Diaphragmatic paralysis

Federico Minen
Andreu Roca Bajona
30th September 2016
The story of JM

❖ Antenatal diagnosis of TGA with IVS and ?ASD

❖ Born at C&W via ventouse on 23/08
❖ Deeply desat after birth (pre-ductal 18%) -> started on Prostin infusion
❖ Postnatal Echo: no ASD -> failed attempt of BAS.
❖ Transferred to us, at arrival pre ductal sats 40-65% on prostin infusion.
❖ BAS 12 hrs after birth with quick improvement in sats
Pre-op

❖ D3: ?NEC (large bowel loops on AXR, high lactate) -> PipTaz + Gent, kept
intubated and NBM
❖ D5: extubated
❖ D11: Arterial switch operation + coronary arteries translocation + ASD closure
(direct suture)
❖ Prolonged bypass time due to abnormal coronary anatomy - single coronary
origin from Ostium 2
❖ 1 LA line, Atrial pacing wires, 4 drains: pericardial, mediastinal, right and left
pleurals, 1 PD cath
Clinical improving

❖ Post-op Echo: moderate BVF

➢ LV function moderately impaired
➢ RV impaired with high pressures -> iNO

❖ D14: iNO stopped

❖ D17: chest closure.
❖ D19: switched to CPAP. CXR: left lung more congestive. Pleural drains 10-20
mls each
❖ D20: extubated onto nCPAP, tolerating but FiO2 increased to 30%
❖ D21: L drain lost accidentally
Worsening again

❖ D22: off CPAP but after 12 hrs deterioration -> CPAP

❖ D23: CXR lung collapse with high left hemidiaphragm -> BiPAP
❖ D25: Lung USS -> Paradoxical movement of the Lt diaphragm
❖ D30: reintubated (tachypnoea, increased WOB) ->left diaphragm plication
❖ D31: CRE sepsis (R drain swab + BC)
❖ D33: extubated onto nCPAP
The story of EP

❖ Born at Luton 23/08

❖ Postnatal diagnosis of suspected TAPVD -> transferred to RBH D3 of age
❖ Echo at arrival:
➢ 4 pulmonary veins join to form a confluence and descend via vertical vein and join the IVC to
drain to the right atrium. Suspicion of obstruction in the liver.
➢ Interatrial communication with a R>L shunt
➢ Severe TR with a PG of 71 mmHg (systolic blood pressure 45)
➢ Right ventricle significantly dilated
➢ Small PDA shunting right to left
Post-op

❖ D4: TPVPC repair, ASD patch closure

❖ 4 drains: pericardial, mediastinal, right and left pleurals (+ PD)
❖ Platelet induced pulm. hypertension episode -> iNO started
❖ D7: left pneumothorax, quickly resolving
❖ D12: skin closure
❖ D14 chest closure - chylothorax
❖ D18: commenced on PipTaz and Teic for suspected sepsis (Klebsiella growing
from ETT secretions)
❖ D20: desats, increased FiO2 -> CXR: left lung “worsening”. Poor transmission of
sounds in the left base. USS: small pleural fluid left base (0.4cm).

Left pleural drain migrated -> self-removed.

❖ D24: still ventilator dependant.

➢ ?paradoxical movement when on CPAP-bag
➢ USS: Lt diaphragmatic movement diminished but not paradoxical -> incomplete palsy

❖ D27: USS: Lt hemidiaphragm paresis, not moving with inspiration

❖ D32: left hemidiaphragm plication
❖ D36: extubated onto nCPAP
A little bit of theory...
The septum transversum is first recognizable at the third week
of gestation as a mass of mesoderm located ventral to the
cervical somites. During the fourth week, it descends and
partially separates the thoracic and peritoneal cavities. The
openings that remain between the thorax and abdomen are the
pleuroperitoneal ducts.
During the fifth week of gestation, myoblasts and neuronal
elements from the third, fourth, and fifth cervical somites
penetrate the developing diaphragm as the downward migration
of the septum transversum continues. As a result, the phrenic
nerve is comprised of the third, fourth, and fifth cervical nerve
roots [2]. The migration continues until the eighth week of
gestation. At that time, the septum transversum fuses with the
pluripotential membranes and the mesenchyme ventral to the
esophagus, closing the pleuroperitoneal ducts.
DIAPHRAGM PHYSIOLOGY

- It decreases intrapleural pressure.

- It expands the rib cage by

generating positive intra abdominal
pressure.
Differences
● Flatter shape.

Laplace P = 2T / r
Differences
● Flatter shape.

● Highly compliant rib cage

Differences
● Flatter shape.

● Highly compliant rib cage

● Horizontal configuration of the ribs

 Impaired more in the prone than
supine position.
Differences
In one study, diaphragm thickness and
● Flatter shape. shortening were assessed by ultrasound in
healthy term infants.
● Highly compliant rib cage In the prone position, the diaphragm was
thicker and shorter at both end-expiratory and
end-inspiratory volumes, and diaphragm
● Horizontal configuration of the ribs shortening was greater during tidal breathing,
compared to the supine position. The reduced
resting diaphragmatic length in the prone
● Recumbent posture position would be expected to impair
diaphragmatic strength, while the additional
shortening during tidal breathing would
increase diaphragmatic work
Rehan VK, Nakashima JM, Gutman A, et al. Effects of the
supine and prone position on diaphragm thickness in
healthy term infants. Arch Dis Child 2000; 83:234.
Differences
● Flatter shape.

● Highly compliant rib cage

● Horizontal configuration of the ribs

● Recumbent posture

● Rib cage attachments

The attachments of the diaphragm to the rib cage render the neonatal diaphragm less efficient than that of the adult. In the newborn, muscle fibers from the anterior
diaphragm radiate laterally and attach to the xiphoid process and the seventh rib [7]. Fibers from the posterior diaphragm join the eleventh rib, while posterolateral fibers
blend with the transverse abdominal muscles. The large angle of insertion into the lateral wall limits the zone of apposition. As a result, most of the movement of the
neonatal diaphragm is in the posterior portion, in contrast to the piston-like movement of the adult diaphragm.
Gibson GJ. Diaphragmatic paresis: pathophysiology, clinical features, and investigation. Thorax 1989; 44:960.
Differences
● Flatter shape.

● Highly compliant rib cage

● Horizontal configuration of the ribs

● Recumbent posture

● Rib cage attachments

● Fiber tipe II < I

ETIOLOGY
● Birth injury
○ Brachial plexus palsy is associated in 80 to 90 percent of cases, while diaphragmatic paralysis occurs
in approximately 5 percent of brachial plexus injuries.

● Cardiothoracic surgery
○ 0.5-6% of cardiac surgeries (studies diffear).
■ Tetralogy of Fallot (31.5%)>>>BT shunt (11.1%)>>> VSD w PA plasty (11.1%)
■ Requiring plication: BT shunt (23.8%)>> ASO(19%), Fallot (11.9%).
Akay TH, Ozkan S, Gultekin B, Uguz E, Varan B, Sezgin A, et al. Diaphragmatic paralysis after cardiac surgery in children: Incidence, prognosis and surgical
management. Pediatr Surg Int. 2006;22:341–6.

● Other procedures:
○ Cannulation of the internal jugular or subclavian vein
○ Insertion of chest tubes
○ Other thoracic surgeries

● Neuromuscular disorders (rare)

Onset

- Suspected when the infant cannot be weaned from the ventilator or develops
respiratory distress after extubation

- Some infants present with complications of diaphragmatic paralysis:

- Atelectasis
- Pleural effusions
- Aspiration
- Respiratory infections
Signs
● Respiratory compromise

● Kienbock's sign: Paradoxical movement of the thorax

● "belly dancer's sign": the umbilicus moves toward the affected side during
inspiration

● Gastrointestinal symptoms.
○ Tiring during feedings
○ Left-sided diaphragmatic paralysis: frequent regurgitation
Diagnostic
Diagnostic

Echocardiography Fluoroscopy
Diagnostic
● Bedside
● No ionizing radiation
● Ultrasound has been
shown to be similar in Fluoroscopy
accuracy to most other
imaging modalities for
diaphragm ● Echography is more reliable in
assessment. Echocardiography detecting hemidiaphragms that
require surgical plication
Miller SG, Brook MM, Tacy TA. Reliability of two-dimensional echocardiograp
hy in the assessment of clinically significant abnormal hemidiaphragm motio
Ultrasonographic evaluation of diaphragmatic motion. Gerscovich EO, n in pediatric cardiothoracic patients: Comparison with fluoroscopy. Pediatr
Cronan M, McGahan JP, Jain K, Jones CD, McDonald CJ Ultrasound Med. Crit Care Med 2006; 7:441
2001 Jun; 20(6):597-604.

Diagnosis of abnormal diaphragm motion after cardiothoracic surgery: ultrasound performed by a cardiac intensivist vs. fluoroscopy. Sanchez de Toledo J, Munoz R, Landsittel D, Shiderly
D, Yoshida M, Komarlu R, Wearden P, Morell VO, Chrysostomou C Congenit Heart Dis. 2010 Nov-Dec; 5(6):565-72
DIAPHRAGMATIC USS

- Supine position

- Focus mainly on the posterior and lateral parts of the diaphragm

- which are the muscular crural components innervated by the phrenic nerve, rather than the anterior
central tendon seen in fluoroscopy, which moves 40% less with respiration
Invasively ventilated patients
- Spontaneous respiration to help identify the moving diaphragm
- Ultrasonographic Evaluation of Diaphragmatic Motion Eugenio O. Gerscovich, MD, Michael Cronan, RDMS, John P. McGahan, MD, Kiran
Jain, MD, C. Darryl Jones, MD,
Craig McDonald, MD. American Institute of Ultrasound in Medicine • J Ultrasound Med 20:597–604, 2001
- Necessary to briefly disconnect the ventilator to assess the patient's spontaneous respiratory efforts. Diaphragmatic paralysis: the use of M mode
ultrasound for diagnosis in adults. Lloyd T, Tang YM, Benson MD, King S. Spinal Cord. 2006 Aug;44(8):505-8. Epub 2005 Dec 6.

- In patients undergoing in assisted mechanical ventilation, diaphragm thickening is a

reliable indicator of respiratory effort, whereas diaphragm excursion should not be
used to quantitatively assess diaphragm contractile activity.
- Umbrello M, Formenti P, Longhi D, et al. Diaphragm ultrasound as indicator of respiratory effort in
critically ill patients undergoing assisted mechanical ventilation: a pilot clinical study. Critical Care.
2015;19(1):161. doi:10.1186/s13054-015-0894-9.
CPAP patients
The expected effect of PEEP/CPAP both in invasive and non-invasive ventilation is to
increase functional residual capacity (FRC), maintaining alveolar recruitment;

The corresponding increase in lung volumes lowers diaphragmatic dome.

This effect can result in a decreased diaphragmatic excursion, not related to

diaphragmatic dysfunction but to a caudal displacement of diaphragmatic dome at the end
of expiration.

The variable that better helps to quantify the impact of patient effort in the generation of
tidal volumes is the thickening fraction (TF = thickness at end-inspiration–thickness at
end-expiration/thickness at end-expiration)
Vivier E, Mekontso Dessap A, Dimassi S, Vargas F, Lyazidi A, Thille AW, Brochard L (2012) Diaphragm ultrasonography to
estimate the work of breathing during noninvasive ventilation. Intensive Care Med 38:796–803
 Sanchez de Toledo J, Munoz R, Landsittel D,
Shiderly D, Yoshida M, Komarlu R, Wearden P,
Morell VO, Chrysostomou C. Diagnosis of
Who can perform US? abnormal
cardiothoracic
diaphragm
surgery:
motion after
Ultrasound
performed by a cardiac intensivist vs.
fluoroscopy. Congenit Heart Dis. 2010 Nov-
Dec;5(6):565–572.
DESIGN:
Prospective study in consecutive pediatric patients with suspected abnormal diaphragmatic motion after cardiothoracic surgery. All patients
underwent fluoroscopy and ultrasound study of the diaphragm. Ultrasound was performed by a pediatric cardiac intensivist and a trainee. Kappa
statistic was calculated to assess concordance between both ultrasound readings. Sensitivity, specificity, and positive and negative predictive values
(PPV and NPV) were calculated to assess accuracy of each ultrasound test in predicting fluoroscopy results.

RESULTS:
Twenty-five patients with median age 3 months (12 days-11 years) and median weight of 3.8 kg (2.5-29 kg) were included. The ultrasound
diagnosis of the cardiac intensivist was perfectly accurate (100% sensitivity, specificity, and PPV and NPV) in predicting fluoroscopy results. The
ultrasound performed by the trainee achieved 85.7% sensitivity, 94.4% NPV, and 100% specificity relative to fluoroscopy. The interoperator reliability
of chest ultrasound was 0.89 (95% confidence interval 0.69-1). Delay between clinical suspicion and the diagnostic tests was 15 minutes (5 minutes-
2.5 hours) for ultrasound and 17 hours (60 minutes-82 hours) for fluoroscopy (P < 0.001).

CONCLUSIONS:
Chest ultrasound performed by cardiac intensivists allows for an early and accurate diagnosis of abnormal diaphragmatic motion, as evidenced by
their ability to predict fluoroscopy findings in pediatric cardiothoracic patients. Training in ultrasound-guided assessment of diaphragmatic
motion should be reinforced during pediatric cardiac intensive care fellowship.
How to perform the diaphragmatic US?

Sarwal A, Walker FO, Cartwright MS. Neuromuscular Ultrasound for Evaluation of the Di
aphragm. Muscle & nerve. 2013;47(3):319-329. doi:10.1002/mus.23671.
Management
1) Immediate diaphragmatic plication to reduce the need for mechanical ventilation,
duration of hospital stay and pulmonary infections

2) Diaphragmatic plication after 2–4 weeks, which is the usual time for spontaneous
recovery

3) Conservative, non-operative approach using prolonged mechanical ventilation, as

improvement can occur up to 2 months, thus avoiding operative/postoperative
complications
Spontaneous recovery timing
- Haller et al. suggested a trial of continuous positive airway pressure (CPAP) for 4-6 weeks
during which the diaphragmatic function is presumed to improve with conservative
management.
Haller JA Jr, Pickard LR, Tepas JJ, Rogers MC, Robotham JL, Shorter N, et al. Management of diaphragmatic paralysis in infants with
special emphasis on selection of patients for operative plication. J Pediatr Surg 1979;14:779-85.
- Watanabe et al reported recovery of the affected diaphragm between 5 and 51 days indicating
that recovery is an unpredictable phenomenon. Further follow-up suggested that 16% never
recovered.
Watanabe T, Trusler GA, Williams WG, Edmonds JF, Coles JG, Hosokawa Y. Phrenic nerve paralysis after pediatric cardiac surgey:
Restrospective study of 125 cases. J Thorac Cardiovasc Surg 1987;94:383-8.
- Iverson showed that in many cases of traumatic injury to the phrenic nerve, normal
diaphragmatic function could be expected to return after 6-12 months.
Iverson LI, Mittal A, Dugan DJ, Samson PC. Injuries to the phrenic nerve resulting in diaphragmatic paralysis with special reference to stretch
trauma. Am J Surg 1976;132:263-9.
- Mickell noted radiographic or fluoroscopic resolution of PNP in 95% of children up to 3.5 years
after operation
Agressive aproach
- Some authors recommend that diaphragmatic plication should be performed as soon
as the diagnosis of DP has been confirmed.
Stauffer UG, Rickham PP. Acquired eventration of the diaphragm in the newborn. J Pediatr Surg 1972;7:635-40. Back to cited text no. 24

Late surgical plication may be jeopardized by atrophy of the diaphragm which may even
preclude successful surgical plication.
Case based aproach
- Most authors recommend a waiting period of 1-6 weeks in anticipation of potential
spontaneous recovery.
Indications for diaphragmatic plication in children with diaphragmatic palsy after open-heart surgery

- Talwar S, Agarwala S, Mittal CM, Choudhary SK, Airan B. Diaphragmatic palsy after cardiac surgical procedures in patients with congenital heart. Annals of Pediatric Cardiology. 2010;3(1):50-57.
doi:10.4103/0974-2069.64370.
- Tonz M, von Segesser LK, Mihaljevic T, Arbenz U, Stauffer UG, Turina MI. Clinical implications of phrenic nerve injury after pediatric cardiac surgery. J Pediatr Surg 1996;31:1265-7.
- Serraf A, Planche C, Lacour Gayet F, Bruniaux J, Nottin R, Binet JP. Post cardiac surgery phrenic nerve palsy in pediatric patients. Eur J Cardiothorac Surg 1990;4:421-4.
- Watanabe T, Trusler GA, Williams WG, Edmonds JF, Coles JG, Hosokawa Y. Phrenic nerve paralysis after pediatric cardiac surgey: Retrospective study of 125 cases. J Thorac Cardiovasc Surg
1987;94:383-8
- Mickell JJ, Oh KS, Siewers RD, Galvis AG, Fricker FJ, Mathews RA. Clinical implications of postoperative unilateral phrenic nerve paralysis. J Thorac Cardiovasc Surg 1978;76:297-304.
Univentricular patients
The negative intrathoracic pressure assumes significance in ensuring optimal systemic
venous and pulmonary arterial circulation.

In the presence of DP, these patients have higher Fontan pressures, ↑ morbidity, pleural
effusions, ascites, duration of hospital stay...

These are likely to improve with early diaphragmatic plication as has been demonstrated
Amin Z, McElhinney DB, Strawn JK, Kugler JD, Duncan KF, Reddy VM, et al. Hemidiaphragmatic paralysis increases postoperative morbidity after a
modified Fontan operation. J Thorac Cardiovasc Surg 2001;122:856-62.

Ovroutski S, Alexi-Meskishvili V, Stiller B, Ewert P, Abdul-Khaliq H, Lemmer J, et al. Paralysis of the phrenic nerve as a risk factor for suboptimal
Fontan hemodynamics. Eur J Cardiothorac Surg 2005;27:561-5
Talwar S, Agarwala S, Mittal CM, Choudhary SK, Airan B.
Diaphragmatic palsy after cardiac surgical procedures in
patients with congenital heart. Annals of Pediatric
Cardiology. 2010;3(1):50-57. doi:10.4103/0974-
2069.64370.
Conclusions
DP is not uncommon following open-heart surgery in children

DP can be a cause of significant morbidity and mortality.

A high-index of suspicion is required for timely diagnosis and the management has to be
individualized depending on the overall clinical scenario.

Patients undergoing univentricular repair should have early diaphragmatic plication.

What could be improved in our cases?

- Do we explore diaphragmatic paralysis signs before extubation?

- Can diaphragm paralysis be confused for LTRI?
- Do we have to put patients on the bag to perform the UUSS if they are in CPAP?
- When to plicate a diaphragm?