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intensive care unit (ICU) stay at birth for congestive heart failure
related to patent ductus arteriosus (PDA) and ventriculoseptal
defect (VSD). She underwent PDA coiling at 9 months of age. Her
VSD closed spontaneously. She had no history of cleft lip/palate or
any other craniofacial anomalies though her facial features were
noted to be dysmorphic. Genetics work up was negative for the
22q11 deletion. Speech pathology evaluation revealed severe
nasality and speech delay consistent with VPI. She underwent two
years of speech therapy with little improvement prior to ENT
evaluation. Our initial exam was significant for 3+ tonsils and
moderate adenoid tissue. Nasopharyngoscopy demonstrated good
coronal closure but persistent 2–3 cm central gap with attempted
sagittal velopharyngeal closure. Our patient underwent tonsillec-
tomy without adenoidectomy 2 months prior to VPI repair.
Based on the patient’s preoperative nasopharyngoscopy, which
noted a central gap on sagittal closure, pharyngeal flap surgery was
expected to provide increased tissue in the sagittal place of the
nasopharynx and improvement in her VPI. The resulting nasal
ports could be closed with considerably less sagittal movement of
the lateral pharyngeal wall during swallowing and speech. The
patient underwent
a superior-based pharyngeal flap in the
Fig. 1. Plain anterior/posterior (AP) CXR. Arrow indicates mild pneumomediastinum
and pneumopericardium. The CXR also demonstrates opacification in the left lower
and middle lobes and right lung base.
operating room. General endotracheal (ET) anesthesia was induced
per the pediatric anesthesia team. The technique was, briefly, as
follows: two vertical incisions were made in the posterior
pharyngeal wall from the level of C1 to the larynx. They were
connected with a horizontal incision at the larynx and the flap was
elevated in the prevertebral plane. The flap was inset into the soft
palate and the flap donor site was partially closed with vicryl
interrupted sutures and over-sewn uvula. The nasopharyngeal
ports were noted to be 4 Â 5 mm bilaterally and patent. The
patient was extubated without difficulty but did require positive
pressure mask ventilation after ET tube removal to maintain
adequate oxygen saturations. The procedure was without intra-
operative complication and the patient was transferred to the
pediatric ICU (PICU) for recovery with a tongue stay suture in place
and on room air.
After these interventions, the patient’s oxygen saturations
improved to >90% and she tolerated weaning of ventilator settings.
The patient continued to require dopamine infusion on POD#3
for hypotension and bradycardia. She continued to have desatura-
tions despite FiO2 80% and gentamycin was added to her
therapeutic regimen for radiographic evidence of aspiration. An
orogastric tube was placed easily by pediatric otolaryngology and
enteral feeds were initiated. The patient required placement of a
second chest tube on POD#4 for new development of right-sided
pneumothorax associated with worsening cervical subcutaneous
emphysema. Her respiratory status subsequently improved and
In the PICU, the patient received IV clindamycin 10 mg/kg q8 h,
dexamethasone 1 mg/kg q8 h, and morphine 0.1 mg/kg q2 h as
needed. Within hours of arrival to the PICU, she began having
desaturations to the high 70 s. She had difficulty tolerating her oral
secretions and required frequent suctioning. Chest X-Ray (CXR)
demonstrated mild pneumopericardium (Fig. 1). She was placed on
a humidified face tent with O2 4 l/min and a precedex drip was
started per the PICU staff. On the morning of post-operative day
(POD) #1, the patient was tachypneic to >60 breaths/min. She
received respiratory treatments but her saturations and respirato-
ry rate remained labile; her oxygen requirement continued to
increase.
Early in the morning of POD#2, the patient became increasingly
lethargic with increased work of breathing and desaturations to
the 70 s. CXR revealed cervical emphysema, pneumomediastinum,
pneumocardium, small bilateral pneumothoraces, and bilateral
airspace disease (Fig. 2). Cardiopulmonary resuscitation was
started. Her oxygen saturations did not improve despite positive
pressure mask ventilation. The decision was made to reintubate
the patient as she continued to deteriorate through the resuscita-
tive efforts. Maximal ventilator settings (FiO2 100%, postitive end-
expiratory pressure (PEEP) 8, peak pressure 30 s) yielded oxygen
saturations in the 80 s. Flexible bronchoscopy was performed via
the endotracheal tube by pediatric otolaryngology; mucus
plugging was not identified and removal of ventilator support
caused the patient to rapidly desaturate to the 40 s. A dopamine
drip was started for hypotension. Pediatric cardiology was
consulted and an echocardiogram revealed excellent cardiac
function without evidence of tamponade physiology. Pediatric
surgery placed a left-sided chest tube to relieve the pneumothorax.
Fig. 2. Plain AP CXR Thick arrow indicates pneumomediastinum and
pneumopericardium. Dashed arrows indicate small bilateral pneumothoraces.
Fine arrows indicate subcutaneous emphysema in the supraclavicular soft tissues of
the neck bilaterally.
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