Appendicitis and its associated mortality and morbidity in infants up to 3 months of age:A systematic review

Background and Aims: Although appendicitis is rare in young infants, the reported mortality is high. Primary aim of this systematic review was to provide updated insights in the mortality and morbidity (postoperative complications, Clavien-Dindo grades I–IV) of appendicitis in infants ≤3 months of age. Secondary aims comprised the evaluation of patient characteristics, diagnostic work-up, treatment strategies, comorbidity, and factors associated with poor outcome. Methods: This systematic review was reported according to the PRISMA statement with a search performed in Pubmed, Embase and Web of Science (up to September 5th 2022). Original articles (published in English ≥1980) reporting on infants ≤3 months of age with appendicitis were included. Both patients with abdominal appendicitis and herniated appendicitis (such as Amyand's hernia) were considered. Data were provided descriptively. Results: In total, 131 articles were included encompassing 242 cases after identification of 4294 records. Overall, 184 (76%) of the 242 patients had abdominal and 58 (24%) had herniated appendicitis. Two-hundred (83%) of the patients were newborns (≤28 days) and 42 (17%) were infants between 29 days and ≤3 months of age. Either immediate, or after initial conservative treatment, 236 (98%) patients underwent surgical treatment. Some 168 (69%) patients had perforated appendicitis. Mortality was reported in 20 (8%) patients and morbidity in an additional 18 (8%). All fatal cases had abdominal appendicitis and fatal outcome was relatively more often reported in newborns, term patients, patients with relevant comorbidity, nonperforated appendicitis and those presented from home. Conclusion: Mortality was reported in 20 (8%) infants ≤3 months of age and additional morbidity in 18 (8%). All patients with fatal outcome had abdominal appendicitis. Several patient characteristics were relatively more often reported in infants with poor outcome and adequate monitoring, early recognition and prompt treatment may favour the outcome.</p

The Diagnostic Accuracy of Serum Alpha-Fetoprotein Levels in Follow-up for Recurrence of Sacrococcygeal Teratoma; a Nationwide Review of SCT Cases in the Netherlands

Background: Serum alpha-fetoprotein (AFP) is often used as tumour marker for recurrent sacrococcygeal teratoma (SCT). We aimed to assess the normal dynamics of serum AFP levels after initial resection and diagnostic accuracy of serum AFP levels the follow-up for recurrence in SCT. Methods: This retrospective study included 57 patients treated for SCT in the six pediatric surgical centers in the Netherlands from 1980 to 2018. Main results: 57 patients were included in the study of whom 19 children developed 20 recurrences at a median of 14.0 months after initial resection. No significant difference was found in serum AFP level dynamics between the recurrence and non-recurrence group after initial resection (p = 0.950). Serum AFP levels did not significantly increase before recurrence (p = 0.106) compared to serum AFP levels of children without recurrence at the same time. However, serum AFP levels did significantly increase in malignant recurrences (n = 7) (p = 0.03) compared to patients without recurrence. A cut-off value of 55 μg/L was found to be predictive for recurrent SCT with an Area Under the Curve (AUC) of 0.636 with sensitivity of 50% and specificity of 100%. Conclusion: Dynamics of serum AFP levels are not different between patients with and without recurrence after initial resection of SCT. Serum AFP levels are not predictive for mature or immature recurrent SCT and normal AFP levels do not rule out recurrent SCT. However, serum AFP levels exceeding 55 μg/L can indicate recurrent SCT, especially malignant recurrences.</p

Opposite incidence trends for differentiated and medullary thyroid cancer in young dutch patients over a 30‐year time span

Thyroid cancer is the most common endocrine malignancy in children. A rising incidence has been reported worldwide. Possible explanations include the increased use of enhanced imaging (leading to incidentalomas) and an increased prevalence of risk factors. We aimed to evaluate the incidence and survival trends of thyroid cancer in Dutch children, adolescents, and young adults (0–24 years) between 1990 and 2019. The age-standardized incidence rates of differentiated thyroid cancer (DTC, including papillary and follicular thyroid cancer (PTC and FTC, respectively)) and medullary thyroid cancer (MTC), the average annual percentage changes (AAPC) in incidence rates, and 10-year overall survival (OS) were calculated based on data obtained from the nationwide cancer registry (Netherlands Cancer Registry). A total of 839 patients aged 0–24 years had been diagnosed with thyroid carcinoma (PTC: 594 (71%), FTC: 128 (15%), MTC: 114 (14%)) between 1990 and 2019. The incidence of PTC increased significantly over time (AAPC +3.6%; 95%CI +2.3 to +4.8), the incidence rate of FTC showed a stable trend ((AAPC −1.1%; 95%CI −3.4 to +1.1), while the incidence of MTC decreased significantly (AAPC: −4.4% (95%CI −7.3 to −1.5). The 10-year OS was 99.5% (1990–1999) and 98.6% (2000–2009) in patients with DTC and 92.4% (1990–1999) and 96.0% (2000–2009) in patients with MTC. In this nationwide study, a rising incidence of PTC and decreasing incidence of MTC were observed. For both groups, in spite of the high proportion of patients with lymph node involvement at diagnosis for DTC and the limited treatment options for MTC, 10-year OS was high

Risk factors for enterocolitis in patients with Hirschsprung disease:A retrospective observational study

Introduction: Hirschsprung-associated enterocolitis (HAEC) accounts for substantial morbidity and mortality in patients with Hirschsprung disease (HD). The aim of this study was to identify incidence of pre- and postoperative HAEC in our consecutive cohort and to identify patient and clinical characteristics that are associated with developing postoperative HAEC and HAEC-free interval. Material and methods: A retrospective cohort study was performed with all 146 HD patients treated between 2000 and 2017. Data were retrieved from the medical records. HAEC was defined as presence of clinical signs of bowel inflammation, that required treatment with intravenous antibiotics and admittance to the hospital during at least two days. To identify risk factor for HAEC, patients with and without a history of postoperative HAEC were compared. Kaplan-Meier and Cox-regression were used to assess HAEC free intervals before and after surgery. Results: Out of 146 patients, 12 patients had pre-operative HAEC (8%) and 31 patients had postoperative HAEC (21%). Median preoperative HAEC free interval was 112 days (IQR 182 days). Length of hospital stay due to readmissions was longer for patients with a history of postoperative HAEC compared to patients without a history of postoperative HAEC (9.5 vs 16 days, U = 1872.5, p = 0.047). Median postoperative HAEC free interval was 226 days. Of the patients who had postoperative HAEC, 66% had their first episode within the first year after surgery and that the incidence of HAEC declined over follow-up. Conclusions: HAEC incidence was relatively low in our population. No patient or clinical characteristics were associated with the risk of postoperative HAEC

Comparison of laparoscopic and open pyloromyotomy: Concerns for omental herniation at port sites after the laparoscopic approach

Pyloromyotomy is a common surgical procedure in infants with hypertrophic pyloric stenosis and can be performed with a small laparotomy or laparoscopically. No specific complications have been documented about one of the approaches. We aim to study (severity of) complications of pyloromyotomy and to compare complications of both approaches. Children undergoing pyloromyotomy between 2007 and 2017 were analyzed retrospectively. Complication severity was classified using the Clavien-Dindo classification. We included 474 infants (236 open; 238 laparoscopic). 401 were male (85%) and median (IQR) age was 33 (19) days. There were 83 surgical complications in 71 patients (15.0%). In the open group 45 infants (19.1%) experienced a complication vs. 26 infants in the laparoscopic group (10.5%)(p = 0.013). Severity and quantity of postoperative complications were comparable between both groups. Serosal tears of the stomach (N = 19) and fascial dehiscence (N = 8) occurred only after open pyloromyotomy. Herniation of omentum through a port site occurred only after laparoscopy (N = 6) and required re-intervention in all cases. In conclusion, the surgical complication rate of pyloromyotomy was 15.0%. Serosal tear of the stomach and fascial dehiscence are only present after open pyloromyotomy and omental herniation after laparoscopy respectively. The latter complication is underestimated and requires attention

A Delphi analysis to reach consensus on preoperative care in Infants with hypertrophic pyloric stenosis

Introduction  Infantile hypertrophic pyloric stenosis (IHPS) is a common gastrointestinal condition that can lead to metabolic alkalosis and, if uncorrected, to respiratory complications. A standardized approach to correct metabolic derangements and dehydration may reduce time until pyloromyotomy while preventing potential respiratory complications. Such an evidence-based policy regarding preoperative care is absent. We aim to formulate a recommendation about preoperative care for infants with IHPS using the Delphi technique. Materials and Methods  The RAND/UCLA appropriateness method was used to reach international consensus in a panel of pediatric surgeons, pediatric anesthetists, and pediatricians. Statements on type and frequency of blood sampling, required serum concentrations before pyloromyotomy and intravenous fluid therapy, were rated online using a 9-point Likert scale. Consensus was present if the panel rated the statement appropriate/obligatory (panel median: 7-9) or inappropriate/unnecessary (panel median: 1-3) without disagreement according to the interpercentile range adjusted for symmetry formula. Results  Thirty-three and twenty-nine panel members completed the first and second round, respectively. Consensus was reached in 54/74 statements (73%). The panel recommended the following laboratory tests and corresponding cutoff values prior to pyloromyotomy: pH ≤7.45, base excess ≤3.5, bicarbonate &lt;26 mmol/L, sodium ≥132 mmol/L, potassium ≥3.5 mmol/L, chloride ≥100 mmol/L, and glucose ≥4.0 mmol/L. Isotonic crystalloid with 5% dextrose and 10 to 20 mEq/L potassium should be used for fluid resuscitation. Conclusion  Consensus is reached in an expert panel about assessment of metabolic derangements at admission, cutoff serum concentrations to be achieved prior to pyloromyotomy, and appropriate intravenous fluid regime for the correction of dehydration and metabolic derangements in infants with IHPS.</p

Histopathology of human small intestinal and colonic ischemia-reperfusion: Experiences from human IR-models

Intestinal ischemia-reperfusion (IR) injury is a frequent, but potentially life-threatening condition. Although much has been learned about its pathophysiology from animal IR models, the translation to the human setting is imperative for better understanding of its etiology. This could provide us with new insight into development of early detection and potential new therapeutic strategies. Over the past decade, we have studied the pathophysiology of human small intestinal and colonic ischemia-reperfusion (IR) in newly developed human in vivo IR models. In this review, we give an overview of new insights on the sequelae of human intestinal IR, with particular attention for the differences in histopathology between small intestinal and colonic IR

Ultrasonography guided puncture and dilatation in membranous rectal atresia

Purpose: Rectal atresia (RA) is a rare type of anorectal malformation (ARM), occurring in 1–2% of ARM. Discussion remains on optimal treatment strategy for RA. The aim of this study was to present a minimally invasive method to treat patients with membranous RA: ultrasonography guided puncture and dilatation. Cases: Three children are described (1 female, and 2 male). All patients were diagnosed with membranous RA, and treated with ultrasonography guided puncture and dilatation within 48 hours after birth. The procedure was performed under sedation and was uncomplicated in all patients. Follow-up showed good bowel function in 2 of 3 patients. One patient had persistent constipation needing laxative treatment up to the age of 6 years. Conclusions: Rectal atresia is extremely rare, and little is known on optimal treatment methods. Ultrasonography guided puncture and dilatation is a minimally invasive technique to potentially adequately treat patients with membranous RA. In our patients, this treatment strategy has shown to be effective with good long-term outcomes regarding bowel function. Further research should be performed to further investigate optimal treatment for the different types of RA. Level of Evidence: