Discussion
This case highlights a unique presentation of pneumoperitoneum secondary
to PI with concurrent focal pneumatosis cystoides. The concurrent
presence of pneumatosis cystoides occurring from outside of the GI
tract, the diaphragm in this case, has not been documented in the
literature. This finding prompts several questions regarding the
pathogenesis, diagnosis, and management of PI.
The patient’s clinical course was complicated by recurrent abdominal
symptoms and multiple interventions. Despite initial surgical management
of the malrotation, the patient continued to experience symptoms,
necessitating further investigation and repeat procedures. The
persistence of symptoms and the discovery of diaphragmatic pneumatosis
cystoides suggest that the initial surgery may not have fully addressed
the underlying pathophysiology. Alternatively, the waxing and waning
nature of symptoms could be indicative of chronic PI. Surgical
intervention remains the mainstay of treatment for PI when complications
such as bowel obstruction or perforation are
present.11 In this case, the decision to perform a
bowel resection and resect the diaphragmatic cysts was guided by the
acute nature of the presentation, despite the lack of clear perforation.
The patient’s postoperative recovery was complicated by persistent
symptoms, highlighting the need for a comprehensive approach to
postoperative symptom management.
The etiology of PI remains idiopathic in many cases.10Our patient had no apparent underlying cause of the PI, as autoimmune
disease (e.g., Crohn’s disease) and causes of barotrauma are unlikely,
despite history of endoscopy and colonoscopy procedures prior to
presentation. Notably, the patient had a history of Coronavirus disease
2019 (COVID-19) prior to the clinical course, raising the question of a
potential association between viral infection and PI. A case of PI has
been reported following COVID-19 infection with
pneumonia,10 however involved different clinical and
anatomic manifestations than our patient. The proposed mechanism for
COVID-19-related PI involves disruption of gut flora due to viral
infection. Other potential infectious associations with PI include
cytomegalovirus in post-lung transplant patients,16cryptosporidiosis and human immunodeficiency virus
(HIV),17 and Clostridium difficile, rotavirus, and
adenovirus in children.18 An infectious cause is not
likely in this patient due to his normal white blood cell count and lack
of infectious signs in the pathologic studies. Additionally, the patient
was not immunocompromised and did not have any risk factors for an
opportunistic infection.
The significance of the diaphragmatic pneumatosis cystoides is
uncertain. Abnormalities of the diaphragm were not specifically noted
during his initial surgery, thus the time of onset of this cyst is
unknown and could theoretically be sequelae of the pneumatosis cystoides
intestinalis, which is unlikely given the negative margins of the PI.
The occurrence of pneumatosis cystoides in two separate locations, the
diaphragm and intestine, suggests a possible systemic/tissue
predisposition to cyst formation. Collagen vascular disorders were
considered but are unlikely due to the absence of characteristic
clinical signs. This presentation raises the possibility of an
unidentified connective tissue disorder or a rare presentation of an
existing one.
An important consideration in this case is the patient’s history of
congenital malrotation, which was surgically corrected prior to
presenting with PI. While malrotation is most commonly diagnosed in
early childhood with other concurrent anomalies, many cases have also
been reported in adolescents and adults.19 PI is not a
commonly reported complication after Ladd’s procedure in
children,20 however limited research has investigated
long-term outcomes of the Ladd’s procedure performed in adults.
This case highlights the need for further investigation into the
pathogenesis and optimal management strategies for PI, particularly in
atypical presentations, to improve patient outcomes and expand our
understanding of this rare condition. Additionally, future studies
should evaluate the long-term outcomes of Ladd’s procedure when
performed in adults. Genetic and pathology studies may uncover
underlying connective tissue disorders predisposing patients to cyst
formation, particularly in the context of congenital malrotation.
Long-term follow-up of patients with PI is essential to monitor for
recurrence and manage chronic symptoms. Multidisciplinary care involving
gastroenterologists, surgeons, and infectious disease specialists may
improve outcomes in complex and unique cases.