Case Report: A successful surgical approach towards rare congenital left diaphragmatic hernia associated with respiratory distress syndrome

Introduction

Congenital diaphragmatic hernia (CDH) is a condition in which partial or total agenesis of the diaphragm occurs, resulting in the thoracic and abdominal cavities becoming connected. Nearly five people are affected among every 10000 live births. It is more prevalent in males and with multiple pregnancies.¹ The primary pathology behind this disease is the impaired development or defective fusion of the pleuroperitoneal membrane.² Worldwide, survival rates increased to 50 % due to medical and surgical care advancements and enhanced diagnostic methods. It mostly depends on primary pulmonary hypoplasia, hypertension, and heart failure.³ The time of surgical repair also affects later morbidities. Also, initial stabilisation at birth, early diagnosis, and surgical intervention give positive outcomes.⁴

Patient and observation

Diagnostic assessment

In laboratory investigations, patient values were described in Table 1.

Table 1. Laboratory blood investigation of a patient with diaphragmatic hernia.

Sr. No	Name of investigation	Patient value	Normal value
1.	Haemoglobin	9.4 g/dl	14-24 g/dL
2.	Leukocytes	7,600 cells/mm3	9000-30,000/mm3
3.	Random blood sugar	98 mg/dl	80-90 mg/dL
4.	Serum sodium	134 mEq/L	135-145 mEq/L
5.	Serum potassium	3.4 mEq/L	3.7-5.9 mEq/L
6.	Creatinine	0.1 mg/dL	0.3-1.5 mg/dL

On radiological examination, the thorax left hemithorax showed multiple gaseous distended bowel loops within, with preserved peristaltic movement. Also, 4×2 cm defects were identified, which shows a left diaphragmatic hernia (Figure 1).

Figure 1. The ultrasound report shows a 4×2 cm defect present over the left diaphragm.

Therapeutic intervention

The newborn infant experienced difficulty in maintaining proper oxygen levels, leading the physician to initiate ventilator support. Following appropriate medical intervention, the infant’s condition became stable, prompting the physician to plan a surgical procedure for repairing a diaphragmatic hernia. The surgery occurred on the second day after the infant’s condition had stabilized. The surgical closure of the 4×2 cm defect was conducted on the left diaphragm using intermittent sutures. However, post-surgery, the abdominal contents unexpectedly breached into the thoracic cavity, necessitating the rescheduling of a second surgical procedure. After an interval of eight days, the second surgery was carried out, during which a compound flap was introduced into the abdomen. This step was completed smoothly, indicating an uneventful surgical procedure and a favourable prognosis.

Discussion

The most common type of CDH is the left-sided posterolateral defect, and it is reported in 85% of cases, while only 12% are represented on the right side. Anterior defects through the foramen Morgagni are less commonly reported in only 1-5% of patients with CDH. These defects contribute to the right sides.⁵

Advancement in fatal ultrasonography means 60% of CDH cases are diagnosed before the foetus’s birth.⁶ After birth, the child’s diagnosis is confirmed with the clinical and radiological examinations and symptoms that appear after a few hours of the birth. 5-25% of CDH cases present late, usually diagnosed during routine check-ups, after injury, or as complications such as necrosis, perforations, intestinal blockage, peritonitis, or respiratory or digestive discomfort.⁷

Symptoms include abdominal or chest pain, wheezing, dyspnoea, cough, apnoea, nausea, and vomiting.⁸ Respiratory symptoms most commonly occur in younger patients and are more frequent in right-sided CDH. At the same time, gastrointestinal symptoms are found in older patients and associated with left sides CDH cases.⁹

Patients diagnosed with CDH must be transferred to the surgical unit for further management and to avoid subsequent consequences. Treatment of patients in delayed presenting cases requires the stable condition of the baby and prompt surgical interventions.¹⁰ Open surgery is most commonly preferred, even though there is evidence of some reported cases managed through the laparoscopic approach. Delayed presented cases have a better outcome than those diagnosed antenatally or just after birth. Management of both cases varies. The survival rate is approximately 97-100% compared to neonatal CDH, with patients having a good recovery rate and fewer complications after surgery.¹¹

The mortality rate attributed to Congenital Diaphragmatic Hernia (CDH) varies significantly among different medical centres and remains notably elevated, despite the widespread adoption of innovative therapeutic approaches. The ECMO report indicates that specialised referral centres with considerable expertise have demonstrated the potential to save many infants. The survival rate for newborns delivered alive ranges approximately from 60% to 80%.¹² Various novel therapeutic strategies have been introduced for infants facing this condition, albeit without adequately controlled research to validate their effectiveness.

There is currently no substantiated evidence supporting the notion of “maturing” the fetal lung. Administering prenatal corticosteroids after the 34th week of pregnancy might be deemed necessary in cases of CDH-related pregnancies. However, this approach is not currently recommended. Simultaneously, there might be advantages to scheduling the delivery of a fetus with CDH. Nevertheless, there is a lack of controlled studies endorsing the timing of such deliveries on an elective basis or favouring a cesarean section over a vaginal delivery.¹³

Conclusion

Congenital Diaphragmatic Hernia usually occurs in neonates, but some cases can be diagnosed in older children. CDH is a therapeutic challenge. Although CDH can be diagnosed antenatally by ultrasonography and surgical repair is being done in many centres, the morbidity and mortality are still high because many aspects of the disease are still unknown, so more research and clinical studies need to be done for a better understanding of disease and adequate management.

Consent

Written informed consent for publication of their clinical details and clinical images was obtained from the patient’s guardian.