Critically Ill Children in Pediatric Surgery. No disclosures to report.

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Critically Ill Children in Pediatric Surgery Hillary J. Collyer RN, MSN, CPNP, CCRN Pediatric Surgery Nurse Practitioner Hasbro Children s Hospital Disclosure Information Speaker: Hillary Collyer No disclosures to report. Accreditation Statement APSNA is an approved provider of continuing nursing education by NAPNAP. The NAPNAP contact hours is accepted by ANCC. Objectives Describe surgical conditions that place patients at risk of becoming critically ill Identify patient populations at greatest risk of becoming critically ill Discuss the management of these critically ill patients, Review radiologic findings associated with these disease processes 1

NAPNAP Boston The Pediatric Surgery Patient Age Spectrum: Premature infant to adolescent Diagnoses specific to age Bowel obstructions Congenital abnormalities must always be considered when encountering a newborn or young infant with unexplained GI symptoms Neonatal Bowel Obstruction 1. Pyloric Stenosis 2. Malrotation with Midgut Volvulus 3. Intestinal Atresias 4. Necrotizing Enterocolitis 5. Meconium Disease 6. Hirschsprung s Disease 7. Anorectal Malformations 2

American Pediatric Surgical Nurses Association 23rd Annual Scientific Conference May 26-29, 2014 Phoenix, AZ Child & Adolescent Bowel Obstructions 1. 2. 3. 4. Appendicitis Intussusception Incarcerated Hernia Meckel s Diverticulum 5. Malrotation with Midgut Volvulus 6. Tumors The Vomiting Infant Hypertrophic Pyloric Stenosis 3

Incidence One of the most common surgical conditions of the infant Occurs 1-4:1000 live births 4:1 Male to Female ratio Risk factors: Family history, race, younger maternal age, 1 st born infant, maternal feeding patterns 1 Etiology- Unknown Presentation Functional gastric outlet obstruction 2 Full-term infant between the ages of 2-8 weeks Classic Presentation- Nonbilious vomiting which increases in amount and force as obstruction increases è projectile, hunger despite emesis Physical exam- well appearing Late Presentation Persistent vomiting with late diagnosis results in: Cachectic appearing infant with weight loss 3 Large losses of gastric secretions causing metabolic disturbances Gastritis with hematemesis Palpable pyloric mass Olive Peristaltic waves Lower incidence secondary to prompter diagnosis before protracted vomiting 4 Altit G, Milot M. N Engl J Med 2013 4

Differential Diagnosis **Gastroesophageal Reflux **Milk Intolerance Viral Gastroenteritis Malrotation with Midgut Volvulus Incarcerated Hernias Increased Intracranial Pressure Metabolic Disorders Diagnosis Gold Standard- Ultrasound 5 Diagnostic criteria: Muscle thickness: > 3 mm Total thickness: 14 mm Length of Canal: 16 mm Diagnosis: Ultrasound 5

Diagnosis: KUB Diagnosis: UGI Pre-op Considerations Not an operative emergency Mainstay of therapy is resuscitation followed by surgical management NPO +/- Gastric decompression 6

Metabolic Disturbances Metabolic Disturbances 6 : Hypochloremia <100 mmol/l Hypokalemia <4.5 mmol/l Metabolic Alkalosis ph > 7.45 HCO 3 > 30 BE > +3 Jaundice If sustained may turn to acidosis Initial Resuscitation Usually achieved within 24 hours of presentation Basic metabolic panel followed by resuscitation to correct abnormalities Initially 10 to 20ml/kg NSB IVF 1 ¼- 2x Maintenance rate, IVF with KCL Basic Metabolic panel Q6h until normalized Respiratory Depression Alkalosis-induced central respiratory depression 7 ñ HCO 3 Respiratory pauses & Apnea-like events Tactile Stimulation Supplemental Oxygen Intubation 7

Surgical Treatment 1 st surgical treatment by Lobker in 1898- Unsuccessful Pyloromyotomy technique first introduced by Ramstedt in 1911 Extramucosal longitudinal myotomy of the pylorus 8 Open v Laparoscopic Open Pyloromyotomy RUQ transverse incision/supraumbilical incision Pylorus is exteriorized through incision Longitudinal serosal incision in the pylorus Blunt dissection to divide the firm pyloric fibers Completed when all fibers have been divided the entire length of the incision Laparoscopic Pyloromyotomy 1 st laparoscopic pyloromyotomy in 1991 9 Minimally invasive approach Typically 3 port sites Accomplish same results as open approach Studies have shown no difference in complications 8

Post-Op Considerations Remain NPO for approximately 6 hours post-op Post-pyloromyotomy feeding regimens Recent studies support ad lib feeds 1 Metabolic derangement pre-op affect post-op feeding tolerance Acetaminophen for pain Outcomes Malrotation/Midgut Volvulus Incidence Occurs 1:6000 live births 1% of the total population 2:1 Male to Female ratio Intestinal rotation and fixation is an orderly sequence of embryologic events in early fetal development 10 Disruption of these steps leads to rotational anomalies 9

Normal Rotation & Fixation Nonrotation Midgut Volvulus: Presentation Incidence of volvulus: Presents during the first week in 50%, in the first month in 75%, in the first year in 15%, small amount later in life Cardinal sign is bilious emesis B/C of devastating outcomes, volvulus is presumed diagnosis until proven otherwise 1 Surgical Emergency!! 10

Presentation Symptoms vary with progression of volvulus Acute onset bilious emesis Scaphoid abdomen è increasing abdominal distention Abdominal tenderness Late signs: Abdominal wall erythema Hematemesis Melena Laboratory findings: Leukocytosis v Leukopenia Hyperkalemia Thrombocytosis Metabolic Acidosis Mesenteric vascular compromiseè peritonitis è sepsisè shockè death Diagnosis: UGI Upper GI (UGI): Gold Standard Evaluate position of the duodenojejunal junction Normal è Diagnosis: UGI Abnormal Findings: Duodenojejunal junction to the right of the spine Obstruction of the duodenum Coil Spring, Corkscrew, or Beak appearance of the obstructed proximal jejunum 11

Diagnosis: KUB Plain Abdominal Radiograph (2 View): Nonspecific Double Bubble Sign Gasless abdomen OR ENTIRELY NORMAL Diagnosis: Ultrasound UGI R GB SMV Duodenum Antrum SMA L Ultrasound Vessel orientation SMV anterior R of SMA Malrotation: SMV L of SMA R SMV L SMA Diagnosis: Ultrasound UGI GB Duodenum Ultrasound Vessel orientation SMV anterior R of SMA Malrotation: SMV L of SMA Swirl or Whirlpool Sign 12

Pre-Op Considerations Early diagnosis is imperative Aggressive resuscitation with electrolyte correction NGT decompression IV broad-spectrum antibiotics (Gram +,Gram -, and Anaerobic Flora) IMMEDIATE SURGICAL INTERVENTION Ladd s Procedure 6 Key Elements in Operative Correction of Malrotation 1 : 1. Entry into abdominal cavity and evisceration 2. Counterclockwise detorsion of bowel 3. Division of Ladd s cecal bands 4. Broadening small bowel mesentery 5. Incidental appendectomy 6. Placement of small bowel along the right lateral gutter and large colon along the left lateral gutter Ladd s Procedure 13

Intra-Op Considerations Necrotic or ischemic bowel may involve isolated segments or entire midgut Limited resection and second-look operation in 24-48 hours Abdominal compartment syndrome may necessitate temporary closure (silo, patch) 1 Intra-Op Considerations 14

Entire Midgut Loss Occasionally complete infarction of the midgut, resulting in entire midgut loss Ethical issues remain controversial 3 Abdominal closure and comfort care Longterm TPN & eventual intestinal and multivisceral transplantation 1 Post-Op Considerations Continued resuscitation to correct hypovolemia, metabolic acidosis NGT decompression until return of bowel function +/- Antibiotics Respiratory support Pain management Special Populations: Adhesive Bowel Obstruction in Infants, Children & Adolescents 15

Adhesive Bowel Obstructions Most common cause of bowel obstruction 12 Occurs in 1-6% of children Rate depends on initial type of operation Conservative v Operative management still controversial in this age group 11 Special Populations: Patients with Intellectual Disabilities Intellectual Disability Challenges include: Can have extensive PMHx & PSHx- Multiple abdominal surgeries Nonverbal patients- difficult to ascertain history Sometimes difficult physical exam secondary to body habitus, scoliosis Pain is difficult to assess Often present late in course of illness Radiographic imaging difficult to interpret 16

American Pediatric Surgical Nurses Association 23rd Annual Scientific Conference May 26-29, 2014 Phoenix, AZ Intellectual Disability Intellectual Disability Meteorism 17

Collaborative Care ICU Collaboration Multidisciplinary Team Care Complex, Multisystem dysfunction References Holcomb, G.W., & Murphy, J.P. (Eds.). (2010). Ashcraft s Pediatric Surgery. Philadelphia,PA: Saunders Elsevier. Piroutek, M., Brown, L., & Thorp, A. (2012). Bilious vomiting does not rule out infantile hypertrophic pyloric stenosis. Clinical Pediatrics, 51(3), 214-218. McCollugh, M., & Sharieff, G.Q. (2006). Abdominal pain in children. Pediatric Clinics of North America, 53, 107-137. Papadakis, K., Chen, E., Luks, F., Lessin, M., Wesselhoeft, C., & DeLuca, F. (1999). The changing presentation of pyloric stenosis. American Journal of Emergency Medicine, 17(1), 67-69. Chen, E., Luks, F., Gilchrist, B., Wesselhoeft, C., & DeLuca, F. (1996). Pyloric stenosis in the age of ultrasonography: Fading skills, better patients? Journal of Pediatric Surgery, 31(6), 829-830. Glatstein, M., Carbell, G., Boddu, S. Bernardini, A., & Scolnik, D. (2011). The changing clinical presentation of hypertrophic pyloric stenosis: The experience of a large, tertiary care Pappano, D. (2011). Alkalosis-induced respiratory depression from infantile hypertrophic pyloric stenosis. Pediatric Emergency Care, 27(2), 124. pediatric hospital. Clinical Pediatrics, 50(3), 192-195. St. Peter, S., Holcomb, G., Calkins, C., Murphy, J., Andrews, W., Sharp, R., Snyder, D., & Ostlie, D. (2006). Open versus laparoscopic pyloromyotomy for pyloric stenosis. Annals of Surgery, 244(3), 363-370. Askew, N. (2010). An overview of infantile hypertrophic pyloric stenosis. Paediatric Nursing, 22(8), 27-30. Juang, D., & Snyder, C.L. (2012). Neonatal Bowel Obstruction. Surgery Clinics of North America, 92, 685-711. Lautz, T., Raval, M., Reynolds, M. & Barsness, K. (2011). Adhesive small bowel obstruction in children and adolescents: Operative utilization and factors associated with bowel loss. Journal of the American College of Surgeons, 212(5), 851-865. Jackson, P.G., & Raiji, M. (2011). Evaluation and management of intestinal obstruction. American Family Physician, 83(2), 159-165. 18