Division of pediatric surgery

1. Division of pediatric surgery Interesting Case Studies in Pediatric Surgery Michelle L. Ceo, RN, CPN, MSN, CPNP Stony Brook Children’s

2. objectives Discuss diagnoses and treatment of: Abdominal trauma Achalasia Duodenal stenosis Spontaneous pneumothorax Improve surgical outcomes of the infant, child and adolescent patient.

3. Case study 1 EG is a 7 year old female with a past medical history of ADHD on Concerta. She fell off the monkey bars 3 days prior to presentation. She had complaints of mild left sided abdominal pain, but was otherwise acting normally, tolerating PO. On day of presentation to the ED she had complaints of severe abdominal pain, and emesis x 1.

4. Case study 1 EG was seen at the outside hospital where an abdominal CT scan was performed and showed a Grade IV splenic laceration. Patient was transferred to Stony Brook Children’s as a Pediatric Code T. Labs in ED: H/H 11.5/34.4 Plan was to admit the patient to the General Pediatric floor for Serial Abdominal exams and CBC’s.

5. Case study 1 Pediatric abdominal trauma is typically blunt in nature, with the spleen being the most common organ injured. Non-operative management is employed in over 95% of patients.

6. Spleen laceration grading system

7. Spleen laceration protocolstony brook children’s hospital

8. Case study 1 On admission day #1 patient had some complaints of abdominal pain in the morning, H/H was 9.5/28.2. 14 hours later, In the afternoon patient had complaints of severe abdominal pain, nausea, vomiting. EG became very pale, and a rapid response was called. BP 81/48 HR 152. Patient received 1L NS bolus, CBC, Type and Cross STAT. H/H 6.7/18.8. Patient was emergently brought to the OR for a splenectomy.

9. Case study 1 Intra operatively patient received 5 units PRBC, 3 units FFP, I unit platelets, I units of cryoprecipitate and 1200cc NS. Post operatively patient did well and was discharged home on POD 9.

10. Literature review Surg Case Rep. 2018 Surg Case Rep. 2018 Jul 11;4(1):75. doi: 10.1186/s40792-018-0477-5. A rare mechanism of delayed splenic rupture following the nonoperative management of blunt splenic injury in a child. Shinkai T1, Ono K2, Masumoto K2, Urita Y2, Gotoh C2. Author information Abstract BACKGROUND: Nonoperative management (NOM) has been established as the standard treatment for isolated blunt organ injury in hemodynamically stable pediatric patients. Although delayed splenic rupture or bleeding is a rare complication in NOM, it is an issue that many pediatric surgeons are greatly concerned about. We herein report a rare pediatric case concerning the mechanisms involved in delayed splenic rupture after NOM. CASE PRESENTATION: A 9-year-old boy with severe abdominal pain was transferred to our hospital. Twenty-one hours before the admission, he had been kicked in the region of his left lateral abdomen. Contrast-enhanced abdominal computed tomography revealed a severe intra-parenchymal hematoma and multiple lacerations of the spleen with a large amount of hemoperitoneum without active bleeding. His condition was diagnosed as a grade III injury on the AAST splenic injury scale. After fluid resuscitation, his vital signs became stable. The patient was treated with NOM in our intensive care unit. However, suddenly after defecation (72 h after the injury), he started complaining of severe abdominal pain and left shoulder pain. His blood pressure dropped to 70/35 mmHg, and he started to lose consciousness. Abdominal ultrasonography (US) revealed increased ascites. Fluid resuscitation and blood transfusion were performed. His symptoms and abdominal US findings suggested that splenic re-bleeding had caused delayed splenic rupture to occur. Emergency splenectomy was performed. The resected spleen was enlarged with a large parenchymal hematoma. The posterior-lateral side of the splenic capsule was ruptured. CONCLUSIONS: The mechanism of delayed splenic rupture in our case was considered to be the result of a tear in the subcapsular hematoma caused by stretching the splenocolic ligament related to a bowel movement during defecation. Although delayed splenic rupture or bleeding is unpredictable, it is very important to understand the mechanisms and to educate the family of the children with splenic injuries of the warning signs of delayed rupture or bleeding.

11. Literature review Surg Case Rep. 2018 A rare mechanism of delayed splenic rupture following the non-operative management of blunt splenic injury in a child. Shinkai T1, Ono K2, Masumoto K2, Urita Y2, Gotoh C2. Conclusions: Rupture typically occurs 48-72 hours after injuries in cases diagnosed with delayed splenic rupture. Jet et al described four cases of delayed splenic rupture that occurred 4 to 20 days post-injury. Davis et al reviewed cases of delayed splenic bleeding and found that they occurred 2 to 28 days post injury. Patient’s with symptoms of increased pain, pallor, dizziness, vomiting, and worsening shoulder pain must return to hospital. Treatment options of delayed splenic rupture include: Splenectomy- 18 patients splenic artery embolization- performed in one case. MUST BE HEMODYNAMICALLY STABLE Observation- one case- MUST BE HEMODYNAMICALLY STABLE

12. Case study 2 CR is a 12 year old female that presents to the outpatient pediatric surgery office. She has a long history of intermittent abdominal pain, vomiting of feculent material and being underweight

13. Case study 2 Pediatric GI performed an upper GI series which demonstrated a stricture in the duodenum and an enlarged stomach. She underwent two upper endoscopies with dilation ofthe duodenal stenosis. Her symptoms reoccurred within a few weeks after dilation.

14. CR CT scan

15. Duodenal stenosis Duodenal atresia is a disease found in newborn infants, children and adolescents. It is the absence or complete atresia of a portion of the lumen within the first part of the small intestine duodenum. There may also be partial obstruction due to narrowing (stenosis) of the duodenum.

16. Case study 2 CR underwent an exploratory laparotomy, duodenal dudenumonstomy. Post operatively the patient did exceedingly well. She tolerated all foods with no further vomiting. At diagnosis (September 2018) patient weighed 58lbs, as of February 2019 patient now weighs 71lbs.

17. Literature review Medical Journal or Cairo University Vol. 81, No. 1, September 609-617, 2013 Congenital Duodenal Stenosis: Early and Late Presentation Medical Journal or Cairo University Vol. 81, No. 1, September 609-617, 2013 Conclusions: Duodenal stenosis can present in neonates, infants, children and adults. Late presentation occurs due to incomplete nature of the obstruction, negligence of parents or expectancy of the diagnosis. Age of Distribution (total 13 cases): Up to 1 Month- 4 cases 1 Month-1 year- 4 cases 1-2 years- 3 cases More then 2 years- 2 cases

18. Case study 3 HV is a 12 year old female that presents to the office with complaints of difficulty swallowing. She notes a sensation of difficulty swallowing with pain in the upper chest and throat. She then began having associated emesis and regurgitation of food.

19. Esophageal manometry She was evaluated by Pediatric GI. She underwent an upper endoscopy, and esophageal manometry. She was diagnosed with achalasia.

20. Esophogram

21. esophogram Birds Beak Appearance

22. Achalasia J Gastrointestinal Surgery (2017) 21:207-214 Achalasia is a rare idiopathic disease of esophageal motility characterized by a failure of the esophageal gastric junction to relax during swallowing, combined with a peristalsis of the esophageal body. The lower esophageal sphincter is hypertensive in about 50% of patients. Dysphasia, regurgitation, retrosternal pain, heartburn, respiratory symptoms, and weight loss are the most common symptoms.

23. Case study 3 HV underwent a laparoscopic Heller-myotomy with a Dor Fundoplication. Post operatively patient did well was started on a soft regular diet and was slowly advanced to a full regular diet, with no further complaints of dysphasia or vomiting.

24. Heller Myotomy and dor fundoplication

25. Literature review Surgical Endoscopy 14 December 2018 SurgEndosc. 2018 Dec 14. doi: 10.1007/s00464-018-06625-6. Heller myotomy is the optimal index procedure for esophageal achalasia in adolescents and young adults. Hung YC1, Westfal ML1,2, Chang DC1, Kelleher CM3,4,5. Author information Abstract BACKGROUND: There is limited and conflicting data on the optimal intervention for the treatment of achalasia in adolescents and young adults (AYA), Heller myotomy (HM), esophageal dilation (ED) or botulinum toxin injection (botox). The goal of this study is to determine the most appropriate index intervention for achalasia in the AYA population. METHODS: We completed a longitudinal, population-based analysis of the California (2005-2010) and New York (1999-2014) statewide databases. We included patients 9-25 years old with achalasia who underwent HM, ED or botox. Comparisons were made based on the patients' index procedure. Rates of 30-day complications, long-term complications, and re-intervention up to 14 years were calculated. Cox regression was performed to determine the risk of re-intervention, adjusting for patient demographics. RESULTS: A total of 442 AYAs were analyzed, representing the largest cohort of young patients with this disease studied to date. Median follow-up was 5.2 years (IQR 1.8-8.0). The overall rate of re-intervention was 29.3%. Rates of re-intervention for ED and botox were equivalent and higher than HM (65.0% for ED, 47.4% for botox and 16.4% for HM, p < 0.001). Ultimately, 46.9% of ED and botox patients underwent HM. The overall short-term complication rate was 4.3% and long-term, 1.9%. There was no difference in the short-term and long-term complication rates between intervention groups (p > 0.05). On adjusted analysis, ED and botox were associated with increased risks of re-intervention when compared to HM (HR 5.9, HR 4.8, respectively, p < 0.01). Black patients were found to have a risk of re-intervention twice that of white patients (HR 2.0, p = 0.05). CONCLUSIONS: HM has a similar risk of complications but a significantly lower risk of re-intervention when compared to ED and botox. Based on our findings, we recommend HM as the optimal index procedure for AYAs with achalasia.

26. Literature review Surgical Endoscopy 14 December 2018 Heller myotomy is the optimal index procedure for esophageal achalasia in adolescents and young adults. Hung YC1, Westfal ML1,2, Chang DC1, Kelleher CM3,4,5. Conclusions: 442 patients with achalasia underwent surgical intervention. 75.8% underwent hellermyotomy, 18.3% underwent esophageal dilation or botox injections. Overall re intervention rate was 29.3%, but significantly higher in endoscopic dilation and botox injection (p=0.43) vs hellermyotomy (p<0.001).

27. Literature review Journal of Gastrointestinal Surgery (2017) 21:207-2014 POEM vs Laparoscopic Heller Myotomy and Fundoplication: Which Is Now the Gold Standard for Treatment of Achalasia? Marco G. Patti1 & Ciro Andolfi2 & Steven P. Bowers3 & Nathaniel J. Soper4 J GastrointestSurg (2017) 21:207–214 DOI 10.1007/s11605-016-3310-0 Conclusion: POEM (Per Oral Endoscopic Myotomy) is the latest innovation in the treatment of achalasia. The procedure is performed mid esophagus and a submucosal tunnel is created between muscularis and mucosa down onto the stomach. The inner circular layer of muscle is then divided using electrosurgery of the distal esophagus and down onto the stomach for a distance of 2-3 cm. The mucosotomy is then closed with clips or endoscopic sutures. 1991 the first thoracocscopicmyotomy for achalasia in the USA. POEM procedure was first described in 2010. Success rate from Laparoscopic Heller Myotomy with partial fundoplication at one year is 90%. There are no long term outcome studies of POEM beyond 2-3 years. There might be a higher rate of GERD and recurrent dysphasia with POEM requiring re surgical intervention.

28. Case study 4 DW is a 16 year old male that presents to the emergency department with complaints of recent URI, cough and shortness of breath. He has a past medical history of asthma, factor 5 Leiden deficiency (not on anticoagulation), aortic dilation, and spontaneous pneumothorax treated non operatively with a chest tube one month prior in another state.

29. chest x-ray with pneumothorax

30. Spontaneous pneumothorax Spontaneous pneumothorax: No clinical signs or symptoms in primary spontaneous pneumothorax until a bleb ruptures and causes pneumothorax. Typically the result is acute onset of chest pain and shortness of breath. The highest incidence of primary spontaneous pneumothorax in the pediatric population is tall, thin adolescent males, aged 13-16 years.

31. DW chest x-ray with chest tube in placeresolved pneumothorax

32. Case study 4 DW underwent a left sided VATS (video assisted thoracoscopic surgery)with chemical (doxycycline) and mechanical pleurodesis and blebectomy.

33. Vats with pleurodesis

34. Case study 4 Post operatively the patient did well. Chest tube was removed on POD 2. Chest x-ray post chest tube removal demonstrated small apical pneumothorax. No further surgical intervention or chest tube placement was needed.

35. Post op chest x-raychest tube removedsmall apical pneumothorax

36. Case study 4 DW followed up in the office for a post operative visit. He had no further episodes of spontaneous pneumothorax. Chest x-ray demonstrated resolution of apical pneumothorax.

37. Post op chest x-ray pneumothorax resolved

38. Literature review Journal of Laparoendoscopic & Advanced Surgical Techniques 2018 J LaparoendoscAdvSurg Tech A. 2018 Dec 28. doi: 10.1089/lap.2018.0629. Current Practice in the Management of Spontaneous Pneumothorax in Children. Williams K1,2, Baumann L2,3, Grabowski J2,4, Lautz TB2,4. Author information Abstract INTRODUCTION: There is a lack of pediatric-specific guidelines for the workup and management of primary spontaneous pneumothorax (PSP) in children. The aim of this study was to describe current practices among North American pediatric surgeons. MATERIALS AND METHODS: An online survey comprising 18 questions was sent out through the American Pediatric Surgical Association Outcomes and Clinical Trials Committee to all members. Bivariate analysis was performed using Chi-square analysis. RESULTS: A total of 287 surveys were completed (33% response rate). For a first episode of PSP, 57% of surgeons opt for chest tube drainage, 4% for upfront video-assisted thoracoscopic surgery (VATS), 3% for needle aspiration, and 29% for only oxygen administration. Eighty-one percent of surgeons report that the size of the pneumothorax influences management. However, neither practice setting (P = .87) nor years in practice (P = .11) correlated with initial management strategy. For patients with a persistent air leak after chest tube placement, there is wide variation in duration of observation before performing VATS, with 40% operating after 3 days, but 21% waiting at least 5 days. The use of chest computed tomography (CT) is also highly variable. Eighty-two percent of respondents perform surgery only after the second episode of PSP. Most perform a stapled apical blebectomy and mechanical pleurodesis for both initial and recurrent PSP. CONCLUSION: There is significant variation among pediatric surgeons in the management of spontaneous pneumothorax, including the use of CT, timing of operation, and duration of observation for air leak before performing surgery. Prospective data are needed to better inform guidelines and standardize practice.

39. Literature review Journal of Laparoendoscopic & Advanced Surgical Techniques 2018 Current Practice in the Management of Spontaneous Pneumothorax in Children. Williams K1,2, Baumann L2,3, Grabowski J2,4, Lautz TB2,4. J LaparoendoscAdvSurg Tech A. 2018 Dec 28. doi: 10.1089/lap.2018.0629. Initial Management: Oxygen administration 29% Needle Aspiration 3% Chest tube 57% Upfront VATS 4% Factors Promoting VATS during initial admission: Persistent air leak 94% Failure of complete lung expansion 57% Recurrence after chest tube removal 78%

40. Literature review Journal of Laparoendoscopic & Advanced Surgical Techniques 2018 Current Practice in the Management of Spontaneous Pneumothorax in Children. Williams K1,2, Baumann L2,3, Grabowski J2,4, Lautz TB2,4. J LaparoendoscAdvSurg Tech A. 2018 Dec 28. doi: 10.1089/lap.2018.0629. Observation of air leak before preforming VATS (days) 1 day 2% 2 days 27% 3 days 39% 4 days 11% 5 or more days 20% Influence of size of pneumothorax Strong 43% Moderate 38% None 19%

41. Literature review Journal of Laparoendoscopic & Advanced Surgical Techniques 2018 Current Practice in the Management of Spontaneous Pneumothorax in Children. Williams K1,2, Baumann L2,3, Grabowski J2,4, Lautz TB2,4. J LaparoendoscAdvSurg Tech A. 2018 Dec 28. doi: 10.1089/lap.2018.0629. Timing of Surgery Second episode 82% Third or more episodes 4% Operative Management: VATS was favored operative approach vs. thoracotomy for both initial and recurrent spontaneous pneumothorax. Most surgeons perform a surgical blebectomy and mechanical pleurodesis. Agent for Chemical Pleurodesis Talc 70% Minocycline 0% Doxyclycline 30%

42. Literature review American Journal of Respiratory and Critical Care Medicine Vol 164, No. 9, November 01, 2001 Talc Should Not Be Used for Pleurodesis in Patients with Nonmalignant pleural effusions. American Journal of Respiratory and Critical Care Medicine Vol 164, No. 9, November 01, 2001 Conclusions: Talc can be used for patients with malignant recurrent effusions. Talc can cause an increase risk of malignant mesothelioma.

43. Thank you! Division of Pediatric Surgery Stony Brook Children's Christopher Muratore, MD Division Chief Richard Scriven, MD Charles Coren, MD Helen Hsieh, MD, PhD Erica Gross, MD Michelle Ceo, CPNP Joanne Camarda, CPNP

44. Thank you!