A case report on dravet syndrome complicated by multidrug- resistant pneumonia, sepsis, and severe ARDS
Rajamma1*, Pavan2, Vijayakumari. D3, Shalini H S4
1PICU Incharge, Kauvery Hospital, Electronic city, Bengaluru
2PICU Consultant, Kauvery Hospital, Electronic city, Bengaluru
3Nurse Educator, Kauvery Hospital, Electronic city, Bengaluru
4Chief Nursing Officer, Kauvery Hospital, Electronic city, Bengaluru.
*Correspondence
Introduction
Dravet syndrome is a severe developmental and epileptic encephalopathy characterized by recurrent seizures, developmental impairment, and increased susceptibility to complications during acute illnesses. Children with complex neurological disorders may have impaired airway protection, recurrent aspiration, and dependence on respiratory support, increasing their risk of severe lower respiratory tract infections.
This case describes a child with dravet syndrome who presented with severe pneumonia complicated by sepsis, septic shock, Acute Respiratory Distress Syndrome (ARDS), and polymicrobial infection, including carbapenem-resistant Acinetobacter baumannii and Burkholderia cepacia. The child also had a history of tracheostomy and home BiPAP support, with suspected aspiration contributing to the respiratory illness. The clinical course was further complicated by persistent respiratory distress requiring invasive ventilation and subsequently High-Frequency Oscillatory Ventilation (HFOV).
The case highlights the challenges of managing severe respiratory infection in a neurologically compromised child and emphasizes the importance of multidisciplinary care, early recognition of deterioration, appropriate antimicrobial therapy, respiratory support, and close monitoring.
History of Presenting Illness
The child presented with a history of fever, cough, and cold for 3 days, accompanied by fast breathing. On the morning of admission, she had two episodes of abnormal movements involving both her upper and lower limbs. She was brought to the emergency room and subsequently admitted to the PICU for further management.
Prior to admission, the child was on home BiPAP support (ST mode, settings 15/7) via a tracheostomy tube. Her home medications included nebulized tobramycin and budesonide, cotrimoxazole (Septran) prophylaxis, and antiepileptic drugs (levetiracetam, valproate, cannabidiol, and clobazam) via PEG tube.
A history of similar episodes, aspiration pneumonia was suspected. Consequently, all medications were switched to injectable formulations, and the child was kept NPO.
Issues Addressed in PICU
Respiratory System
BiPAP support was continued initially at the home settings. A chest X-ray revealed bilateral infiltrates, prompting the initiation of injectable piperacillin-tazobactam and amikacin alongside other symptomatic treatments. Endotracheal (ET) cultures and a Bio Fire panel were sent; the Bio Fire panel returned positive for Acinetobacter baumannii and Pseudomonas species.
On Day 3 of admission, the child developed increased work of breathing and worsening respiratory distress. A repeat chest X-ray showed progression of the bilateral infiltrates. BiPAP settings were increased to 18/8. Due to unresolving distress, she was transitioned to invasive ventilation (SIMV, PRVC-PS mode) with Sedo analgesia. An Arterial Blood Gas (ABG) analysis indicated mild-to-moderate ARDS, which subsequently progressed to severe ARDS. Due to escalating ventilator requirements, High-Frequency Oscillatory Ventilation (HFOV) was initiated on Day 5 and titrated based on serial blood gases and chest X-ray findings.
Serial X-rays demonstrated diffuse infiltrates alongside persistent tachypnoea and fever spikes. Immunomodulation was considered, and IVIG (1g/kg) was administered on Day 6 of admission. The child had a stormy PICU course on HFOV, complicated by respiratory acidosis that was managed by optimizing ventilator settings.Upon clinical and radiological improvement, a trial of conventional ventilation was successful, and she was transitioned back to conventional modes.
On Day 11/7, the child developed new-onset tachypnoea. Other causes were ruled out, and a repeat chest X-ray showed no new worsening. A paediatric pulmonology opinion was sought, and nebulization’s (Levo salbutamol, budesonide, 7% hypertonic saline, and N-acetylcysteine) were added pending further workup. Investigations revealed AFB-positive ET secretions, with the ET culture suggesting atypical Mycobacteria (PCP PCR and CBNAAT were negative).
Because the child continued to do well on conventional ventilation, a BiPAP trial was initiated, and she was gradually transitioned to BiPAP support. She has been tolerating BiPAP well since then without any fresh issues, currently on ST mode with settings of 18/8.
The background of Dravet syndrome and multiple infections noted during this admission, cystic fibrosis was suspected. A re-analysis for cystic fibrosis was requested using an existing sample from 2024 at Med Genome, which returned a negative result.
Because the tracheostomy tube had been in place for more than 6 weeks, it was electively changed to a 5.5 cuffed tracheostomy tube on 11/8/26 in the PICU. This was performed under procedural sedation and strict monitoring, and the child withstood the procedure well. A repeat chest X-ray confirmed proper tube placement and showed persisting bilateral infiltrates.
Ventilation Timeline
| Mode | From | To |
|---|---|---|
| BiPAP | 29/6 | 30/6 |
| PRVC-PS | 30/6 | 3/7 |
| HFOV | 3/7 | 10/7 |
| PRVC-PS | 10/7 | 17/7 |
| SIMV | 17/7 | 21/7 |
| BiPAP | 21/7 | Continue |
Cardiovascular System
The child was initially hemodynamically stable but developed signs of septic shock on Day 3 of admission, requiring the initiation of noradrenaline. Central and arterial lines were secured for close monitoring. Hypotension subsequently worsened, necessitating fluid boluses and adrenaline support. An echocardiogram (done on 2/7) showed good biventricular contractility with a normal cardiac index. Once the shock resolved, inotropic support was gradually weaned and successfully stopped on 11/7/26.
The child experienced episodes of bradycardia during her stay. An ECG showed sinus bradycardia, and Point-Of-Care Ultrasound (POCUS) confirmed good cardiac contractility. A metabolic panel revealed hypomagnesemia; following correction, no further bradycardic episodes were noted.
The child is currently hemodynamically stable with no further cardiovascular issues.
Central Nervous System
All antiepileptic drugs (AEDs) were converted to injectable formulations, while the overall regimen initially remained unchanged. Upon initiation of conventional ventilation, she was started on a morphine infusion, followed by fentanyl and dexmedetomidine infusions, as frequent intermittent ketamine boluses were required for adequate Sedo analgesia. Sedo analgesia was gradually weaned with intermittent breaks and eventually stopped. Oral clonidine was added for ongoing sedation support.
Sedo analgesia Timeline
| Drug | Start | Stop |
|---|---|---|
| Inj. Morphine | 2/7 | 3/7 |
| Inj. Fentanyl | 4/7 | 16/7 |
| Inj. Dexmedetomidine | 3/7 | 14/7 |
A paediatric neurology opinion was obtained, and an EEG was reported as normal. Valproate was discontinued due to hepatic dysfunction, thrombocytopenia, and trough levels in the high-normal range. Serum ammonia was normal. An MRI of the brain with spectroscopy is planned once the child is fully stable. As per neurology advice, a mitochondrial cocktail and topiramate were started on Day 11/7.
Upon repeat neurology review (21/7), AED doses were adjusted according to the child’s updated weight: topiramate was increased, while clobazam and levetiracetam were reduced. The child tolerated the lower doses well.
On 23/7/26, the child had one episode of a generalized tonic-clonic seizure. Following this, doses of levetiracetam, topiramate, and clobazam were increased, and an opinion from Dr. Gauri Krishna was advised.
An earlier episode of a left focal convulsion occurred on 15/7/26; this was successfully aborted with lorazepam, and the corresponding labs sent during the episode were normal. All AEDs were adjusted to strict round-the-clock timings. Zonisamide and perampanel were subsequently added following consultation with the paediatric neurologist.
A subsequent paediatric neurology reviews suggested tapering and stopping topiramate, alongside increasing the zonisamide dose. A further review by Dr. Gauri Krishna was also advised.
On 28/7, the child had another seizure lasting less than 30 seconds, which was aborted with lorazepam; dosages were adjusted accordingly after Dr Shalini mam opinion.
Renal Function
Urine output remained adequate throughout the PICU stay, and Renal Function Tests (RFTs) remained within normal limits. A low-dose furosemide infusion was started during a period of fluid overload and was stopped after one day once a satisfactory negative fluid balance was achieved. Thereafter, the child passed urine adequately. Regular RFTs were conducted due to the prolonged antibiotic course and remained normal throughout her stay.
Liver Function
Liver Function Tests (LFTs) initially showed worsening bilirubin and transaminase levels with rising GGT. A bedside ultrasound of the abdomen and pelvis indicated acute hepatic parenchymal changes. Valproate levels sent concurrently were in the high-normal range, prompting its discontinuation. Liver function gradually improved thereafter. There were no bleeding manifestations, and the coagulation profile and serum ammonia levels were normal. No further LFT derangements were noted.
Sepsis / Infectious Disease
Initial labs showed isolated thrombocytopenia (platelet count 1.01 lakh/µL), with other parameters within the normal range. The child was started on injectable piperacillin-tazobactam, amikacin, and oseltamivir. Subsequent labs indicated a downward trend in total leukocyte counts and platelets. An ET Bio Fire panel grew Acinetobacter baumannii and Pseudomonas species with NDM and VIM resistance genes. Antibiotics were escalated to polymyxin and aztreonam due to worsening tachypnoea and fever spikes, with tigecycline added later.
A chest X-ray showed fluffy infiltrates alongside persistent fever and thrombocytopenia, raising suspicion for a fungal infection, so amphotericin B was added. An ET culture grew Carbapenem-Resistant Acinetobacter Baumannii (CRAB), prompting the addition of sulbactam for persistent symptoms. Sulbactam was stopped once repeat cultures turned negative. Given the underlying syndrome, immunocompromised state, and worsening lung disease, cotrimoxazole prophylaxis was escalated to a high treatment dose.
Subsequent cultures grew Burkholderia cepacia. Antibiotics were changed to ceftazidime-avibactam and aztreonam, which were continued for 2 weeks due to the ongoing lung disease. Injectable polymyxin B was stopped once repeat blood cultures showed no growth.
With the onset of new tachypnoea, vancomycin was added for gram-positive coverage. A subsequent flu panel and ET culture grew Acinetobacter species and Non-Tuberculous Mycobacteria (NTM). Anti-tubercular therapy (levofloxacin, amikacin, ethambutol) was initiated, and vancomycin was switched to linezolid for improved coverage. Species identification of the ET isolates confirmed Mycobacterium abscessus. Rifampicin was added once liver parameters improved.
Rifampicin and ethambutol were stopped once the species was confirmed as Mycobacterium abscessus. Repeat labs displayed a pancytopenia picture; an infectious disease opinion advised withholding linezolid and Septran while continuing other medications. Follow-up labs showed recovering cell counts, including an improving platelet count.
Antibiotic Profile
Summary of antimicrobial agents administered during the PICU course:
| Antibiotic / Agent | Start | Stop | Duration | Indication |
|---|---|---|---|---|
| Inj.Piperacillin-Tazobactam | 29/6 | 30/6 | 2 days | Empirical, pneumonia |
| Inj. Amikacin | 29/6 | 30/6 | 2 days | Empirical, pneumonia |
| Inj. Fluconazole | 29/6 | 30/6 | 2 days | Antifungal prophylaxis |
| Syp. Oseltamivir (Fluvir) | 29/6 | 30/6 | 2 days | Empirical antiviral cover |
| Inj. Colistin | 30/6 | 30/6 | 1 day | MDR gram-negative cover |
| Syp.Cotrimoxazole (prophylactic) | 1/7 | 6/7 | 6 days | PJP prophylaxis |
| Inj. Polymyxin B | 30/6 | 13/7 | 14 days | CRAB / NDM-VIM resistant organisms |
| Inj.Aztreonam (1st course) | 30/6 | 1/7 | 2 days | MDR gram-negative cover |
| Inj. Tigecycline | 2/7 | — | Ongoing | MDR gram-negative cover |
| Inj. Amphotericin B | 3/7 | 12/7 | 10 days | Suspected fungal infection |
| Inj. Sulbactam | 4/7 | 8/7 | 5 days | CRAB |
| Inj.Cotrimoxazole (treatment) | 7/7 | 31/7 | 25days | Escalated prophylaxis → treatment dose |
| Inj.Ceftazidime-Avibactam | 6/7 | 20/7 | 15 days | Burkholderia cepacia |
| Inj.Aztreonam (2nd course) | 6/7 | 20/7 | 15 days | Burkholderia cepacia |
| Inj. Vancomycin | 13/7 | 16/7 | 4 days | Gram-positive cover, later switched |
| Inj. Amikacin (ATT) | 14/7 | 11/8 | 29days | Atypical Mycobacteria (M. abscessus) |
| Inj. Levofloxacin | 14/7 | — | Ongoing | |
| (Orally from 27/7) | Atypical Mycobacteria (M. abscessus) | |||
| Tab. Ethambutol | 14/7 | 28/7 | 15 days | Atypical Mycobacteria (M. abscessus) |
| Inj. Linezolid | 16/7 | 31/7 | 17days | Switched from vancomycin; withheld due to thrombocytopenia |
| Tab. Rifampicin | 21/7 | 28/7 | 7days | Atypical Mycobacteria (M. abscessus), started after LFTs improved |
| Syp. Azithromycin | 31/7 | — | Ongoing | Atypical Mycobacteria (M. abscessus) and immunomodulation effect |
Microbiology reports
| Date | Sample / Test | Result |
|---|---|---|
| 29/6/26 | ET culture | Acinetobacter baumannii |
| 29/6/26 | Flu / respiratory panel | Acinetobacter baumannii and Pseudomonas sp. |
| 29/6/26 | Urine culture | No growth |
| 1/7/26 | Fungal stain, ET secretions | No fungal elements seen |
| 1/7/26 | Blood culture | Burkholderia cepacia |
| 3/7/26 | ET culture | Gram-positive cocci (normal flora) |
| 9/7/26 | Blood culture | No growth |
| 13/7/26 | Blood culture | No growth |
| 14/7/26 | Gram stain, ET secretions | Gram-positive cocci with moderate gram-negative bacilli |
| 14/7/26 | AFB stain, ET secretions | Positive |
| 14/7/26 | PCP PCR | Negative |
| 14/7/26 | Flu / respiratory panel | Acinetobacter baumannii |
| 14/7/26 | ET culture | Atypical Mycobacterium |
| 14/7/26 | PCR / molecular ID | Mycobacterium abscessus |
Haematology
The child received a Packed Red Blood Cell (PRBC) transfusion due to low haemoglobin and to optimize oxygen-carrying capacity while on HFOV. She also received platelet transfusions on three separate occasions for thrombocytopenia. Follow-up routine labs later revealed a drop in haemoglobin, prompting an additional PRBC transfusion. A peripheral smear did not show any significant changes.
Ophthalmology
The child developed exposure keratitis, prompting an ophthalmology consultation. Lacrigel, moxifloxacin eye drops, and lubricant (Refresh) eye drops were advised, leading to clinical improvement. The lubricants were discontinued once the condition sufficiently improved.
Metabolic
The child had persistent hypokalemia throughout much of the PICU stay, which was successfully corrected with oral and intravenous supplementation. Renal function remained normal throughout. Vitamin D3, B12, and serum ammonia levels were all normal.
Dr. Gauri Krishna (Paediatric Geneticist) was consulted following the neurology review. She advised stopping the combined metabolic cocktail and administering the individual components separately at higher doses. She also recommended sending a plasma amino acid profile and performing an MRI of the brain with spectroscopy.
Serum ammonia and folate levels were normal, but lactate was on the higher side. The initial reticulocyte count was normal (1.2%), and a peripheral smear was suggestive of normocytic normochromic anaemia. A repeat reticulocyte count, obtained post-transfusion, was in the higher range.
The plasma amino acid profile returned abnormal, and a repeat assessment of ammonia and lactate was subsequently normal. Urine for orotic and organic acids was sent and suggested an ornithine transcarbamylase (OTC) deficiency. Dr. Gauri Krishna advised close follow-up and planned for a re-analysis of the existing genetic data. Consequently, the parents were instructed to collect the raw genetic data from the MedGenome lab to forward to her; this process is currently underway.
Gastrointestinal Tract (GIT)
The child experienced intermittent episodes of loose stools during her stay, which were managed symptomatically. Perianal rashes are present but show a reducing trend.
Nutrition
The child was initially kept NPO with restricted intravenous fluids. Enteral feeds were introduced on Day 5 of admission and advanced gradually. The feeds were well tolerated and steadily stepped up. Daily weight gain was monitored and dietician reference taken for the same. Currently, she is tolerating 150 ml via PEG tube every 3 hours as advised, along with nutritional supplements.
Lines / Invasive Catheters
| Line / Catheter | Secured | Removed | Duration |
|---|---|---|---|
| Right femoral CVP line | 1/7/26 | 7/7/26 | 7 days |
| Left arterial line | 2/7/26 | 14/7/26 | 13 days |
| Left femoral CVP line | 7/7/26 | 22/7/26 | 16 days |
| Foley's catheter (1st) | 2/7/26 | 11/7/26 | 10 days |
| Foley's catheter (2nd) | 11/7/26 | 17/7/26 | 7 days |
| PICC line (Right basilic vein) | 5/7/26 | Discharged with PICC Line | - |
The PICC line was secured in the OR under the guidance of an interventional radiologist. An X-ray was performed to confirm proper placement. The patient was advised to continue BIPAP Support, PEG feeding, PICC Line care and Tracheostomy care, along with prescribed antibiotics and Medications as per discharge plan.
Discussion
This case demonstrates the complexity of managing a critically ill child with an underlying neurological disorder and chronic respiratory support requirements. The child’s Dravet syndrome, tracheostomy, impaired airway protection, and suspected aspiration were important predisposing factors for recurrent respiratory infections and pneumonia.
Initially, the child required BiPAP support. Chest radiography showed bilateral infiltrates, and antimicrobial therapy was initiated. Respiratory cultures subsequently identified carbapenem-resistant Acinetobacter baumannii and Burkholderia cepacia, highlighting the difficulty of treating healthcare-associated and multidrug-resistant infections. Management therefore required infectious disease consultation and appropriate antimicrobial selection based on microbiological findings.
Despite initial management, the child developed increasing work of breathing and worsening respiratory distress. Progression of bilateral infiltrates and ABG findings indicated worsening respiratory failure and ARDS. Escalation from non-invasive ventilation to invasive mechanical ventilation was therefore required. With increasing ventilatory requirements, HFOV was initiated, demonstrating the need for timely escalation of respiratory support in severe paediatric ARDS.
The child’s prolonged and complicated clinical course also illustrates the importance of multidisciplinary collaboration. Involvement of paediatric pulmonology, infectious disease, paediatric neurology, ENT, and genetics allowed different aspects of the child’s condition to be addressed. Management of seizures, airway and respiratory problems, infection, nutritional considerations, and the suspected metabolic/genetic condition required coordinated care.
Another important aspect is the prevention of recurrent aspiration and infection. In children with neurological impairment and tracheostomy, careful airway management, secretion clearance, aspiration precautions, appropriate feeding practices, infection-control measures, and regular reassessment of respiratory status are essential.
Overall, this case emphasizes that children with complex neurological and respiratory conditions can deteriorate rapidly during infections. Early identification of respiratory deterioration, serial clinical and radiological assessment, appropriate microbiological investigations, antimicrobial stewardship, lung-protective ventilation, and multidisciplinary decision-making are essential components of successful critical care management.
Conclusion
This case highlights the severe complications that can occur when a child with Dravet syndrome and chronic respiratory support develops pneumonia, particularly in the presence of aspiration risk and multidrug-resistant organisms. The progression from pneumonia to sepsis, septic shock, and severe ARDS required rapid escalation of respiratory support, including invasive ventilation and HFOV.
The case reinforces the importance of early recognition of deterioration, timely escalation of respiratory support, culture-guided antimicrobial therapy, aspiration prevention, and coordinated multidisciplinary management. Continuous monitoring and individualized care are essential to improve outcomes in children with complex neurological and respiratory disorders.
