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Laparoscopic repair of an excessive Morgagni hernia in an adult presenting as upside-down stomach

Highlights • Morgagni hernias represents a rare subtype of diaphragmatic congenital hernias manifesting usually during early childhood.• An innocuous or asymptomatic presentation render diagnosis of Morgagni hernias a challenging procedure.• In our case, an adult female patient presented with a giant Morgagni hernia, initially diagnosed as a paraesophageal hernia.• Early treatment of such cases is advised due to potential fatal complications. Laparoscopic surgery is considered the best approach.


Introduction
Morgagni hernia (MH) is an uncommon type of congenital diaphragmatic hernia, referring to the protrusion of abdominal viscera inside the thoracic cavity, through a defect in the anterior diaphragm, known as foramina of Morgagni [1]. It is a condition diagnosed usually during childhood, with only a small number of cases being observed in the adult population. Herniated abdominal contents usually include the colon, small bowel or omentum, while the absence of symptoms or obscure abdominal presentation in adult patients, often lead to misdiagnosis [1].
In this paper, we present the very uncommon case of a patient suffering from a huge Morgagni hernia, with portions of the stomach, small bowel and omentum protruding inside the right thoracic cavity. A review of the literature is also performed regarding pre-sentation and management of this rare medical condition. This work has been reported in line with the SCARE criteria [2].

Case presentation
A 68-year-old female patient was admitted to our department after progressive epigastric discomfort, along with nausea, gastric reflux and episodes of vomiting for the past four months. Her medical history included diabetes mellitus and no previous abdominal surgery. Her vital signs were normal, while physical examination was insignificant, with a mild discomfort during epigastric palpation but no abdominal pain.
Laboratory examinations where within normal limits, with a hematocrit of 39.7%, and a hemoglobin of 12.4 mg/dL. Chest radiograph uncovered the presence of a large diaphragmatic hernia, with gastric portions protruding inside the right thoracic cavity. A further CT scan initially revealed a massive paraoesophageal hiatal hernia, with part of the stomach body, antrum and duodenum along with part of the greater omentum protruding inside the right thoracic cavity (Fig. 1). After written consent, patient admitted to   surgery, for laparoscopic repair of an excessive paraoesophageal hiatal hernia.
Upon entering the abdominal cavity, a huge anterior diaphragmatic defect was identified, with part of the greater omentum, small bowel loops, and gastric portions protruding through it inside the right thoracic cavity. No adhesions were encountered, so manipulation of the hernia's sac contents and their reduction back in the abdominal cavity was easily achieved through laparoscopy. Excision of the sac was next performed, with a tension-free closure of the diaphragmatic defect and reinforcement of the diaphragmatic wall with the fixation of an intraperitoneal non-absorbable mesh. The esophageal hiatus along with both right and left diaphragmatic crura and esophagogastric junction were identified in their normal anatomical position, so no further antireflux operation (360 • or partial) was performed (Fig. 2).
Post-surgical period was uneventful for the patient. She resumed normal diet on the 2nd post-operative day and was discharged on the 4th. During 6-month follow up she reported no further symptoms related to gastroesophageal reflux, while a plain chest x-ray revealed no malposition of the abdominal viscera.

Discussion
Morgagni hernia is defined as the protrusion of abdominal viscera inside the thoracic cavity through an anterior defect of the diaphragm known as foramina of Morgagni. It represents an uncommon type of congenital diaphragmatic hernias (CDH), manifesting in less than 5% of such cases [1]. Formation of the defect is presumed to take place during gestation, when the diaphragm fails to normally close. Herniated abdominal contents usually include parts of omentum, stomach, colon and small bowel, while herniation takes place in the right hemithorax in up to 90% of cases [1,3]. Cardiorespiratory distress and gastrointestinal symptoms often lead to the diagnosis and treatment of MH in early childhood, leaving a notion that cases of MHs in adult population present either asymptomatic or manifesting as a mild abdominal or retrosternal discomfort [4]. In a large analysis regarding Morgagni hernias in adult population involving a total of 298 patients, Horton et al. found that 72% of adult patients presented with hernia related symptoms during time of diagnosis, with pulmonary specific symptoms being observed in 36% of these patients [1]. Other reported cases of MH in adult patients are also related with pulmonary or gastric symptoms during initial diagnosis. A few cases of reported MH describe patients with acute abdominal symptoms due to gastric obstruction [5][6][7].
Paraesophageal hernias are classified into three types, according to the anatomic dislocation of the esophagogastric junction and parts of the stomach, in relation to the diaphragm [8]. Type I refers to the esophagogastric junction being above the diaphragm. In type II hernias, the esophagogastric junction is normally positioned below the diaphragm, but part of the stomach protrudes intrathoracically through the diaphragmatic crura, while in type III both the esophagogastric junction and stomach segments slide through the diaphragm inside the thoracic cavity. In rare cases of hiatal herniation, the entire stomach along with bowel loops may protrude through a diaphragmatic defect inside the posterior mediastinum, a condition known as 'upside down' stomach. Rotation of the stomach may also be observed, resulting in the reversal of greater and lesser curvatures [8].
This innocuous and vague clinical manifestation of both MH and paraesophageal hernias often leads to misdiagnosis. Arora et al. [3], reported a rare case of left sided strangulated Morgagni hernia with bowel necrosis, which was initially diagnosed as pneumonia, while in another case, Kim et al. reported the presence of a MH manifesting as chronic dyspnea in an elderly patient [5]. In addition, the controversial manifestation of MH in adult population has led to the conclusion by some authors, that MH is mostly an acquired condition rather than a true congenital hernia [5]. An increase in abdominal pressure seems to be the leading point regarding intrathoracic herniation of abdominal viscera, while it is also suggested in the medical literature that an additional hereditary predisposition in weakening of the anterior part of the diaphragm perhaps plays a role when formation of a Morgagni hernia takes place in an adult [5,9].
In our case, the presence of an upside-down stomach falsely led to the initial diagnosis of a paraoesophageal hernia, while intraoperatively it was identified that the abdominal contents were protruding inside the right thoracic cavity through an anteriorsided Morgagni hernia, making for a pretty unusual case.
Plain chest radiograph along with computed tomography play an important role in the diagnosis, identification of the hernia's contents and further management of this condition [8].
In order to avoid complications such as gastric obstruction, incarceration or gangrene, most authors agree on a prompt repair of MHs, even on asymptomatic patients [4,10]. Trans-thoracic or trans-abdominal open surgery was initially considered the best approach for surgical management of diaphragm related hernias, although a paradigm shift from open surgery to minimal invasive laparoscopic techniques has evolved in the 21st century, reducing intraoperative morbidity, postoperative complications and hospital stay [11,12].

Conclusion
Summarizing, diagnosis of MH requires a high degree of clinical suspicion, due to its innocuous and vague presentation. Prompt surgical repair is advised, and although only a small number of reported MH cases have been repaired through laparoscopy, this technique is considered the best approach in the hands of an experienced surgeon.