Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey.

BACKGROUND: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. METHODS: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010-2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). RESULTS: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day-18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. CONCLUSIONS: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

Thrombotic Features as the Primary Cause of SARS-CoV-2 Related Acute Abdomen in Children.

OBJECTIVES: We performed a retrospective case control study to evaluate the histological characteristics of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive pediatric patients undergoing laparoscopic exploration for acute abdomen symptoms. To our knowledge this is the first study that analyzes histopathological characteristics of abdominal tissues in SARS-CoV-2 children. STUDY DESIGN: We enrolled 8 multisystem inflammatory syndrome in children (MIS-C) patients and 4 SARS-CoV-2 positive patients who underwent intestinal resection versus 36 control appendectomies from 2 pediatric tertiary referral centers between March 2020 and July 2021. Surgical resection samples were evaluated on several histological sections focusing on general inflammatory pattern and degree of inflammation. Peculiar histological features (endotheliitis and vascular thrombosis) were semi-quantitatively scored respectively in capillary, veins, and arteries. RESULTS: All SARS-CoV-2 related surgical samples showed thrombotic patterns. Those patterns were significantly less frequent in SARS-CoV-2 negative appendectomies ( P = 0.004). The semi-quantitative score of thrombosis was significantly higher ( P = 0.002) in patients with SARS-CoV-2 related procedures. CONCLUSIONS: Our results showed that SARS-CoV-2 can cause thrombotic damage in abdominal tissues both in the acute phase of the infection (SARS-CoV-2 related appendectomies) and secondary to cytokine storm (MIS-C).

The Role of Manual Detorsion in Pediatric Testicular Torsion During the Global COVID-19 Pandemic: Experience From 2 Centres.

OBJECTIVE: To evaluate the role of emergency manual detorsion as first line management for testicular torsion in the context of the COVID-19 pandemic. METHODS: This retrospective observational study includes 90 pediatric patients ≤14 years old with diagnosis of testicular torsion made at 2 tertiary centers between October 2020 and June 2022. Variables examined included age, presentation delay, surgical wait time, number of attempts at manual testicular detorsion, and manual testicular detorsion success. All patients finally underwent surgery, including contralateral testicular fixation. Outcomes included predictors of successful manual detorsion, testicular findings at surgery, and operation time. RESULTS: Mean (SD) age at diagnosis was 11.51 (2.64) years. Mean presentation delay was 11.76 (13.79) hours. Detorsion was attempted in 72 (80%) patients, resulting successful in 58 (80.5%). Surgical wait time after successful manual detorsion was 22.85 (16.94) hours. On multivariable analysis, successful manual detorsion was associated with a presentation delay<6 hours (odds ratios [OR] 0.154, 95% confidence intervals (CI) 0.036-0.655, P = 0.01) and absence of scrotal edema (OR 0.171, 95% CI 0.038-0.769, P = 0.02). Vice versa, a heterogeneous echo-texture (OR 0.57, 95% CI 0.007-0.461, P = 0.007) and absent blood flow on Doppler ultrasound scan (OR 0.256, 95% CI 0.067-0.971, P = 0.045) were significantly associated with the likelihood of manual detorsion failure. CONCLUSION: In our experience, manual detorsion provided safe and effective emergency treatment for pediatric testicular torsion, especially in absence of edema and when presentation delay is <6 hours. This maneuver should be more widely attempted immediately after diagnosis as temporizing rescue.

Hepatic hemangiomas in childhood: the spectrum of radiologic findings. A pictorial essay.

Hepatic hemangiomas (HHs) are the most common benign liver tumors in infancy, but despite their frequent occurrence, their radiologic appearance may be particularly challenging to assess, owing to unique anatomical and development features that distinguish their subtypes in the pediatric population. There are 3 main patterns of the lesions recognized in HHs: focal, multifocal and diffuse. Medical care strategies range from simple observation to surgery, and a timely diagnosis is crucial to establish the most appropriated clinical management and therapy. Ultrasound (US) is typically the first level examination of pediatric vascular anomalies, able to meet a satisfactory diagnosis and has proved very helpful to assess for involution through serial imaging. CT and MRI are second-level methods seldom performed, offering an anatomical panoramic view and clarification when US is non-conclusive. Histologic confirmation is rarely required. HHs show a variable sonographic appearance, with hypoechoic (mostly) or hyperechoic solitary\multifocal mass and wide features. Hepatic vessels assessment reveals an abnormal flow, enlarged vasa, decreased resistive index (RI) values, scarce blood supply distally to lesions and hyper vascular regions inside them. In this pictorial essay, we review HHs subtypes in further details, illustrate US, CT and MRI findings and clarify a recurrent dispute over the existing terminology.

Long gap esophageal atresia repair using the foker tecnique: a 19 years experience.

Esophageal atresia (EA) with a 3-cm gap or longer and a very short distal segment represents the extreme of this disorder's spectrum, the treatment of which can be challenging. Management of newborns with long-gap esophageal atresia (LGEA) remains a challenge for pediatric surgeons. The inclusion criteria in the study were as follows: long gap esophageal atresia, Foker technique, at least 10 years follow up. We sought to examine our experiences with LGEA using Foker technique. RESULTS: From 2001 to 2020 were operated for esophageal atresia at the UOC Surgery of the AORN Santobono 290 children of whom 16 patients meet our inclusion criteria. 6 had a type I EA, 2 had a type II and 8 had a type III. Mean gap length was 4 cm (range 3-5), In 9 patients gastrostomy and Foker technique was performed in the same time (around the 1stto the 4th day of life), while in 7 patients gastrostomy was performed in 1st-2nd day of life and the Foker technique was delayed due to clinical needs with a mean of 76 days (range 26-96 days) . In the study group, 11/16 (68,75%) patients underwent multiple endoscopic dilatations ( means 5 sessions per patient); 3 out of 16 cases of early complications occurred: 2 perforations and 1 dehiscence. Gastroesophageal reflux was common during the first 6 years of life and 2 had a fundoplication due to gastroesophageal reflux disease resistant to maximal medical therapy and 1 had an esophageal diverticulum at 5 years. 7 out of 16 (43,7%) (average age 13,5 years) perform therapy cycles with pump inhibitors for an average period of 12 weeks. CONCLUSIONS: Foker procedure for esophageal lengthening in patients with LGEA was successful in mobilizing the esophagus and obtaining a repair in the majority of our patients after 12 to 15 days of traction and it should be considered in LGEA cases to preserve the native esophagus as often as possible.

Giant malignant sacrococcygeal germ cell tumor in a newborn: A rare case report.

Malignant germ cell tumors constitute about 3%-4% of all neoplasms occurring before the age of 15. They arise in the ovaries, the testes, and in several other locations, including the lower back, the chest, the brain, and the abdomen. In infants and young children, the sacrococcygeal region is the most common site for extragonadal germ cell tumors, and teratomas account for the vast majority of sacrococcygeal germ cell tumors. Neonatal sacrococcygeal teratomas are usually benign and rarely they may contain a malignant component that is predominantly a yolk sac tumor. In this article, we describe a rare case of a male newborn with a giant sacrococcygeal mixed germ cell tumor composed of grade 3 immature teratoma and malignant yolk sac elements.

Value of intestinal ultrasound in pediatric magnet ingestion: a case report.

Ingestion of magnetic foreign bodies in pediatric settings has become more common in the last years due to the marketing of various toys containing small magnetic parts. Most magnets, especially if a single element is ingested, usually pass through the gastrointestinal tract without complications. However, ingestion of multiple magnets or magnets and small metallic components may require a prompt intervention due to the risk of attraction across bowel layers, leading to pressure necrosis, perforation, and even death. Routinely, serial radiological evaluations are needed to follow the progression of magnets through the intestine, while the role of small bowel ultrasound is regarded as marginal. Here we report a case of a 5-years old boy who ingested 8 magnets and in which small bowel ultrasound was pivotal for the correct assessment of magnets location to correct address the surgical approach.

Early diagnosis of Meigs syndrome in children A case report and a review of the literature.

Meigs syndrome is a rare disease defined by the coexistence of benign ovarian neoplasm, ascites and hydrothorax, which mainly affects women over the age of 30. This clinical condition refers only to cases in which the ovarian neoformation is a fibroid, a thecoma, a granulosa cell tumor or a Brenner tumor with disappearance of symptoms and effusions after removal of the neoplasm. Meigs syndrome is most frequently characterized by the presence of an ovarian fibroid, which in childhood is very rare and not commonly associated with the disease. In this article we report the case of an 11- year-old girl who came to our observation for a high fever for five days accompanied by cough and abdominal pain; imaging methods revealed bilateral hydrothorax, ascites, and a voluminous expansive right ovarian formation. On histological examination, the mass showed a cellular fibroid and the diagnosis of Meigs syndrome was made. Furthermore, we present a review of the literature aimed at detecting the state of knowledge on this disease in pediatric age, giving particular emphasis to the condition for which, in the presence of pleural effusion and ascites, an ovarian neoformation is not necessarily malignant. KEY WORDS: CT, Meigs syndrome, Pediatric, Pelvic mass, Ultrasounds.

Pseudopapillary solid tumour of the pancreas in paediatric age: description of a case report and review of the literature.

Pseudopapillary solid tumour of the pancreas is a rare neoplasm that mainly affects young women in the second and third decade of life and less frequently children; originates from the exocrine component of the pancreas; and is characterized by slow growth, low potential for malignancy, and excellent prognosis following complete surgical resection. The tumour often presents as an asymptomatic abdominal mass that is accidentally detected during radiological investigations performed for other reasons. In this article, we report the clinical case of a 10-year-old girl who came to our observation for pain in the left hypochondrium, which had arisen for a week following a trauma; the imaging methods revealed a voluminous expansive pancreatic formation in the abdomen; on histological examination, the mass was a solid pseudopapillary tumour. Furthermore, we present a review of the literature aimed at highlighting the salient features of this neoplasm in paediatric age.

Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 81 patients.

BACKGROUND: Despite significant developments in understanding the pathological anatomy and physiology of Hirschsprung's disease (HD), the follow-up of children with HD remains far from clear. Treatment results for HD are not uniformly successful and the interpretation of the result by the parents and child may differ from that of the surgeon. Our goal is to analyze quality of life and differences in functional outcomes in children operated on using the pediatric incontinence / constipation scoring system (PICSS) validated in a regulatory group. METHODS: A retrospective questionnaire was performed for all children operated for HD between 1995 and 2017 at the Pediatric Surgery Unit. The use of PICSS defined children with continence or incomplete constipation. RESULTS: 125 patients were operated on for HD between 1995 and 2017 in our Pediatric Surgery Unit and 81 families gave their consent to participate. The median age at the interview was 94.5 months (range 12-283 months). Complete aganglionosis was in 2% of cases. Treatments included a Soave procedure in 96%. Colostomy was performed in 15.8% (13/81). Decompressive enterostomy was performed in a child. The rate of normal bowel function was 66.6% (n = 54). The percentage of cases with incomplete continence and with constipation were 4.9% (n = 4) and 16% (n = 13), respectively. CONCLUSIONS: HD results aren't always as good as surgeons may perceive; long-term follow-up is important. Over time, most schoolage children improve significantly for fecal continence, but it can also be acquired in late adolescence.

Isolated intestinal Ganglioneuromatosis: case report and literature review.

BACKGROUND: Intestinal Ganglioneuromatosis (IG) is a rare disorder of the enteric nervous system. In pediatric age it is often associated with genetic syndromes such as Neurofibromatosis 1 (NF1), multiple endocrine neoplasia type 2B (MEN2B) and Cowden syndrome (PTEN mutation), and ganglioneuromas (GNs) may be sometimes the first sign of the disease. Isolated GNs are rare and sporadic. Clinical symptom vary and depend on the size and on the location of the GNs. This disorder affects intestinal motility and it, consequently, causes changes in bowel habits, abdominal pain, occlusive symptoms and rarely lower gastrointestinal bleeding secondary to ulceration of the intestinal mucosa. On the other hand, patients can remain asymptomatic for many years. CASE PRESENTATION: We describe a 9-year-old boy referred to our emergency department for right lower quadrant abdominal pain. No familial history for gastrointestinal disorders. No history of fever or weight loss. At physical examination, he had diffused abdominal pain. Abdominal ultrasonography showed a hypoechoic formation measuring 41.8 mm by 35 mm in the right lower quadrant of the abdomen. Routine blood tests were normal, but fecal occult blood test was positive. Abdominal TC confirmed the hypodense formation, of about 5 cm in transverse diameter, in the right hypochondrium that apparently invaginated in the caecum-last ileal loop. Colonoscopy showed in the cecum an invaginated polypoid lesion of the terminal ileal loop. Laparoscopic resection of the polypoid lesion was performed. Histological diagnosis of the large neoplasm observed in the terminal ileum was diffuse ganglioneuromatosis. NF1, RET and PTEN gene tests resulted negative for specific mutations. At the 1 year follow-up, the patient presented good general condition and blood tests, fecal occult blood test, esophagogastroduodenoscopy, colonoscopy and MR-enterography were negative. CONCLUSIONS: Only few cases are reported in literature of IG in pediatric age. Although rare, the present case suggests that this disorder must be taken in consideration in every patient with GI symptoms such as abdominal pain, constipation, lower intestinal bleeding, in order to avoid a delayed diagnosis.

Omental Infarction: An Underrecognized Cause of Right-Sided Acute Abdominal Pain in Children.

OBJECTIVE: To evaluate the prevalence of omental infarction (OI) in children with suspected appendicitis, the role of ultrasonography (US) in its diagnosis and management and the efficacy of conservative management. METHODS: Consecutive children with suspected acute appendicitis were prospectively enrolled. Ultrasonography was performed at baseline, during follow-up, before the discharge, and at 15-day intervals until US findings of OI disappeared. All children with a diagnosis of OI were treated conservatively. RESULTS: One hundred ninety-nine children (91 male; age range, 3-15 years) were evaluated. Eighty-four patients had acute appendicitis. Omental infarctions were found in 14 children (8 male; mean age, 9.8 ± 2.6 years), with an incidence of 7%. Ultrasonography depicted an echogenic mass consistent with OI in all children. Ultrasonography detected in 8 patients a normal-looking appendix, whereas in other 6 patients, it identified neither appendix nor indirect signs of acute appendicitis. A normal appendix has been detected by US during follow-up in 2 of these 6 patients. During follow-up, US finding of OI disappeared in all cases and no signs of acute appendicitis or other disease occurred. All 14 OIs were treated conservatively, with no reported complications. CONCLUSIONS: Omental infarction is an underestimated cause of abdominal pain in children accounting for 7% of patients with suspected appendicitis. Ultrasonography is a useful method for the diagnoses and to guide clinical management of OI. Conservative therapy is a safe option for the management of OI.