Anesthetic management for double lung transplantation in an 8-year-old male with extreme malnutrition (BMI 11.4 kg/m²) following hematopoietic stem cell transplantation: a case report

Introduction

Pediatric lung transplantation (LTx) is the definitive treatment for children with end-stage lung diseases (1-3). It remains exceptionally rare, accounting for only 1.8% of the total lung transplant volume in China, according to 2019–2023 data from the China Lung Transplantation Registry (CLTR) (4). Within this niche cohort, bronchiolitis obliterans syndrome (BOS) following hematopoietic stem cell transplantation (HSCT) is a primary indication. Despite advances in surgery and immunosuppression, perioperative management remains challenging, particularly in patients with severe malnutrition (5). Malnutrition in this population involves a significant depletion of lean mass and fat stores; up to 59% of cystic fibrosis (CF) and 39% of non-CF recipients are underweight at the time of surgery (6).

Although nutritional status typically recovers 12–24 months post-transplant, current International Society for Heart and Lung Transplantation (ISHLT) guidelines still classify a body mass index (BMI) below 16–17 kg/m² as a high-risk factor or relative contraindication (2). Poor nutritional reserves impair respiratory mechanics, drug metabolism, and wound healing, complicating anesthesia and prolonging intensive care unit (ICU) stays (6). A recent multi-institutional analysis of the United Network for Organ Sharing (UNOS) registry [1986–2020] by Heidel et al. quantified this hazard in pediatric candidates (aged 2–18 years). Their findings demonstrated that patients listed with a severely low BMI percentile (<3rd) exhibited significantly worse overall survival regardless of transplantation status (P=0.009) and lower graft survival (P=0.034) compared to those with a normal BMI percentile (5th–85th). Furthermore, even when compared to the low BMI cohort (3rd–5th percentile), graft survival in the severely low BMI group remained significantly worse (P=0.040) (7).

Despite this well-documented mortality hazard, clinical evidence guiding the specific anesthetic management of candidates with an extremely low BMI (<16 kg/m²) is scarce. In accordance with the CARE guidelines, we report the successful perioperative management of an 8-year-old child with severely low BMI (11.4 kg/m²) undergoing bilateral LTx for post-HSCT BOS, focusing on specific strategies used to address malnutrition-associated risks. We present this article in accordance with the CARE reporting checklist (available at https://tp.amegroups.com/article/view/10.21037/tp-2025-1-781/rc).

Case presentation

Patient history and clinical course

Chief complaint: progressive chest tightness and dyspnea for 11 months, 3 years following HSCT.

An 8-year-old male was admitted in November 2022 for LTx evaluation due to progressive respiratory failure secondary to BOS. The patient’s history began in June 2019 with a diagnosis of acute myeloid leukemia (AML). Following induction chemotherapy (decitabine and IA regimen), he underwent haploidentical allogeneic HSCT (allo-HSCT) in July 2019. The donor was his father (5/10 HLA match), and post-transplant immunosuppression included cyclosporine, methylprednisolone, and mycophenolate mofetil.

Sixteen months post-HSCT (November 2020), the patient developed exertional dyspnea. Pulmonary function tests (PFTs) revealed a severe obstructive ventilatory defect: forced expiratory volume in the first second (FEV₁) 0.22 L (22.9% predicted), forced vital capacity (FVC) 0.35 L (30.2% predicted), and FEV₁/FVC ratio 64.72%. These findings were consistent with BOS, prompting a switch from cyclosporine to tacrolimus. Despite modified immunosuppression, his condition deteriorated. By July 2021, nocturnal oxygen therapy (1 L/min) was initiated due to worsening cough and sputum production, maintaining oxygen saturation (SpO₂) between 92% and 100%. In May 2022, his exercise tolerance decreased significantly, and SpO₂ dropping to 90% during activity. Follow-up PFTs in June 2022 showed progressive deterioration: FEV1 0.23 L (22% predicted), FVC 0.63 L (47% predicted), and FEV₁/FVC ratio 37%.

Two weeks prior to the LTx surgery (December 2^nd, 2022), the dyspnea intensified, necessitating continuous home oxygen therapy (2 L/min). The patient became symptomatic after walking less than 50 meters, with SpO₂ dropping to 85–88%. Birth and family histories were noncontributory.

Preoperative assessment

Vital signs

Temperature 37 ℃, HR 124 bpm (sinus tachycardia), RR 20 bpm, and BP 114/80 mmHg. SpO₂ was maintained at 97–100% on 3 L/min via nasal cannula.

Physical examination

The patient appeared cachectic with profound muscle wasting, consistent with a state of severe malnutrition (height 113 cm, weight 14.5 kg, BMI 11.4 kg/m²). The thoracic cage was symmetrical with coarse breath sounds and scattered dry rales noted bilaterally. Ocular secretions were observed in the left eye. Skin examination revealed generalized brownish hyperpigmentation and desquamation, typical signs of chronic cutaneous graft-versus-host disease (GVHD). Cardiac auscultation revealed a regular rhythm without pathological murmurs (P2 < A2). Peripheral pulses were regular and synchronous.

Preoperative investigations

Laboratory evaluation revealed mild anemia (hemoglobin 112 g/L) with normal leukocyte (7.7×10⁹/L, 71.8% neutrophils, 16.9% lymphocytes) and platelet (323×10⁹/L) counts. Cardiac biomarkers (Troponin-I 0.000 ng/mL, Pro-BNP 38 pg/mL) were normal, though CK-MB was 28 U/L. Comprehensive infectious disease screening (respiratory pathogen antibodies, EBV-DNA, and SARS-CoV-2 PCR) were negative. A transthoracic echocardiogram revealed sinus tachycardia with otherwise normal cardiac structure and function. Abdominal and peripheral vascular ultrasounds were unremarkable. Contrast-enhanced chest computed tomography (CT) confirmed severe, extensive bilateral bronchiectasis (Figure 1). Psychosocial evaluation cleared the patient for transplantation. The Nutritional Risk Screening (NRS-2002) score was 6, and the BODE index was 10, underscoring the severity of nutritional depletion and poor respiratory prognosis.

Figure 1 Preoperative contrast-enhanced chest CT demonstrating severe, extensive bilateral bronchiectasis. CT, computed tomography.

To definitively establish the diagnosis of BOS and rule out other late-onset post-HSCT pulmonary complications, a comprehensive differential diagnosis was conducted. First, extramedullary leukemic pulmonary infiltration secondary to AML relapse was considered clinically unlikely due to normal leukocyte and platelet counts, indicating sustained hematological remission. Second, infectious etiologies were effectively excluded by negative broad-spectrum screening and the absence of acute infectious signs. Third, idiopathic pneumonia syndrome (IPS) was ruled out by the late onset (>1 year post-HSCT) and chronic disease progression, which lacked the acute diffuse alveolar damage typical of IPS. Finally, bronchiolitis obliterans organizing pneumonia (BOOP) was differentiated based on physiological and radiological phenotypes. Unlike BOOP, which typically presents with a restrictive ventilatory defect and patchy consolidations or ground-glass opacities on imaging, our patient exhibited a severe, irreversible obstructive pattern on PFTs and progressive air trapping with extensive bronchiectasis on chest CT. These findings, combined with evidence of chronic cutaneous GVHD, strongly supported the definitive diagnosis of BOS.

Anesthetic management

The patient was positioned with a 20° head-up tilt and pre-oxygenated via mask. Standard monitoring was established, supplemented by bispectral index (BIS) and regional cerebral oxygen saturation (rSO₂). Under ultrasound guidance, a left radial arterial line was placed using local anesthesia (1% lidocaine) for continuous blood pressure and advanced hemodynamic monitoring (cardiac output, cardiac index, stroke volume variation, and systemic vascular resistance index).

Anesthesia induction utilized midazolam (0.05 mg/kg), propofol (1.5 mg/kg), sufentanil (0.8 µg/kg), and rocuronium (0.6 mg/kg). Airway access was secured with a 5.5-mm ID reinforced endotracheal tube via video laryngoscopy, precise positioning was confirmed via fiberoptic bronchoscopy. A pediatric transesophageal echocardiography (TEE) probe was inserted to monitor ventricular function and pulmonary venous flow. Mechanical ventilation was initiated with a fraction of inspired oxygen (FiO₂) of 1.0, tidal volume (VT) of 8 mL/kg, respiratory rate (RR) of 16 bpm, an inspiratory-to-expiratory (I:E) ratio of 1:2, and a positive end-expiratory pressure (PEEP) of 5 cmH₂O.

Following induction, ultrasound-guided central venous access was established via the left internal jugular vein (triple-lumen) and right internal jugular vein (Swan-Ganz catheter). A femoral venous catheter was placed for potential continuous renal replacement therapy (CRRT). Radial arterial and pulmonary arterial blood gases were analyzed prior to incision to optimize electrolytes and ventilation. Anesthesia was maintained with target-controlled infusions of propofol (9–15 mg/kg/h), sufentanil (0.5–2 µg/kg/h), and cisatracurium (0.06–0.12 mg/kg/h), strictly titrated to maintain a BIS of 40–60.

Surgical course and hemodynamics

A bilateral anterior thoracotomy (clamshell incision) revealed diffuse emphysematous changes and hyperinflation consistent with BOS; minimal adhesions were noted. Two critical hemodynamic events occurred during vascular clamping:

• Right pulmonary artery (PA) clamping: induced significant hypotension (113/74 to 60/42 mmHg) and mild bradycardia (111 to 107 bpm). Hemodynamic stability was restored following a 5 µg norepinephrine bolus.
• Left atrial clamping: during partial clamping for the left pulmonary vein anastomosis, profound hypotension (94/45 to 44/27 mmHg) and severe bradycardia (154 to 47 bpm) immediately ensued. The clamp was immediately released. Following a 5 µg epinephrine bolus and hemodynamic recovery (116/50 mmHg, HR 144 bpm), the atrium was successfully re-clamped.

Methylprednisolone (150 mg) was administered 15 minutes prior to reperfusion. Post-anastomosis TEE confirmed patent pulmonary venous flow (right: 129 cm/s; left: 89.7 cm/s) without stenosis. The chest was closed following rigorous hemostasis.

Total intraoperative intake was 1,400 mL [200 mL red blood cells (RBCs), 700 mL 5% albumin, 250 mL 20% albumin, 100 mL balanced crystalloid, 150 mL antibiotics]. Total output was 1,200 mL (400 mL blood loss, 800 mL urine).

Short-term and long-term outcome

The patient was transferred to the ICU in stable condition, extubated on postoperative day 2, and discharged from the ICU on day 6.

Follow-up through April 2025 indicated highly favorable recovery. The patient’s somatic growth demonstrated a robust catch-up trajectory: height and weight increased from 113 cm and 14.5 kg (BMI 11.4 kg/m²) preoperatively to 134 cm and 32.5 kg (BMI 18.10 kg/m²) by March 2025 (Figure 2).

Figure 2 Longitudinal BMI trajectory of the patient before and after double lung transplantation. The solid line indicates the patient’s actual BMI. The shaded area and grey dashed line represent the normal reference range (3rd–97th percentiles, P₃–P₉₇) and median (50th percentile, P₅₀), respectively, based on the 2000 CDC growth charts for age- and sex-matched males. The vertical red dashed line marks the time of transplantation. Pre-transplantation baseline and latest follow-up anthropometric parameters are annotated to highlight catch-up growth. BMI, body mass index; CDC, Centers for Disease Control and Prevention; LTx, lung transplantation.

Follow-up PFT on January 15, 2025, confirmed normal ventilatory capacity (FVC 2.08 L, 103.62% predicted; FEV₁ 1.87 L, 111.00% predicted; FEV₁/FVC ratio 90.04%), along with normalized small airway and diffusion functions. The clinical timeline of disease progression and treatment is illustrated in Figure 3.

Figure 3 Clinical timeline of disease progression and treatment. allo-HSCT, allogeneic hematopoietic stem cell transplantation; AML, acute myeloid leukemia; BMI, body mass index; BOS, bronchiolitis obliterans syndrome; LTx, lung transplantation; PFT, pulmonary function test.

Ethical statement

All procedures performed in this study were in accordance with the Institutional Ethics Committee of the Second Affiliated Hospital, Zhejiang University School of Medicine (No. 2025-1484) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient’s parent/guardian for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

This case report describes the successful perioperative management of an 8-year-old male with severe malnutrition (BMI 11.4 kg/m²) undergoing LTx for post-HSCT BOS. While LTx is the standard of care for end-stage lung disease, severe cachexia poses a significant clinical dilemma. Traditionally, a low BMI (<16–17 kg/m²) is classified as a relative or absolute contraindication due to perceived prohibitive risks of poor wound healing and mortality (2,7,8). Our successful outcome aligns with Ramos et al., who demonstrated that severely underweight CF recipients (BMI <17 kg/m²) achieved post-transplant survival rates comparable to normal-weight recipients, suggesting that extreme somatic depletion alone should not preclude listing for transplantation (8). However, the decision to proceed with surgery without prolonged nutritional optimization was driven by the urgency of the patient’s respiratory failure, and this patient’s history of HSCT and signs of chronic GVHD added systemic complexity. While Khubutiya et al. advocate for preoperative percutaneous endoscopic gastrostomy (PEG) to optimize BMI prior to listing (9), the rapid progression of respiratory failure in our patient necessitated an immediate surgical rescue. Consistent with observations by Chohan et al. (10), our case demonstrates that while high nutritional risk scores accurately stratify baseline vulnerability, the associated “frailty” with low BMI is functionally reversible if the patient can be safely bridged through the immediate surgical insult via meticulously tailored anesthetic management.

The primary anesthetic challenge stems from the “double-hit” of a catabolic state and severely limited physiological reserve. Jomphe et al. highlight that the immediate post-transplant phase is characterized by a hypermetabolic state and increased nutritional demands due to wound healing and immunosuppression (11). Extreme sarcopenia and concurrent hypoproteinemia fundamentally alter drug pharmacokinetics by reducing plasma protein binding. This increases the free plasma fraction of highly protein-bound agents such as propofol and sufentanil, dramatically elevating the risk of profound hypotension during induction, significantly elevating the risk of relative overdose and profound cardiovascular depression. We utilized BIS monitoring to precisely titrate anesthetic depth (12,13). This targeted approach prevented drug accumulation in the sarcopenic body, facilitating rapid wake-up and extubation on postoperative day 2, which contrasts with the prolonged ventilation typically anticipated in malnourished cohorts.

Pathophysiologically, severely malnourished children often present with an “atrophic heart”, characterized by diminished myocardial mass, blunted catecholamine responsiveness, and limited ventricular reserve. This fragility extends to the intraoperative period, manifesting as an intolerance to myocardial depressants and vascular clamping. During transplantation, surgical maneuvers such as PA clamping abruptly increase right ventricular (RV) afterload, which directly precipitated the severe hemodynamic instability observed during PA and left atrial clamping (12,14). In our patient, this mechanical stress precipitated acute RV strain and hemodynamic collapse. Although a nationwide survey indicates that TEE is utilized in only a minority of cases in China (15), it is an essential tool for real-time evaluation of cardiac function and pulmonary venous flow. The continuous TEE allowed us to early identification of clamping intolerance, and differentiate between absolute hypovolemia and mechanical outflow obstruction during clamping, providing decisive guidance for preemptive vasopressor support (norepinephrine/epinephrine). This underscores the necessity of aggressive, preemptive hemodynamic support and close communication between the surgeon and anesthesiologist.

Furthermore, pediatric LTx recipients require exceptionally precise fluid regulation due to their limited systemic blood volume. While excessive fluid administration drives primary graft dysfunction (PGD), aggressive restriction exacerbates organ hypoperfusion and metabolic acidosis. We advocate for a “staged” fluid strategy:

• Pre-reperfusion phase: fluid infusion must proactively account for basal fluid requirements (BFR) and anticipated surgical losses to prevent hemodynamic collapse during positional changes or vascular clamping.
• Post-reperfusion phase: fluid intake must be tightly controlled. High-concentration albumin is utilized to maintain intravascular oncotic pressure, combined with diuretics to achieve a negative fluid balance (16).

Postoperative recovery in such patients relies on a seamless transition to nutritional rehabilitation. As Motz et al. noted, standard predictive equations often fail in malnourished pediatric transplant recipients, making precise resting energy expenditure (REE) assessment crucial (17). Although indirect calorimetry was not utilized, our strategy of aggressive early extubation (day 2) facilitated the rapid resumption of spontaneous breathing and enteral nutrition, which remains a cornerstone of holistic multidisciplinary care for complex pediatric recipients (18).

Limitations

The primary limitation of this report is its single-case nature. The observed hemodynamic responses to vascular clamping may vary depending on the specific etiology of pulmonary hypertension and baseline RV function in other candidates. Additionally, the lack of preoperative invasive hemodynamic data limits the precise quantification of the patient’s baseline cardiac reserve.

Conclusions

Our experience demonstrates that the frailty associated with extreme malnutrition (BMI <16 kg/m²) in pediatric LTx is not a universally prohibitive barrier, but rather a rigorously manageable risk. Success depends on a multidisciplinary team (MDT) framework that replaces rigid anthropometric cutoffs with precision, physiology-based protocols. The integration of TEE-directed hemodynamic support to manage clamping intolerance and BIS-guided anesthetic titration to prevent drug accumulation serves as the critical pillar for surviving the acute surgical insult. This tailored approach may effectively bridge these “unfit” candidates to the postoperative phase, where long-term nutritional and functional recovery is attainable.

Acknowledgments

The authors thank the patient and his family for providing written informed consent for the publication of their clinical data and accompanying images.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://tp.amegroups.com/article/view/10.21037/tp-2025-1-781/rc

Peer Review File: Available at https://tp.amegroups.com/article/view/10.21037/tp-2025-1-781/prf

Funding: This study was supported by the Zhejiang Provincial Medical and Health Science and Technology Plan (No. 2025KY855), Zhejiang Clinovation Pride (No. CXTD202501020), the National Clinical Key Specialty Construction Project of China 2021 (No. 2021-LCZDZK-01), and the Zhejiang Key Laboratory of Pain Perception and Neuromodulation.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-2025-1-781/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the Institutional Ethics Committee of the Second Affiliated Hospital, Zhejiang University School of Medicine (No. 2025-1484) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient’s parent/guardian for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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