Spontaneous Acquired Diaphragmatic Hernia: Case Series and Review of Literature

Abstract

Congenital diaphragmatic hernia (CDH) and acquired diaphragmatic hernia (ADH) are secondary to a weakness or opening in the diaphragm. Both are types of internal hernia. The abnormality can range from a minor defect in the posterior edge of the diaphragm to the entire loss of it. CDH usually leads to lung hypoplasia associated with pulmonary hypertension and immaturity, as well as cardiac dysfunction. In ADH, pathophysiology is secondary to herniation and its pressure effects. We report a series of two cases of spontaneous ADH (SADH). Spontaneous acquired rupture of the diaphragm can occur after exercise, coughing, labor (delivery), or even without any significant preceding event. It is uncertain if SADH is caused by a preexisting weak region in the diaphragm, a loss of muscle coordination during vigorous exertion, or both. It is a potentially fatal surgical emergency requiring a high index of clinical judgment in the appropriate clinical setting. SADH acts mechanically by compressing the lung. It is an infrequent entity. This case series and review concisely illustrate the genesis, diagnosis, and treatment of spontaneous diaphragmatic hernia.

INTRODUCTION

A spontaneous acquired diaphragmatic hernia (SADH) happens when the peritoneal viscera or contents herniate into the chest through the diaphragm. Only 31 cases being reported in the literature till 2009, hence SADH is a sporadic or rare disorder. It is a rare entity with significant mortality and morbidity. Hence, a high level of alertness is strongly advised to avoid the devastating death rate of more than 30% and morbidity associated with delayed and missed identification.[1]

Congenital diaphragmatic hernia (CDH) is caused by a defective diaphragm, allowing peritoneal contents to herniate into the chest cavity, disrupting the function of the lungs and embryonic growth. The disorder can manifest as a single lesion or as part of a syndrome. The incidence of CDH affects <5 per 10,000 births. African Americans are a less affected population, and males are affected more than females. Acquired diaphragmatic hernia (ADH) is usually secondary to direct or indirect trauma in the form of either penetrating or blunt trauma. Other causes of ADH include iatrogenic.[2] It is interesting to note that the cause of SADH is uncertain and could be due to a preexisting weak region in the diaphragm, a loss of muscle coordination during vigorous exertion, or a combination of both factors.[3]

CASE REPORT

Two patients were admitted to the surgery department with spontaneously acquired diaphragmatic ruptures. Both patients, one female of 45 and one male of 52 years old, did not have any history of trauma or any preceding event. The female patient is a farmer by occupation and the male one is a manual laborer; both have work hard with physical exertion like lifting heavy weight. The female presented with pain in the left hypochondria and lumbar region for 10 days; on examination, she was found to have a soft, nontender abdomen with reduced air entry in the left lower lung field. An intraoperative posterolateral defect of 5.8 cm was noted in the left side of the diaphragm, with herniation of small bowel loops, transverse colon, omentum, and left kidney into the thoracic cavity. The second patient, a male, presented with diffuse pain in the abdomen for 2 days. On examination, he was found to have a soft, nontender abdomen with reduced air entry in the left lower lung field and bowel sounds heard in the left lower lung field. Both these patients were treated by open diaphragmatic hernia repair with composite mesh.

SADH was identified on chest X-ray. The diagnosis was confirmed with contrast-enhanced computed tomography (CECT). CECT images are depicted in Figure 1 for the first patient for the second patient. Intraoperative details are illustrated in Figures 2 and 3. The postoperative course was uneventful. Both were followed up after one month without any evidence of recurrence.

Figure 1.

Contrast-enhanced computerized tomography images both (a and b) show evidence of diaphragmatic hernia, yellow arrowhead shows hernia contents and green suggestive of defect

Figure 2.

(a and b) Intraoperative details of the female patient. The green arrow in image (a) depicts the defect in diaphragm and yellow arrow in (b) shows repair with composite mesh

Figure 3.

(a and b) Intraoperative details of the male patient. The green arrow in image (a) depicts the defect in the diaphragm and yellow arrow in (b) shows repair with composite mesh

The details are summarized in Table 1.

Table 1:

Case details

Age/gender	Preceding event	Clinical features	Imaging	Treatment	Follow-up at 1 month
45/female	No preceding event	C/O pain in left hypochondria and lumbar region for 10 days No history of trauma PA - soft, nontender Chest - reduced air entry in left lower lung field	Poster lateral defect of 5.8 cm in the left side of the diaphragm, with herniation of small bowel loops, transverse colon, omentum, left kidney into thoracic cavity	Laparoscopic converted to open diaphragmatic hernia repair	No recurrence
52/male	No preceding event	C/O diffuse pain abdomen for 2 days No C/O dyspnea, constipation No history of trauma PA - soft, no tender Chest- bowel sounds heard in the left lower lung field	Defect of 10 cm × 12 cm in left hemi diaphragm, with herniation of stomach, GJ junction, transverse colon, small bowel loops, left kidney (with simple cyst)	Open diaphragmatic hernia repair	No recurrence

DISCUSSION

The septum transversum (ventral and pericardial portion), pleura-peritoneal membrane, or canal and striated muscles contribute to form the diaphragm during embryonic development. The canals normally close around the eighth week of pregnancy. Failure to close results in developmental anomalies or defects. Fennertus described diaphragmatic hernia in 1541 for the first time, whereas Ambroise Pare described traumatic diaphragmatic hernia in 1579. In 1690, Lazarus Riverius described a CDH in a 24-year-old man at postmortem. McCauley described the physical anatomy associated with CDH in a newborn baby in 1754. Victor Alexander Bochdalek described Bochdalek hernia for the first time in 1848. CDH neonates have a triad of respiratory distress, obvious dextrocardia, and a scaphoid abdomen. Adults with congenital heart disease may have occasional abdominal pain, vomiting, dyspnea, and chest pain. Acute manifestation can be caused by strangulation of the hernia contents. A positive pressure gradient between the peritoneal and pleural compartments with a range of 8–21 cm H20 can push abdominal contents to herniate through the diaphragm defect.[4]

Of the 28 cases found up to 2009 in the literature, the demographic features found are described. The age range was 3–74 years, and males were affected more than females (Seventeen were males, and 11 were females); only three children were affected out of all. Out of these 28 patients, the preceding event was coughing in most of the patients, followed by strenuous exercise like heavy weight lifting, and in minorities, vomiting, and normal vaginal delivery. In one patient, it was after a massage; no preceding history was present in six. The most common presentation of SADH was chest and abdominal pain, nausea and vomiting, breathlessness, and chest swelling. Chest X-ray was the primary initial investigation to diagnose, followed by confirmation with CECT. Two patients were initially treated with chest tube insertion, leading to bowel and stomach perforation. In these 2 patients, the intercostal drain was inserted as it was considered pneumothorax. Patients were diagnosed with chest X-ray and not undergone confirmatory CECT. All patients’ diagnoses were confirmed during surgery except for one, established with an autopsy study. Nineteen (70%) patients had hernias on the left side; one was bilateral, and the rest affected the right dome of the diaphragm. The defects ranged in size from 2 cm to 16 cm. There were 22 peripheral (79%) and 6 central weaknesses of the diaphragm. The stomach, large bowel, greater and lesser omentum, small bowel, spleen, and liver were among the hernia contents. Ten hernias had a single organ; nine contained two organs, and seven contained three organs. There was one death on admission, and there were three postoperative deaths.[3] However, Losanoff et al.[3] not mentioned about recurrence in their review. For 9 (32%) sufferers of SADH, coughing was the prior symptom, followed by physical activity for 6 (21%) patients, normal vaginal delivery for 4 (14%), nausea and vomiting for 2 (7%), and massage in 1 (4%); for the one comatose patient, no history was given.[3] Five (18%) patients had no prior event noticed.

Three more cases of SADH have been reported till date: Darocki and Medak[5] reported a case of SADH in an elderly female, who presented with difficulty breathing in the emergency room. Physical examination with auscultation reveals absent air entry in her left chest. Chest X-ray was suggestive of SADH. She underwent a successful surgical intervention.

Ghidirim et al.[6] presented a case of spontaneous diaphragm rupture in an 18-year-old patient with chest pain. He had not given any history of trauma. The chest X-ray was suggestive of herniation of the stomach and bowel in the left chest, compressing the lung and leading to its collapse. Surgery was performed to repair a left-sided diaphragm after the reduction of the hernia. The third case was reported by Fenner et al.,[7] with coughing as a preceding event. A 66-year-old man had a recurrent cough, shortness of breath, and fever. Although the initial CECT scan was unremarkable, the follow-up computed tomography scan revealed SADH of the left diaphragm with involvement of the left colic flexure, abdomen, and most of the omentum. Emergency laparotomy confirmed SADH. The anterior part of the torn diaphragm was repaired with composite mesh. Along with this, a right hemicolectomy with anastomosis was performed.

Dubhashi et al.[4] presented a case of Bochdalek hernia with incarceration of the colon in a young female. The repair was performed with nonabsorbable suture similar to the present case series. Hamoudi et al.[8] reported a case of a woman of 40 admitted to an emergency, a week after a full-term home delivery. She was having chest pain with abdominal pain, vomiting, and breathlessness. Chest radiography reveals abdominal contents in the left thoracic cavity. It was suggestive of SADH on surgery and was found to have a left Bochdalek hernia with stomach, large bowel, and spleen herniation into the left thoracic cavity. SADH during delivery is a dangerous but uncommon condition that necessitates prompt treatment to prevent further complications.

We report two cases with spontaneous diaphragm rupture without any preceding event. As per the literature review, 31 cases of SADH have been reported.[3,6,7] Out of these, most of the patients had preceding events such as cough, vomiting, physical exercise, and delivery. No preceding event was identified in six patients,[3] as in the present case series.

CONCLUSION

Spontaneous rupture of the diaphragm is indeed rare but can present with acute and severe symptoms. Emergency manifestations include sudden onset of intense chest or abdominal pain, difficulty breathing, and rapid breathing. Red flag signs are persistent coughing, worsening breathlessness, and abdominal tenderness.

Diagnosis can be challenging, but imaging plays a crucial role. Chest X-ray may show abnormal positioning of abdominal organs in the chest cavity. Contrast-enhanced computed tomography (CECT) provides detailed images of the diaphragm and surrounding structures, aiding in diagnosis. Ultrasound can also be helpful, especially in unstable patients.

Prompt evaluation is essential in the emergency setting. Suspected cases require urgent surgical intervention as the mainstay of treatment. Delay in diagnosis and management can lead to complications such as respiratory distress, abdominal organ dysfunction, and even death. Therefore, maintaining a high index of suspicion and rapid diagnostic workup are crucial for timely intervention and improved outcomes.

Research quality and ethics statement

The authors followed applicable EQUATOR Network (http://www.equator-network.org/) guidelines, notably the CARE guideline, during the conduct of this report.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.