with a CDH.3. Congenital diaphragmatic hernias that present during the perinatal period are usually associated with some degree of pulmonary hypoplasia.
Surg Today (2005) 35:149–152 DOI 10.1007/s00595-004-2875-5
Congenital Diaphragmatic Hernia Complicated by Tension Gastrothorax During Gastroscopy: Report of a Case Anestis Ninos1, Evagelos Felekouras1, Gerasimos Douridas1, Enkel Ajazi1, Anna Manataki2, Stefanos Pierrakakis1, and Nikolaos Setakis1 Departments of 1 Surgery and 2 Radiology, Thriassion General Hospital, Athens, Greece
Abstract Bochdalek’s hernia typically manifests early in childhood, but it is uncommon in adults. We report the case of a 77-year-old woman who suffered sudden acute cardiorespiratory distress during an upper gastrointestinal endoscopy, caused by a massive gastrothorax, confirmed by a chest radiograph and thoracic computed tomography. Laparotomy revealed a posterolateral diaphragmatic defect and an acutely distended stomach occupying the left hemithorax, caused by an incomplete organoaxial volvulus of the organ. The stomach was reduced and secured inside the abdominal cavity by a Nissen fundoplication and the defect was repaired with interrupted sutures. Unfortunately, the patient succumbed to generalized sepsis resulting from Pneumocystis carinii bilateral pneumonia. To our knowledge, this is the first reported case in the English literature of an adult with a congenital posterolateral hernia presenting in this manner. However, it demonstrates that a symptomatic congenital diaphragmatic hernia should be surgically repaired to prevent the numerous complications that may arise, many of which can prove fatal even in an adult. Key words Congenital diaphragmatic hernia · Bochdalek hernia · Acute tension gastrothorax · Pneumocystis carinii pneumonia
Introduction Congenital diaphragmatic hernia (CDH) usually manifests in the early neonatal period, with life-threatening complications occurring in the first hours of life. HowReprint requests to: A. Ninos, 18 Levidiou Str., 131.21 Ilion, Athens, Greece Received: October 15, 2002 / Accepted: November 4, 2003
ever, the diagnosis may be delayed beyond the neonatal period in some cases.1 We report our experience of treating a 77-year-old woman with a CDH who suffered severe cardiorespiratory distress during an upper gastrointestinal endoscopy, caused by a massive gastrothorax.
Case Report A 77-year old woman presented to our hospital with a 15-day history of upper abdominal discomfort and occasional nausea, which had become worse during the preceding 24 h. She reported having experienced similar symptoms in the past, which had been attributed to gastritis, but not specifically investigated. Her medical history was otherwise unremarkable. Chest X-ray (Fig. 1) showed a high left hemidiaphragm, but her biochemistry and hematology profile were within the normal range. An upper gastrointestinal endoscopy was arranged for the following day because of the nature and severity of her epigastric symptoms. The endoscope was passed under direct vision and views of the upper esophagus were normal. The instrument was advanced into the lower esophagus but excessive insufflation of air was necessary to negotiate the acute bend in the gastroesophageal junction. Steering the instrument into the stomach proved challenging. The distance between the stomach entry and the pyloric ring was obviously abnormal and shortened. We abandoned the endoscopic procedure at this point and removed the scope because the patient had suddenly become acutely distressed, with inspiratory stridor, cyanosis, and signs of shock. The urgent withdrawal of the endoscope precluded any systematic attempt to deflate the stomach. Faced by a rapidly deteriorating clinical state with severe hypotension (blood pressure 60/ 40 mmHg) and abnormal blood gases (SO2 89%; PO2 57 mmHg; PCO2 72 mmHg), a decision was made to
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intubate and ventilate. We resuscitated the patient with copious intravenous fluids and inotrope support. Her vital signs gradually stabilized and the blood gases normalized. Chest X-ray and a thoracic computed tomography (CT) scan (Fig. 2) showed a gastrothorax and a large left-sided diaphragmatic defect. The dilated stomach seemed to distort and compress both the heart and lungs, and extended into almost the entire chest cavity.
Fig. 1. Chest radiograph hemidiaphragm
showing
a
high-sited
left
Repeated attempts to insert a nasogastric tube were unsuccessful. The patient remained ventilated and was operated on a few hours later. Laparotomy was done through a midline incision. The intra-abdominal organs were normal, but a large posterolateral diaphragmatic defect was found, through which almost all of the stomach herniated into the left hemithorax (Fig. 3a,b). The stomach was distended and fixed by multiple adhesions, and was herniating intrathoracically. There was a partial gastric organoaxial volvulus. Adhesion-lysis was done; then the organ was deflated, reduced, and secured in its intra-abdominal
Fig. 2. Computed tomography scan showing the herniated stomach compressing and distorting the endothoracic structures
b
a Fig. 3. a Intraoperative photograph showing a large posterolateral diaphragmatic defect, through which the stomach herniated into the hemithorax. b Graphic representation of the intraoperative photograph, depicting the defect in black
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position. The procedure was completed with a Nissen fundoplication and the diaphragmatic defect was repaired with interrupted nonabsorbable sutures. The immediate postoperative recovery was uneventful and the patient was transferred to the intensive care unit. However, she became pyrexic (39°C) on the second postoperative day and chest radiographs showed bilateral pneumonia. Bronchoalveolar lavage confirmed a Pneumocystis carinii infection. The patient remained intubated for the following 3 days and was treated with intravenous trimethoprim and sulfamethoxazole at doses of 20 and 100 mg/kg, respectively. Despite this treatment, her cardiorespiratory status gradually deteriorated and she finally succumbed to the uncontrollable chest infection on the fourth postoperative day.
Discussion Between the eighth and tenth week of intrauterine life, the diaphragm is normally formed by fusion of the pleuroperitoneal membrane and the transverse septum. Failure of this process results in a continuous peritoneopleural cavity along the posterior abdominal wall. This posterolateral diaphragmatic or Bochdalek defect allows the intra-abdominal viscera to enter the thoracic cavity.2 Most of the reported cases involved the left side. In our patient, the defect included the lumbodorsal space of the diaphragm and extended anteriorly, affecting nearly half of the hemidiaphragmatic space. The posterolateral location of the defect and the lack of any history of trauma helped us to differentiate this type of hernia from a paraesophageal hiatal hernia which is much more common in her age group. Moreover, there was no distinct sac, consistent with the fact that this element is found in only 10%–15% of patients with a CDH.3 Congenital diaphragmatic hernias that present during the perinatal period are usually associated with some degree of pulmonary hypoplasia. Few of the 3%–30% of CDHs that present after the newborn period are associated with any form of pulmonary defect.4 Most of these patients have either an asymptomatic herniation or a defect without any herniated viscera. However, an incidental rise in intra-abdominal pressure for any reason causes the herniation that elicits the symptoms.5 According to the existing literature, delayed symptoms are primarily cardiorespiratory in infants and young children, and gastrointestinal in older children.6 Investigation usually includes chest radiography, contrast studies, CT, and upper gastrointestinal endoscopy.7 Our patient complained of a long history of epigastric discomfort that had recently become worse. This presentation convinced us to perform gastroscopy as the first-line diagnostic procedure. Computed tomo-
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graphy would have followed to further investigate the high-sited left hemidiaphragm. Numerous complications of a delayed CDH have been reported, including small or large bowel obstruction and strangulation, acute appendicitis with malrotation, splenic torsion, gastric volvulus and perforation, acute pneumothorax, and gastrothorax.8–11 A posterolateral congenital diaphragmatic defect may become symptomatic later in life,12–14 causing abdominal or respiratory symptoms. However, to our knowledge there have been no other reported cases of adults suffering cardiorespiratory collapse caused by a gastrothorax precipitated by a left congenital diaphragmatic defect. Diaphragmatic defects can be operated on by either an open or a laparoscopic approach.15,16 A primary repair is usually attempted first, but in as many as 53% of cases, primary closure is impossible because of the size of the defect, and a mesh repair or autologous flap is necessary. Although a subject of debate,17 gastroesophageal reflux (GER) is very common after CDH repair,18 with a reported incidence of up to 62%.19 It has been suggested that plication of the previously paralyzed diaphragm reinforces the GER driving forces, while weakening the antireflux barrier;20 therefore, an additional procedure to prevent the lower esophagus from sliding might be indicated.21 Although there is a limited amount of data on the treatment of adults with CDH, we performed a Nissen fundoplication with diaphragmatic repair for our patient, based on our relative evidence with neonates. Our patient may have suffered from short occasional episodes of endothoracic gastric dilatation, partial volvulus, or both, which resolved spontaneously. The final episode of cardiorespiratory collapse, which occurred during the gastroscopy session, was triggered by the inadvertent overinflation of the stomach. The gastric overdilatation that occurred intrathoracically restricted her cardiorespiratory function. This mechanism of cardiorespiratory compromise is similar to that which occurs in patients with tension pneumothorax. Although most adults with CDH present with more chronic abdominal symptoms, a case of acute respiratory failure occurring in a patient with right-sided adult Bochdalek hernia has been reported.22 Unfortunately, our patient succumbed to an opportunistic infection, well known to affect AIDS patients and other immunocompromised individuals. Considering that the patient had no history of an immunosuppressive element, we speculate that the P. carinii pneumonia occurred in the context of a disturbed immune status caused by the endoscopic complications, extended surgery, and her need for intensive interventional care. In conclusion, a high index of suspicion is needed to diagnose CDH after the newborn period. A high-sited
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hemidiaphragm may indicate a congenital diaphragmatic defect, in which case endoscopy should not be performed. Symptomatic CDH should be surgically repaired to prevent the numerous complications that may arise, many of which can prove fatal, even in an adult.
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