RESUMEN
Congenital portosystemic shunts (CPSS) or congenital extrahepatic portosystemic shunts (CEPS) is a rare malformation. This congenital anomaly presents with a diverse array of clinical manifestations, ranging from asymptomatic to severe complications such as cardiac failure, pronounced pulmonary hypertension, and widespread pulmonary arteriovenous malformations. CPSS increases the risk of developing benign or malignant liver tumors, including nodular regenerative hyperplasia, focal nodular hyperplasia, hepatic adenoma, hepatocellular carcinoma, and hepatoblastoma. We report a case of a 15-month-old boy, identified with Abernethy's malformation type Ib, who presented with an abdominal mass during a follow-up. A comprehensive assessment established a diagnosis of hepatoblastoma. The patient was transferred to a specialized liver transplant center for further treatment and management. This is a review of literature highlighting the complexity of Abernethy malformation and its associated risk of liver tumors.
RESUMEN
Granulicatella adiacens is a rare variant of the Streptococcus bacteria. When isolated, G. adiacens has been described in cases of endocarditis and bacteremia, but less commonly seen in isolated pyogenic infections. We report a case of a parapharyngeal abscess caused by G. adiacens in an otherwise healthy 10-month-old infant, which was successfully treated with antibiotics and surgical drainage. To the best of our knowledge, this is the first described case of a pediatric deep soft tissue neck infection caused by G. adiacens with one other report in an adult. Additionally, of all localized infections from this bacteria, this is only the second reported case in the pediatric population. We also include an evidence-based literature review of the clinical presentation, microbiology, imaging modalities, and management approach to deep neck infections (DNIs).
RESUMEN
Background The use of circular frames in correcting lower limb deformity is well-established and has evolved dramatically over the years. Three new frames have been introduced recently, and this study is set to compare them in terms of accuracy and efficiency in correcting a similar long bone deformity. These frames are the Taylor Spatial Frame (TSF; Smith & Nephew, London, United Kingdom), the Truelok Hexapod System (TL-HEX; Orthofix, Lewisville, Texas), and Orthex (OrthoPediatrics, Warsaw, Indiana). Methods This is a biomechanical study comparing the above three types of circular frames to correct similar deformities in Sawbones models. The deformities that are compared were: (1) 30° valgus deformity of the distal femur; (2) 30° varus deformity of the proximal tibia. Each frame was applied to the deformed bone in the standard way that we apply to normal bone. X-rays were taken before and after the deformity correction. The frames' software was used to estimate the deformities. The variations between the software's estimations and the known bone deformities were compared. Residual deformity after initial correction and the number of re-programmings was compared among these three frames. The least residual deformity and re-programming is the favorable outcome. Results All the Sawbones models had a 30° actual coronal angulation. The Orthex software estimated the deformity at around 25.35° (SD 4.6), TSF 25.6° (SD 2), and TL-HEX 29.87° (SD 2.1). One-way analysis of variance (ANOVA) showed a significant difference in the findings (P-value 0.014). Accuracy was measured by comparing the residual deformity in angulation in the coronal plane after the first and second correction. The Orthex median residual deformity was 1°, TSF was 2.5°, and TL-HEX was 3° with a range of less than 5° for all of them. The independent samples Kruskal-Wallis test shows that there is no significant difference between the three groups (P=0.549). The frequency of strut changes required throughout the correction was not significant among the three frames using the Fisher exact test (P=0.336). TSF struts are not designed to be readjusted. Conclusion The three frames were comparable in terms of accurate correction of the two deformities, strut changes, and strut adjustments. The TL-HEX frame software was superior to other frames in terms of analyzing the deformity but the difference, although statistically significant clinically, was not.
RESUMEN
Neuroendocrine cell hyperplasia of infancy (NEHI) is a recently reported condition and commonly missed. A general pediatrician who encounters an infant with an insidious onset of breathlessness, hypoxemia, and failure to thrive should think through a diagnosis of NEHI when common respiratory diseases are excluded. Lung biopsy is regarded as the diagnostic gold standard for NEHI and typically demonstrates increased numbers of neuroendocrine cells (NECs) in otherwise near-normal lung tissues. However, classic high-resolution computed tomography (HRCT) findings can enable to establish the diagnosis without the need for a biopsy. This case shows typical chest imaging findings of NEHI with a brief review of the literature.
