Tiffany Sinclair

The first 100 infant thoracoscopic lobectomies: Observations through the learning curve and comparison to open lobectomy

OBJECTIVE The objective of the study is to describe our initial 100 attempted infant thoracoscopic lobectomies for asymptomatic, prenatally diagnosed lung lesions, and compare the results to contemporaneous age-matched patients undergoing open lobectomy. BACKGROUND Infant thoracoscopic lobectomy is a technically challenging procedure, which has only gained acceptance worldwide in recent years. METHODS This is a retrospective review of all patients undergoing thoracoscopic or open lung lobectomy between March 2005 and January 2014. Included were all asymptomatic infants younger than 4months. Excluded were patients undergoing emergent lobectomy and patients with isolated extralobar bronchopulmonary sequestrations. RESULTS A total of 100 attempted thoracoscopic lobectomies were compared with 188 open lobectomies. In the thoracoscopic group, mean age and weight at surgery were 7.3weeks and 4.8kg, mean operative time was 185minutes, and mean hospital stay was 3days. Twelve cases were converted to open (12%). Ten conversions occurred within the first third of the series and none in the last third. There were no mortalities. There were no differences between the thoracoscopic and open groups in perioperative complications or hospital stay. There was a significant difference in the operative time: 111minutes vs. 185minutes (open vs. thoracoscopic; p<0.001). There was a higher mean end-tidal carbon dioxide (ETCO2) and lower mean peripheral capillary oxygen saturation (SpO2) in the thoracoscopic group versus the open group (51.7 versus 38.6mmHg and 97.5 versus 99.1%, respectively). CONCLUSION In high volume centers, the learning curve of thoracoscopic lobectomy can be overcome and the procedure can be performed with equivalent outcomes and, in our opinion, superior cosmetic results to open lobectomy.

Acylcarnitine Profiles Reflect Metabolic Vulnerability for Necrotizing Enterocolitis in Newborns Born Premature

Objective To evaluate the association between newborn acylcarnitine profiles and the subsequent development of necrotizing enterocolitis (NEC) with the use of routinely collected newborn screening data in infants born preterm. Study design A retrospective cohort study was conducted with the use of discharge records for infants born preterm admitted to neonatal intensive care units in California from 2005 to 2009 who had linked state newborn screening results. A model‐development cohort of 94 110 preterm births from 2005 to 2008 was used to develop a risk‐stratification model that was then applied to a validation cohort of 22 992 births from 2009. Results Fourteen acylcarnitine levels and acylcarnitine ratios were associated with increased risk of developing NEC. Each log unit increase in C5 and free carnitine /(C16 + 18:1) was associated with a 78% and a 76% increased risk for developing NEC, respectively (OR 1.78, 95% CI 1.53‐2.02, and OR 1.76, 95% CI 1.51‐2.06). Six acylcarnitine levels, along with birth weight and total parenteral nutrition, identified 89.8% of newborns with NEC in the model‐development cohort (area under the curve 0.898, 95% CI 0.889‐0.907) and 90.8% of the newborns with NEC in the validation cohort (area under the curve 0.908, 95% CI 0.901‐0.930). Conclusions Abnormal fatty acid metabolism was associated with prematurity and the development of NEC. Metabolic profiling through newborn screening may serve as an objective biologic surrogate of risk for the development of disease and thus facilitate disease‐prevention strategies.

Initial experience with peroral endoscopic myotomy for treatment of achalasia in children

BACKGROUND Achalasia is a primary esophageal motility disorder characterized by aperistalsis of the esophagus and failed relaxation of the lower esophageal sphincter that presents rarely in childhood. The peroral endoscopic myotomy (POEM) procedure is an emerging treatment for achalasia in adults that has recently been introduced into pediatric surgical practice. METHODS This is a prospective case series of all children referred to Stanford University Lucile Packard Childrens Hospital with manometry-confirmed achalasia who underwent a POEM procedure from 2014 to 2016. RESULTS We enrolled 10 subjects ranging in age from 7 to 17years (M=13.4). The mean pre- and 1-month post-procedure Eckardt scores were 7 (SD=2.5) and 2.4 (SD=2) (p<0.001), respectively. The median procedure time for the entire cohort was 142min (range 60-259min) with ongoing improvement with increased experience (R2=0.6, p=0.008). There were no major adverse events. CONCLUSION The POEM procedure can be successfully completed in children for the treatment of achalasia with demonstrated short-term post-operative improvement in symptoms. The adoption of advanced endoscopic techniques by pediatric surgeons may enable development of unique intraluminal approaches to congenital anomalies and other childhood diseases. LEVEL OF EVIDENCE Treatment Study - Level IV.

The challenges of closing an ileostomy in patients with total intestinal aganglionosis after small bowel transplant

We present the case of a 14-year-old male with a history of small bowel transplantation for long segment Hirschsprung’s disease who underwent Duhamel ileorectal pull-through procedure. In post-transplant, the patient had no restrictions and was not TPN-dependent. To improve his quality of life, he and his family were interested in closing the ileostomy and undergoing pull-through surgery. The complexity of the case includes the presence of an aganglionic rectal segment—a short root of the mesentery due to the small bowel transplant—and significant immunosuppression. At the moment, he is continent, doing well, and has not had any remarkable complications.

Pleomorphic myxoid liposarcoma in an adolescent with Li-Fraumeni syndrome

We present the case of a 15-year-old female with a right perineal mass that was found to be pleomorphic myxoid liposarcoma, a recently recognized, rare subtype of liposarcoma. The patient had a strong family history of malignancy and genetic screening revealed a pathogenic TP53 mutation consistent with Li–Fraumeni syndrome.

Advanced minimal access surgery in infants weighing less than 3 kg: A single center experience

BACKGROUND Minimal access surgery (MAS) has gained popularity in infants less than 5kg, however, significant challenges still arise in very low weight infants. STUDY DESIGN A retrospective chart review was performed to identify all infants weighing less than 3kg who underwent an advanced MAS or equivalent open procedure from 2009 to 2016. Advanced case types included Nissen fundoplication, duodenal atresia repair, Ladd procedure, congenital diaphragmatic hernia repair, esophageal atresia/tracheoesophageal fistula repair, diaphragmatic plication, and pyloric atresia repair. A comparative analysis was performed between the MAS and open cohorts. RESULTS A total of 45 advanced MAS cases and 17 open cases met the inclusion criteria. Gestational age and age at operation were similar between the cohorts, while infants who underwent open procedures had significantly lower weight at operation (p=0.003). There were no deaths within 30days related to surgery in either group. Only 3 MAS cases required unintended conversion to open. There were 2 (4.4%) postoperative complications related to surgery in the MAS cohort and 2 (11.8%) in the open cohort. CONCLUSION Advanced MAS may be performed in infants weighing less than 3kg with low mortality, acceptable rates of conversion, and similar rates of complications as open procedures. TYPE OF STUDY Prognosis study. LEVEL OF EVIDENCE Level III.

Endoscopic Division of Duodenal Web Causing Near Obstruction in 2-Year-Old with Trisomy 21

INTRODUCTION Surgical intervention for duodenal atresia most commonly entails duodenoduodenostomy in the neonatal period. Occasionally, type I duodenal atresia with incomplete obstruction may go undiagnosed until later in life. Endoscopic approach to dividing intestinal webs has been reported as successful in patients as young as 7 days of age, and can be a useful modality particularly in patients with comorbidities who may not tolerate open or laparoscopic surgery. METHODS A 2-year-old female with a history of trisomy 21 and tetralogy of Fallot underwent laparoscopic and endoscopic exploration of intestinal obstruction as seen on upper gastrointestinal series for symptoms of recurrent emesis and weight loss. After laparoscopy confirmed a duodenal web as the cause of intestinal obstruction, endoscopic division of the membrane was carried out with a triangle tip electrocautery knife and 15 mm radially dilating balloon. RESULTS The patient tolerated the procedure well, and also tolerated full age-appropriate diet by time of discharge on postoperative day 2. She remains asymptomatic as of 6 months postoperatively. CONCLUSIONS This report describes a successful endoscopic approach for definitive treatment of a duodenal web in a 2-year-old girl with trisomy 21, and laparoscopy confirmed no intraabdominal obstructive process or complication from endoscopy. Endoscopy enables minimal recovery time and suggests an improved method of duodenal web division over pure surgical intervention.

Gastrointestinal Mucormycosis Initially Manifest as Hematochezia from Arterio-Enteric Fistula

A 41-year-old man was evaluated in the emergency department for acute hematochezia accompanied by hemorrhagic shock. Ten months prior to presentation, he had received a hematopoietic stem cell transplant from an HLA-matched, related donor for Philadelphia chromosome-positive B cell acute lymphoblastic leukemia (ALL). He also had a history of Stage III rectal cancer treated with neo-adjuvant chemoradiation (cetuximab/capecitibine), low anterior resection, and adjuvant chemotherapy (capecitabine/oxaliplatin) 5 years prior to transplantation. On presentation, there was no evidence of either malignancy. After stabilization with massive transfusion of blood products, computed tomography (CT) angiography of the abdomen and pelvis demonstrated an arterio-enteric fistula between the left common iliac artery and the sigmoid colon (Fig. 1). Interventional radiology (IR) emergently performed angiography followed by successful deployment of a left common iliac artery stent (Fig. 2). The patient had experienced cardiopulmonary arrest twice during the resuscitation which led to multi-organ failure, need for vasopressor support, and continuous renal replacement therapy. Once stabilized, he underwent flexible sigmoidoscopy that demonstrated friable and ulcerated mucosa in the sigmoid colon; biopsies were consistent with invasive mold infection (Fig. 3). Amphotericin B, caspofungin, and posaconazole were started for presumed mucormycosis. During the first three hospital days, he developed small, tender, violaceous nodules along the medial aspect of his left thigh, calf, and foot. Biopsy of the sentinel lesion also confirmed fungus. As these lesions were in the same arterial distribution, radical surgical debridement of all involved tissues was still recommended. CT of the thorax, CT of the head, and magnetic resonance imaging (MRI) of the sinuses confirmed that there were no other areas of dissemination. A multidisciplinary, staged resection was planned. First, a left femoral artery–femoral artery bypass with a polytetrafluoroethylene graft was performed. Next, at exploratory laparotomy, an infected left common and internal iliac artery, sigmoid colon, segment of small intestine, left kidney, and ureter were identified. He underwent excision of left common and external iliac artery and stent, total abdominal colectomy, left nephron-ureterectomy, and temporary abdominal closure. Two days later, he underwent intra-peritoneal washout with amphotericin B (two liters of 5 mg/ml amphotericin B solution), creation of an end ileostomy, and abdominal wall closure. Final pathology from colonic biopsies demonstrated fungal colitis with extensive necrosis. Organisms were J. M. Cloyd (&) T. Sinclair A. Shelton Division of General Surgery, Department of Surgery, Stanford University, 300 Pasteur Dr., MC5641, Stanford, CA 94305, USA e-mail: jcloyd@stanford.edu