Aggressive papillary thyroid carcinoma in a child with type 2 congenital generalized lipodystrophy

SUMMARY Thyroid carcinoma (TC) is rare in children, particularly in those aged < 10 years. Several studies have demonstrated a correlation between neoplasms and hyperinsulinemia and insulin resistance, which are often associated with a higher risk for and/or aggressiveness of the neoplasm. Congenital generalized lipodystrophy (CGL) with autosomal recessive inheritance is a rare disease and is characterized by the lack of adipose tissue, severe insulin resistance, and early metabolic disturbances. Here, we reported a rare case of a type 2 CGL in a girl who presented with a papillary TC (PTC) at the age of 7 years. She had no family history of TC or previous exposure to ionizing radiation. She had a generalized lack of subcutaneous fat, including the palmar and plantar regions, muscle hypertrophy, intense acanthosis nigricans, hepatomegaly, hypertriglyceridemia, severe insulin resistance, and hypoleptinemia. A genetic analysis revealed a mutation in the BSCL2 gene (p.Thr109Asnfs* 5). Ultrasound revealed a hypoechoic solid nodule measuring 1.8 × 1.0 × 1.0 cm, and fine needle aspiration biopsy suggested malignancy. Total thyroidectomy was performed, and a histopathological examination confirmed PTC with vascular invasion and parathyroid lymph node metastasis (pT3N1Mx stage). This is the first report to describe a case of differentiated TC in a child with CGL. Severe insulin resistance that is generally observed in patients with CGL early in life, especially in those with type 2 CGL, may be associated with this uncommon presentation of aggressive PTC during childhood.

Transposição jejunal com anastomose microvascular adicional (supercharge) como opção salvadora pós-esofagectomia total complicada / Jejunal transfer with additional microvascular anastomosis (supercharge) as a recovery option for post total esophagectomy complicated

Introdução: Os pacientes submetidos à esofagectomia geralmente necessitam de reconstrução do trânsito alimentar substituindo o esôfago por algum outro órgão. Porém, uma das principais complicações dessa cirurgia é a necrose do órgão transposto por deficiência vascular, impondo morbimortalidade aos pacientes acometidos. Objetivo: Apresentar uma nova técnica. Métodos: Apresentamos a experiência de um procedimento cirúrgico inovador, utilizado para resgate de esofagectomia complicada com necrose de tubo gástrico transposto. Dada a absoluta impossibilidade do paciente alimentar-se, optou-se por realizar uma transposição jejunal pré-esternal em Y en Roux, com anastomose microvascular adicional (supercharge), devolvendo ao paciente a capacidade de alimentação por via oral e melhora da sua qualidade de vida. Resultados: Este relato, o primeiro da América Latina, demonstra que a transposição jejunal com anastomose microvascular adicional (supercharge) é uma boa opção para reconstrução do trânsito digestivo para os casos de necrose de tubo gástrico transposto.

Introduction: The patients who were submitted to esophagectomy usually need have their route of nutrition restored by having their esophagus replaced by another organ. However, one of the main complications resulted from such procedure is the transplanted organ necrosis, as a result of vascular deficiency, causing patients morbidity. Objective: To present a new technique. Methods: We present the experiment of an innovator surgical procedure which is used as a recovery from esophagectomy complicated by gastric feeding tube necrosis. As the patience is completely unable to feed by himself, we decided to perform a Roux-en-Y presternal jejunal transplantation with additional microvascular anastomosis (supercharge) giving back to the patient the ability of getting fed orally, improving his life quality. Results: This report, which is the first in Latin America, shows that the jejunum transplant with additional microvascular anastomosis is a good option to the recovery of the regular route of nutrition in cases of gastric feeding tube necrosis.

Nervo laríngeo inferior não-recorrente / Non-recurrent inferior laryngeal nerve

Introdução: A lesão do nervo laríngeo inferior é uma das complicações dos procedimentos cirúrgicos da glândula tireóide. O risco torna-se maior quando o nervo, que normalmente é recorrente, apresenta variação anatômica, constituindo o nervo laríngeo inferior não-recorrente. Essa variação apresenta-se mais comumente à direita (0,2% a 4%) e, de forma mais rara, à esquerda (0,07%) e está associada a alterações anatômicas de artérias subclávias. O diagnóstico pode ser realizado no pré-operatório através de exames de imagem, o que não é rotina nos serviços de cirurgia. Pacientes e método: Relatamos a detecção intra-operatória do nervo laríngeo inferior não recorrente, todos à direita, em cinco pacientes submetidas à tireoidectomia total. O nervo foi identificado e preservado estruturalmente em todas as pacientes, apesar das dificuldades técnicas para sua localização. Resultados: Exames de videolaringoestroboscopia pós-cirúrgicos confirmaram a preservação funcional dos nervos. Conclusão: O cirurgião que realiza procedimentos de cervicotomia (tireoidectomia, em especial) deve estar alerta a respeito da existência dessa rara variação anatômica e da importância da identificação sistemática do nervo laríngeo inferior, de modo a evitar sua lesão acidental.

Introduction: The injury of the inferior laryngeal nerve is a complication of the surgical procedures on the thyroid gland. It occurs with increased frequency when this nerve has an anomalous anatomy and constitutes the non-recurrent inferior laryngeal nerve. This variation, that is associated with anatomic alterations of subclavian arteries, is more frequent at the right side (0.2% to 4%) and occurs less frequently at the left side (0.07%). The diagnosis can be performed preoperatively with image examinations. However, it is not a routine procedure in the surgery services. Patients and methods: We report the intraoperative detection of the non-recurrent inferior laryngeal nerve at the right side in five patients undergoing total thyroidectomy. In despite of the difficulties, the nerve was identified and preserved structurally in all patients. Results: The postoperative direct laringostroboscopy confirmed the functional preservation of the nerves. Conclusion: The surgeons who work with cervicotomy procedures (principally thyroidectomy) must be aware about the existence of this rare anatomic variation and the importance of the systematic identification of the inferior laryngeal nerve in order to avoid its accidental injury.