Transcript Omphalocele Powerpoint

Gabriela Olivas
NNP II
GNRS: 5632
July 12, 2014
Objectives
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Outline maternal history
Maternal and fetal risks and complications
Discuss delivery and stabilization needed for infant
Review admission assessment and diagnostics of affected infant
Review admission diagnoses
Review etiology and pathophysiology of admission diagnoses
Outline initial plan of care
Discuss hospital course by systems
Explain medications used in infant’s treatment
Review pertinent theories and explore current evidence based practice
about Omphaloceles
 Discuss family interventions
 Outline infant’s discharge plan and follow-up
 Review summary
Maternal History
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Age: 21 Prenatal Care: yes. Where? UTMB clinic
Now G 2, P 2, Ab 0, LC 2
Blood Type/IAT: B positive/IAT negative
Prenatal Labs:
Syphilis IgG: negative
Hep Bs Ag: negative
GBS: negative
HIV: negative
Other Infections:
None
Social History:
None
Other Pregnancy Problems:
Anemia of mother in pregnancy, antepartum
Maternal/Fetal Risks &
Complications
 Maternal Anemia
 Fetus at increased risk for decreased RBC volume,
hemoglobin, iron stores, and cord ferritin levels and an
increased risk of iron deficiency anemia.
 Risk of Rupture of Omphalocele
 This increases risk of infection for fetus.
 Increases risk of intestinal necrosis, if intestines
becomes twisted or blood supply is decreased.
(Blackburn, 2013)
Delivery and Stabilization
 NICU team called to delivery due to Omphalocele with
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portion of liver, two vessel umbilical cord noted on
prenatal diagnostics
AROM at delivery with clear fluid.
Mode of Delivery: C-Section, Previous cesarean
Apgar's: 1 minute 7, 5 minutes 8
Resuscitation: basic stimulation and basic suction and O2
by face mask/nasal cannula (2L at 100%), likely TTN
Transition: respiratory distress, likely TTN, requiring
oxygen (2L at 100%)
Admission Assessment
 Birth Length: 49 cm
 Birth Head Circumference: 35.5
 Gestational Age: (Dates) Gestational Age: 38w4d
 (exam) Gestational Age: 39w
 Weight: 3550 g (7 lb 13.2 oz) (62%*, Z = 0.32)
 Vital signs
O2 sats: 99%
HR: 138
RR: 47
Temp: 37 degree Celsius
BP: 66/39 (47)
Admission Assessment continued
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General: active, in no distress and nasal cannula in place
Skin: well perfused without rashes or hematomas
Head and Neck: sutures open, fontanel soft, normal facies, palate intact
Eyes: red reflex intact bilaterally, no discharge
Chest/Lungs: symmetrical, breath sounds present and equal bilaterally and
wet crackles bilaterally
Heart: regular rate and rhythm, no murmur; pulses palpable
Abdomen: omphalocele present, approximately 8cmX8cm, including
intestines and liver
Cord: 2 vessels
Genitalia: normal external female genitalia
Extremities: no deformities, normal range of motion, hips stable, clavicles
intact
Neurologic: responsive to stimuli
Back: no defect, anus patent and normally placed
Diagnostics
 Babygram on admission: “The patient is rotated, the lungs are mildly
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hazy. No pneumothorax or pleural effusion is seen. The orogastric tube
terminates in the stomach. Midline abdominal soft tissue shadow is
seen likely represent the omphalocele.”
Blood Cultures drawn.
ABG:
ph: 7.36
Pco2: 45
P02:131
HCo3: 25
BE: -0.8
CBC:
WBC: 10.7
Hct: 39.2
Hgb: 13.5
Plt: 134
Segs: 32
Bands: 5
Lymphs:52
Monos: 5
Eos: 2
Lytes:
Na: 138
K: 5
CO2: 104
Cl:23
Mg: 1.8
Phos: 6
Gluc: 63
Ca: 9.7
BUN: 6
Cr: 0.7
Bili (uncon.): 4 Bili (con.): 0.7
Prenatal Diagnostics
 Omphalocele containing liver was seen on fetal
ultrasound on 04/29/14
 Mom declined amniocentesis.
(Velasco-Sanchez, 2007)
Primary Admission Diagnoses
 Term 38 week, 4 day appropriate for
gestational age female , DOL o
 Omphalocele
 Suspected Transient Tachypnea of the
Newborn
(Khan, Sabih, Thomas, MacDonald, & Chandramohan, 2013)
Etiology of Omphalocele
 Incidence: Small omphaloceles occur with a rate of 1
case in 5000 live births. Large omphaloceles occur with
a rate of 1 case in 10,000 live births.
 The etiology of omphalocele is not known.
 Various theories have been postulated; these include
failure of the bowel to return into the abdomen by 1012 weeks, failure of lateral mesodermal body folds to
migrate centrally, and persistence of the body stalk
beyond 12 weeks' gestation.
(Khan, Sabih, Thomas, MacDonald, & Chandramohan, 2013)
(Mann, Blinman, & Wilson, 2008)
Omphalocele Images
(Omphalocele [Fetal MRI], 2014)
(Omphalocele [Photograph], 2014)
Pathophysiology of Omphalocele
 Omphalocele is a midline defect in the anterior
abdominal wall that results in herniation of abdominal
contents into a membrane-covered sac.
 The contents of the sac, which is composed of an inner
layer of peritoneum and an outer layer of amnion, can
include solely intestine (small defect) or can also
contain liver and stomach (large defect).
 Rupture of the sac increases the risk of infection and
can lead to intestinal or hepatic trauma, but worse,
destroys options for delayed closure strategies.
(Thigpen, 2013)
Pathophysiology of Omphalocele
continued. . .
 Omphaloceles may range between 2 to 15 cm in size.
 These two extremes reflect the difference in the time
at which normal emybryogenesis is interrupted.
 If the interruption is early, around 3-4 weeks when
unfolding is in its last stages, the defect is large.
 If the interruption occurs at about 9-10 weeks when
migration is generally completed, the defect is smaller.
(Mann et al., 2008)
Pathophysiology of Omphalocele
continued. . .
 Beginning at the 6th postconception week, rapid
elongation of the gut and increased liver size result in
crowding of the intraabdominal space.
 As a result, intestinal loops are pushed out of the
abdominal cavity into the proximal umbilical ring.
 During the 10th week, the intestine returns to the
abdominal cavity and the process is completed by the 12th
week.
 Persistence of intestine or the presence of other abdominal
viscera in the umbilical cord results in an omphalocele.
(Mann et al., 2008)
Pathophysiology of Omphalocele
continued. . .
 The embryogenesis of this defect remains to be clear;
however, it is thought that there is a failure of abdominal
wall closure at the umbilical ring that results from a defect
in lateral folding in the embryo.
 Although omphaloceles can occur as isolated anomalies, up
to 70% of these defects can be associated with other
malformations and can be attributed to a single gene
disorder, chromosomal abnormalities, or genetic
syndromes
(Mann et al., 2008)
Etiology of Transient Tachypnea of
the Newborn
 Transient tachypnea of the newborn (TTN) is a self-
limiting disorder that requires minimal intervention,
and resolves over a 24- to 72-h period without
significant morbidity.
 TTN is common physiologic disorder of the newborn
resulting from pulmonary edema secondary to
inadequate or delayed clearance of fetal alveolar fluid.
 Incidence: 5.7 per 1000 births in term infants
 Risk factors include: premature or elective cesarean
delivery without labor.
(Abu-Shaweesh, 2011)
(Yurdakök, 2010)
Pathophysiology of Transient
Tachypnea of the Newborn
 Transition to air breathing requires rapid clearance of
fetal lung fluid, which is mediated primarily by
transepithelial sodium reabsorption through
amiloride-sensitive sodium channels in the alveolar
epithelial cells.
 This is likely facilitated by the changes in the
maternal-fetal hormonal milieu that normally
accompany the onset of spontaneous labor at term.
(Gomella, Cunnningham, & Eyal, 2013)
Pathophysiology of Transient
Tachypnea of Newborn continued…
 Disruption or delay in clearance of fetal lung liquid
results in transient pulmonary edema that categorizes
TTN.
 Retained fluid accumulates in the peribronchiolar
lymphatics and bronchovascular spaces, causing
compression and bronchiolar collapse with areas of air
trapping and hyperinflation.
 These changes result in a net decrease in lung
compliance accounting for clinical manifestations of
TTN.
(Gomella et al., 2013)
Initial Plan of Care
 Routine nursery care: check maternal labs, Hepatitis B vaccine, erythromycin
ophthalmic ointment, Vitamin K, OAE, and pulse oximetry screening
 Cord blood type and DAT if applicable
 CBC and blood culture; begin Ampicillin and Gentamicin
 Capillary blood gas
 OG tube to gravity
 BMP, Mg, Phos, Bili, CBC Q am
 Gentamicin peak and trough after 3rd dose
 Baby gram for respiratory distress and omphalocele
 Order Echo per Surgery to evaluate heart for defects
 Continue oxygen 2L at 100%, weaning as tolerated and monitor saturations
 NPO; IVF (D10W) at 80 mL/kg/day
 Consider consult(s) to: Pedi surgery and Pedi Cardiology
 Surgery to reduce and close omphalocele on Friday
 Apply Bacitracin to defect and cover with gauze or may use saline covered
gauze to keep it moist per Pedi. Surgery
Hospital Course by Systems
 Respiratory:
 06/25/14-06/26/14: HFNC 2 L, 100% Fi02
 06/27/14-06/28/14: Intubated for surgical repair
 06/28/14-07/01/14: HFNC 2 L, 21% Fi02
 Cardiac:
 06/26/14: Echo results:
Secundum ASD; Mod to Large PDA
 07/08/14: Repeat Echo:
Secundum ASD; Small PDA; thickened
aortic/pulmonic/mitral valves without
stenosis or regurgitation
Hospital Course by Systems cont.
 GI/FEN:
 06/25/14: Admit lytes and Patient NPO. Pedi. Surgery
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consulted for large Omphalocele
06/26/14-present: TPN/IL
06/27/14: Surgical repair and closure of Omphalocele
07/03/14-present: Continuous feeds initiated of
EBM/Pregestimil 20 cal via NG
07/04/14: PO feed attempted x 2 (poor)
07/14/14: Feeds currently at 82 ml/kg/day; Total Fluids at
130 ml/kg/day
Hospital Course by Systems cont.
 Hematology:
 06/25/14: On admission: Hct: 39.2/Hgb: 13.5
 06/27/14: Post surgical repair (minimal blood loss): Hct:
37.6/Hgb: 13.7
 06/25/14-present: Patient has not needed blood
transfusion.
 ID:
 06/25/14: Blood culture drawn, infant started on
Ampicillin & Gentamicin.
 06/27/14: Blood culture negative. Antibiotics D/C`ed.
 06/27/14: Day of Surgery - Clindamycin ordered.
Hospital Course by Systems cont.
 GU:
 06/25/14- present: Unremarkable
 CNS:
 06/25/14- present: Unremarkable
 Musculoskeletal:
 06/25/14- present: Unremarkable
 Ophthalmology:
 06/25/14- present: Unremarkable
Hospital Course by Systems cont.
 Developmental:
 07/09/14:
OT consulted for poor PO feeding
and developmental evaluation. OT to
see patient 3 times a week and work
on nonnutritive feeding
 Lines:
 06/25/14-06/27/14: PIV
 06/27/14-present: Right neck Broviac
 Labs:
 06/25/14-present: CBC, BMP, Mg, Phos, Bili Q Tue/Fri
Medications
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06/25/14-06/27/14: Gentamicin 4 mg/kg IV Q 24 hr
06/25/14-06/27/14: Ampicillin 100 mg/kg IV Q 12 hr
06/25/14-06/25/14: D10 W 80 ml/kg/day
06/25/14-06/25/14: Phytonadione 1 mg IM x 1 dose
06/25/14-06/25/14: Erythromycin Ophthalmic (5%)
ointment x 1 to both eyes
06/26/14-present: TPN/IL
06/27/14-07/02/14: Midazolam 0.1 mg/kg Q 4 h for
agitation
06/27/4-07/13/14: Clindamycin 10 mg/kg IV Q 8 hr
07/04/14-07-04/14: Hepatitis B Vaccine 5 mcg IM x 1
Pertinent Theories & Evidenced
Based Practice
 The goals of omphalocele repair are (1) return of the viscera to
the abdominal cavity and (2) closure of both fascia and skin.
 In 1948: Dr Robert Gross used skin flaps to close omphaloceles.
 Dr.Gross mobilized and closed only the skin over the defect,
preserving the sac beneath, but making no attempt to reduce the
viscera into the abdominal cavity. Later, the resulting large
ventral hernia would be closed at a second stage. Although
survival was improved, this technique did little to increase the
intraabdominal space, the viscera remain largely outside the
abdominal cavity, in a skin-covered sac, leaving final closure a
problem.
 Since then, surgeons have devised a number of techniques that
produce better results and can be selected as indicated.
(Holcomb, Murphy, Ostile, 2014)
Pertinent Theories & Evidenced
Based Practice continued. . .
 Direct Closure:
 For small (<5 cm defect in a full term infant) omphaloceles (including
‘hernia of the cord’), direct closure is the best method.
 Staged Reduction and Closure:
 Larger omphaloceles that have either a very large abdominal defect or a
large amount of viscera protruding through a smaller defect cannot be
closed primarily. In these cases, the surgeon applies some type of
temporary coverage that includes some form of pressure (gravity and
compression) that slowly and gradually drive the viscera back into the
abdomen as edema diminishes, the babies lose body water, and the
abdominal wall stretches and grows.
 The oldest variation of this method that is still used today was described by
Schuster (1967). The Schuster repair consists of sewing sheets of Teflon or
Silastic mesh to the rectus sheath and gradually tightening the closure of
the two sheets over around 7 – 10 days until the midline fascia can be closed
primarily. This mesh dwells beneath the abdominal skin, which is reopened
with each tightening procedure every 2–3 days. The sac is typically left
intact.
(Mann et al., 2008)
Pertinent Theories & Evidenced
Based Practice continued. . .
 Scarification treatment (also known as ‘paint and wait’)
 Some omphalocele patients are too unstable for any surgical
intervention. For example, a 2-kg 32-week EGA-premature
neonate with pulmonary hypoplasia, a patent ductus
arteriosus (PDA), and a large omphalocele containing most of
the abdominal viscera will not tolerate even the moderate
pressures required for staged reduction. In this and similar
cases, the safest course is ‘paint and wait’.
 Here, the sac is coated with an antimicrobial agent that allows
the sac to toughen into an eschar. As the sac contracts and the
baby grows, the viscera are very slowly returned to the
abdominal cavity. Much later, the large ventral hernia can be
closed primarily or with a biocompatible membrane
 Currently, the antimicrobial agent used is Betadine .
(Holcomb et al.,2014)
Family Interventions
 Mother and father were both concerned immediately after
birth, questions answered and plan of care explained.
Infant shown to mother prior to transfer to NICU for
further evaluation. Father accompanied infant to NICU and
further questions were answered. After recovery, mother
and father visited infant in the NICU, asked appropriate
questions regarding Omphalocele, date of surgery and
what to expect postoperatively. Pedi Surgery spoke in depth
with parents regarding surgery, and post op expectations.
Parents verbalized understanding and have been very
involved since birth.
 Parents go daily to visit patient. They hold patient
whenever possible. Parents are knowledgeable and ask
appropriate questions.
Infant’s Discharge Plan & Follow Up
 Patient is still working on feeds and getting off TPN/IL, but
there are things to keep in mind to start getting patient
home such as:
 ABR, Car Seat Challenge, CCHD screen prior to discharge.
 Keep vaccinations up to date (Hepatitis B given 07/04/14).
 Follow up on newborn screen results.
 Parents should complete CPR and room in prior to discharge.
 Pediatric Cardiology wants to follow up with patient and
repeat Echo in one month (tentatively 08/08/14).
 Pediatric Surgery needs to follow up with patient outpatient
once patient goes home.
 Pediatrician follow up should be 2-3 days after discharge.
(Hynes & Andrews, 2012)
Summary
 Omphalocele is one of the most common abdominal wall
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defects.
A priority when dealing with omphaloceles is preventing
the rupture of the sac.
Although omphaloceles can occur as isolated anomalies, up
to 70% of these defects can be associated with other
malformations.
Omphaloceles are treated by direct closure, staged
reduction and closure or scarification.
Survival of neonates with omphalocele is 90% when there
are no other associated anomalies.
(Holcomb et al.,2014)
(Mann et al., 2008)
References
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Abu-Shaweesh, J.M. (2011). The Respiratory system. In R. J. Martin, A. A. Fanaroff, & M. C. Walsh (Eds.),
Neonatal-Perinatal Medicine: Diseases of the fetus and infant (9th ed., pp. 1162-1163). Philadelphia,
PA: Mosby Elsevier.
Blackburn, S. (2013). Maternal, Fetal, & Neonatal Physiology (4rd edition). St. Louis, Missouri: Saunders Elsevier.
Gomella, T. L, Cunningham, M. D. & Eyal, F. G. (2013). Neonatology: Management, procedures, on-call problems,
diseases and drugs (7th Ed.). New York: McGraw Hill Education
Holcomb, G.W., Murphy, P.J., & Ostile, D.J. (2014). Ashcraft’s Pediatric Surgery. Retrieved from http://www.inkling.
com/read/ holcomb-ashcrafts-pediatric-surgery-6th/chapter-48/omphalocele
Hynes, R.A., & Andrews, T.M.(2012). Discharge planning. In J. P. Cloherty, E. C. Eichenwald, A. R. Hansen, &
A. R. Stark (Eds.), Manual of neonatal care (7th ed.). (pp. 203-218). Philadelphia, PA: Lippincott Williams &
Wilkins.
Khan, A.N., Sabih, D., Thomas, N., M.acDonald, S., & Chandramohan, H. (2013). Omphalocele. Medscape. Retrieved
from http://emedicine.medscape.com /article/404182-overview
Mann, S., Blinman, T.A., & Wilson, D. (2008). Prenatal and postnatal management of omphalocele. Prenatal
Diagnosis.
28, 626-632. DOI:10.1002/pd.2008
Omphalocele [Fetal MRI]. (2014). Retrieved from http://www.ummchealth.com/Health_are_Services/
Womens_Care/Adult/Fetal_Medicine/Special_Delivery_Planning/Omphalocele.aspx
Omphalocele [Photograph]. (2014). Retrieved from http://wcaap.org/view-newsletter/5/
Thigpen, J. (2013). Gastrointestinal system. In C.Kenner & J.W. Lott (Eds.). Comprehensive neonatal care: an
interdisciplinary approach (4th ed., pp. 93-133). Philadelphia: Saunders Elsevier.
Velasco-Sanchez, M.A. (2007). Omphalocele. [Ultrasound], Retrieved from http:// sonoworld.com/fetus
/page.aspx?id=3052
Yurdakök, M. (2010). Transient tachypnea of the newborn: what is new?. Journal of Maternal-Fetal and
Neonatal Medicine, 23(S3), 24-26.