Surg Today (2005) 35:149–152DOI 10.1007/s00595-004-2875-5

Congenital Diaphragmatic Hernia Complicated by TensionGastrothorax 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

ever, the diagnosis may be delayed beyond the neonatalperiod in some cases.1 We report our experience oftreating a 77-year-old woman with a CDH who sufferedsevere cardiorespiratory distress during an uppergastrointestinal endoscopy, caused by a massivegastrothorax.

Case Report

A 77-year old woman presented to our hospital with a15-day history of upper abdominal discomfort and occa-sional nausea, which had become worse during the pre-ceding 24h. She reported having experienced similarsymptoms in the past, which had been attributed togastritis, but not specifically investigated. Her medi-cal history was otherwise unremarkable. Chest X-ray(Fig. 1) showed a high left hemidiaphragm, but her bio-chemistry and hematology profile were within the nor-mal range. An upper gastrointestinal endoscopy wasarranged for the following day because of the natureand severity of her epigastric symptoms.

The endoscope was passed under direct vision andviews of the upper esophagus were normal. The instru-ment was advanced into the lower esophagus but exces-sive insufflation of air was necessary to negotiate theacute bend in the gastroesophageal junction. Steeringthe instrument into the stomach proved challenging.The distance between the stomach entry and the pyloricring was obviously abnormal and shortened. We aban-doned the endoscopic procedure at this point and re-moved the scope because the patient had suddenlybecome acutely distressed, with inspiratory stridor, cy-anosis, and signs of shock. The urgent withdrawal of theendoscope precluded any systematic attempt to deflatethe stomach. Faced by a rapidly deteriorating clinicalstate with severe hypotension (blood pressure 60/40mmHg) and abnormal blood gases (SO2 89%; PO2

57mmHg; PCO2 72 mmHg), a decision was made to

Reprint requests to: A. Ninos, 18 Levidiou Str., 131.21 Ilion,Athens, GreeceReceived: October 15, 2002 / Accepted: November 4, 2003

AbstractBochdalek’s hernia typically manifests early inchildhood, but it is uncommon in adults. We report thecase of a 77-year-old woman who suffered suddenacute cardiorespiratory distress during an upper gastro-intestinal endoscopy, caused by a massive gastrothorax,confirmed by a chest radiograph and thoracic computedtomography. Laparotomy revealed a posterolateral dia-phragmatic defect and an acutely distended stomachoccupying the left hemithorax, caused by an incompleteorganoaxial volvulus of the organ. The stomach wasreduced and secured inside the abdominal cavity bya Nissen fundoplication and the defect was repairedwith interrupted sutures. Unfortunately, the patientsuccumbed to generalized sepsis resulting fromPneumocystis carinii bilateral pneumonia. To ourknowledge, this is the first reported case in the Englishliterature of an adult with a congenital posterolateralhernia presenting in this manner. However, it demon-strates that a symptomatic congenital diaphragmatichernia should be surgically repaired to prevent the nu-merous complications that may arise, many of whichcan 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 mani-fests in the early neonatal period, with life-threateningcomplications occurring in the first hours of life. How-

150 A. Ninos et al.: Gastrothorax Complicating Endoscopy

intubate and ventilate. We resuscitated the patient withcopious intravenous fluids and inotrope support. Hervital signs gradually stabilized and the blood gases nor-malized. Chest X-ray and a thoracic computed tomogra-phy (CT) scan (Fig. 2) showed a gastrothorax and alarge left-sided diaphragmatic defect. The dilated stom-ach seemed to distort and compress both the heart andlungs, and extended into almost the entire chest cavity.

Repeated attempts to insert a nasogastric tube wereunsuccessful. The patient remained ventilated and wasoperated on a few hours later.

Laparotomy was done through a midline incision.The intra-abdominal organs were normal, but a largeposterolateral diaphragmatic defect was found, throughwhich almost all of the stomach herniated into the lefthemithorax (Fig. 3a,b). The stomach was distended andfixed by multiple adhesions, and was herniating intra-thoracically. There was a partial gastric organoaxial vol-vulus. Adhesion-lysis was done; then the organ wasdeflated, reduced, and secured in its intra-abdominal

Fig. 1. Chest radiograph showing a high-sited lefthemidiaphragm

Fig. 2. Computed tomography scan showing the herniatedstomach compressing and distorting the endothoracicstructures

Fig. 3. a Intraoperative photograph showing a large posterolateral diaphragmatic defect, through which the stomach herniatedinto the hemithorax. b Graphic representation of the intraoperative photograph, depicting the defect in black

a b

151A. Ninos et al.: Gastrothorax Complicating Endoscopy

position. The procedure was completed with a Nissenfundoplication and the diaphragmatic defect wasrepaired with interrupted nonabsorbable sutures.

The immediate postoperative recovery was unevent-ful and the patient was transferred to the intensive careunit. However, she became pyrexic (39°C) on the sec-ond postoperative day and chest radiographs showedbilateral pneumonia. Bronchoalveolar lavage confirmeda Pneumocystis carinii infection. The patient remainedintubated for the following 3 days and was treated withintravenous trimethoprim and sulfamethoxazole atdoses of 20 and 100mg/kg, respectively. Despite thistreatment, her cardiorespiratory status gradually dete-riorated and she finally succumbed to the uncontrol-lable 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 thepleuroperitoneal membrane and the transverse septum.Failure of this process results in a continuousperitoneopleural cavity along the posterior abdominalwall. This posterolateral diaphragmatic or Bochdalekdefect allows the intra-abdominal viscera to enter thethoracic cavity.2 Most of the reported cases involvedthe left side. In our patient, the defect included thelumbodorsal space of the diaphragm and extended an-teriorly, affecting nearly half of the hemidiaphragmaticspace. The posterolateral location of the defect and thelack of any history of trauma helped us to differentiatethis type of hernia from a paraesophageal hiatal herniawhich is much more common in her age group. More-over, there was no distinct sac, consistent with the factthat this element is found in only 10%–15% of patientswith a CDH.3

Congenital diaphragmatic hernias that present duringthe perinatal period are usually associated with somedegree of pulmonary hypoplasia. Few of the 3%–30%of CDHs that present after the newborn period areassociated with any form of pulmonary defect.4 Most ofthese patients have either an asymptomatic herniationor a defect without any herniated viscera. However, anincidental rise in intra-abdominal pressure for any rea-son causes the herniation that elicits the symptoms.5

According to the existing literature, delayed symptomsare primarily cardiorespiratory in infants and youngchildren, and gastrointestinal in older children.6

Investigation usually includes chest radiography, con-trast studies, CT, and upper gastrointestinal endo-scopy.7 Our patient complained of a long history ofepigastric discomfort that had recently become worse.This presentation convinced us to perform gastroscopyas the first-line diagnostic procedure. Computed tomo-

graphy would have followed to further investigate thehigh-sited left hemidiaphragm.

Numerous complications of a delayed CDH havebeen reported, including small or large bowel obstruc-tion and strangulation, acute appendicitis with malrota-tion, splenic torsion, gastric volvulus and perforation,acute pneumothorax, and gastrothorax.8–11 A postero-lateral congenital diaphragmatic defect may becomesymptomatic later in life,12–14 causing abdominal or re-spiratory symptoms. However, to our knowledge therehave been no other reported cases of adults sufferingcardiorespiratory collapse caused by a gastrothorax pre-cipitated by a left congenital diaphragmatic defect.

Diaphragmatic defects can be operated on by eitheran open or a laparoscopic approach.15,16 A primary re-pair is usually attempted first, but in as many as 53% ofcases, primary closure is impossible because of the sizeof the defect, and a mesh repair or autologous flap isnecessary.

Although a subject of debate,17 gastroesophageal re-flux (GER) is very common after CDH repair,18 with areported incidence of up to 62%.19 It has been suggestedthat plication of the previously paralyzed diaphragmreinforces the GER driving forces, while weakening theantireflux barrier;20 therefore, an additional procedureto prevent the lower esophagus from sliding might beindicated.21 Although there is a limited amount of dataon the treatment of adults with CDH, we performed aNissen fundoplication with diaphragmatic repair for ourpatient, based on our relative evidence with neonates.

Our patient may have suffered from short occasionalepisodes of endothoracic gastric dilatation, partial vol-vulus, or both, which resolved spontaneously. The finalepisode of cardiorespiratory collapse, which occurredduring the gastroscopy session, was triggered by theinadvertent overinflation of the stomach. The gastricoverdilatation that occurred intrathoracically restrictedher cardiorespiratory function. This mechanism ofcardiorespiratory compromise is similar to that whichoccurs in patients with tension pneumothorax.Although most adults with CDH present with morechronic abdominal symptoms, a case of acute respira-tory failure occurring in a patient with right-sided adultBochdalek hernia has been reported.22

Unfortunately, our patient succumbed to an opportu-nistic infection, well known to affect AIDS patients andother immunocompromised individuals. Consideringthat the patient had no history of an immuno-suppressive element, we speculate that the P. cariniipneumonia occurred in the context of a disturbed im-mune status caused by the endoscopic complications,extended surgery, and her need for intensiveinterventional care.

In conclusion, a high index of suspicion is needed todiagnose CDH after the newborn period. A high-sited

152 A. Ninos et al.: Gastrothorax Complicating Endoscopy

hemidiaphragm may indicate a congenital diaphrag-matic defect, in which case endoscopy should not beperformed. Symptomatic CDH should be surgically re-paired to prevent the numerous complications that mayarise, many of which can prove fatal, even in an adult.

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