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 Table of Contents  
CLINICAL IMAGE
Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 128-129

Late Onset Morgagni Hernia Associated with Xeroderma Pigmentosum


1 Department of General Paediatrics, Institute of Child Health and Hospital for Children, Chennai, Tamil Nadu, India
2 Department of Paediatric Pulmonology, Institute of Child Health and Hospital for Children, Chennai, Tamil Nadu, India

Date of Submission31-Jan-2023
Date of Decision26-Mar-2023
Date of Acceptance06-Apr-2023
Date of Web Publication24-May-2023

Correspondence Address:
Dr. R Shyamsundar
5 G Orchid, Ceebros Gardens, Vembuliamman Koil Street, Virugambakkam, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2772-5170.377515

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How to cite this article:
Shyamsundar R, Abinesh R, Arunagirinathan V, Balaji S. Late Onset Morgagni Hernia Associated with Xeroderma Pigmentosum. Indian Pediatr Case Rep 2023;3:128-9

How to cite this URL:
Shyamsundar R, Abinesh R, Arunagirinathan V, Balaji S. Late Onset Morgagni Hernia Associated with Xeroderma Pigmentosum. Indian Pediatr Case Rep [serial online] 2023 [cited 2023 Jun 6];3:128-9. Available from: http://www.ipcares.org/text.asp?2023/3/2/128/377515

An 11-year-old girl, presented with dry cough, mild fever, and abdominal pain for 2 weeks. She had a known diagnosis of xeroderma pigmentosum with photophobia along with generalized freckling and xerosis of skin, since 1.5 years of age, associated with progressive hearing loss and cognitive decline. There was no positive family history. On examination, general condition was stable, with pulse 128/min, respiratory rate 28/min, oxygen saturation 98% on room air, and blood pressure 100/70 mmHg. Chest examination revealed decreased breath sounds and dullness over the right infraaxillary, inframammary, and interscapular regions. Her hemogram showed a total white blood cell count of 16,000 cells/cumm, with 78% neutrophils, hemoglobin 11 g/dl, and platelets 2.5 L/cumm. Chest X-ray revealed a homogenous opacity in the right lower lobe suggestive of consolidation with pleural effusion. A diagnosis of community-acquired pneumonia with synpneumonic effusion was made and the child was initiated on oral amoxicillin-clavulanate on an outpatient basis. Unfortunately, the child did not come for a follow-up until 6 months had passed, when she presented again with a similar clinical picture. Chest radiography, this time, revealed a similar opacity in the right lower lobe, though smaller than the original patch.

Considering her underlying immunodeficient status, a possibility of persistent pneumonia was suspected. Serial roentgenograms taken thereafter showed nonregression of the opacity despite improvement in clinical status. Tuberculosis work-up was negative. Ultrasonography of chest was then done, which demonstrated bowel loops within the chest cavity. A subsequent right lateral chest X-ray showed multiple air-fluid levels in the chest. A barium meal study revealed coils of intestine within the right lower hemithorax [Figure 1] and [Figure 2]. Chest X-rays taken during previous hospital visits, 3 years back however did not show these features, thereby confirming a diagnosis of Morgagni hernia with delayed onset, mimicking persistent pneumonia.[1],[2],[3]
Figure 1: Chest X-ray showed multiple air fluid levels in the chest

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Figure 2: Barium meal study revealed coils of intestine within right lower hemithorax

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A literature search did not reveal any case of xeroderma pigmentosum associated with Morgagni hernia. Diaphragmatic hernias are of four types (i) Bochdalek hernia, where the defect is posterolateral, (ii) hiatal hernia, where the defect is at the esophageal hiatus, (iii) paraesophageal hernia, where the defect is located adjacent to the esophageal hiatus, and (iv) Morgagni hernia, in which the defect is found in an anterior and retrosternal location, and was first described by Morgagni in 1769. It is the rarest of all four comprising only 2%–5% of all congenital diaphragmatic hernias.[4] Morgagni hernia is a type of diaphragmatic hernia where the defect lies in the anterior portion of diaphragm due to failure of the pars terminalis part of the costochondral arches to fuse with the pars sternalis on the right side. Delayed diagnosis can happen in Morgagni hernia since these defects are small, having a hernia sac, but can grow over time due to increases in intra-abdominal pressure, causing weakness of the diaphragm.[5] Precipitating factors could be trauma, obesity, chronic constipation, or chronic cough, none of which were found in this child. Neuropathy, causing weakness of diaphragm may have precipitated the event, though the child had no other features of neuropathy. Definitive treatment is surgical repair even if asymptomatic, to prevent future risks of incarceration.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hashmi MU, Ullah K, Tariq A, et al. Morgagni-Larrey Hernia: A possible cause of recurrent lower respiratory tract infections. Cureus 2019;11:E4035.  Back to cited text no. 1
    
2.
Picard E, Ben Nun A, Fisher D, et al. Morgagni hernia mimicking pneumonia in Down syndrome. J Pediatr Surg 2007;42:1608-11.  Back to cited text no. 2
    
3.
Supomo S, Darmawan H. A rare adult morgagni hernia mimicking lobar pneumonia. Turk J Surg 2022;38:98-100.  Back to cited text no. 3
    
4.
Svetanoff WJ, Rentea RM. Morgagni hernia. In: Statpearls. Treasure Island (Fl): Statpearls Publishing; 2023.  Back to cited text no. 4
    
5.
Loong TP, Kocher HM. Clinical presentation and operative repair of hernia of Morgagni. Postgrad Med J 2005;81:41-4.  Back to cited text no. 5
    


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