|Year : 2022 | Volume
| Issue : 2 | Page : 86-88
A case report on late-onset congenital diaphragmatic hernia in adolescence
Pawan Sharma, Simarjit Singh Rehsi, Abhishek Das, Vipin Venugopal Nair
Department of Surgery, Armed Forces Medical College (AFMC), Pune, Maharashtra, India
|Date of Submission||05-Jun-2021|
|Date of Decision||12-Aug-2021|
|Date of Acceptance||20-Aug-2021|
|Date of Web Publication||22-Oct-2021|
Dr. Vipin Venugopal Nair
Department of Surgery, Armed Forces Medical College (AFMC), Pune 411040, Maharashtra
Source of Support: None, Conflict of Interest: None
Congenital diaphragmatic hernia is a life-threatening anomaly with the high mortality rate in infancy. Very rarely they remain silent and manifest in the adult life. Once they manifest, the symptoms vary and are not easy to diagnose. The entity also has associated problems in the form of hypoplastic lungs and loss of domain of the abdomen. In such cases, the diagnosis is most often based on clinical suspicion and radiological confirmation. The treatment options are open abdominothoracic procedures or laparoscopic procedures. Once hernia is reduced, the defect is closed and should be reinforced with prosthetic mesh. Here we present a case of congenital diaphragmatic hernia in a teenager presenting with cardiac symptoms. The challenges faced in managing the case are discussed.
Keywords: Congenital diaphragmatic hernia (CDH), diaphragmatic defect, laparoscopy, mesh repair
|How to cite this article:|
Sharma P, Rehsi SS, Das A, Nair VV. A case report on late-onset congenital diaphragmatic hernia in adolescence. Int J Abdom Wall Hernia Surg 2022;5:86-8
| Key Messages:|| |
- The clinical presentation of congenital diaphragmatic hernia presenting after childhood is nonspecific.
- The most important challenge during management will be the expansion of the hypoplastic lung and lack of abdominal volume (loss of domain).
- Laparoscopic mesh repair is presently the treatment of choice.
| Introduction|| |
Congenital diaphragmatic hernia (CDH) is a life-threatening congenital anomaly. Most of the times, it is manifested during the neonatal period with a severe respiratory compromise and with an associated mortality of 40%–60%. Rarely the presentation is late. In such cases, the symptoms are misleading. We report a case of nearly asymptomatic teenager with clinical presentation mimicking cardiac disease. On evaluation, viscera and spleen were found inside the thoracic cavity.
| Case History|| |
A 15-year-old boy presented to the medical outpatient department with complaints of progressive dyspnea on mild-to-moderate exertion over the preceding 6 months. On clinical evaluation, decreased breath sounds were found on auscultation over the left side of the chest. Other general systemic examinations were normal. There was no history of syncope, palpitation, orthopnea, or abnormal respiratory sounds. Routine chest radiogram taken revealed multiple air lucencies seen in the left hemithorax in a linear fashion, suggestive of bowel loops. Further evaluation with high-resolution computerized tomography (CT) revealed a large defect in the left subdiaphragmatic region with herniation of small and large bowel loops and the spleen up to mid-left chest. There was mild mediastinal shift toward right and atelectasis of left lower lung lobe. The echocardiography was normal.
He underwent laparoscopic mesh repair of posterolateral diaphragmatic defect under general anesthesia. Intraoperatively there was (1) posterolateral Bochdalek hernia with a defect measuring 8 cm × 3 cm, (2) left thoracic cavity containing large and small bowel loops with spleen, and (3) left lung visualized as minimally expanded [Figure 1]. All the contents were reduced from the thoracic cavity. Due to underdeveloped abdominal cavity there was difficulty in reducing the viscera. There was not enough space for pneumoperitoneum. The defect was approximated using intracorporal prolene sutures. A 15 cm × 15 cm composite polypropylene mesh was placed under the defect for reinforcing the tissue repair. A prophylactic chest tube drain was inserted into the left hemithorax [Figure 2].
|Figure 1: Preoperative chest radiograph: (A) posteroanterior view and (B) lateral view showing herniated bowel loops in the thoracic cavity|
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|Figure 2: Intraoperative laparoscopic placement of 15 cm × 15 cm composite polypropylene mesh|
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Postoperative recovery was uneventful. The patient started on oral diet from the third postop day (POD). Intrabdominal drain was removed on the fourth POD. Port-site staples were removed on the ninth POD and chest drain was removed on the twelfth POD. Wound healed well and the patient was discharged on the fourteenth POD. On 1-month and 3-month follow-up, the patient recovered completely with full lung expansion [Figure 3].
|Figure 3: Postoperative posteroanterior view radiograph on postoperative day 12 showing chest drain in situ with fully expanded left lung|
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| Discussion|| |
A congenital defect in the diaphragm causing protrusion of viscera into the thoracic cavity is called a CDH. CDH is classified as anterior Morgagni’s hernia and posterolateral Bochdalek hernia. Other variants are eventration of the diaphragm and central tendon. The reason for this is hernia formation that precedes lung development causing pulmonary hypoplasia and respiratory distress. This defect is uncommon in teenage populations. In adolescence, CDH can present as cardiac disease, pleural effusion, respiratory disorders, abdominal disorders, or in combination. Many times, late CDH was treated as gastroenteritis, respiratory disorders, and even tuberculosis.
Mostly it is manifested as an incidental finding during radiological examination for some other problem. Otherwise, it may become symptomatic due to pressure effects inside the thoracic cavity or secondary to incarceration, strangulation, and visceral rupture inside the chest cavity. In our case, the chest X-ray during evaluation for breathing difficulty led us to the diagnosis. In most cases, the displaced organ is the stomach, followed by the colon, spleen, small intestine, and ureter. The preferred diagnostic modality is CT. The reported sensitivity and specificity of CT scanning is 78% and 100%, respectively. Surgical repair is recommended in all adult CDH patients to prevent life-threatening complications. Laparoscopic repair of a Bochdalek hernia is considered safe and is the recommended elective surgery. Primary closure of the congenital defect using nonabsorbable suture and mesh reinforcement over the defect is the surgery of choice. It is associated with low morbidity and mortality and has an excellent long-term prognosis with a low recurrence rate.,
The type of mesh used in repair of CDH varies from nonabsorbable prolene to partially absorbable dual mesh. Many studies advocate the use of dual mesh with bowel-protective layering facing the viscera as the best prosthetic option. However, there are many articles in literature that state the successful use of polypropylene mesh in repair of CDH in adults. Coated prolene mesh was found to be safe for use in adult cases.,,,,
Apart from laparoscopic repair other options available are laparotomy and thoracotomy approach. Compared to laparoscopic surgery the long-term morbidity is high in open approach. A short-term surgical complication is pneumothorax and is seen in 17.5%–36.1%. In our case, we inserted a chest tube prophylactically on the left side. The problems faced by our team in the management of CDH were (1) atypical clinical presentation, (2) the hypoplastic lung causing anesthesia risks, and (3) lack of operative space in the abdominal cavity after reduction of thoracic content.
| Conclusion|| |
Late-onset CDH is a rare disease with atypical presentation. These patients present over a period with varied subtle signs. Early diagnosis is the key to successful management. Hypoplastic lung may cause minor problems, but overall lung expansion is excellent post-reduction of hernia. Laparoscopic repair is a better option than open procedure with excellent exposure and may have additional benefits, such as decreased pain, shorter hospitalization, and improved cosmesis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/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.
Ethical policy and institutional review board statement
Institutional ethical committee approval was taken prior to the study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Shin HB, Jeong YJ Late presenting congenital diaphragmatic hernia misdiagnosed as a pleural effusion: A case report. Medicine (Baltimore) 2020;99:e20684.
Rattan KN, Singh J, Dalal P Diagnostic challenges in late-presenting congenital diaphragmatic hernia: A 16-year experience from tertiary care centre in north India. Trop Doct 2019;49:138-41.
Elhalaby EA, Abo Sikeena MH Delayed presentation of congenital diaphragmatic hernia. Pediatr Surg Int 2002;18:480-5.
Vega MT, Maldonado RH, Vega GT, Vega AT, Liévano EA, Velázquez PM Late-onset congenital diaphragmatic hernia: A case report. Int J Surg Case Rep 2013;4:952-4.
Schumacher L, Gilbert S Congenital diaphragmatic hernia in the adult. Thorac Surg Clin 2009;19:469-72.
Malekzadegan A, Sargazi A Congenital diaphragmatic hernia with delayed presentation. Case Rep Surg 2016;2016:7284914.
Kunduru N, Digvijoy S, Gangadhar RG, Venu MT, Suryaramachandra VG, Jagan MR, et al
. Surgical management of adult diaphragmatic hernia: An institutional experience. Int J Contemp Med Surg Radiol 2018;3:B54-7.
Chatterjee D, Ing RJ, Gien J Update on congenital diaphragmatic hernia. Anesth Analg 2020;131:808-21.
Kesieme EB, Kesieme CN Congenital diaphragmatic hernia: Review of current concept in surgical management. Int Sch Res Notices 2011;8:2011.
Kao AM, Ayuso SA, Huntington CR, Sherrill WC, Cetrulo LN, Colavita PD, Todd Heniford B J Laparoendosc Adv Surg Tech 2021;7:814-9.
Târcoveanu E, Georgescu Ș, Vasilescu A, Andronic D, Dănilă N, Lupaşcu C, et al
. Laparoscopic management in Morgagni hernia: Short series and review of literature. Chirurgia (Bucur) 2018;113:551-7.
Ambrogi V, Forcella D, Gatti A, Vanni G, Mineo TC Transthoracic repair of Morgagni’s hernia: A 20-year experience from open to video-assisted approach. Surg Endosc 2007;21:587-91.
Caprotti R, Mussi C, Scaini A, Angelini C, Romano F Laparoscopic repair of a Morgagni–Larrey hernia. Int Surg 2005;90:175-8.
Han S, Lee S, Son J, Seo JM, Lee SK Pneumothorax is a significant risk factor for mortality in congenital diaphragmatic hernia: A single center experience. Adv Pediatr Surg 2018;24:68-75.
[Figure 1], [Figure 2], [Figure 3]