Oomphalocele : A clinical case study

Omphalocele: A clinical case studyGabriela OlivasNNP IIGNRS: 5632July 12, 2014

ObjectivesOutline maternal historyMaternal and fetal risks and complicationsDiscuss delivery and stabilization needed for infantReview admission assessment and diagnostics of affected infantReview admission diagnosesReview etiology and pathophysiology of admission diagnosesOutline initial plan of careDiscuss hospital course by systems Explain medications used in infants treatmentReview pertinent theories and explore current evidence based practice about OmphalocelesDiscuss family interventionsOutline infants discharge plan and follow-upReview summary

Maternal HistoryAge: 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: negativeHep Bs Ag: negativeGBS: negativeHIV: negativeOther Infections: None Social History: None Other Pregnancy Problems: Anemia of mother in pregnancy, antepartum

Maternal/Fetal Risks & ComplicationsMaternal AnemiaFetus 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 OmphaloceleThis 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 portion of liver, two vessel umbilical cord noted on prenatal diagnosticsAROM 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 signsO2 sats: 99%HR: 138Temp: 37 degree CelsiusRR: 47BP: 66/39 (47)

Admission Assessment continuedGeneral: 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

DiagnosticsBabygram 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: 7.36Pco2: 45P02:131HCo3: 25BE: -0.8CBC: WBC: 10.7Hct: 39.2Hgb: 13.5Plt: 134Segs: 32Bands: 5Lymphs:52Monos: 5Eos: 2Lytes:Na: 138K: 5CO2: 104Cl:23Mg: 1.8Phos: 6Gluc: 63Ca: 9.7BUN: 6Cr: 0.7Bili (uncon.): 4 Bili (con.): 0.7

Prenatal DiagnosticsOmphalocele containing liver was seen on fetal ultrasound on 04/29/14Mom declined amniocentesis.

(Velasco-Sanchez, 2007)Primary Admission DiagnosesTerm 38 week, 4 day appropriate for gestational age female , DOL oOmphalocele Suspected Transient Tachypnea of the Newborn

Etiology of OmphaloceleIncidence: 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)

(Khan, Sabih, Thomas, MacDonald, & Chandramohan, 2013)(Khan, Sabih, Thomas, MacDonald, & Chandramohan, 2013)Omphalocele Images

(Omphalocele [Fetal MRI], 2014)

(Omphalocele [Photograph], 2014)Pathophysiology of OmphaloceleOmphalocele 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 NewbornTransient 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 infantsRisk factors include: premature or elective cesarean delivery without labor.

(Abu-Shaweesh, 2011)(Yurdakk, 2010)

Pathophysiology of Transient Tachypnea of the NewbornTransition 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 continuedDisruption 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)19Initial Plan of CareRoutine 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 gravityBMP, 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 SystemsRespiratory:06/25/14-06/26/14: HFNC 2 L, 100% Fi0206/27/14-06/28/14: Intubated for surgical repair06/28/14-07/01/14: HFNC 2 L, 21% Fi02Cardiac:06/26/14: Echo results: Secundum ASD; Mod to Large PDA07/08/14: 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 consulted for large Omphalocele06/26/14-present: TPN/IL 06/27/14: Surgical repair and closure of Omphalocele07/03/14-present: Continuous feeds initiated of EBM/Pregestimil 20 cal via NG07/04/14: 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: 13.506/27/14: 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 feedingLines:06/25/14-06/27/14: PIV06/27/14-present: Right neck Broviac Labs:06/25/14-present: CBC, BMP, Mg, Phos, Bili Q Tue/Fri

Medications06/25/14-06/27/14: Gentamicin 4 mg/kg IV Q 24 hr 06/25/14-06/27/14: Ampicillin 100 mg/kg IV Q 12 hr06/25/14-06/25/14: D10 W 80 ml/kg/day 06/25/14-06/25/14: Phytonadione 1 mg IM x 1 dose06/25/14-06/25/14: Erythromycin Ophthalmic (5%) ointment x 1 to both eyes06/26/14-present: TPN/IL06/27/14-07/02/14: Midazolam 0.1 mg/kg Q 4 h for agitation06/27/4-07/13/14: Clindamycin 10 mg/kg IV Q 8 hr07/04/14-07-04/14: 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 (