2019 Research Forum

Serghei Burcovschii MD R1, Shrey Patel MS III, Nakisa Kia’i MS III, Ahmad Malik MS III, Hannah Schuett MS III, Basel Shoua MD R3, Tushar Bajaj MD R1, Mohamed Hammami MD Ivemark Syndrome – Surgical Evaluation Studies/Imaging Introduction

Conclusions Ivemark Syndrome is a form of heterotaxy characterized by situs ambiguous including: intestinal malrotation, centrally located liver, severe spleen hypoplasia or asplenia, centrally located heart and other cardiac malformations, and multiple lung malformations. Overall, there is a failure to establish normal left- right asymmetry at birth. When evaluating these patients for surgical management, careful consideration must be taken regarding the specific malformations in each individual and the respective possible associated complications.

CT CHEST - Diffuse right and left lung centrilobular/tree-in-bud nodular densities most pronounced at the lung bases with more consolidative airspace disease seen in both lower lobes along with diffuse bronchial wall thickening. Findings are suggestive of endobronchial spread of infection/multifocal pneumonia. Mediastinal and hilar lymphadenopathy, likely reactive. Suggestion of abdominal situs. Enlargement of the main pulmonary trunk suggests underlying pulmonary hypertension. CHEST XRAY - Improved aeration of the right lung base, with persistent mild interstitial opacification/consolidation, with likely some chronic component.

This case explores medical clearance and surgical preoperative evaluation in an adult- aged patient with Ivemark Syndrome Type II, which has not been greatly reported upon in medical literature, as the majority of cases do not survive the first year of life.

Purpose

The patient is a 33 year-old Male with Ivemark Syndrome Type II, pulmonary hypertension and interstitial lung disease who presents to the Emergency Department after a ground level fall resulting in a displaced oblique fracture of the proximal tibial fibular diaphysis. Prior to admission the patient reported a four month history of worsening dyspnea at rest and upon exertion. On admission, the patient was found to be hypercapnic with an arterial blood gas PCO2 of 128 mmHg. ICU was consulted to evaluate the patient's hypercapnia which was being treated via BiPAP. Furthermore, Orthopedic Surgery evaluated the patient for possible open reduction internal fixation of his fracture; however, in the setting of acute hypercapnia, surgery was delayed. Ultimately, the patient decided against surgical intervention and opted to be casted to prevent complications of surgery.

Results Surgical Intervention for patients with Ivemark Syndrome are deemed high risk due to their congenital heart problems, and a myriad of pulmonary problems. The patient presented in this case was found to have an 18.4% risk for serious complications based on the National Surgical Quality Improvement Program (NSQIP), which is above the average risk of 4%. NSQIP calculations also reported a total risk for any complication 20.3% for this patient, which is above the average of 4.6%. Patients with interstitial lung disease secondary to Ivemark Syndrome must have a careful preoperative evaluation along with targeted laboratory and imaging studies. Chest computed tomography (CT) is commonly used to evaluate risk for perioperative complications as well as viability of anesthesiology. The patient’s CT Chest revealed bronchial wall thickening, main pulmonary artery enlargement - indicating pulmonary hypertension, as well as mediastinal and hilar adenopathy. Additionally, multiple bilateral miliary nodules and coarctation of the aorta was suggested. These findings on CT are consistent with a high risk of complications during surgery.

References

1. Jane Simpson and Hans Zellweger, Familial Occurrence of Ivemark Syndrome with Splenic Hypoplasia and Asplenia in Sibs, J Med Genet. 1973 Sep; 10(3): 303–304. 2. Diaz-Fuentes, Gilda, et al. “Perioperative Evaluation of Patients with Pulmonary Conditions Undergoing Non-Cardiothoracic Surgery.” Health Services Insights , vol. 9s1, 9 Nov. 2016, doi:10.4137/hsi.s40541.

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