doi:10.1111/j.1447-0756.2010.01451.

J. Obstet. Gynaecol. Res. Vol. 37, No. 7: 709714, July 2011

Diaphragmatic hernia during pregnancy: A case report with a review of the literature from the past 50 years
jog_1451 709..714

Yue Chen, Qiannan Hou, Zhu Zhang, Jian Zhang and Mingrong Xi
West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China

Abstract
Diaphragmatic hernia is a rare complication during pregnancy. Only 30 reports have been published on this subject in English between 1959 and 2009. Due to misdiagnoses and management delays, diaphragmatic hernia usually presents itself as a life-threatening emergency. Here, we present a case report of a patient with a traumatic diaphragmatic hernia who became acutely symptomatic during pregnancy. The diaphragmatic hernia was managed successfully, and we describe the presentation, management and outcome of this case. We also present a review of all of the reported cases of diaphragmatic hernias complicating pregnancy that have been published in English during the past 50 years. Key words: complication of pregnancy, diagnosis, diaphragmatic hernia, treatment.

Introduction
A diaphragmatic hernia (DH) is a condition that occurs when the abdominal viscera shifts into the thoracic cavity. It is classied into three categories: congenital, acquired and traumatic. DH is a rare complication during pregnancy, which is associated with poor or complex outcomes, particularly when early surgical interventions are not undertaken. The clinical presentation of a DH during pregnancy can range from being completely asymptomatic throughout pregnancy to producing acute intestinal obstruction during any trimester. These variations in the clinical presentation of a DH during pregnancy lead to challenges in diagnosing this condition. Generally, the preferred technique for diagnosis is plain radiography, even during pregnancy. Surgery is often recommended during the second trimester, when fetal organogenesis is complete and the fetus is relatively stable.13 Pregnant women with an asymptomatic DH in their third trimester should be closely monitored and treated conservatively until the fetus reaches maturity. Then an elective cesarean section may be

performed. In symptomatic cases, immediate repair should be undertaken regardless of fetal status. Here, we describe a patient at 23+3 weeks gestation with a traumatic DH. Following the case report, we present a review of all of the reported cases of diaphragmatic hernias complicating pregnancy published in English between 1959 and 2009.

Case Report
A 26-year-old woman (gravida 4, para 0) at 23+3 weeks gestation presented with a four-day history of persistent abdominal pain at the umbilical and epigastric regions of the abdomen after eating. Laboratory tests performed at a local hospital showed normal hemodiastase and mildly elevated leukocyte and neutrophil levels. An abdominal B-ultrasound showed that the intestinal canal was engorged, dilated and aperistaltic. After three days of anti-inammatory therapy, the patient was transferred from her local hospital to our hospital. She reported a persistent, vague pain radiating to her left shoulder. The patient reported that she underwent laparoscopic surgery for pelvic

Received: January 24 2009. Accepted: August 10 2010. Reprint request to: Dr Mingrong Xi, Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China. Email: qmrjzz@126.com These authors contributed equally to the work.

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inammatory disease because of secondary infertility two years previously and was informed following surgery that an old DH was present. Upon further review, she recalled that she was in a car accident when she was seven years old, which had resulted in a pelvic fracture and abdominal injuries. She received conservative treatment for her injury, and since the accident she had experienced slight abdominal pain radiating to her left shoulder. Upon physical examination, the patient showed stable vital signs. Her abdomen was elevated with high tension and she experienced tenderness over the epigastrium. We observed weak bowel sounds and doubtful signs of shifting dullness by percussion. The fetal heart rate was 145 beats per minute. No signs or symptoms of dyspnea or miscarriage were observed. The laboratory tests measured 11.6 109/L white blood cells (WBC), neutrophils 88.9%, 64 U/L blood amylase and 55 U/L blood lipase. The abdominal ultrasound was normal except for the presence of intestinal tympanites. Enhancement computed tomography (CT) revealed that the stomach was dilated and a portion of the small intestinal loops was located within the chest. Intestinal obstruction was also visible (Fig. 1). Due to concerns that the patient had a DH with gastric and intestinal obstruction, an emergency exploratory thoracotomy and laparotomy were performed. The exploratory procedures indicated that more than half of the stomach, spleen, transverse colon and part of the small intestine were trapped in the left hemithorax through a 6 8 cm laceration in the patients diaphragm. Additionally, adhesions were present on the diaphragm. The small intestine was clearly dilated with gas and uid. During surgery, the viscera were gently returned to the abdomen and the laceration in the diaphragm was repaired. Any manipulation toward the uterus was carefully avoided. Gastrointestinal decompression and thoracic close drainage were performed at the end of the surgery. Following surgery, the patient was transferred to the ICU for further monitoring. Three days later, the patient complained of weak, irregular uterine contractions with a bloody vaginal discharge. The fetal movements were still present. Upon obstetric examination, there were no audible fetal heart beats. Uterine contractions could not be felt because of the high abdominal tension. The patients cervical effacement was approximately 50% without dilatation. The fetal presentation remained in S-1. Unfortunately, several hours later, an ultrasound examination demonstrated fetal death. Five days later, vaginal delivery was accomplished at 24+3 weeks gestation in the hospital. The

Figure 1 Three-dimensional reconstruction of the computed tomography data shows the herniated stomach and intestine in the left hemithorax. The heart and trachea are pushed to the right side of the thorax.

patients postpartum course was smooth and she was discharged from the hospital 14 days later (Fig. 2).

Review and Discussion

In 1824, Ashley Cooper divided diaphragmatic hernias into three categories: (i) congenital, which are due to defects in the diaphragm arising from faulty embryologic development; (ii) acquired, which develop at points of anatomical weakness (e.g. at the esophageal hiatus, or the aortic or caval openings); and (iii) traumatic, which are caused by rents in the diaphragm arising from direct or indirect trauma.4 They are rare complications during pregnancy; from 1959 to 2009, only 30 cases of such hernias during pregnancy were reported in English. Nine of these patients had congenital hernias and two of these nine cases had

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Table 1 Clinical features of 30 reviewed cases of diaphragmatic hernia (DH) complicating pregnancy1528 Mean age (years) Gestational age when DH was identied Second trimester Third trimester Postpartum Mean (weeks) Gravida and para Primigravida Multiple pregnancy without delivery Multipara Positive traumatic history Congenital diaphragmatic hernia Cryptogenic Patient symptoms Vomiting Abdominal pain Dyspnea Shoulder pain Cardiopalmus Chest pain Operation type Laparotomy Thoracotomy Thoracolaparotomy Laparoscopy Delivery method Vaginal delivery after repair Simultaneously with repair Operation after delivery Cesarean section after repair Herniated organ Colon Stomach Intestine Omentum Spleen Liver Location of DH orice Left hemidiaphragm Right hemidiaphragm Mortality Child Mother 27.4 11 (37%) 15 (50%) 4 (13%) 27 10 (36%) 18 (64%) 9 (32%) 12 (40%) 9 (30%) 9 (30%) 18 (60%) 17 (57%) 17 (57%) 9 (30%) 7 (23%) 4 (13%) 14 (48%) 8 (28%) 6 (21%) 1 (3%) 10 (37%) 6 (22%) 6 (22%) 5 (19%) 23 (77%) 17 (57%) 9 (30%) 7 (23%) 4 (13%) 2 (7%) 26 (87%) 4 (13%) 4 (13%) 3 (10%)

Figure 2 Chest X-ray illustrating the patients recovery one week after surgery.

received hernia repair surgery as infants. Twelve cases had traumatic hernias (83% were caused by left chest punctures), but the hernia type was unknown for the other nine cases. Our patient would be classied in the traumatic category due to her history. Of the reviewed cases, no patient complained of symptoms before pregnancy, since their diaphragm defects were too small to cause clinical signs. The patients sought treatment when their symptoms appeared during gestation or postpartum. During gestation, the gradually enlarging gravid uterus increases the abdominal pressure, the increasing progestogen levels leads to diaphragmatic muscle relaxation and ligament suppleness.5 During delivery, the sharp rise in abdominal pressure pushes the diaphragm up, while the initial downward force and diaphragmatic muscle contractions push the diaphragm down. These two opposite forces can tear the diaphragm at its weakest points, causing hernias in patients with diaphragmatic defects. The clinical presentation of maternal hernias during pregnancy varies widely. The symptoms range from acute or chronic upper gastrointestinal pain, vomiting, dyspnea and chest pain to life-threatening complica-

tions, such as obstruction, strangulation, ischemia or necrosis of herniated viscera with cardiovascularrespiratory decompensation.3,6,7 Based on our review, the most common symptoms were vomiting (60%), abdominal pain (57%), dyspnea (57%), radiating shoulder pain (30%), cardiopalmus (23%), thoracalgia (13%) and hypotension (10%) (Table 1). Of the 30 reviewed cases, 15 (50%) were misdiagnosed because of atypical symptoms and the lack of chest radiographs. In traumatic DH cases, such as our patient, it is also necessary to record a detailed medical history.

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The three most common clinical manifestations of DH are: Nausea and vomiting Abdominal pain Dyspnea.

Nausea and vomiting While the majority of women (85%) suffer from nausea and vomiting during pregnancy, these symptoms are usually mild and self-limiting, ending before the 12th week of gestation. Only 0.31.0% of pregnant women develop hyperemesis gravidarum, which is often confused with early incarcerated maternal DH.8 In the 30 reviewed cases, most of the DH patients who experienced nausea showed these symptoms during their third trimester. Along with vomiting, other signs of obstruction or compression may be present simultaneously, such as a colicky abdominal pain and dyspnea. Abdominal pain To diagnose a DH, it is important to characterize the patients abdominal pain in terms of the location, quality, intensity, radiation patterns, exacerbating and alleviating factors, and the changes in these characteristics with movement. The abdominal pain associated with an obstructive DH may be mild or severe, and either persistent or intermittent. Generally, the pain is located in the left upper quadrant at the defect location. For a correct diagnosis, the abdominal pain associated with the condition must be differentiated from abdominal pain caused by other diseases, such as pancreatitis, appendicitis, and renal and biliary colic. Patients with acute pancreatitis often suffer from boring, midline pains, which radiate to the back and are accompanied by vomiting and fever. Pancreatitis can be diagnosed or ruled out by performing serum amylase measurements. In pregnant patients with appendicitis, the abdominal pain is located in the lower right quadrant in approximately 82.2% of patients.9 Leukocyte counts are typically a useful marker for inammatory diseases; however, leukocyte levels are also elevated during pregnancy. Renal and biliary colic can also produce waxing and waning pain intensities; for instance, the abdominal pain associated with urolithiasis radiates from the back or abdomen to the groin.10 Dyspnea Dyspnea is a common symptom during pregnancy that can be caused by many diseases, including pneumonia,

severe pleurisy, asthma, amniotic embolism and heart disease. Pneumonia and pleurisy patients usually exhibit a fever and elevated leukocyte level. The possibility of asthma must be considered when a young pregnant woman presents with serious paroxysmal dyspnea. Amniotic embolism may be the rst consideration when a postpartum woman exhibits respiratory distress. According to our literature review, 50% (2/4) of the postpartum women with DH displayed respiratory distress. In these cases, a complete physical examination of the chest should be performed, including inspection, palpation, percussion and auscultation. If an abnormal respiratory murmur is observed and there is normal thrombin function, then chest radiography is recommended. To establish a diagnosis of DH, various auxiliary examinations may by used, such as plain thoraco-abdominal radiography, uoroscopy, thoracoabdominal ultrasound, barium studies, thoracoabdominal CT and magnetic resonance imaging (MRI). For pregnant women, however, it is important to select the most appropriate and least invasive imaging technique. Plain radiography is usually the preferred technique to diagnose DH, but for pregnant patients, there is a reluctance to expose the unborn child to the dangers of radiation. This can lead to difculties in diagnosing DH, as in our case. The low radiation exposure from diagnostic procedures (0.12 mSv) during pregnancy typically does not increase the risk of congenital anomalies.11 Although CT and MRI provide a better view of the abdominal organs and diaphragmatic defect, the radiation dosage of CT is much higher than that of plain radiography, and MRI is believed to affect fetal development. Moreover, the cost of MRI is an obstacle, especially in developing countries. Since most DH misdiagnoses result from misreading plain radiographs, we present a summary of how to correctly interpret the imaging ndings. (i) If the predominant bowel is herniated, then thin-walled translucencies that look like bubbles will be present. The diagnostic clues are connecting bowel segments between the abdominal and herniated bowel loops, which pass through the diaphragmatic defect. (ii) When the spleen and/or the omentum are herniated, the presence of the pneumonic consolidation (a mass lesion with small translucencies) can be confused with a pneumatocele. Fever, cough and related laboratory changes can be used to differentiate pneumonia from pneumatocele or loculated pyopneumothorax. (iii) If the stomach is herniated and lled with gas, then a large air-lled translucency will appear on the

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radiograph. The translucency may simulate a pneumothorax or a congenital cystic lesion of the lung. If the stomach is lled with uid, then the imaging may show a large opacication containing a large translucency.12 A feeding tube and contrast materials can be applied to obtain the correct diagnosis. Ultrasound can serve as a complementary technique in determining the hernial and pleural effusion contents. CT and MRI may be used to observe the defect and the organs inside the hernia. Operative repair is necessary in cases of diaphragmatic disruption. Operative repair approaches include abdominal, thoracic and combined methods. An abdominal approach facilitates the resection and repair of the affected organs, and a thoracic approach facilitates access to the defect for repair.13 Treating pregnant patients with DH is always challenging, since selecting the appropriate treatment depends on the clinical presentation and the gestational age. If a hernia is detected in the rst or second trimesters, then it should be repaired during the second trimester when organogenesis is complete. This timing will prevent progressive intrathoracic displacement of the viscera following the enlargement of the uterus, which can result in strangulation.4 During the later stages of pregnancy, the viscera are relocated from their proper domain, so operative closure may be extremely difcult.4 Diaphragmatic contraction and movement during labor may further enlarge the defect. If an asymptomatic DH is detected during the third trimester, the patient should be closely monitored and conservatively treated as long as the maternal and fetal status is stable. For patients with hernia symptoms, who show no signs of strangulation, conservative management should be selected, which involves close monitoring, nasogastric suction and uid infusion. Once the fetus is mature, the baby should be delivered by Caesarean section and the hernia should be repaired during the procedure. When signs of obstruction or strangulation are apparent, regardless of gestational age, surgical treatment should be performed immediately to avoid fetal and maternal mortality, which can be as high as 3550%.4,14 The leading causes of death are hypoxia and acidosis, which may result from respiratory distress, gastrointestinal perforation or cardiovascular shock.15 Gastric decompression may improve the clinical condition of the patient, allowing surgery to be delayed until antenatal corticosteroids are administered.14 Women with a repaired DH can undergo labor and deliver vaginally because the uterine contractions

alone do not increase the abdominal pressure and are unlikely to break the repaired hernia.14 In these cases, the fetal heart rate, maternal blood pressure and oxygenation, and other symptoms of precipitous labor should be closely monitored during labor. During the second stage of labor, obstetric forceps and a vacuum extractor can be applied to prevent the patient from bearing down and increasing her intra-abdominal pressure.14 A diaphragmatic hernia is a rare and severe condition during pregnancy that often leads to maternal and fetal deaths. In the reviewed literature, a DH has led to maternal deaths in 10.0% (3/30) of the reports, and to fetal deaths in 13.3% (4/30) of the cases. In our patient, the DH was detected two years previously during laparoscopic surgery. Regretfully, both the obstetrician/ gynecologist and the patient herself did not recognize the potential problems associated with the DH during pregnancy, which ultimately led to fetal death. In conclusion, once a DH is identied, repair surgery should be performed immediately, even if there are no symptoms present at the time. For patients who desire fertility, it is essential that the hernia be repaired prior to pregnancy to avoid its possible rupture during gestation.

Acknowledgments
We thank Ting Lai and Minmin Hou (West China Second University Hospital) for their critical reading of this manuscript. This work was supported by the West China First University Hospital.

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