Miguel Guelfand · EUPSA Webinar "GIANT OMPHALOCELE II"
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Video64 min·Published Jun 2024Older

EUPSA Webinar "GIANT OMPHALOCELE II"

With Dr. Marlinda Van der Kamp & Dr. Miguel Guelfand & Dr. Hester Langenfeld · hosted by Dr. Augusto Zani
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What the experts said42 expert statements · 1 host summary
The non-surgical silo technique was developed by Dr. Cristóbal Avello from Colombia approximately 20 years ago and modified by Dr. Guelfund's team starting 7 years ago
ClinicalMiguel Guelfand
In the series of 50 patients, 98% required only one surgery for closure
ClinicalMiguel Guelfand
The silo should be placed as soon as possible after birth to prevent the amnion from drying, which facilitates reduction
ClinicalMiguel Guelfand
The technique uses Duoderm hydrocolloid dressing shaped as a T, with the stem attached to the patient's skin and the flaps wrapped around the omphalocele
ClinicalMiguel Guelfand
Plastic umbilical cord clamps should be replaced with suture or elastic band, as the umbilical cord is not always the apex of the omphalocele
ClinicalMiguel Guelfand
The silo is reduced by 0.5 inch (1 centimeter) every day or every other day depending on the patient's respiratory condition
ClinicalMiguel Guelfand
In the first 2-3 years of using the protocol, patients were kept completely paralyzed and intubated; in the last 4-5 years, the protocol changed to sedation only with paralysis used only during silo reduction
ClinicalMiguel Guelfand
Amnion inversion is performed when the silo is completely flat, typically within 1-10 days, to test how the baby will tolerate primary closure
ClinicalMiguel Guelfand
95% of patients achieved anatomical closure without mesh; approximately 2-3 patients required mesh reinforcement and one required Gore-Tex mesh for closure
ClinicalMiguel Guelfand
In the 50-patient series, median silo reduction time was 5 days, amnion inversion time was 5 days, and time to closure was 12 days
ClinicalMiguel Guelfand
Complications in the series included one hemoperitoneum (due to delayed silo placement on day 3-4 with dry amnion), three local infections treated with antibiotics, one high intra-abdominal pressure (resolved by retrieving reduction 1cm), and one central line infection
ClinicalMiguel Guelfand
There was no mortality related to the silo technique; four mortalities occurred related to other malformations
ClinicalMiguel Guelfand
The Rotterdam case was a 35-week, 2400g female with giant omphalocele (8cm) containing liver, stomach, and small bowel, plus cardiac defect
ClinicalHester Langenfeld
In the Rotterdam case, bladder pressure was 17 with some urination beside the catheter, and there was bulging of the Duoderm on the side
ClinicalHester Langenfeld
The spatula moved upward on the liver side because tape at the ends caused the middle to distend when pressed
ClinicalHester Langenfeld
On day 5, the omphalocele had yellow appearance but did not look infected; cultures were negative initially
ClinicalHester Langenfeld
The amnion leaked fluid, but cultures remained negative until later in the course
ClinicalHester Langenfeld
Enteral feeding via nasogastric tube was started on day 12 while the defect was still large
ClinicalHester Langenfeld
Botox was administered on day 15 due to concerns about achieving primary closure
ClinicalHester Langenfeld
The patient was ventilated but not paralyzed throughout treatment, with CO2 consistently high at 8-10
ClinicalHester Langenfeld
Serratia infection occurred on the skin/sac but the patient was not systemically ill and did not receive antibiotics
ClinicalHester Langenfeld
Attempted amnion inversion on days 23-30 resulted in blue legs, tachycardia, and CO2 rising to 12, requiring abandonment of closure attempt
ClinicalHester Langenfeld
The patient had lung hypoplasia contributing to inability to tolerate closure
ClinicalHester Langenfeld
On day 30, treatment was switched to Mepitel (silicone dressing), after which the patient was extubated 10 days later (day 40)
ClinicalHester Langenfeld
By day 45, the patient was on Optiflow, drinking 20mL per feeding three times daily, with chronic hypercapnia (CO2 of 8)
ClinicalHester Langenfeld
Clinical signs are used to guide progression rather than direct intra-abdominal pressure measurement; the clinician observes the patient for 10-20 minutes after each reduction
ClinicalMiguel Guelfand
When progression is inadequate, complete paralysis for 24 hours over 7 days is used to facilitate reduction
ClinicalMiguel Guelfand
The amnion is not cleaned during dressing changes; only the skin is cleaned to ensure good traction of the Duoderm
ClinicalMiguel Guelfand
The silo is changed every 4-5 weeks because it loses traction as it gets wet
ClinicalMiguel Guelfand
Patients are not fed during the reduction phase to avoid bowel distention
ClinicalMiguel Guelfand
Amnion inversion should not be attempted until the silo is completely flat, as premature inversion will not be tolerated
ClinicalMiguel Guelfand
A protocol is being developed to use Botox from day one, and one patient has been treated with in utero Botox in Colombia
ClinicalMiguel Guelfand
The technique is a pushing mechanism, not pulling; all force goes into the abdomen rather than using traction to the ceiling
ClinicalMiguel Guelfand
The technique requires thoughtful discussion with NICU doctors and nurses about goals and time frames; if no progress occurs within 7 days, the approach should be changed
ClinicalMiguel Guelfand
Three patients with ruptured amnion at birth were treated by suturing the amnion and then placing the silo on top
ClinicalMiguel Guelfand
For narrow-neck mushroom-shaped defects, the technique can be attempted, but if no progression occurs within 7-10 days, the approach should be changed
ClinicalMiguel Guelfand
Component separation has been performed in 3 patients between 2 and 4 weeks of age with good results and no lateral hernias
ClinicalMiguel Guelfand
The first component separation cases were performed with a plastic surgeon experienced in the technique in larger patients
ClinicalMiguel Guelfand
For patients with severe pulmonary hypoplasia or cardiac conditions, the silo can be placed but reduction delayed for 5-7 days until the patient stabilizes, then progression is very slow and gentle
ClinicalMiguel Guelfand
Reduction rate varies by patient: some can tolerate 2 centimeters per day, others only 1 centimeter per week, depending on comorbidities and development of pulmonary hypertension
ClinicalMiguel Guelfand
Without sedation, the baby will continue putting pressure on the silo, and if feeding is started, bowel distention will occur, making the process take years rather than weeks
ClinicalMiguel Guelfand
Two centers in the US (Boston and Nationwide) have adopted the technique over the last 2 years with good results after virtual consultation for initial cases
ClinicalMiguel Guelfand
According to literature, patients with giant omphalocele and lung hypoplasia requiring long-term ventilation have a median ventilation time of 100 days
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