Surg Today DOI 10.1007/s00595-014-1001-6
Case Report
Diaphragmatic hernia caused by heterotopic endometriosis in Chilaiditi syndrome: report of a case Naoki Haratake · Koji Yamazaki · Yasunori Shikada
Received: 20 February 2014 / Accepted: 30 June 2014 © Springer Japan 2014
Abstract A 50-year-old Japanese female was admitted to our hospital because of ileus due to Chilaiditi syndrome. Her symptoms did not improve with conservative therapy, so chest and abdominal computed tomography (CT) was performed on the fifth day after hospital admission. A diagnosis of incarceration of a right diaphragmatic hernia was established in the coronal view of CT, and emergency surgery was performed. A dilated loop of small intestine was seen in the right thoracic cavity, which was strangulated by the 2-cm defect in the diaphragm. Primary closure of the diaphragm was performed. Approximately 80 cm of the terminal ileum showed obvious ischemic changes, and it was cut and reconstructed. Postoperatively, the patient made an uneventful recovery and was discharged on the 15th postoperative day. A histological examination of the specimen of the diaphragm around the hernia orifice showed the presence of a small cystiform glandular system with hemorrhage and congestion. These findings indicated that heterotopic endometriosis was present in the diaphragm. This report describes the first known case of right diaphragmatic hernia secondary to heterotopic endometriosis in a patient with Chilaiditi syndrome. The clinical course and management of affected patients and a literature review of these three unusual conditions are discussed.
Introduction
Keywords Diaphragm hernias · Endometriosis · Chilaiditi syndrome
Case report
N. Haratake (*) · K. Yamazaki · Y. Shikada Department of General Surgery, Saiseikai Fukuoka General Hospital, Fukuoka 810‑0001, Japan e-mail: [email protected]
This report describes the first known case of right diaphragmatic hernia complicated by heterotopic endometriosis and Chilaiditi syndrome. A right diaphragmatic hernia is a rare disease, the etiology of which is usually related to congenital, post-traumatic or iatrogenic factors [1–3]. Heterotopic endometriosis is the presence of ectopic endometrial tissue, and usually manifests as pelvic endometriosis. While intrathoracic endometriosis is very rare [4, 5], pleural or diaphragmatic endometriosis can cause catamenial pneumothorax, particularly in the right thorax. Ectopic endometrial tissue can also fenestrate the diaphragm as a result of hormonal stimulation. Chilaiditi syndrome is a rare intestinal anomaly of hepatodiaphragmatic interposition of the colon or small intestine. Most patients with this condition are asymptomatic throughout their lives. However, affected patients can rarely develop abdominal pain, distention, vomiting, anorexia, constipation and obstruction. The present report describes the case of a patient with Chilaiditi syndrome in which heterotopic endometriosis caused a defect in the right diaphragm, resulting in a right diaphragmatic hernia.
A 50-year-old Japanese female presented to our Emergency Department with right upper abdominal pain and nausea. Her past medical history included Chilaiditi syndrome and heterotopic endometriosis, but she had no history of laparotomy. She had experienced repeated ileus over the past decade due to the Chilaiditi syndrome, but each episode responded to conservative treatment. She had developed
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heterotopic endometriosis during the pre-menopausal period, and had undergone two bullectomy procedures for right catamenial pneumothorax when she was 38 and 39 years old, respectively. A chest and abdominal X-ray revealed intestinal gas in the upper right abdomen, which was a typical finding of Chilaiditi syndrome (Fig. 1). She was diagnosed with ileus due to Chilaiditi syndrome and
Surg Today
was treated conservatively with bowel rest and nasogastric tube decompression. However, her symptoms did not improve, and chest and abdominal computed tomography (CT) was performed. A right diaphragmatic hernia was suspected based on the coronal view of the CT. There was a defect in the top of the right diaphragm, through which the small intestine entered the right thoracic cavity (Fig. 2). A diagnosis of incarceration of a right diaphragmatic hernia was established, and emergency surgery was performed on the fifth day after hospital admission. A 5-cm incision was made at the level of the right eighth intercostal space. A dilated loop of small intestine was seen in the right thoracic cavity, which was strangulated by the 2-cm defect in the diaphragm (Fig. 3). After reducing
Fig. 1 A chest roentgenogram shows bowel gas in the right upper abdomen
Fig. 2 A coronal view of the chest and abdominal computed tomography showed a diaphragmatic defect through which the small intestine entered the right thoracic cavity (arrow)
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Fig. 3 a A dilated loop of small intestine was seen in the right thoracic cavity. b The 2-cm defect in the diaphragm forming the hernia orifice
Surg Today
Fig. 4 A glandular system with fibrosis, chronic inflammation, hemorrhage and congestion was present in the resected diaphragm (arrow) (H&E stain, ×400)
the small intestine into the abdomen, the edge of the diaphragm forming the hernia orifice was biopsied in light of the patient’s past history of heterotopic endometriosis. The diaphragm around the hernia orifice had become atrophied, which allowed the small defect to form. Primary closure of the diaphragm was performed. A median incision was subsequently made in the upper abdomen. Approximately 80 cm of terminal ileum showed obvious ischemic changes. After the ileum was cut and reconstructed, the surgery was completed. A histological examination of the specimen of the diaphragm showed the presence of a small cystiform glandular system with fibrosis, chronic inflammation, hemorrhage and congestion (Fig. 4). These findings indicated that heterotopic endometriosis was present in the diaphragm. Postoperatively, the patient made an uneventful recovery and was discharged on the 15th postoperative day in good condition. Because she had already reached menopause, she did not receive any hormone therapy. On follow-up, she was well and without symptoms.
Discussion A right diaphragmatic hernia is a rare condition. Most are congenital in nature and can induce neonatal respiratory distress [1]. Chest trauma or complications of medical procedures are well-known causes of right diaphragmatic hernia in the adult population [2, 3]. Although heterotopic endometriosis can result in a defect in the right diaphragm, it rarely causes a diaphragmatic hernia, because the liver overlays the defect [4, 5]. However, in patients with Chilaiditi syndrome, a right diaphragmatic hernia can develop because there is an interposition of the colon or small intestine between the liver and right diaphragm.
Therefore, the bowel can enter the thoracic cavity if there is a defect in the diaphragm. In the present case, the simultaneous presence of heterotopic endometriosis and Chilaiditi syndrome allowed the development of a right diaphragmatic hernia. This is first known case of a right diaphragmatic hernia caused by heterotopic endometriosis in a patient with Chilaiditi syndrome. Because the histological examination of the specimen of the diaphragm showed the presence of a small cystiform glandular system (i.e., ectopic endometrial tissue), we were able to prove that heterotopic endometriosis was the cause of the diaphragmatic hernia. In addition, considering that the patient had already reached menopause, and the diaphragm around the hernia orifice had become atrophied, which led to the formation of the small defect, it was concluded that the defect had been made previously by the heterotopic endometriosis, and the bowel had entered the thoracic cavity through the existing defect accidentally. In this case, because the patient had experienced repeated episodes of ileus due to Chilaiditi syndrome, a right diaphragmatic hernia was not initially suspected. Indeed, in the context of a typical sign of Chilaiditi syndrome (i.e., dislocation of the bowel above the liver), the diagnosis of a diaphragmatic hernia was difficult to make by plain X-rays. However, the coronal view of CT accurately visualized the focal defect in the right diaphragm through which the bowel had entered the right thoracic cavity in this case. Therefore, CT is considered to be an excellent modality for diagnosing a diaphragmatic hernia. In summary, this report documents an extremely rare occurrence of right diaphragmatic hernia caused by heterotopic endometriosis in a patient with Chilaiditi syndrome. Clinicians should be aware that patients with a history of heterotopic endometriosis and Chilaiditi syndrome are at risk of developing a right diaphragmatic hernia. Conflict of interest Naoki Haratake and co-authors have no conflicts of interest.
References 1. Newman BM, Afshani E, Karp MP, Jewett TC Jr, Cooney DR. Presentation of congenital diaphragmatic hernia past the neonatal period. Arch Surg. 1986;121:813–6. 2. Hanna WC, Ferri LE, Fata P, Razek T, Mulder DS. The current status of traumatic diaphragmatic injury: lessons learned from 105 patients over 13 years. Ann Thorac Surg. 2008;85:1044–8. 3. Soufi M, Meillat H, Le Treut YP. Right diaphragmatic iatrogenic hernia after laparoscopic fenestration of a liver cyst: report of a case and review of the literature. World J Emerg Surg. 2013;8:2. 4. Chatra PS. Thoracic endometriosis: a case report. J Radiol Case Rep. 2012;6:25–30. 5. Cooper MJ, Russell P, Gallagher PJ. Diaphragmatic endometriosis. Med J Aust. 1999;171:142–3.
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Case Report
Diaphragmatic hernia caused by heterotopic endometriosis in Chilaiditi syndrome: report of a case Naoki Haratake · Koji Yamazaki · Yasunori Shikada
Received: 20 February 2014 / Accepted: 30 June 2014 © Springer Japan 2014
Abstract A 50-year-old Japanese female was admitted to our hospital because of ileus due to Chilaiditi syndrome. Her symptoms did not improve with conservative therapy, so chest and abdominal computed tomography (CT) was performed on the fifth day after hospital admission. A diagnosis of incarceration of a right diaphragmatic hernia was established in the coronal view of CT, and emergency surgery was performed. A dilated loop of small intestine was seen in the right thoracic cavity, which was strangulated by the 2-cm defect in the diaphragm. Primary closure of the diaphragm was performed. Approximately 80 cm of the terminal ileum showed obvious ischemic changes, and it was cut and reconstructed. Postoperatively, the patient made an uneventful recovery and was discharged on the 15th postoperative day. A histological examination of the specimen of the diaphragm around the hernia orifice showed the presence of a small cystiform glandular system with hemorrhage and congestion. These findings indicated that heterotopic endometriosis was present in the diaphragm. This report describes the first known case of right diaphragmatic hernia secondary to heterotopic endometriosis in a patient with Chilaiditi syndrome. The clinical course and management of affected patients and a literature review of these three unusual conditions are discussed.
Introduction
Keywords Diaphragm hernias · Endometriosis · Chilaiditi syndrome
Case report
N. Haratake (*) · K. Yamazaki · Y. Shikada Department of General Surgery, Saiseikai Fukuoka General Hospital, Fukuoka 810‑0001, Japan e-mail: [email protected]
This report describes the first known case of right diaphragmatic hernia complicated by heterotopic endometriosis and Chilaiditi syndrome. A right diaphragmatic hernia is a rare disease, the etiology of which is usually related to congenital, post-traumatic or iatrogenic factors [1–3]. Heterotopic endometriosis is the presence of ectopic endometrial tissue, and usually manifests as pelvic endometriosis. While intrathoracic endometriosis is very rare [4, 5], pleural or diaphragmatic endometriosis can cause catamenial pneumothorax, particularly in the right thorax. Ectopic endometrial tissue can also fenestrate the diaphragm as a result of hormonal stimulation. Chilaiditi syndrome is a rare intestinal anomaly of hepatodiaphragmatic interposition of the colon or small intestine. Most patients with this condition are asymptomatic throughout their lives. However, affected patients can rarely develop abdominal pain, distention, vomiting, anorexia, constipation and obstruction. The present report describes the case of a patient with Chilaiditi syndrome in which heterotopic endometriosis caused a defect in the right diaphragm, resulting in a right diaphragmatic hernia.
A 50-year-old Japanese female presented to our Emergency Department with right upper abdominal pain and nausea. Her past medical history included Chilaiditi syndrome and heterotopic endometriosis, but she had no history of laparotomy. She had experienced repeated ileus over the past decade due to the Chilaiditi syndrome, but each episode responded to conservative treatment. She had developed
13
heterotopic endometriosis during the pre-menopausal period, and had undergone two bullectomy procedures for right catamenial pneumothorax when she was 38 and 39 years old, respectively. A chest and abdominal X-ray revealed intestinal gas in the upper right abdomen, which was a typical finding of Chilaiditi syndrome (Fig. 1). She was diagnosed with ileus due to Chilaiditi syndrome and
Surg Today
was treated conservatively with bowel rest and nasogastric tube decompression. However, her symptoms did not improve, and chest and abdominal computed tomography (CT) was performed. A right diaphragmatic hernia was suspected based on the coronal view of the CT. There was a defect in the top of the right diaphragm, through which the small intestine entered the right thoracic cavity (Fig. 2). A diagnosis of incarceration of a right diaphragmatic hernia was established, and emergency surgery was performed on the fifth day after hospital admission. A 5-cm incision was made at the level of the right eighth intercostal space. A dilated loop of small intestine was seen in the right thoracic cavity, which was strangulated by the 2-cm defect in the diaphragm (Fig. 3). After reducing
Fig. 1 A chest roentgenogram shows bowel gas in the right upper abdomen
Fig. 2 A coronal view of the chest and abdominal computed tomography showed a diaphragmatic defect through which the small intestine entered the right thoracic cavity (arrow)
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Fig. 3 a A dilated loop of small intestine was seen in the right thoracic cavity. b The 2-cm defect in the diaphragm forming the hernia orifice
Surg Today
Fig. 4 A glandular system with fibrosis, chronic inflammation, hemorrhage and congestion was present in the resected diaphragm (arrow) (H&E stain, ×400)
the small intestine into the abdomen, the edge of the diaphragm forming the hernia orifice was biopsied in light of the patient’s past history of heterotopic endometriosis. The diaphragm around the hernia orifice had become atrophied, which allowed the small defect to form. Primary closure of the diaphragm was performed. A median incision was subsequently made in the upper abdomen. Approximately 80 cm of terminal ileum showed obvious ischemic changes. After the ileum was cut and reconstructed, the surgery was completed. A histological examination of the specimen of the diaphragm showed the presence of a small cystiform glandular system with fibrosis, chronic inflammation, hemorrhage and congestion (Fig. 4). These findings indicated that heterotopic endometriosis was present in the diaphragm. Postoperatively, the patient made an uneventful recovery and was discharged on the 15th postoperative day in good condition. Because she had already reached menopause, she did not receive any hormone therapy. On follow-up, she was well and without symptoms.
Discussion A right diaphragmatic hernia is a rare condition. Most are congenital in nature and can induce neonatal respiratory distress [1]. Chest trauma or complications of medical procedures are well-known causes of right diaphragmatic hernia in the adult population [2, 3]. Although heterotopic endometriosis can result in a defect in the right diaphragm, it rarely causes a diaphragmatic hernia, because the liver overlays the defect [4, 5]. However, in patients with Chilaiditi syndrome, a right diaphragmatic hernia can develop because there is an interposition of the colon or small intestine between the liver and right diaphragm.
Therefore, the bowel can enter the thoracic cavity if there is a defect in the diaphragm. In the present case, the simultaneous presence of heterotopic endometriosis and Chilaiditi syndrome allowed the development of a right diaphragmatic hernia. This is first known case of a right diaphragmatic hernia caused by heterotopic endometriosis in a patient with Chilaiditi syndrome. Because the histological examination of the specimen of the diaphragm showed the presence of a small cystiform glandular system (i.e., ectopic endometrial tissue), we were able to prove that heterotopic endometriosis was the cause of the diaphragmatic hernia. In addition, considering that the patient had already reached menopause, and the diaphragm around the hernia orifice had become atrophied, which led to the formation of the small defect, it was concluded that the defect had been made previously by the heterotopic endometriosis, and the bowel had entered the thoracic cavity through the existing defect accidentally. In this case, because the patient had experienced repeated episodes of ileus due to Chilaiditi syndrome, a right diaphragmatic hernia was not initially suspected. Indeed, in the context of a typical sign of Chilaiditi syndrome (i.e., dislocation of the bowel above the liver), the diagnosis of a diaphragmatic hernia was difficult to make by plain X-rays. However, the coronal view of CT accurately visualized the focal defect in the right diaphragm through which the bowel had entered the right thoracic cavity in this case. Therefore, CT is considered to be an excellent modality for diagnosing a diaphragmatic hernia. In summary, this report documents an extremely rare occurrence of right diaphragmatic hernia caused by heterotopic endometriosis in a patient with Chilaiditi syndrome. Clinicians should be aware that patients with a history of heterotopic endometriosis and Chilaiditi syndrome are at risk of developing a right diaphragmatic hernia. Conflict of interest Naoki Haratake and co-authors have no conflicts of interest.
References 1. Newman BM, Afshani E, Karp MP, Jewett TC Jr, Cooney DR. Presentation of congenital diaphragmatic hernia past the neonatal period. Arch Surg. 1986;121:813–6. 2. Hanna WC, Ferri LE, Fata P, Razek T, Mulder DS. The current status of traumatic diaphragmatic injury: lessons learned from 105 patients over 13 years. Ann Thorac Surg. 2008;85:1044–8. 3. Soufi M, Meillat H, Le Treut YP. Right diaphragmatic iatrogenic hernia after laparoscopic fenestration of a liver cyst: report of a case and review of the literature. World J Emerg Surg. 2013;8:2. 4. Chatra PS. Thoracic endometriosis: a case report. J Radiol Case Rep. 2012;6:25–30. 5. Cooper MJ, Russell P, Gallagher PJ. Diaphragmatic endometriosis. Med J Aust. 1999;171:142–3.
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