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Omphalocele: A clinical case study

Omphalocele: A clinical case study. Gabriela Olivas NNP II GNRS: 5632 July 12, 2014. Objectives . Outline maternal history Maternal and fetal risks and complications Discuss delivery and stabilization needed for infant Review admission assessment and diagnostics of affected infant

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Omphalocele: A clinical case study

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  1. Omphalocele: A clinical case study Gabriela Olivas NNP II GNRS: 5632 July 12, 2014

  2. Objectives • 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 pathophysiologyof 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

  3. Maternal History • 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

  4. 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)

  5. Delivery and Stabilization • NICU team called to delivery due to Omphalocele with 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%)

  6. 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 Temp: 37 degree Celsius RR: 47 BP: 66/39 (47)

  7. Admission Assessment continued • 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 genitaliaExtremities: no deformities, normal range of motion, hips stable, clavicles intact • Neurologic: responsive to stimuli • Back: no defect, anus patent and normally placed

  8. Diagnostics • Babygram on admission: “The patient is rotated, the lungs are mildly 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: 134Segs: 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

  9. Prenatal Diagnostics • Omphalocele containing liver was seen on fetal ultrasound on 04/29/14 • Mom declined amniocentesis. (Velasco-Sanchez, 2007)

  10. Primary Admission Diagnoses • Term 38 week, 4 day appropriate for gestational age female , DOL o • Omphalocele • Suspected Transient Tachypnea of the Newborn

  11. (Khan, Sabih, Thomas, MacDonald, & Chandramohan, 2013) (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 10-12 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)

  12. Omphalocele Images (Omphalocele [Fetal MRI], 2014) (Omphalocele [Photograph], 2014)

  13. 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)

  14. 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)

  15. 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)

  16. 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)

  17. 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)

  18. 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)

  19. 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 peribronchiolarlymphatics 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)

  20. 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 Ampicillinand 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 Bacitracinto defect and cover with gauze or may use saline covered gauze to keep it moist per Pedi. Surgery

  21. 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

  22. Hospital Course by Systems cont. • GI/FEN: • 06/25/14: Admit lytes and Patient NPO. Pedi. Surgery 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

  23. 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.

  24. 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

  25. 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

  26. Medications • 06/25/14-06/27/14: Gentamicin4 mg/kg IV Q 24 hr • 06/25/14-06/27/14: Ampicillin100 mg/kg IV Q 12 hr • 06/25/14-06/25/14: D10 W 80 ml/kg/day • 06/25/14-06/25/14: Phytonadione1 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: Midazolam0.1 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

  27. 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)

  28. 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)

  29. 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 ductusarteriosus (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)

  30. 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.

  31. 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)

  32. Summary • Omphalocele is one of the most common abdominal wall 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)

  33. References • 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 (7thEd.). 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.

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