Post-Traumatic Presentation of Congenital Diaphragmatic Hernia in Adulthood: A Case Report

INTRODUCTION

A diaphragmatic hernia (DH) refers to a defect in the diaphragm that allows abdominal contents to protrude into the thoracic cavity.

It is usually a congenital anomaly often associated with a high neonatal mortality. The general incidence of congenital DH (CDH) ranges from 1/2000 to 1/5000 live births. However, in 5–25% of instances, it can also occur late in life as a late-presenting DH.¹

It is more likely to happen at sites of potential weakness over embryological fusion sites due to a sudden rise in the pleuroperitoneal pressure gradient, typically induced by trauma, pregnancy, etc. A late-presenting DH can be a considerable diagnostic challenge due to non-specific symptoms. We present a case of a 60-year-old female with adult-onset DH.

CASE REPORT

A female patient, 60 years of age, presented to the General Surgery OPD complaining of pain in the upper central abdomen and respiratory distress for the past 2 years.

The pain was localised to the epigastrium with an insidious onset and gradual progression. The pain was periodic and was described as dragging, which she described as a heavy and uncomfortable sensation in her abdomen. The pain aggravated sometimes after taking a heavy meal and spontaneously subsided within the next 2–3 h. She also experienced occasional breathlessness post-prandially and during normal household chores. She had a chronic mild cough with no expectoration or hemoptysis. She also reported an unusual sensation of fullness in her upper abdomen and chest after taking a meal.

Two years ago, she experienced blunt trauma to the abdomen when she was attacked by a bull. She was treated conservatively at a nearby primary healthcare centre and recovered without complication. She had moderate truncal obesity and on examination, breath sounds were slightly reduced on the left side with no other abnormalities.

A straight X-ray of the chest revealed an elevated left hemidiaphragm with a slight mediastinal shift to the right. The left costophrenic and cardiophrenic angles were blunt [Figure 1], and the findings were suggestive of a diaphragmatic pathology.

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Figure 1:

Chest X-ray showing elevation of the left dome of the diaphragm with a right mediastinal shift (white arrow).

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High-resolution computed tomography (CT)-thorax revealed intra-abdominal contents in the left hemithorax and marked elevation of the left hemidiaphragm with thinned out muscle fibers [Figure 2]. CT-Abdomen revealed a 6.2 cm defect in the posterolateral aspect of the diaphragm. Multiple viscera were seen to herniate to the thoracic cavity [Figure 3], thereby ruling out differentials such as phrenic nerve palsy, subpulmonic abscess, or an enlarged spleen. There was no evidence of obstruction or strangulation.

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Figure 2:

High-resolution computed tomography of the thorax showing intra-abdominal contents in the left hemithorax (white arrow).

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Figure 3:

Computed tomography of the abdomen showing a breach in the continuity of the diaphragm with multiple abdominal viscera herniating into the thoracic cavity (white arrow).

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The patient was admitted and optimised for surgery with a routine pre-operative workup. General anesthesia was given with endotracheal intubation. A left subcostal incision was made, and on exploration of the abdomen, the small intestine, transverse colon, spleen, and left kidney with its vascular pedicles were seen to herniate through the diaphragm into the thoracic cavity [Figure 4]. After restoring all the structures sequentially to their respective positions, a linear rent of about 6 cm was seen in the posterolateral region of the left hemidiaphragm. An intercostal drain (ICD) was placed in 5^th Intercostal space and primary repair of the rent was done in two layers using a 1-0 polypropylene suture [Figure 5]. The abdomen was closed in layers after establishing hemostasis. Post-operative recovery was uneventful. ICD removed after 7 days of surgery, and the post-operative chest X-ray showed normally expanded lungs with corrected mediastinal shift without any intestinal shadow in the thoracic cavity.

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Figure 4:

Intraoperative image showing the spleen herniating through the rent in the left dome of the diaphragm (white arrow).

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Figure 5:

Intercostal drain was placed, and diaphragmatic rent was repaired in two layers (white arrow).

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DISCUSSION

The thoracic cavity is separated from the abdominal cavity by the diaphragm. From the 4^th week of intrauterine life, the diaphragm develops from four embryonic structures: (1) the Pars diaphragmatica of septum transversum, ventrally, (2) the pleuroperitoneal membranes, postero-laterally, (3) the dorsal mesentery of the esophagus, dorsally, and (4) shelf-like projections, peripherally. The process of development is completed by the end of 12^th week. A CDH can occur due to improper closure of the pleuroperitoneal canal, which can lead to the herniation of abdominal organs into the thorax. CDH is most commonly detected in neonates, making a prevalence of 1 in 4000 live births.² Clinical presentations can include severe respiratory distress with cough, dyspnea, frequent chest infections, and localisation of discomfort to the chest or abdomen.

CDH presents late in 5–25% of adults,¹ most commonly due to the rupture of the peritoneal sac containing the abdominal organs resulting from increase in intra-abdominal pressure from factors such as blunt trauma, obesity, pregnancy or labor. The congenital defect may initially be blocked by a solid organ which can later be displaced, followed by herniation of abdominal contents into the thorax.^3,4

Late presentation of CDH poses a significant clinical challenge, with diagnostic failure rates reported to be as high as 38%.⁵ A delay in diagnosis of diaphragmatic injury can range from 18 h to 15 days.⁶ It either presents on the chest X-ray of an asymptomatic patient as incidental detection of abdominal organs in the thoracic cavity, or presents with vague non-specific symptoms. On the other hand, patients can present with an acute abdomen associated with shortness of breath due to complications such as incarceration, strangulation, or visceral perforation within the thoracic cavity. Diagnosis may be missed if the herniation is intermittent.⁷

CDH can primarily be diagnosed by visualising bowel loops in the thoracic cavity on an X-ray of the chest and abdomen. In patients of DH with acute intestinal obstruction, X-ray may also reveal air-fluid levels in the thorax. However, computed tomography remains the gold standard investigation for diagnosis and assessment.^8,9 Magnetic resonance imaging is a useful alternative for examining the whole of the diaphragm.¹⁰

The most common type of CDH is through the foramen of Bochdalek, which is a defect in the posterolateral aspect of the diaphragm. It is more frequently encountered on the left side (85%).¹¹ Typically, left-sided hernias contain the stomach, spleen, small intestine, and colon, whereas right-sided hernias contain the liver and intestine. Herniation of the kidney is a very uncommon finding; the incidence is estimated to be <0.25%.¹²

Surgery is the management of choice in an adult CDH, even if asymptomatic. It is done to avoid additional herniations and associated complications.¹³ The surgical approach can either be transabdominal or transthoracic for emergency and elective settings, respectively. The transabdominal route through an upper midline incision is preferred in cases of intestinal obstruction.¹⁴ In our case, a subcostal incision was chosen for better surgical exposure and to reduce the risk of incisional hernias due to muscle damage. After reducing the hernia contents, they should be systematically inspected for signs of compromised viability, serosal breach or frank perforation. A non-absorbable suture or mesh of adequate size is used for closure of the diaphragmatic defect in a tension-free manner. Laparoscopic and thoracoscopic correction of CDH is also a viable option.^15,16

Mortality following elective repair of CDH is <4%.¹⁷ Nonetheless, in cases of delayed diagnosis or post-operative complications, mortality can go up to 32%.¹⁸ Therefore, the patient’s post-operative care is extremely important in the first few weeks. In case of compromised respiratory function, positive pressure ventilation followed by active chest physiotherapy is recommended.²

CONCLUSION

Usually occurring in neonates, DH may occur in adults due to an increase in pleuroperitoneal pressure gradient along embryological fusion points due to trauma, pregnancy, etc. Untreated long-standing cases may also present with acute symptoms. Due to its non-specific symptoms, adult-onset DH often presents a diagnostic challenge. Detailed history taking, clinical examination, a high level of diagnostic vigilance supplemented with appropriate radiological support, along with necessary surgical and post-operative care is the optimal modus operandi.