seen in the thorax. Up-Low GIS series can be used in suspicious cases or differential diagnoses even though these findings can also be seen on PA-AC or CT. Before surgery, additional pathologies such as malrotation can be found using the Up-Low GIS series.
Patients with right and left late-presenting BDH have been compared in a small number of studies in the literature. Eighty percent of people with right-sided BDH, according to the literature, have liver hernias. Patients with left diaphragmatic hernias had 26% stomach, 33% small intestine, and 42% large bowel hernias. These findings suggest that Up-Low GIS studies are more beneficial for patients with left late-presenting BDH [2,14]. 20% of the patients in our study who had left BDH underwent the Up-Low GIS Series.
Because the liver prevents herniation up the GIS, respiratory symptoms predominate in right diaphragmatic hernias as well [5]. While 86% of patients with right late-presenting BDH had respiratory symptoms, 74% of patients with left late-presenting BDH had respiratory symptoms, according to our study. On the left late-presenting BDH in our study, GIS symptoms were primarily observed.
Early surgical intervention is necessary to prevent complications as soon as the diagnosis is made. Patients with later-presenting BDH have a better prognosis because they have less pulmonary hypoplasia [15,16]. For this, laparotomy or MIS is preferred. In our study, we mostly performed it with laparotomy. However, in recent years we have performed MİS in selected cases. We preferred thoracoscopy as the minimally invasive method in patients with right late-presenting BDH. We preferred thoracoscopy or laparoscopy in patients with left late-presenting BDH. However, further studies with a higher number of patients are needed in this regard.
A hernia sac is observed in 7-32% of patients [17]. In our study, the incidence of hernia sac was 33%, which is consistent with the literature.
In the study by Kim et al. [11], it was reported that the rate of additional anomalies was 23%. In our study, while malrotation was seen in 30% of the patients, additional anomalies were observed in 9% of the patients.
In this study; recurrence and mortality were not observed. The reported mortality rate for CDH with a late presentation ranges from 0% to 18% [5,18,19,20]. In our series, Brid ileus developed in one patient and surgery was performed and corrected with a bridectomy. Recurrence occurred in two patients, re-operation was performed and mash was used in one. one patient died due to multiple anomalies in the sixth postoperative month.
The low mortality rate in our series is likely a lower rate of misdiagnosis and a low rate of pulmonary hypoplasia. We think it to proper surgical technique, appropriate intensive care support, and multidisciplinary work.
The limitation of our study is the low number of patients who underwent MIS surgery. In this regard, there is a need for patient numbers to be compared with higher patient numbers.