Progressive Metabolic Dysfunction and Nutritional Variability Precedes Necrotizing Enterocolitis.
2020; 12 (5)
Necrotizing Enterocolitis (NEC) is associated with prematurity, enteral feedings, and enteral dysbiosis. Accordingly, we hypothesized that along with nutritional variability, metabolic dysfunction would be associated with NEC onset. Methods: We queried a multicenter longitudinal database that included 995 preterm infants (<32 weeks gestation) and included 73 cases of NEC. Dried blood spot samples were obtained on day of life 1, 7, 28, and 42. Metabolite data from each time point included 72 amino acid (AA) and acylcarnitine (AC) measures. Nutrition data were averaged at each of the same time points. Odds ratios and 95% confidence intervals were calculated using samples obtained prior to NEC diagnosis and adjusted for potential confounding variables. Nutritional and metabolic data were plotted longitudinally to determine relationship to NEC onset. Results: Day 1 analyte levels of alanine, phenylalanine, free carnitine, C16, arginine, C14:1/C16, and citrulline/phenylalanine were associated with the subsequent development of NEC. Over time, differences in individual analyte levels associated with NEC onset shifted from predominantly AAs at birth to predominantly ACs by day 42. Subjects who developed NEC received significantly lower weight-adjusted total calories (p < 0.001) overall, a trend that emerged by day of life 7 (p = 0.020), and persisted until day of life 28 (p < 0.001) and 42 (p < 0.001). Conclusion: Premature infants demonstrate metabolic differences at birth. Metabolite abnormalities progress in parallel to significant differences in nutritional delivery signifying metabolic dysfunction in premature newborns prior to NEC onset. These observations provide new insights to potential contributing pathophysiology of NEC and opportunity for clinical care-based prevention.
View details for DOI 10.3390/nu12051275
View details for PubMedID 32365850
Surgery for adrenocortical carcinoma: When and how?
Best practice & research. Clinical endocrinology & metabolism
Adrenocortical carcinoma (ACC) is a rare malignancy that is frequently asymptomatic at presentation, yet has a high rate of metastatic disease at the time of diagnosis. Prognosis is overall poor, particularly with cortisol-producing tumors. While the treatment of ACC is guided by stage of disease, complete surgical resection is the most important step in the management of patients with primary, recurrent, or metastatic ACC. Triphasic chest, abdomen, and pelvis computer tomography (CT) scans and 18F flourodeoxyglucose positron emission tomography CT scanning are essential for accurate staging; moreover, MRI may be helpful to identify liver metastasis and evaluate the involvement of adjacent organs for operative planning. Surgical resection with negative margins is the single most important prognostic factor for survival in patients with ACC. To achieve the highest rate of R0 resection, open adrenalectomy is the gold standard surgical approach for confirmed or highly suspected ACC. It is extremely important that the tumor capsule is not ruptured, regardless of the surgical approach used. The best post-operative outcomes (complications and oncologic) are achieved by high-volume surgeons practicing at high-volume centers.
View details for DOI 10.1016/j.beem.2020.101408
View details for PubMedID 32265101
- Correction to: Delayed appearance of mature ganglia in an infant with an atypical presentation of total colonic and small bowel aganglionosis: a case report. BMC pediatrics 2019; 19 (1): 172
Delayed appearance of mature ganglia in an infant with an atypical presentation of total colonic and small bowel aganglionosis: a case report.
2019; 19 (1): 93
BACKGROUND: Total colonic and small bowel aganglionosis (TCSA) occurs in less than 1% of all Hirschsprung's disease patients. Currently, the mainstay of treatment is surgery. However, in patients with TCSA, functional outcomes are often poor. A characteristic transition zone in TCSA can be difficult to identify which may complicate surgery and may often require multiple operations.CASE PRESENTATION: We present the case of a male infant who was diagnosed with biopsy-proven total colonic aganglionosis with extensive small bowel involvement as a neonate. The patient was diverted at one month of age based on leveling biopsies at 10cm from the Ligament of Treitz. At 7months of age, during stoma revision for a prolapsed stoma, intra-operative peristalsis was observed in nearly the entire length of the previously aganglionic bowel, and subsequent biopsies demonstrated the appearance of mature ganglion cells in a previously aganglionic segment.CONCLUSIONS: TCSA remains a major challenge for pediatric surgeons. Our case introduces new controversy to our understanding of aganglionosis. Our observations warrant further research into the possibility of post-natal ganglion maturation and encourage surgeons to consider a more conservative surgical approach.
View details for PubMedID 30953480
Comparison of laparoscopic and open pediatric inguinal hernia repairs at two institutions.
Pediatric surgery international
BACKGROUND/PURPOSE: The proposed benefits of laparoscopic inguinal hernia repair in the pediatric population include easier access to the contralateral groin and avoidance of manipulation of the spermatic cord; however, some studies also report higher recurrence rates. Due to these differences, the traditional open technique is still used by many pediatric surgeons. The objective of this study is to compare the outcomes of two institutions that employed different techniques.METHODS: We retrospectively reviewed pediatric patients who had open repair of inguinal hernias at hospital A or laparoscopic repair at hospital B. Data collection included age of patients, laterality, operative time, and complications.RESULTS: From 2010 to 2015, 154 patients underwent open repair at hospital A and 220 patients underwent laparoscopic repair at hospital B. The mean operative time was 52min for the open technique and 23min for the laparoscopic technique (p<0.01). There were 2.6% complications and 0.65% recurrences with the open technique, compared to 4.6% complications and 2.7% recurrences with the laparoscopic technique (p>0.2).CONCLUSION: Laparoscopic hernia repairs at hospital B are associated with shorter operative times and have similar outcomes as open repairs at hospital A. A prospective study with both techniques done at the same institution is warranted.
View details for PubMedID 30317375
Endoscopic Excision of Benign Facial Masses in Children: A Review of Outcomes.
Journal of laparoendoscopic & advanced surgical techniques. Part A
Benign masses of the eyebrow and forehead are common in pediatric patients and can result in facial asymmetry, discomfort, or super-infection. Excision is classically conducted via an incision directly over the mass, which can produce sub-optimal cosmesis. Recently, an endoscopic approach using pediatric brow-lift equipment has been adopted. We reviewed our center's experience with endoscopic removal of benign facial lesions and compared these cases with an equivalent series of open cases.A retrospective chart review was conducted to identify pediatric cases of endoscopic and open removal of benign eyebrow or forehead lesions at our institution from 2009 to 2016. Clinical and cosmetic outcomes were reviewed.A total of 40 endoscopic and 25 open cases of excision of benign facial lesions in children were identified. For the patients who underwent endoscopic excision, the majority (85%) presented with a cyst located at the eyebrow. Histologic examination revealed 36 dermoid cysts (90%), 2 epidermal cysts, and 2 pilomatrixomas. Of the 36 cases with post-operative follow-up, 32 patients (89%) had an uncomplicated recovery with good cosmesis. Two patients had an eyebrow droop that resolved without intervention. One patient had localized numbness overlying the site, but no motor deficits. One patient presented with a recurrent dermoid cyst that required open resection. For the patients who underwent open excision, the majority (52%) had dermoid cysts located at the eyebrow. Of the 22 cases with follow-up, 20 of the patients had an uncomplicated recovery (90%). Comparing the rate of complications, there was no statistically significant difference between the two groups (P = 1.0).Endoscopic excision of benign forehead and eyebrow lesions in pediatric patients is feasible and yields excellent cosmetic results. When compared with open excision, complication rates are similar between both approaches and a facial scar can be avoided with an endoscopic approach.
View details for PubMedID 29446701
The challenges of closing an ileostomy in patients with total intestinal aganglionosis after small bowel transplant
PEDIATRIC SURGERY INTERNATIONAL
2018; 34 (1): 113–16
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.
View details for PubMedID 29170900
Advanced minimal access surgery in infants weighing less than 3kg: A single center experience.
Journal of pediatric surgery
Minimal access surgery (MAS) has gained popularity in infants less than 5kg, however, significant challenges still arise in very low weight infants.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.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.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.Prognosis study.Level III.
View details for DOI 10.1016/j.jpedsurg.2017.05.006
View details for PubMedID 28549685
Regional block via continuous caudal infusion as sole anesthetic for inguinal hernia repair in conscious neonates.
Pediatric surgery international
2017; 33 (3): 341-345
The use of general anesthesia in young children has come under increasing scrutiny due to its potential long-term neurotoxic effects. Meanwhile, regional anesthesia for surgical procedures in neonates has many advantages, including preservation of respiratory status and faster return to feeding. We describe the successful use of 3% 2-chloroprocaine administered via continuous caudal infusion as the sole anesthetic agent during elective surgical procedures in infants.A retrospective chart review of all patients who underwent elective surgical procedures under continuous caudal regional anesthetic at a single institution was performed. Thirty patients (27 males, three females) were identified: 28 patients underwent inguinal hernia repairs. Caudal anesthesia was established via continuous infusion of 3% 2-chloroprocaine through an indwelling catheter.Successful analgesia by regional block alone was achieved in all patients for the duration of each surgical procedure without need for rescue anesthesia. Mean operative time was 49 min. Patients were able to return to feeding immediately after surgery and were ready for discharge home within that day.Continuous caudal infusion of chloroprocaine is a safe and effective way to maintain adequate analgesia for elective surgeries in infants. This successful regional approach obviates the use of general anesthetic which reduces post-operative recovery time and avoids concerns for neurotoxicity.
View details for DOI 10.1007/s00383-016-4027-6
View details for PubMedID 27873010
Pleomorphic myxoid liposarcoma in an adolescent with Li-Fraumeni syndrome.
Pediatric surgery international
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.
View details for DOI 10.1007/s00383-017-4063-x
View details for PubMedID 28160093
Acylcarnitine Profiles Reflect Metabolic Vulnerability for Necrotizing Enterocolitis in Newborns Born Premature.
journal of pediatrics
2017; 181: 80-85 e1
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.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.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).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.
View details for DOI 10.1016/j.jpeds.2016.10.019
View details for PubMedID 27836286
Initial experience with peroral endoscopic myotomy for treatment of achalasia in children.
Journal of pediatric surgery
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.This is a prospective case series of all children referred to Stanford University Lucile Packard Children's Hospital with manometry-confirmed achalasia who underwent a POEM procedure from 2014 to 2016.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.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.Treatment Study - Level IV.
View details for PubMedID 28827050
Endoscopic Division of Duodenal Web Causing Near Obstruction in 2-Year-Old with Trisomy 21
JOURNAL OF LAPAROENDOSCOPIC & ADVANCED SURGICAL TECHNIQUES
2016; 26 (5): 413-417
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.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.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.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.
View details for DOI 10.1089/lap.2015.0462
View details for Web of Science ID 000376469600014
View details for PubMedID 26913816
The first 100 infant thoracoscopic lobectomies: Observations through the learning curve and comparison to open lobectomy
JOURNAL OF PEDIATRIC SURGERY
2015; 50 (11): 1811-1816
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.Infant thoracoscopic lobectomy is a technically challenging procedure, which has only gained acceptance worldwide in recent years.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.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).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.
View details for DOI 10.1016/j.jpedsurg.2015.05.015
View details for Web of Science ID 000367580400001
View details for PubMedID 26100691
- Gastrointestinal Mucormycosis Initially Manifest as Hematochezia from Arterio-Enteric Fistula DIGESTIVE DISEASES AND SCIENCES 2014; 59 (12): 2905-2908
- Gastrointestinal mucormycosis initially manifest as hematochezia from arterio-enteric fistula. Digestive diseases and sciences 2014; 59 (12): 2905-2908