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Indian Pediatr 2013;50: 242-243 |
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Respiratory Flutter Syndrome in a Neonate
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Nalinikanth Panigrahy, Poddutoor Preetham Kumar and Dinesh Kumar Chirla
From Department of Neonatology, Rainbow Children’s
Hospital and Perinatal Centre, Hyderabad. Andhra Pradesh, India.
Correspondence to: Dr. Poddutoor Preetham Kumar,
Department of Neonatology, Rainbow Children’s Hospital and Perinatal
Centre, Hyderabad. Andhra Pradesh, India.
Email: [email protected]
Received: July 04, 2012;
Initial review: August 21, 2012;
Accepted: August 31, 2012.
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Respiratory flutter or diaphragmatic flutter is a rare disorder
characterized by involuntary, high frequency contractions of the
diaphragm. Only 7 cases are reported in infants till date. The present
case presented with life threatening respiratory distress immediately
after birth. In view of high respiratory rate of 120-154 per minute,
clinical and fluoroscopic evidence of diaphragmatic contraction and
absence of any obvious CNS, cardiovascular and respiratory pathology,
respiratory flutter was diagnosed. It was also associated with dysphagia
and laryngomalacia. The patient was managed with prolonged continuous
positive airway pressures (CPAP) with partial success, but symptoms
improved with use of chlorpromazine.
Key words: Diaphragmatic flutter, Newborn,
Respiratory distress.
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Respiratory or diaphragmatic flutter
(DF) is a heterogeneous neurologic disorder, rarely reported
in neonates, infants and children. It is characterized by
rapid, involuntary high frequency contractions of the
diaphragm (35-480/min), often superimposed on normal
diaphragmatic excursion; and considered to be a form of
myoclonus [1-3]. The reported respiratory patterns of DF are
highly variable. It may occur in inspiration, expiration or
in both phases of respiration, generally asynchronous with
heart rate and usually resolves during sleep, but may
persist within all stages of sleep. The duration of DF may
vary from days to 18 years but typical to this disease, gas
exchange (CO2
and O2)
abnormalities are absent. Since the first case report in
1723, a case of self diagnosis by Leeuwenhoek [4], only 7
cases are reported in infants [2, 3, 5]. We report an infant
with diaphragmatic flutter associated with dysphagia and
laryngomalacia.
Case Report
This full term male baby was delivered by
elective caesarian section with birthweight of 3100g to a
non-consanguineously married 23 years old primipara with no
antenatal risk factors and normal antenatal ultrasound
scans. Baby cried immediately after birth. At 2 hours of
life, baby was shifted to NICU for excessive drooling of
saliva and respiratory distress. Baby was intubated and
ventilated at the referral NICU for 5 days, but in view of
extubation failure twice, he was referred to our unit on 6 th
day of life. On examination, baby was hemodynamically stable
and systemic examination was normal. He was lethargic, tone,
and reflexes were poor, and pupils were normal. Baby was
ventilated on SIMV mode, weaned and extubated to CPAP over
next 48 hours. During ventilation, his respiratory rate was
very high, irrespective of SIMV rate and level of sedation.
In view of history of extubation failure, retrognathia and
excessive pooling of secretions and recurrent upper lobe
collapse of lungs, airway evaluation was done. Direct
laryngoscopy revealed moderate laryngomalacia and omega
shaped epiglottis; bronchoscopy was normal. Expanded newborn
screening with immunoreactive trypsinogen (IRT) was normal.
MRI brain, nerve conduction study and electromyography were
normal. Sepsis work-up was negative. 2D echo cardiography
was normal. During CPAP, respiratory distress persisted with
a biphasic pattern of respiratory rate ranging from 40 – 154
per minute. Excessive secretions and respiratory distress
was persistent even after 10 days of CPAP use. During
clinical observation, it was noted that baby was "appearing
frightened". Respiratory rate monitored with impendence
plethysmography (Philips Intellivue MP 40 monitor) revealed
a respiratory rate of >150 per minute. Video recording of
respiratory movement showed high frequency like wiggle of
chest and abdomen with interposed normal breathing.
Video-fluoroscopic study showed a breathing rate of 160
breaths per minute with oscillatory movement of both sides
of diaphragm. It also revealed oropharyngeal dysphagia
characterized by a delay in swallow onset and silent
aspiration in absence of cough and slow progression of dye
in esophagus. Secretions decreased with glycopyrrolate but
respiratory distress persisted. Characteristically, through
out the course of 10 days of CPAP, arterial blood gases
(ABG) were normal.
In view of high frequency intermittent
contraction of diaphragm as evidenced by clinical
observation impendence plethysmography monitor and
fluoroscopy recording, diaphragmatic flutter was diagnosed.
In view of poor response to continuous CPAP use,
chlorpromazine in a dose of 1.5mg/kg/day in three divided
doses was started through nasogastric tube. Clinical
improvement was noticed over next 48 hours and infant was
weaned from CPAP. Follow-up at 1 month was normal with no
recurrence. Subsequently the child was lost to follow-up.
Discussion
Diagnosis of DF is by visual observation
of diaphragmatic flutter, fluoroscopic imaging, impendence
pneumography, respiratory inductive plethysmography (RIP)
and diaphragmatic electromyography. DF is difficult to
diagnose in infants by physical examination owing to the
normal rapid respiratory rate of infants and difficulty in
differentiating superimposed flutter waves which may be in
the frequency of heart rate. Fluoroscopy can differentiate
diaphragmatic contrations from heart rate [5]. RIP with a
frequency response up to 1800 per minute accurately
diagnoses high frequency DF. Impendence pneumography, though
commonly used in infants, as in our case, has limitation
that it may not detect respiratory rate above 180 per
minute.
Three types of breathing pattern have
been reported in DF. These are tachypnea, dirythmic
breathing superimposed with high frequency waves and with
apnea [6]. In the present case high frequency intermittent
contraction of the diaphragm superimposed on ordinary
respiratory rhythm i.e. "dual rhythms", confirmed by
fluoroscopy and being asynchronous with heart rate ensured a
diagnosis of diaphragmatic flutter.
A complex interaction between swallowing
and medullary breathing centre in brainstem normally
co-ordinates breathing and swallowing. Our infant had
dysphagia supporting the concept of common brainstem
dysfunction seen in these babies. However, normal MRI brain
suggests functional rather than gross anatomic lesion. The
response of DF to different therapies is variable. A range
of therapies are described including diazepam, carbamazepine,
phenytoin, chlorpromazine and even phrenic nerve-crush [2].
Poor response to CPAP prompted us to treat with
chlorpromazine, a centrally acting dopamine antagonist. This
treatment resulted in marked reduction in DF and allowed us
to wean from CPAP within few hours. No side-effects of
chlorpromazine were observed during follow-up.
Contributors: NP was responsible for
data collection and designed the manuscript; PPK: was
responsible for drafting the paper; he will act as guarantor
of the study and CDK helped in manuscript writing. The final
manuscript was approved by all authors.
Funding: None; Competing interests;
None stated.
References
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2. Katz ES, Gauda E, Crawford T, Ogunlesi
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Respiratory flutter syndrome an underrecognized cause of
respiratory failure in neonates. Am J Respir Crit Care Med.
2001;164:1161-4.
3. Riordan LL, Eavey RD, Strieder DJ.
Neonatal diaphragmatic flutter. Pediatr Pulmonol.
1990;8:209-11.
4. Phillips JR, Eldridge FL. Respiratory
myoclonus (Leeuwenhoek’s Disease). N Engl J Med.
1973;289:1394-5.
5. Adams JA, Zabaleta IA, Sackner MA.
Diaphragmatic flutter in three babies with bronchopulmonary
dysplasia and respiratory syncytial virus bronchiolitis.
Pediatr Pulmonol. 1995;19:312-6.
6. Tamaya S, Kondo T, Yamabayashi H. Diaphragmatic
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