Non-thyroidal illness syndrome and SARS-CoV-2-associated multisystem inflammatory syndrome in children.

PURPOSE: COVID-19 disease may result in a severe multisystem inflammatory syndrome in children (MIS-C), which in turn may alter thyroid function (TF). We assessed TF in MIS-C, evaluating its impact on disease severity. METHODS: We retrospectively considered children admitted with MIS-C to a single pediatric hospital in Milan (November 2019-January 2021). Non-thyroidal illness syndrome (NTIS) was defined as any abnormality in TF tests (FT3, FT4, TSH) in the presence of critical illness and absence of a pre-existing hormonal abnormality. We devised a disease severity score by combining severity scores for each organ involved. Glucose and lipid profiles were also considered. A principal component analysis (PCA) was performed, to characterize the mutual association patterns between TF and disease severity. RESULTS: Of 26 (19 M/7F) patients, median age 10.7 (IQR 5.8-13.3) years, 23 (88.4%) presented with NTIS. A low FT3 level was noted in 15/23 (65.3%), while the other subjects had varying combinations of hormone abnormalities (8/23, 34.7%). Mutually correlated variables related to organ damage and inflammation were represented in the first dimension (PC1) of the PCA. FT3, FT4 and total cholesterol were positively correlated and characterized the second axis (PC2). The third axis (PC3) was characterized by the association of triglycerides, TyG index and HDL cholesterol. TF appeared to be related to lipemic and peripheral insulin resistance profiles. A possible association between catabolic components and severity score was also noted. CONCLUSIONS: A low FT3 level is common among MIS-C. TF may be useful to define the impact of MIS-C on children's health and help delineate long term follow-up management and prognosis.

A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Nasal polyposis in children.

Nasal polyposis is a chronic inflammatory disease affecting the nasal cavity and the paranasal sinuses. It is a relatively common disease, occurring in 1-4 % of the general population, but it is rarely described in the pediatric population. Most of the published series include children presenting with other underlying systemic diseases, mainly cystic fibrosis. The aim of the present study was to describe the characteristics of the patients suffering from nasal polyposis, evaluated at the Pediatric Clinic of the University of Pavia (Italy) over the last 17 years. 56 consecutive pediatric patients referring to our Pediatric Department had a diagnosis of nasal poyposis over the last 17 years. All children underwent allergy evaluation, nasal endoscopy, CT scan of the paranasal sinus, and Functional Endoscopic Sinus Surgery. The mean age of the present cohort was of 11.8 years and most of the patients were male. 50% of the patients presented with unilateral, polyposis, mostly with a diagnosis of antrochoanal polyp. 4 patients presenting with bilateral polyposis suffered from cystic fibrosis. Main symptoms at diagnosis included nasal obstruction, snoring and rhinorrhea 32% of the patients presented at least a positivity to skin prick test, for major inhalant and food allergens. Nasal polyposis in children could represent an alert sign for other underlying systemic diseases. Nasal endoscopy should therefore be prescribed when a diagnosis is suspected. To properly treat a patient presenting with nasal polyposis, it is necessary to integrate medical and surgical skills through a multidisciplinary approach.

Adenoids during childhood: the facts.

Adenoids are constantly exposed to viral and bacterial agents as well as to allergens. They play a major role in the upper airways immunity, being effector organs in both mucosal-type and systemic-type adaptive immunity. Because of both their immunological function and their specific location, adenoids are considered to be as reservoirs of viruses and bacteria. Reiterative infections may therefore contribute both to Eustachian tube dysfunction and to tissue hypertrophy. Nasal endoscopy is a key diagnostic tool to detect both adenoid hypertrophy and adenoiditis. Moreover, such a procedure may be very helpful in detecting bacterial biofilms that could justify the concomitant presence of recurrent episodes of otitis media, chronic and occult sinusitis in children. Even though the connection between allergies and adenoidal diseases is not completely clear, allergic diseases cause an inflammatory state that influences adenoidal tissue as well, configuring the picture of allergic adenoiditis, a condition in which adenoid tissue exhibit numerous IgE positive mast cells. Several studies are still needed to better understand the relationship between allergies and infections and the influence they play on adenoids during childhood.

The evolution of the adenoidectomy: analysis of different power-assisted techniques.

Techniques and instruments for adenoidectomy have considerably changed over the years. With the introduction in Otolaryngology of power-assisted instruments for endoscopic sinus surgery, the classic adenoidectomy performed with curette or adenotome has evolved, with an improved patients' outcome and a better satisfaction of the surgeon. The purpose of this article is to describe and critically analyze the literature reports about different methods of power-assisted adenoidectomy. We performed a literature search (Medline) to identify all available reports. We discussed the surgical techniques and reviewed advantages and disadvantages of each method. The techniques can be schematically divided in non-endoscopic, usually performed with a laryngeal mirror, and endoscopic-assisted. The endoscopic control can be obtained either trans-nasally or trans-orally, as well as the microdebrider can be inserted in the nasal cavity or maneuvered through the oral cavity. Some authors reported the usage of the power-assisted instruments in performing the entire adenoidectomy; while, according with other authors, the microdebrider can be used as a step of the surgical procedure, for a combined adenoidectomy. In conclusion, all the methods seem to be safe and effective, and the personal experience of the surgeon should guide the choice of the instruments. However, we personally consider the endoscopic techniques as the most suitable, and among these the Transoral Endonasal-Controlled Combined Adenoidectomy (TECCA) should be considered as the most ergonomic technique to perform a power-assisted adenoidectomy.

Lymphangiomas of the head and neck in children.

Lymphangiomas are rare benign congenital tumours, involving both the head and the neck and causing obstructing symptoms in the upper airways as well as aesthetic anomalies. In recent years, sclerosing therapy with OK-432 has become the treatment of choice in the management of these lymphatic malformations. Nonetheless, surgery still seems to be the therapy advocated for resolution of symptoms. Herein, three cases of lymphangiomas involving the head and the neck are described and a review of the English scientific literature is outlined.

Giant omphaloceles with a small abdominal defect: prenatal diagnosis and neonatal management.

A giant omphalocele is a liver-containing protrusion through an abdominal defect wider than 5 cm in diameter. The giant form with a small abdominal wall defect is a rare condition which, to our knowledge, has not been described previously. We describe three cases with the typical features of elongated vascular liver pedicle and angiomatosis of the hepatic portal system. The abnormal liver organogenesis, due to extra-abdominal development, represented a significant risk factor for hepatic thrombosis after visceral reduction and liver rotation. All the neonates underwent surgery on the first day of postnatal life. One died because of a postoperative liver infarction, and the survivors needed prolonged respiratory support. Prenatal sonographic features, timing, delivery, type of surgical repair, and postnatal outcome are reviewed. A prenatal sonographic diagnosis could be useful to evaluate the abdominal ring and serial ultrasound examinations are recommended to detect promptly ominous signs of hepatic and bowel damage. Color Doppler may be useful to assess the anatomy of the abdominal vessels and their relationships with the herniated organs, although it was not used in any of the cases reported here. This congenital malformation might be considered as a pathological entity separate from giant omphalocele with large abdominal defect, with a severe prognosis due possibly to its different embryological development.

Acute airway obstruction in an infant with Pierre Robin syndrome after palatoplasty.

This report describes a complication of post-operative oedema of the palate, tongue and pharynx after a Perko-revised cleft palate repair, which resulted in a life-threatening airway obstruction in an infant with Pierre Robin syndrome. Although infants experiencing airway problems after Wardill-Kilner, von Langenbeck and Furlow palatoplasty have been described, airway complications in a group of Perko-revised repair children have not been previously reported. We speculate that this complication, which occurred in the absence of a history of previous airway problems, is due to prolonged operating time and excessive pressure exerted on the base of the tongue by the Kilner-Doughty retractor. Acknowledgments of this risk permits to identify those patients prior to surgery so that they can be managed appropriately.

A new technique of bladder enlargement and continent urinary diversion using the ceco-appendicular unit.

The authors developed a new technique using the cecum with in-situ appendix to simultaneously achieve bladder enlargement and continent urinary diversion (CUD) in five cases of neuropathic bladder or serious complications of abdominopelvic trauma. The cecoplasty provides a large-capacity, low-pressure reservoir; the submucosally-embedded appendix gives complete continence with 3 to 5 intermittent catheterizations daily. It is an alternative to the Mitrofanoff principle when bladder augmentation and CUD are necessary, and easier to achieve when faced with a neuropathic bladder where the thickness of the detrusor makes appendicular implantation difficult.

[Charge syndrome: long-term survival. Report of a case]. / Sindrome di Charge: sopravvivenza a lungo termine. Descrizione di un caso.

Charge association is a relatively rare pattern of congenital anomalies whose incidence is approximately 1:10.000. We describe our experience on a young boy (Cariotype 46-XY) with associated oesophageal atresia survived up to 12 years. We analyse the most relevant difficulties occurred in the treatment of this patient and we underline the importance of performing an early tracheostomy in order to improve the respiratory impairment. The management of this multiple complex malformations requests a multidisciplinary approach during the life.

Carcinoid tumors of the appendix in children.

We report ten cases of carcinoid tumor of the appendix observed in children from 1988 to 1996. The patients included six females and four males with an average age of 13 years at presentation. They were admitted after complaining of pain in the lower abdominal quadrant. In eight children who presented with symptoms of acute appendicitis, the tumor was located at the tip of the appendix. Diagnosis was performed after appendicectomy (AE) and pathologic examination, which revealed a tumor slightly under 1 cm in size. Two other children were admitted with clinical signs of peritonitis due to larger tumors measuring more than 2 cm on the base of the appendix. One patient underwent a cecectomy, the other a right hemicolectomy. For all patients follow-up was 3 years, and all recovered fully. According to these findings and a review of the literature, we suggest conservative surgical procedures in children. More than 70% of these tumors are localized at the tip of the appendix and represent an incidental pathologic finding during AE; AE alone is curative. Patients with a bulky tumor of the appendicular base measuring 2 cm and invading the serosa and mesoappendix without metastases may be treated with a cecectomy; ileocecal resection may be indicated in cases where the tumor has infiltrated tissue beyond the cecum with localized metastases and in patients with incomplete gross resection. Right hemicolectomy is questionable in this age group and restricted to rare conditions.

[Post-traumatic complication of trans-appendiceal cystostomy: urinary peritonitis]. / Une complication post-traumatique de la cystostomie trans-appendiculaire: la péritonite urinaire.

Two children who had undergone a transappendicular urinary diversion (type Mitrofanoff) developed bladder rupture, one following abdominal trauma, 4 months after the operation and the other following traumatic self-catheterization at 4 years. The clinical history and standard radiological examinations (ultrasonography, cystography) confirmed the diagnosis of urinary peritonitis. Emergency surgical repair was possible in both cases with an uneventful postoperative course. This serious and rare complication requires emergency surgery and justifies rigorous selection of children suitable for this type of diversion giving preference to increased bladder neck resistance over bladder neck closure.

Gastric volvulus associated with congenital diaphragmatic hernia, wandering spleen, and intrathoracic left kidney: CT findings.

We present an unusual case of gastric volvulus associated with wandering spleen, a delayed manifestation of congenital diaphragmatic hernia and left intrathoracic kidney. Gastric volvulus should be considered in any infant with unexplained vomiting and left diaphragmatic anomaly: in these patients, developmental disorders of the peritoneal visceral attachments of the left upper abdomen may coexist. The absence of ligamentous connections between the stomach, posterior abdominal wall, and spleen result in wandering spleen. We emphasize prompt surgical therapy to avoid gastric and splenic necrosis. Radiologic findings and the appearance of this complex congenital malformation are reported.

Prenatal management of inherited urogenital malformation: case report.

Documentation of unique kidney renal function early in pregnancy can be helpful in defining prenatal management and therefore in improving prognosis. Antenatal diagnosis of a solitary kidney was performed at 20 weeks' gestation in a foetus with a 1,7 chromosome translocation. Because of the decreasing renal function and the increasing pelvic dilatation, an early in utero stenting was placed at 23 weeks' gestation. Optimal outcome occurred and the baby was delivered at 32 weeks. Complete assessment of the malformation showed a left hydronephrosis due to a megaureter, right renal agenesis with ipsilateral cryprorchidism and agenesis of the right vas deferens. The chromosomic translocation was inherited from the mother who was affected by uterus didelphys, obstructed right hemivagina and right renal agenesis. Renal function of the unique kidney with hydronephrosis can be early diagnosed and promptly treated. This condition should also increase the index of suspicion of underlying genital and chromosomal anomalies.

Abdominal expansion using a polytetrafluoroethylene prosthesis in the treatment of Pepper syndrome.

The authors report three cases of stage IV-S neuroblastoma in infants aged 4, 6, and 8 weeks, who despite chemo- and radiotherapy required surgical intervention to urgently relieve major thoracoabdominal compression secondary to massive hepatomegaly. The results were successful, with abdominal expansion being achieved by the introduction of a polytetrafluoroethylene prosthesis, which was removed during the 2nd, 3rd and 7th postoperative month, respectively, after tumor regression. Two children were in complete remission 32 and 38 months later, the 3rd died after 16 months of tumor progression.

Surgical treatment of diaphragmatic agenesis by transposition of a muscle flap: report on 15 cases.

Between 1987 and 1996 the transposition of a muscle flap using the anterolateral abdominal wall (the internal oblique and transversus abdominus muscle) has been used for the surgical treatment of fifteen cases of diaphragmatic agenesis. There are several advantages of this technique: a more accurate reconstruction of the diaphragmatic dome, a better adapted muscle flap, good tolerance and evolution as a result of the use of autologous material with thus less risk of recurrence and/or infection. The disadvantages are represented by the nine cases of abdominal herniation at the site of the muscle flap, three of which necessitated secondary surgical treatment. Nine children survived this treatment, six died following severe cardio-pulmonary complications associated with this malformation.

[Cystic dysplasia of the rete testis and ipsilateral kidney agenesis in children]. / Dysplasie kystique du rete testis et agénésie rénale ipsilatérale chez l'enfant.

BACKGROUND: Cystic dysplasia of rete testis, a rare abnormality, is often associated with renal agenesis. CASE REPORT: A 5-year-old boy was examined because he presented large scrotum. This was initially considered as hydrocele. Surgery showed a cystic dysplasia of the testis that in this patient was associated with ipsilateral renal agenesis. CONCLUSION: This congenital abnormality, explained on the basis of embryological development, has been reported in 15 children. The testis appears cystic at surgical exploration; echography can affirm diagnosis and MRI can give specific features. A conservative attitude is proposed as this lesion is benign. Long-term follow-up is recommended for possible painful effects or growth of the lesion.

[Modified Dyplay technique in the treatment of anterior hypospadias. Immediate and long term results in 321 cases and review of the literature]. / Technique de Duplay modifiée dans le traitement de l'hypospadias antérieur. Résultats immédiats et à long terme à propos de 321 cas et revue de la littérature.

OBJECTIVES: Description of a surgical technique in the treatment of anterior hypospadias, a frequent malformation for which the treatment is not devoid of complications. Evaluation of the short-term as well as the long-term results. MATERIAL AND METHODS: Retrospective study of 321 patients treated according to this method. 58 patients were reviewed with a mean follow-up of 71 months. RESULTS: The immediate results were marked by the development of 16 fistulas (5%), 4 of which closed spontaneously. Five cases (1.6%) of meatal retraction and 6 meatal stenoses (1.9%) were observed. The overall complication rate requiring specific treatment was 5.6%. The long-term results showed meatal retraction in 20.7% of cases, meatal stenosis in 1.7% of cases and fistula in 3.5% of cases. This is the series with the longest mean follow-up. Comparison with the currently proposed technique suggests that this technique is indicated in the treatment of anterior hypospadias. Long-term analysis of the results strongly encourages regular and prolonged follow-up of children treated for hypospadias in the first 2 years. CONCLUSION: The modified Duplay technique is reliable, can be easily performed, and gives perfectly satisfactory results, even in the long-term, for the treatment of anterior hypospadias.