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Case Report 


Abdullah Saeed Alghamdi et al, 2019;3(11):963–966.

International Journal of Medicine in Developing Countries

A report of two pediatric cases of Morgagni Hernia

Abdullah Saeed Alghamdi1, Muhammad Abdulrahman Alzahrani1, Amjad Saeed Alghamdi1, Yasmin Abdulaziz Yousef2, Ahmed Alawi2

Correspondence to: Abdullah Saeed Alghamdi

*College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.

Email: alghamdi1996 [at] hotmail.com

Full list of author information is available at the end of the article.

Received: 14 August 2019 | Accepted: 20 August 2019


ABSTRACT

Background:

Morgagni hernia (MH) is a rare congenital anomaly of the diaphragm. It is a birth defect characterized by herniation of abdominal organs into the thoracic cavity through a retrosternal diaphragmatic defect which inhibits proper lung formation.


Case presentation:

We report two cases of MH in a set of premature twins aged 9 days, who presented with respiratory distress at King Abdulaziz Medical City, Jeddah. The diagnosis was confirmed by imaging of chest X-ray and CT scan. Both cases were successfully managed through abdominal approach, i.e., laparotomy with surgical treatment of the diaphragmatic deformity.


Conclusion:

The exact pathogenesis of most cases of congenital diaphragmatic hernia (CDH) remains unknown; however, there is an increasing evidence indicating that genetic factors play a pivotal role in formation of CDH. Moreover, its occurrence in a set of twins further supports the genetical theory.


Keywords:

Congenital abnormality, diaphragmatic hernia, Morgagni hernia, twins.


Introduction

Morgagni hernia (MH) is the least common type of congenital diaphragmatic hernia (CDH) with estimated incidence of 3%–5% [1]. It is defined as protrusion of abdominal organs into the thoracic cavity through a retrosternal diaphragmatic lesion [2]. This lesion occurs due to failed fusion between the costal and sternal edges of the diaphragm anteriorly in the midline [3]. MH cases may remain asymptomatic till adulthood; however, when pediatric patients present with MH; atypical clinical presentation could include non-specific gastrointestinal, respiratory, or cardiac symptoms. Therefore, diagnosis is usually delayed [2,4]. The treatment of choice is surgical even in patients who show no symptoms because of increased risk of intestinal obstruction strangulation or incarceration. It is highly uncommon for CDH cases to happen in both of twins, and previously only five pairs were found. Thus, the present study reported a case of male twins who presented with Morgagni hernia and were treated surgically [5].


Case Presentation

Preterm male twin infants with gestational age of 32 weeks were born with asymmetrical intrauterine growth retardation. The premature twins aged 9 days, presented with respiratory distress and tachypnea at King Abdulaziz Medical City-Jeddah which required Nasal continuous positive airway pressure support for 1 day and then breathed normally in room air.

The first twin’s cardiovascular examination was normal, but respiratory examination revealed tachypnea with equal bilateral air entry. Oxygen saturation was >98%. Chest X-ray displayed mediastinal mass with opacifying tissue that was in continuation with the abdominal content which lead to suspicion of diaphragmatic hernia or upward displacement of the diaphragm (eventration). Echocardiogram showed displacement of heart upward by echogenic tissue which resembled the texture of liver, with normal cardiac structure and function. CT chest revealed right anteromedial CDH containing left hepatic lobe and part of right hepatic lobe with associated minimal superior displacement of the heart, this led to confirmation of diagnosis of Morgagni hernia. The patient underwent surgery at day 9 through abdominal approach (laparotomy) under general anesthesia. A transverse subcostal incision was done, 2 cm on each side of the linea alba, and the defect was identified anteriorly just behind the sternum. The liver was retracted downward. The falciform ligament was cut and the umbilical vein was ligated. Treatment was achieved by diaphragm’s approximation to the under surface of the ribs and xiphoid process with permanent sutures as seen in (Figure 1). The patient was then shifted to neonatal intensive care unit (NICU) where there was an uneventful recovery except for one attack of hypotension that responded to a normal saline bolus. The patient was discharged on day 18 postoperative and follow up went well.

The second twin’s cardiovascular examination was normal except apex beat which was elevated. Chest examination showed bilateral equal air entry, and respiratory rate was 50–70 breaths/minute and oxygen saturation >95%. Imaging modalities were done to examine structures within the chest. Mediastinal mass was seen on the X-ray, and echocardiogram showed that the heart was displaced upward with normal cardiac structure and function. Diaphragmatic hernia was suspected as in twin 1, and chest CT was done to confirm the diagnosis. It revealed that part of right hepatic lobe, as well as the whole left hepatic lobe were herniating through an anteromedial defect in the right hemi diaphragm. The herniated liver exerted a mass effect; displacing the heart upwards. No herniated bowel was seen. At day 7 the patient was managed with surgery through abdominal approach (laparotomy) under general anesthesia. A transverse subcostal incision was made, and the liver was found herniating to the chest through small hernia sac, and reduction of the liver into the abdomen was easily achieved. The herniated sac was placated, and from the posterior edge of the defect, diaphragm was sutured to the anterior abdominal wall and xiphoid process (Figure 2). The patient was then shifted to the NICU where ventilation was given for a few days. The patient was discharged on day 17 postoperative and follow up went well.

Figure 1. Surgical repair for twin1 by approximation of the diaphragm to the under surface of the ribs and xiphoid process with permanent sutures.


Discussion

CDH has an estimated occurrence of 1 in 2,000 to 5,000 births [4]. The most common type of CDH is through the postero-lateral foramen of Bochdalek; and only 3%–5% of congenital diaphragmatic hernias occur through the anterior foramen of Morgagni. It originates from failed fusion of the pars sternalis and the 7th costochondral arch, which was defined by Giovanni Morgagni as Larrey’s space in 1769, and cause a deformity in septum transversum [6]. The MH sac may include transverse colon, omentum, and rarely liver and stomach are found [7]. In the present report, it contained a part of right hepatic lobe and complete left lobe. About 90% of Morgagni hernias were right sided in a previous study, like the present case, 2% were left sided and 8% bilateral [4]. The presence of left sided MH is uncommon because of barrier formation by precardial sac on the sternocostal trigon [5]. The clinical manifestations of MH in children are variable. Some are asymptomatic, and their hernias are diagnosed incidentally [8]. They may present with vague symptoms, such as recurrent chest infections, respiratory distress, or gastrointestinal discomfort. However, the most common symptom is pulmonary complaint, which is similar to the present patients’ presentation. Aghajanzadeh et al. [9] indicated that 50% of patients have no symptoms, 17% have respiratory symptoms, and the remaining 14% have gastrointestinal symptoms [9]. Children with MH have an increased risk of associated congenital anomalies. A study found that MH is associated with congenital heart disease in 35%, gut malrotation in 22%, Down’s syndrome in 26% [10]. The possibility of the presence of a genetic factor cannot be completely eliminated even though this disease is believed to be because of a developmental accident.

Figure 2. Surgical repair for twin2 in which the diaphragm was sutured from the posterior edge of the defect to the anterior abdominal wall and xiphoid process.

Passarge recorded unilateral diaphragmatic deformity cases in seven pairs of siblings, and he proposed that a rare recessive gene could be attributed for this condition [11]. Researchers have also reported cases that are similar to Passarge [12]. The diagnosis of MH can be confirmed by revealing retrosternal herniation of bowel loops using a chest X-ray from lateral view. However, it showed mediational mass with opacifying tissue in continuation with abdominal content. Pneumonia enlarged pericardial fat pads, atelectasis, diaphragmatic eventration, pleuropericardial cyst, and mediastinal lipoma are included in differential diagnosis of MH. More importantly, the diagnosis of MH cannot be excluded by a previous normal radiography because sometimes the hernia sac lacks content. Occasionally, the hernia sac may contain a part of the omentum or liver, which might be confused with mass of chest in radiographical studies. In situations like these, useful information can be provided by other radiology modalities, such as ultrasonography, MRI, and CT. However, CT is considered as the gold standard modality in diagnosing and assessing MH and other diaphragmatic deformities [13]. Management of MH is by surgical repair, which can be achieved by suturing diaphragm edge to retrosternal and retro-costal peritoneum and periosteum along its transverse axis [8]. Approximately 50% of the reported hernias are treated with mesh [14]. In some cases, where there is a large lesion and weakness of muscles, mesh has been advocated [4]. There are two approaches to repair Morgagni; thoracic or abdominal, but an abdominal approach is preferred if there is bowel obstruction. Laparoscopy has the advantage of less postoperative ileus, hospitalization period, and pain, it is also associated with a quick recovery of functions. Currently, for the management of hernia sac with laparoscopic repair, no agreement is available.


Conclusion

Morgagni hernia is a congenital diaphragmatic anomaly that occurs rarely, and it is diagnosed incidentally in pediatric age group but may present late in adulthood. Occurrence of this anomaly in both twins is considered very rare and the pathological process of this is still unclear. Involvement of genetic factors in the development of this lesion is suggested by the present case of twins with rare type of CDH. Therefore, genetic implication in this disease needs further clarification and investigation. The diagnosis can be done by using computed tomography. Treatment of hernia can be done immediately by suturing the diaphragm edge to retrosternal and retrocostal periosteum and peritoneum. Although laparoscopic approach is gaining popularity in present practice for pediatric cases, the open transabdominal approach remains the gold standard, with excellent short and longterm results.


List of Abbreviations

CDH congenital diaphragmatic hernia
MH Morgagni hernia

Conflict of interest

The authors declare that there is no conflict of interest regarding the publication of this article.


Funding

None.


Consent for publication

Informed consent was obtained from the parents of the patients.


Ethical approval

Ethical approval is not required at our institution to publish an anonymous case report.


Author details

Abdullah Saeed Alghamdi1, Muhammad Abdulrahman Alzahrani1, Amjad Saeed Alghamdi1, Yasmin Abdulaziz Yousef2, Ahmed Alawi2

  1. College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia
  2. Department of Pediatric Surgery, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia

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How to Cite this Article
Pubmed Style

Alghamdi AS, Alzahrani MA, Alghamdi AS, Yousef YA, Alawi A. A report of two pediatric cases of Morgagni Hernia. IJMDC. 2019; 3(11): 963-966. doi:10.24911/IJMDC.51-1560365818


Web Style

Alghamdi AS, Alzahrani MA, Alghamdi AS, Yousef YA, Alawi A. A report of two pediatric cases of Morgagni Hernia. https://www.ijmdc.com/?mno=52541 [Access: October 15, 2021]. doi:10.24911/IJMDC.51-1560365818


AMA (American Medical Association) Style

Alghamdi AS, Alzahrani MA, Alghamdi AS, Yousef YA, Alawi A. A report of two pediatric cases of Morgagni Hernia. IJMDC. 2019; 3(11): 963-966. doi:10.24911/IJMDC.51-1560365818



Vancouver/ICMJE Style

Alghamdi AS, Alzahrani MA, Alghamdi AS, Yousef YA, Alawi A. A report of two pediatric cases of Morgagni Hernia. IJMDC. (2019), [cited October 15, 2021]; 3(11): 963-966. doi:10.24911/IJMDC.51-1560365818



Harvard Style

Alghamdi, A. S., Alzahrani, . M. A., Alghamdi, . A. S., Yousef, . Y. A. & Alawi, . A. (2019) A report of two pediatric cases of Morgagni Hernia. IJMDC, 3 (11), 963-966. doi:10.24911/IJMDC.51-1560365818



Turabian Style

Alghamdi, Abdullah Saeed, Muhammad Abdulrahman Alzahrani, Amjad Saeed Alghamdi, Yasmin Abdulaziz Yousef, and Ahmed Alawi. 2019. A report of two pediatric cases of Morgagni Hernia. International Journal of Medicine in Developing Countries, 3 (11), 963-966. doi:10.24911/IJMDC.51-1560365818



Chicago Style

Alghamdi, Abdullah Saeed, Muhammad Abdulrahman Alzahrani, Amjad Saeed Alghamdi, Yasmin Abdulaziz Yousef, and Ahmed Alawi. "A report of two pediatric cases of Morgagni Hernia." International Journal of Medicine in Developing Countries 3 (2019), 963-966. doi:10.24911/IJMDC.51-1560365818



MLA (The Modern Language Association) Style

Alghamdi, Abdullah Saeed, Muhammad Abdulrahman Alzahrani, Amjad Saeed Alghamdi, Yasmin Abdulaziz Yousef, and Ahmed Alawi. "A report of two pediatric cases of Morgagni Hernia." International Journal of Medicine in Developing Countries 3.11 (2019), 963-966. Print. doi:10.24911/IJMDC.51-1560365818



APA (American Psychological Association) Style

Alghamdi, A. S., Alzahrani, . M. A., Alghamdi, . A. S., Yousef, . Y. A. & Alawi, . A. (2019) A report of two pediatric cases of Morgagni Hernia. International Journal of Medicine in Developing Countries, 3 (11), 963-966. doi:10.24911/IJMDC.51-1560365818