Congenital cystic lung malformations encompass a diverse group of lesions that arise during in utero development. A variety of pathologic classification schemes have been proposed, but recent advances suggest that gross and microscopic morphologies can often be explained by the underlying mechanism of cyst development. A subset of congenital pulmonary airway malformations, usually with large cysts, is associated with widespread epithelial oncogenic KRAS mutation. These have a long-term risk of developing mucinous adenocarcinoma. Another subset, more commonly with smaller cysts, are associated with bronchial atresia or other mechanisms of obstruction. The histology of atresia specimens overlaps with that of intra- and extra-lobar sequestrations, which additionally have an extra hilum-like structure suggesting that they arise due to an ectopic portion of lung developing without normal fluid-flow in utero. These lesions are not expected to have a long-term risk of cancer but may become infected due to abnormal clearance. Finally, a subset of lesions associated with DICER1 mutation may arise in utero or after birth and are best classified as cystic pleuropulmonary blastoma. The term congenital pulmonary airway malformation is no longer in use for these cysts, which may progress to a solid aggressive sarcoma or may regress to a bland and benign multiloculated cyst. While surgical management may be the same independent of the underlying pathology (open or thoracoscopic lobectomy), post-natal pathologic examination contributes to patient care by establishing a diagnosis that goes beyond cyst size. This may guide future treatment decisions including monitoring, molecular testing, or genetic counseling.
Appendicitis remains the most common surgical emergency in children and adolescents, yet its epidemiology and pathophysiology remain incompletely understood. Studies have demonstrated substantial geographic, racial, temporal, and socioeconomic variation in appendicitis incidence and severity. Advances in population-based investigation, imaging, and microbiome analysis have further challenged the traditional concept of appendicitis as a uniform disease process caused solely by luminal obstruction. Increasing evidence instead supports a multifactorial process influenced by environmental exposures, host susceptibility, microbial factors, and healthcare access. Epidemiologic observations additionally suggest important differences between uncomplicated and perforated appendicitis. Improved understanding of these relationships has important implications for disease classification, risk stratification, and future treatment strategies. This review summarizes the current understanding of the incidence, epidemiologic trends, and pathophysiology of pediatric appendicitis.
It is reasonable to offer nonoperative management (NOM) of acute uncomplicated appendicitis as an alternative treatment option to appendectomy in cases meeting established evidence-based criteria. As public awareness of this option has increased, patients and families are more likely to inquire about NOM. Shared decision-making (SDM) is well suited for instances where there is not one clear recommended treatment option, as it allows the patient and family's preferences and values to be prioritized. When applying SDM to the pediatric patient, both the patient and the family should be included in the decision-making process. We provide case-based examples of SDM for determining the treatment course for pediatric patients with acute uncomplicated appendicitis.
Pediatric intestinal failure resulting from short bowel syndrome remains a significant source of morbidity and mortality despite advances in parenteral nutrition and multidisciplinary intestinal rehabilitation. While native intestinal adaptation provides a foundation for recovery in many patients, those with the most severe forms of short bowel syndrome face limited therapeutic options, including lifelong parenteral nutrition dependence, surgical lengthening procedures, and intestinal transplantation-each associated with substantial complications. The field of bowel regeneration has expanded dramatically over the past two decades, drawing on advances in stem cell biology, biomaterials science, mechanotransduction, and bioengineering. This review examines the current landscape of bowel regeneration strategies through a conceptual framework that progresses from native adaptation, through cellular and biological therapies, to structural and mechanical approaches, and ultimately to tissue engineering as the integration of cellular and structural strategies. Emerging technologies including gene editing for regional reprogramming of intestinal identity, three-dimensional bioprinting, and advanced organoid-scaffold systems are discussed as they relate to the future of clinical translation. Although significant hurdles remain-particularly in vascularization, innervation, and scaling of engineered constructs-the trajectory of the field suggests that regenerative approaches may fundamentally alter the management of pediatric intestinal failure within the coming decades.
