Liver Transplantation in a Child with Kartagener Syndrome: A Case Report

Abstract

Background: Kartagener syndrome (KS) is a rare genetic disorder consisting of the triad of situs inversus, chronic sinusitis, and bronchiectasis. Although there are previous reports regarding the anaesthetic considerations in KS, none have included liver transplantation. Case Presentation: An 11-year-old boy with a diagnosis of KS underwent liver transplantation due to extrahepatic biliary atresia. Previous diagnostic imaging confirmed situs inversus and the absence of an inferior vena cava. The patient's peak airway pressure intermittently increased intraoperatively from 15 to 30 cm H 2 O due to increased pulmonary secretions, which required frequent suctioning of the endotracheal tube. Intraoperative volume resuscitation included 200 mL of 5% albumin, 5 units of erythrocyte suspension and 3 units of fresh frozen plasma. Intermittently, a norepinephrine infusion was required to maintain the MAP. Coagulation function was monitoring using the thromboelastogram to guide the use of blood products including fresh frozen plasma. At the end of the surgery, the patient was transferred to the intensive care unit. He was discharged from the intensive care unit on postoperative day 5, and from the hospital on postoperative day 28. He continues to do well with normal liver function 23 months after surgery. Conclusion: Despite the risk of pulmonary related to airway secretions and exacerbation of hemodynamic instability related to anatomical variations in the inferior vena cava anatomy, KS patients can be safely anesthetized with careful planning and attention of the disease process, even for complex surgical procedures such as liver transplantation.

A Novel DNAH9 Gene Mutation Causing Primary Ciliary Dyskinesia With an Unusual Association of Jejunal Atresia in a Bahraini Child

“…We present a case of PCD with an unusual association with type IIIb jejunal atresia and a novel mutation. Upon reviewing the previously reported cases in the past 12 years, a slightly higher prevalence of PCD among males was noted [ 1 , 2 , 5 - 7 , 11 , 12 ]. Our patient was male, which is in line with the previously reported cases in terms of sex.…”

“…PCD can be associated with other diseases such as central giant cell granuloma, extrahepatic biliary atresia, or duodenal atresia [ 2 , 12 , 19 ]. However, our patient had PCD with jejunal atresia.…”

“…However, our patient had PCD with jejunal atresia. Upon extensive literature search, we found that no study had reported this association before (Table 1 ) [ 1 , 2 , 5 - 7 , 11 , 12 , 18 , 20 ].…”

“…Primary ciliary dyskinesia (PCD) is a rare autosomal recessive genetic disorder [ 1 , 2 ]. No sex predilection exists for PCD [ 3 ].…”

“…In general, the estimated prevalence of the condition is one in 15,000-20,000 individuals [ 4 ]. This disease is caused by a defect in the action of the cilia lining the respiratory tract [ 1 , 2 ]. Ciliary functions are vital in multiple organs including the lungs, the sinuses, hepatobiliary and reproductive organs [ 5 ].…”