Current Pediatric Reviews (v.12, #4)
Meet Our Editorial Board Member by J. A. Bellanti (245-245).
Editorial (Thematic Issue: Thyroid Cancer in Children and Adolescents) by Claudio Spinelli (246-246).
Pediatric Differentiated Thyroid Cancer: When to Perform Conservative and Radical Surgery by Claudio Spinelli, Leonardo Rossi, Jessica Piscioneri, Silvia Strambi, Alessandro Antonelli, Andrea Ferrari, Maura Massimino, Paolo Miccoli (247-252).
Thyroid tumors affect all age groups, including children and adolescents. Malignant cancer of the thyroid is a relatively uncommon disease in pediatric age. In the recent decades the incidence of paediatric differentiated thyroid carcinoma (DTC) has increased. DTC in paediatric age is rare and has an excellent prognosis. Compared to adult counterpart, DTC in childhood presents some different features as follows: larger volume at the diagnosis, more frequent multicentricity (both mono- and bilateral), earlier local involvement of soft tissue of the neck, earlier lymph node involvement, distant metastases 3-4 times more frequent (most often in the lungs and almost always functional) and more common post-operatory recurrence; nevertheless, the prognosis of DTC in childhood is better and the survival greater than in adult. Because of unusual association between aggressive presentation and good prognosis, the choice about the surgical treatment to perform in DTC is debatable, especially between conservative and radical approach in TNM stage I pediatric patients. To date, total thyroidectomy is the operation most often performed in children with DTC, although recently conservative surgery has been performed in solitary unifocal nodule without evidence distant metastases.
Thyroid Ultrasound and Other Imaging Procedures in the Pediatric Age by Federica Guidoccio, Mariano Grosso, Federica Orsini, Giuseppe Boni, Giuliano Mariani, Duccio Volterrani (253-264).
The purpose of this review is to provide a reappraisal of the diagnostic imaging procedures for thyroid carcinoma in pediatric patients, including thyroid ultrasound (US), ultrasound-guided fine-needle aspiration biopsy (FNAB), scintigraphy, radiological techniques (CT, MR), and PET/CT. The most frequent indication for thyroid imaging is characterization of a palpable mass in the neck or thyroid gland. Thyroid US is a first-line examination for visualizing the thyroid gland as it provides anatomic and perfusion information; on the other hand, scintigraphy mostly provides functional information but combined with some anatomic information as well. CT and MRI have a supplemental role in these patients. Furthermore, with the introduction of PET/CT and the development of new imaging agents, nuclear medicine plays an important role in different phases of neoplastic disease in terms of both staging and evaluation of response to medical/surgical treatments.
Papillary Thyroid Carcinoma in Pediatric Age: An Example of a Rare Tumour Managed Within a Cooperative Comprehensive Project by Giovanna Sironi, Andrea Ferrari, Marta Podda, Stefano Chiaravalli, Gianni Bisogno, Giovanni Cecchetto, Maura Massimino (265-271).
Though rare in childhood, thyroid cancers represent the most frequent tumours of endocrine glands in childhood and adolescence. Papillary thyroid carcinoma is a nonmedullary, follicular-derived differentiated tumour of the thyroid. There is still controversy concerning the therapeutic approach to be adopted of PTCs in the paediatric population, regarding the extent of the surgical approach, and the need for radioactive iodine (RAI) therapy. The cooperative TREP project (Tumori Rari in Eta Pediatrica [Rare Tumours in Paediatric Age]) was launched in Italy in 2000 (under the patronage of AIEOP - Associazione Italiana Ematologia Oncologia Pediatrica) with a view to improving both researches on the clinical management of the less common paediatric cancers. The aim of the project is to merge clinical data and elaborate diagnostic and therapeutic guidelines for everyone of these rare tumours within a single framework: of course papillary thyroid carcinoma have been analysed in the TREP project. The cooperative model of TREP led to the establishment of a larger cooperative group, EXPeRT (European Cooperative Study Group for Pediatric Rare Tumors), seeking to create collaboration and communication within European members. EXPeRT aims to promote clinical and biological research in paediatric rare tumours by strengthening collaboration between the founder national groups and international partners. The studies have demonstrated that international cooperation in childhood rare tumours is not only achievable, but may be of great advantage.
Histopathology, Immunohistochemistry and Molecular Biology of Follicular Epithelium-Derived Pediatric Thyroid Carcinomas by Paola Collini, Marta Barisella, Angela Greco (272-279).
Thyroid carcinomas derived from the follicular epithelium are rare in children up to 18 years of age. While the morphological features are superimposable in children and adults, the age at onset is relevant to both the frequency of histotypes and variants, and to their biologic behavior. Papillary carcinomas (PCs) represent more than 90% of cases. Follicular carcinomas (FCs) rarely occur, and they are mostly represented by minimally invasive (encapsulated) FCs (MIFCs). Poorly differentiated carcinomas (PDCs) are very rare, while anaplastic (undifferentiated) carcinomas are practically nonexistent. PCs arisen in children have an overall survival of about 100% independently of the parameters associated with a bad outcome in adults. In pediatric ages the solid/trabecular variant is particularly frequent, together with PC, NOS. The occurrence of a solid/trabecular pattern of growth is one of the most frequent pitfalls in the differential diagnosis with the ominous PDCs. In children even the PC variants reported as high-risk in adults like the tall cell variant, seem to follow an indolent behaviour. MIFC has to be carefully distinguished from follicular adenoma and the encapsulated follicular variant of PC. The high risk, widely invasive FC, which in adults has an overall survival superimposable to PDC, is only occasionally seen in children. In the last decades, in particular following the Chernobyl accident, the pediatric PCs genetics has been extensively studied.
Surgical Management of Medullary Thyroid Carcinoma in Pediatric Age by Claudio Spinelli, Silvia Strambi, Leonardo Rossi, Rossella Elisei, Maura Massimino (280-285).
Medullary thyroid carcinoma (MTC) is a rare, accounting for 5% of thyroid malignancies. It is a neuroendocrine tumor wich origins from thyroid parafollicular cells. It may be sporadic, mostly in adult patients, or inherited as autosomal dominant pattern, mostly in pediatric patients. As familial cancer, MTC may presented isolated as familial medullary thyroid carcinoma (FMTC) - 10% of cases - or, most often, as part of multiple endocrine neoplasm type 2 (MEN 2A or MEN 2B) syndromes - 90% of cases. The therapy for sporadic or hereditary MTC is surgical resection and consists in total thyroidectomy associated with central compartment lymph nodal dissection; the radicality of this intervention is fundamental to obtain a definitive cure. Genetic screening in children of parents with MEN2 or FMTC has led to prophylactic total thyroidectomy. The type of prophylactic surgery required is focus of great attention, and it is reported that total thyroidectomy, central neck dissection associated with parathyroid autotransplantation is the proper management. In children, the recommended age of surgery based on genetic testing is 5 years for patients with MEN 2A and FMTC, and 1 year for children possessing the RET mutation responsible for MEN 2B. The surgical management changes depending on the type of codon mutated.
Clinico-Hematological Features and Management Outcome in Neonatal Malaria: A Nine Years Analysis from North India by Kriti Mohan, B. J. Omar, Rupa D. Singh, Aaradhana, M. M. Maithani, R. N. Chaurasia (286-291).
Background: Malaria is an important cause of death and illness in children worldwide. Most cases of neonatal malaria are misdiagnosed because of lack of specific symptoms and general lack of awareness. Nothing much is known in literature about the hematological changes during malaria infection and outcome of disease in neonates. Neonatal malaria is an underdiagnosed entity. So this hospital based observational study aims to assess diagnostic features of neonatal malaria. Methods: From August 2004 to August 2013, information of all slide positive for malaria cases aged 0 to 28 days admitted to our pediatric hospital was collected and analysed. Results: 28 slide positive cases of neonatal malaria were studied, four out of them were congenital malaria. Fever (93%) was the most common symptom followed by pallor (72%) and diarrhoea (50%). We also found respiratory distress in four (14%) cases. Apart from anemia and atypical lymphocytosis, We also found thrombocytopenia and low hematocrit, MCV and RBC count. Two cases with bleeding manifestations expired during course of treatment. Discussion: Malaria in the first few months of life can simulate transplacentally or postnatally acquired infection such as TORCH, syphilis, neonatal hepatitis and septicemia all having an important symptom complex of fever jaundice, hepatosplenomegaly and anemia. Although in our cases clinical presentation has been similar to septicemia but culture of blood, CSF and urine were sterile. The dilemma of distinguishing neonatal malaria alone versus neonatal sepsis or both existing does not seem to be easily resolved by the use of clinical features alone. The laboratory diagnosis of parasitemia in neonates require special attention in Giemsa staining as well as the technical skill involved in malaria microscopy because parasite densities are low. So high degree of suspicion is needed to diagnose malaria in newborns presenting with fever and anemia.
Dysfunctional Voiding in Pediatrics: A Review of Pathophysiology and Current Treatment Modalities by Maria Fernandez-Ibieta, Lidia Ayuso-Gonzalez (292-300).
In Dysfunctional Voiding (DV), the external urethral sphincter or pelvic diaphragm (consisting of striated muscle) contracts during voiding, contrary to normal physiological mechanism. In Flowmetry plus pelvic Electromyography (EMG), the perineal surface electrodes will show a persistent activity during voiding. The cause is believed to be the persistence of an immature bladder control. The overall prevalence of DV is estimated between 7 and 11% of girls of school age. Symptoms of DV comprise a range of symptoms, deriving from outlet obstruction, incomplete voiding and elevated Post Micturition Residual (PMR): from daytime urine leaks, nocturnal enuresis, Urinary Tract Infections (UTI), or Vesicoureteral reflux (VUR), to the final decompensation of upper urinary tract in extreme cases. The literature reflects a high prevalence of constipation among these patients: between 33 and 56% of dysfunctional voiders are constipated. Initially, therapy of functional voiding disorders such as DV, comprises a series of fundamental principles called Urotherapy or voiding re-education. Currently, Urinary animated Biofeedback (Bfb) is the treatment modality of choice. This therapy discloses patients their own physiological and muscular mechanisms. Majority of the studies on Bfb efficacy reflect an improvement/ cure rate of around 70-80%. Initial flowmetric improvement followed by symptom relief or clinical improvement as the Bfb sessions continue, is likely to occur. It has been observed that there are two variables associated with clinical success of Bfb in DV syndrome: the absence of PMR at the end of treatment and an adequate or even aggressive constipation management.
Child Abuse and Neglect and its Psycho-Physical and Social Consequences: A Review of the Literature by P. Ferrara, C. Guadagno, A. Sbordone, M. Amato, G. Spina, G. Perrone, C. Cutrona, M. C. Basile, F. Ianniello, G. C. Fabrizio, M. Pettoello-Mantovani, A. Verrotti, A. Villani, G. Corsello (301-310).
Child maltreatment is a complex life experience occurs when a parent or caregiver does an intentional or potential damage to a child, including acts of commission and omission. Child abuse is not an uncommon event, but it is not always recognized. Identifying the real number of maltreated children is a challenge because of the large variability in reported prevalence data across studies. Unfortunately, in the United States, it affects 1 in 8 children, by the age of 18 years, annually. Paediatricians may encounter a variety of forms of maltreatment such as neglect, emotional, physical and sexual abuse. These aspects should be recognised, examined and evaluated by employing a systematic approach and focusing on basic needs of children that may not be met. Child maltreatment is a global problem with serious life-long physical and psychological or psychiatric outcomes. It is associated with important economic and social costs (such as physical and mental health, productivity losses, child welfare, criminal justice and special education costs) due to its high prevalence and its long-term and short-term consequences. In the United States, the average cost of nonfatal maltreatment is $210,012 per children and the cost of fatal maltreatment is $1,272,900. General Practitioners are quite prepared to face the problem of child maltreatment: since they have the opportunity to meet several members of the same family, they can detect stressors that put children at risk of maltreatment. All health professionals have the responsibility to protect children from abuse and neglect.
ACKNOWLEDGEMENTS TO REVIEWERS (311-311).