E. Danzer et al.: J Extra Corpor Technol 2025, 57, 168--170 169
25.4 %( using Meyer’ s method [ 4 ]. Additionally, there was evidence of an intrathoracic liver position. The low O / E LHR and O / E TLV, combined with the intrathoracic position of the liver, were indicative of severe pulmonary hypoplasia, known to be associated with significant neonatal morbidity [ 5, 6 ]. The prenatal course was further complicated by hydrops, necessitating fetal thoracocentesis, and amnioreduction at 32 weeks and one day of gestation. She was delivered via urgent cesarean section due to non-reassuring fetal heart tracing at 33 weeks and two days. The birth weight was 2,190 g. She was placed on veno-arterial ECMO within three hours after birth because of worsening hypercarbia and hypoxemic respiratory failure caused by severe pulmonary hypoplasia. The newborn was cannulated using an 8 Fr arterial cannula in the right common carotid artery and an 8 Fr venous cannula in the right internal jugular vein. She underwent primary diaphragmatic repair on day of life( DOL) eight and was successfully weaned from ECMO by day of life 15.
On DOL 152, she developed a urinary tract infection caused by Klebsiella pneumoniae. Adequate antibiotic coverage with cefotaxime was initiated and quickly broadened to vancomycin, flagyl, and cefepime; however, her condition deteriorated rapidly. This deterioration was marked by an acute increase in B-type natriuretic peptide, rising to 3,718 pg / mL, and was accompanied by biventricular dysfunction, right ventricular failure, and mitral valve stenosis on repeat echocardiogram. Despite maximal intensive medical management for severe pulmonary hypertension crisis and associated cardiac dysfunction, she developed worsening hemodynamics, further deterioration of biventricular function, and end-organ dysfunction. This prompted the decision to reinitiate venoarterial ECMO as a bridge to recovery. Transthoracic cardiac cannulation via sternotomy was urgently performed on DOL 158, as ligation of the neck vessels was performed at the time of the neonatal ECMO decannulation. A 10-French return cannula was placed in the ascending aorta, and a 20-French drainage cannula was placed in the right atrium. Her sternum was left open, but the skin was closed around the cannulas. An echocardiogram conducted on full support demonstrated improvement in biventricular function. On DOL 161, she developed oozing from the cannula site, which progressively worsened, leading to hypotension and bradycardia. A repeat echocardiogram revealed a large pericardial effusion with signs of cardiac tamponade; therefore, she underwent an urgent bedside mediastinal washout. Bleeding from the aortic cannulation site was controlled by placing additional purse-string sutures. The remaining course of the repeat ECMO was uneventful, and she was successfully decannulated on day 167. The development of a large left holo-hemispheric extra-axial, subdural multiloculated hematoma with mild mass effect in the adjacent brain parenchyma, prominence of the subarachnoid spaces, and a noticeable loss of brain volume complicated her post-decannulation course. Additionally, surveillance MRI revealed a reduction in white matter and thinning of the corpus callosum with under-opercularization.
Following her second ECMO course, she had ongoing ventilator-dependent respiratory insufficiency, which resulted in the placement of a tracheostomy on DOL 261 after several unsuccessful extubation attempts. Additionally, she experienced failure to thrive, oral aversion, and gastroesophageal reflux disease, leading to gastrojejunostomy placement on DOL 223. Her pulmonary hypertension was managed with inhaled nitric oxide, Sildenafil, and Bosentan. She was eventually discharged to a long-term care facility on DOL 378 for management of persistent pulmonary hypertension, tracheostomy / ventilator-dependent respiratory insufficiency, and feeding intolerance. Additionally, her post-discharge course has been complicated by bilateral conductive hearing loss, hypotonia, and global developmental delay. A formal neurodevelopmental evaluation was conducted at 19 months of age using the Bayley Scales of Infant Development, 3rd Edition. Her cognitive, language, and motor skills are severely delayed, with composite scores of 55, 63, and 58, respectively. She now lives at home with healthcare support. She was eventually weaned off the ventilator during the day, achieved full enteral gastrojejunostomy feeds, and her pulmonary hypertension is managed with Tadalafil and Ambrisentan. She is participating in physical, occupational, and speech therapy and early intervention.
Discussion
To improve decision-making regarding multi-course ECMO in patients with CDH, it is essential to better understand the potential survival benefits, risks, and surgical difficulties associated with this approach. The need for repeat ECMO is rare. Over the past decade, 458 neonates with CDH have been cared for at the Children’ s Hospital of Philadelphia, of which 96 required a single ECMO run. As a result, the prevalence of repeat ECMO in patients who required ECMO at our institution, 1 %, is even lower than the reported rate of 5.3 % in the study group registry [ 3 ]. Given the rarity of repeat ECMO in CDH, establishing clear guidelines in this complex situation remains challenging. We demonstrate that repeat ECMO can be a life-saving strategy for selected CDH patients when a reversible cause for clinical deterioration can be identified. Our findings also show that recannulation at a different site is technically feasible and safe. Our case is distinct from previously reported instances because the need for repeated ECMO cannulation typically occurs within the first week after decannulation [ 3 ]. Early recannulation may indicate that the underlying condition that required the initial ECMO treatment has not fully resolved or could still be linked to the issues from the first ECMO run. In our patient, the original cause for ECMO-- hypoxemic respiratory failure-- had been resolved, and repeat ECMO was necessary due to an acute exacerbation of pulmonary hypertension resulting from urosepsis. We demonstrate that late-term recannulation, even months after the initial cannulation, is possible and can effectively improve management and survival in patients with CDH.
Formulating an appropriate re-cannulation strategy early on is essential. Repeated neck cannulation has been used in cases where the intervals between ECMO courses are short [ 3 ]. However, cannulating the same side after nearly five months, as in our case, was not feasible due to the ligation of the right internal jugular vein and the common carotid artery close to the clavicle. Known factors such as insufficient proximal vessel length, previous tissue trauma, scarring, and increased inflammation at the initial cannulation site also prevented us from using the