Özet

Congenital right diaphragmatic hernia of Bochdalek rarely occurs in adults. Most of them are asymptomatic. In this article, we report a case of a 21-year-old male with rightsided Bochdalek diaphragmatic hernia who presented with abdominal pain and dyspnea. The chest radiography showed features suggestive of right-sided diaphragmatic hernia. This was confirmed on a computed tomography. The patient underwent right posterolateral thoracotomy whereby a 10 cm posterolateral diaphragmatic defect with herniation of the colon and kidney through the opening was found. During surgery the colon and right kidney were reduced into peritoneal cavity, and the diaphragmatic defect was repaired with non-absorbable sutures. The patient's recovery was uneventful. He remained well at six-month follow-up.

Bochdalek first reported herniation in the posterolateral region of the diaphragm in 1848, referred to as ‘Bochdalek hernia'. The hernia defect results in utero from failed closure of the pleuroperitoneal ducts, primitive communications between the pleural and abdominal cavity.[1] It is a common congenital anomaly, occurring in approximately one in 2200 to 12.500 live births, but is widely considered to be extremely rare in adults.[2] Most Bochdalek hernias present with lifethreatening cardiorespiratory distress in the neonatal period, and emergency surgical repair is usually performed in infants. Right-sided Bochdalek hernias are rarer because the right pleuroperitoneal canal closes earlier and the liver buttresses the right hemidiaphragm. Rarely, right-sided Bochdalek hernias remain clinically silent until adulthood. A Medline search of the literature revealed only 14 cases of right-sided Bochdalek's hernia in adults.[3] We report a 21-year-old male with Bochdalek hernia. The right kidney and colon were the herniated organs.

A 21-year-old previously healthy male patient was admitted to the emergency department with periumbilical pain and right-sided lower chest discomfort associated with shortness of breath of two weeks duration. There was no history of thoracic or abdominal trauma. Physical examination revealed stable vital signs. The bowel sounds were audible on the right side of the chest. Posteroanterior and lateral chest X-rays showed loops of the colon above the right hemidiaphragm (Fig. 1a, b). Computed tomography was performed and confirmed a right-sided Bochdalek hernia with colon loops and right kidney within the pleural cavity (Fig. 2a, b). Right thoracotomy was performed on the second hospital day. Herniation of the transverse colon and right-kidney in a hernia sac were observed at the site of the foramen of Bochdalek. The defect measured as 10x8 cm, and its borders were well defined. The lower lobe of the right lung was compressed and atelectatic. The adhesions were carefully released. The colon and right kidney were reduced to the peritoneal cavity after opening the hernia sac. There were no ischemic changes of the colon and kidney. The hernia sac was extracted and was repaired using non-absorbable sutures (Ethicon Prolene, Ethicon Inc. USA). The patient's postoperative recovery was uneventful. Chest tubes were removed on the 3^rd and 4^th days, and he was discharged home on the 6^th postoperative day. He remains well at six-month follow-up.

Fig 1: (a) Posteroanterior chest graphy of the patient. (b) Lateral chest graphy of the patient.

Fig 2: Computed tomography of the patient in (a) paranchimal window, (b) mediastinal window.

Tartışma

The classic congenital diaphragmatic hernia of Bochdalek is a posterolateral defect in the diaphragm thought to be caused by failure of the pleuroperitoneal canal to close at eight weeks' gestation.

The right hemidiaphragm is fully formed before the left side. The defect occurs on the left side 80% of the time and is occasionally bilateral. The hole can range in size from 1- to 2-cm round defect to total absence of the hemidiaphragm. A Bochdalek hernia may cause life-threatening respiratory distress in the first hours or days of life. The defect can cause respiratory distress or feeding intolerance in later infancy or childhood or may be identified on a radiograph obtained for unrelated reasons in an asymptomatic patient. The morbidity and mortality associated with a congenital diaphragmatic hernia are directly related to the age of the patient at presentation.[4] An emergency surgical repair is usually performed in infants. Congenital right diaphragmatic hernia of Bochdalek rarely occurs in adults and it is usually asymptomatic. Usually ventilation is not compromised in Bochdalek hernia discovered during adulthood.[5] P ulmonary symptoms include chest or shoulder pain, shortness of breath, dyspnea, cough. Abdominal or back pain, changes in bowel habits, vomiting, nausea, or abdominal distension are the abdominal symptoms of Bochdalek hernia. As in the present case, the physical examination of Bochdalek hernia in adults is typically misleading. Bochdalek hernia is unexpectedly detected on chest X-rays in patients who are asymptomatic or have no specific symptoms. Chest X-ray shows gas-fluid levels in the chest and thus suggest the diagnosis of diaphragmatic hernia. Thin section CT scanning is highly accurate and should be regarded as the standard method to diagnose a Bochdalek hernia. Our present patient was admitted to the emergency department with such nonspecific symptoms as abdominal pain and dyspnea. Diagnosis is established by plain chest radiography, with definitive confirmation by computed tomography (CT) scanning of the thorax and abdomen. Because our patient did not have any prior history of trauma, we believe that the defect in the diaphragm may have been congenital, for this reason, the hernia is supposed to be Bochdalek type.

The current treatment of choice of a Bochdalek hernia is surgical repair even in asymptomatic cases because of the risk of visceral herniation and strangulation.[6] Surgical treatment encompasses both reduction of the hernia and defect closure. The open thoracic approach has traditionally been performed for rightsided Bochdalek hernias because of superior visibility of the ipsilateral hemidiaphragm and the presence of the liver. In the present case a right posterolateral thoracotomy exposed a perfect view of the diaphragmatic defect and rest of the hemidiaphragm. We transferred the colon and right kidney to the peritoneal cavity. Intrathoracic kidney associated with Bochdalek hernia differs from other intrathoracic renal ectopia, as it tends to be mobile and be easily reduced from thorax to the abdominal cavity with other organs.[7] We repaired the diaphragmatic defect using non-absorbable sutures. Extensive defects may not be repaired with sutures and/or endogenous tissues. Recent research suggests a superiority of nonabsorbable vs absorbable prosthetic materials to achieve durable repairs.[8] These prosthetic materials should be available before surgery.

In conclusion, congenital right diaphragmatic hernia of Bochdalek rarely occurs in adults. Its successful management is based on both emergency diagnosis and timely surgical management. Right sided thoracotomy is recommended as the best chance for a successful outcome, helps to obtain satisfactory results.

Declaration of conflicting interests
The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Funding
The authors received no financial support for the research and/or authorship of this article.

Kaynaklar

1) Schumpelick V, Steinau G, Schlüper I, Prescher A. Surgical embryology and anatomy of the diaphragm with surgical applications. Surg Clin North Am 2000;80:213-39.

2) Yamaguchi M, Kuwano H, Hashizume M, Sugio K, Sugimachi K, Hyoudou Y. Thoracoscopic treatment of Bochdalek hernia in the adult: report of a case. Ann Thorac Cardiovasc Surg 2002;8:106-8.

3) Rout S, Foo FJ, Hayden JD, Guthrie A, Smith AM. Rightsided Bochdalek hernia obstructing in an adult: case report and review of the literature. Hernia 2007;11:359-62.

4) Reynolds M. Congenital posterolateral diaphragmatic hernias and other less common hernias of the diaphragm in infants and children. In: Shields TW, LoCicero J III, Ponn RB, Rusch VW, editors. General thoracic surgery. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 761-71.

5) Skandalakis JE, Gray SW, Ricketts RR. The diaphragm. In: Skandalakis JE, Gray SW, editors. Embryology for surgeons. 2nd ed. Baltimore: Williams & Wilkins; 1994. p. 502-15.

6) Mullins ME, Saini S. Imaging of incidental Bochdalek hernia. Semin Ultrasound CT MR 2005;26:28-36.

7) Obatake M, Nakata T, Nomura M, Nanashima A, Inamura Y, Tanaka K, et al. Congenital intrathoracic kidney with right Bochdalek defect. Pediatr Surg Int 2006;22:861-3.

8) Steinau G, Dreuw B, Schleef J, Treutner KH, Schumpelick V. Diaphragm replacement - an experimental animal study. Hernia 1997;1:123-7.