Diagnosis and management of Intratyroid Ectopic Thymus. / Diagnóstico y manejo del Timo Ectópico Intratiroideo.

INTRODUCTION: Intrathyroidal ectopic thymus (IET) is a rare benign condition caused by the aberrant thymic migra tion during embryogenesis. It is usually incidentally diagnosed as a thyroid nodule. OBJECTIVE: To report the intrathyroidal location of ectopic thymic tissue and to describe the ultrasound findings in children. PATIENTS AND METHOD: Retrospective descriptive review of the medical charts and thyroid ultrasound studies of children with nodular images in the thyroid gland, in a third level national pediatric hospital, from January 2010 to August 2017. Solid hypoecogenic intrathyroid lesions with multiple linear tracts or hyperechogenic points that did not change their characteristics during fo llow-up were considered intrathyroidal thymos. The ultrasound follow-up was performed every 4-6 months. The ultrasound characteristics of the lesions (location, laterality, size and shape), the indi cation of the ultrasound scan and the follow-up time were analyzed. RESULTS: Of 147 patients with thyroid nodules, we identified 12 children with lesions suggestive of an IET (8.1%). The mean age at diagnosis was 3.9 years (range 0-8). It was an incidental finding in all cases. Imaging findings were unilateral in eight patients and bilateral in four patients. All lesions were located in the mid and/or posterior portion of the gland. We adopted a watch-and-wait approach with ultrasound follow-up (mean 2.2 years; range 0.83-4) in all patients except in a 7-year-old boy who presented uncertain findings and underwent surgery, confirming IET in the pathological study. CONCLUSIONS: Thymic inclu sions in the thyroid gland are a rare but increasingly frequent finding, possibly related to the increased use of ultrasound studies. Pediatricians and radiologists should be aware of this entity to differentiate it from other thyroid lesions, avoiding unnecessary studies and/or treatments in these patients.

Combined transvaginal and transabdominal oocyte retrieval in a patient with an ectopic ovary and unicornuate uterus.

OBJECTIVE: To report the utility of combined transvaginal and transabdominal oocyte retrieval in a patient with an ectopic ovary and unicornuate uterus. DESIGN: Video case report with demonstration of oocyte retrieval technique. SETTING(S): University-affiliated fertility center. PATIENT(S): A 35-year-old woman, gravida 0, with a 6-month history of infertility who presented to our center for fertility evaluation. Hysterosalpingography revealed a left unicornuate uterus and patent left fallopian tube magnetic resonance imaging and laparoscopy showed a right ectopic ovary located in the upper abdomen. Her partner was a 36-year-old male with isolated teratozoospermia. The couple did not conceive with intrauterine insemination. INTERVENTION(S): Ovarian stimulation for in vitro fertilization (IVF). Transvaginal retrieval of oocytes from the right ovary was not deemed possible due the anatomic location of the ovary, intervening blood vessels, and limited mobility of the ovary. Institutional review board approval was not required for this case report as per our institution's policy; patient consent was obtained for publication of the case. MAIN OUTCOME MEASURE(S): Transabdominal retrieval of oocytes from the right ovary and transvaginal retrieval of oocytes from the left ovary. RESULT(S): The couple underwent two IVF cycles. Nine oocytes were retrieved during the first IVF cycle: seven transabdominal (right ovary) and two transvaginal (left ovary). All oocytes were mature, and five blastocysts were cryopreserved. Eight oocytes were retrieved during the second IVF cycle, of which five oocytes were retrieved transabdominally from the right ovary, and three oocytes were retrieved transvaginally from the left ovary. All oocytes were mature, and four blastocysts were cryopreserved. A single thawed embryo was transferred in the natural menstrual cycle, which resulted in the live birth of a full-term baby boy weighing 2,410 grams. CONCLUSION(S): The current case highlights the safety and feasibility of combined transvaginal and transabdominal oocyte retrieval in patients with an ectopic ovary located in the upper abdomen.

Ectopic Intravesical Ejaculatory Ducts: Case Report of Bulking Agent Injection for Treatment of Recurrent Epididymitis in a Patient With Anorectal Malformation.

As far as we know this is the first report on bulking agent injection into intravesical ectopic ejaculatory orifices reported in the English literature. During a follow-up period of 23 months, the child was free of episodes of epididymo-orchitis. Deflux injection in this rare anomaly of intravesical refluxing ducts had prevented irreversible damage to the testes from recurrent EO. Thus, it may be a better option than vasectomy when antibiotic treatment fails.

Ectopic cervical thymus in children: Clinical and radiographic features.

OBJECTIVES: Ectopic thymus is rare and can be a diagnostic challenge. This study evaluated the management of children radiographically diagnosed with ectopic cervical thymus. METHODS: A retrospective review of 100 patients was performed. Data related to clinical presentation, radiological imaging, pathology, and management were collected. Changes in lesion volume were tracked over time. Clinical characteristics were compared based on lesion location in the neck using analysis of variance modelling. RESULTS: There were 115 lesions with radiographic features of ectopic cervical thymus (15 children had bilateral lesions). Diagnosis was based on ultrasound in 98% of patients, magnetic resonance imaging in 18%, and computed tomography in 11%. Mean (SD) follow-up duration was 2 (2.2) years. Forty-four percent (51/115) of lesions involved the thyroid gland, 29% (33/115) were in the central neck but separate from the thyroid, 18% (21/115) had mediastinal extension, and 8% (9/115) involved the submandibular region. Location was unclear for two patients. Submandibular lesions were on average 12.4 cm3 larger (95% CI, 8.2, 16.6) than mediastinal lesions at diagnosis, P ≤ .001. Volume of thymic tissue decreased over time, from a mean (standard deviation [SD]) volume of 4.3 cm3 (9.2) at initial ultrasound to 2.7 cm3 (6.1) at final ultrasound (paired t-test, P = .008). Only two patients required surgery: one for compressive symptoms, and the other to rule out malignancy. CONCLUSION: Ninety-eight percent of children with ectopic cervical thymus were managed conservatively without issues. We propose a classification system based on location to ease communication among clinicians and to help follow these lesions over time. LEVEL OF EVIDENCE: 4, case series Laryngoscope, 130:1577-1582, 2020.

Issues and controversies in esophageal inlet patch.

The proximal esophagus is rarely examined, and its inspection is often inadequate. Optical chromoendoscopy techniques such as narrow band imaging improve the detection rate of inlet patches in the proximal esophagus, a region in which their prevalence is likely underestimated. Various studies have reported correlations between these esophageal marks with different issues such as Barrett's esophagus, but these findings remain controversial. Conflicting reports complicate the process of interpreting the clinical features of esophageal inlet patches and underestimate their importance. Unfortunately, the limited clinical data and statistical analyses make reaching any conclusions difficult. It is hypothesized that inlet patches are correlated with various esophageal and extraesophageal symptoms, diagnoses and the personalized therapeutic management of patients with inlet patches as well as the differential diagnosis for premalignant lesions or early cancers. Due to its potential underdiagnosis, there are no consensus guidelines for the management and follow up of inlet patches. This review focuses on questions that were raised from published literature on esophageal inlet patches in adults.

Malignant Transformation of Ectopic Pancreas.

Malignant transformation of ectopic pancreas tissue is a diagnostic challenge as clinical symptoms and radiographic features of these tumors are non-specific. Given the rarity of these lesions, it is usually neither suspected nor included in the diagnostic workup of different tumors. We conducted a comprehensive literature review regarding malignancy arising from ectopic pancreas for a better understanding of its frequency, clinicopathological features, and prognosis. A literature search was performed in three major databases: PubMed, Cochrane, and Web of Science. Fifty-four well-documented cases of malignant ectopic pancreas were identified in the published literature. Our analysis provided the following observations: (1) there was a slight predominance of males over females; (2) most patients with malignant transformation of ectopic pancreas were middle-aged; (3) most commonly, the tumor was located in the stomach; (4) most tumors were adenocarcinomas; (5) most frequently, the malignancy arose within a type I heterotopia according to Heinrich classification; (6) macroscopically, a subepithelial-like appearance was most frequently observed; and (7) improved prognosis for ectopic pancreatic malignancies in comparison with reported survival data for orthotopic pancreatic cancer. Even if the majority of cases of ectopic pancreas are incidental findings and malignant transformation is a rare event, pancreatic heterotopy should be considered as a source of potentially malignant lesions.

Accessory liver within the thoracic cavity.

Ectopic intrathoracic liver tissue is extremely rare. Studies are mainly limited to case reports. In the vast majority of reported cases, a diagnosis of intrathoracic liver tissue was made either after a thoracic surgery or during a postmortem examination. However, once included in differential diagnosis, surgical intervention or biopsy procedures may be avoided with optimal diagnostic approach. In the present study, we conducted a literature review and proposed a new classification method for accessory liver within the thoracic cavity. This approach may provide a better understanding of underlying pathophysiology and aid in determination of optimal diagnostic modality and clinical management of such cases. According to our literature review, type II ectopic liver is the most common subtype followed by types I and III. All types can be definitively diagnosed with imaging modalities. On the other hand, it is important to prevent patients, particularly children, from unnecessary radiation exposure during performance of sophisticated diagnostic imaging modalities. Ultrasound is a safe, low-cost and accessible imaging modality that has not been previously reported in diagnosis of this entity. With addition of Color Doppler Imaging, ultrasound may allow for diagnosis with high precision in types I and II, as demonstrated in the present study. Based on long-term follow-up of a case reported here, this study also illustrates the natural course of this entity via non-operative management. This approach may prevent an unnecessary surgical intervention.

Intrathyroidal thymic tissue in children: Avoiding unnecessary surgery.

BACKGROUND/PURPOSE: Intrathyroidal thymic tissue may be misinterpreted as a thyroid lesion in children, leading to invasive tests or resection. We sought to describe the characteristic imaging features of these lesions and to evaluate the safety of non-operative management. METHODS: A retrospective review of all patients less than 18years old with intrathyroidal thymic tissue from 2000 to 2016 was performed. Data collection included patient demographics, imaging results, interventions, and outcomes. RESULTS: Eleven patients were identified using institutional radiology and pathology databases. Median patient age and lesion size at presentation were 5years old (range 2 to 8years old) and 0.9cm (range 0.4 to 9.2cm), respectively. Six lesions were incidentally identified, six were left-sided, and the most common location was the lower pole. Ultrasonographic features were reproducible and included well demarcated (10/11), hypoechoic lesions (11/11), containing punctate/linear internal echoes (11/11), and occasional mild hypervascularity (6/11). All cases demonstrated interval size and echotexture stability over a median surveillance period of 3years (range 1 to 8years). While 9 patients were simply observed, the first patient in this series underwent excision, while another had a fine needle aspiration to confirm pathology. LEVEL OF EVIDENCE: Study of diagnostic test, Level IV. CONCLUSION: Intrathyroidal thymic tissue has typical clinical and sonographic characteristics which allow for appropriate diagnosis and avoids thyroid resection.

[Oral and dental expression of thyroid diseases]. / Manifestations buccodentaires des maladies thyroïdiennes.

Oral disorders were observed in children with congenital hypothyroidism, lately apparent because of difficulties of feeding, swallowing disorders, and macroglossia. Macroglossia was also a component of the severe acquired myxedema, particularly observed in hypothyroidism related to autoimmune atrophic thyroiditis beyond menopause. Lingual ectopy in children and adults could determine respiratory gene or swallowing difficulties, and were detectable by visual examination and the endobuccal touch. Expression of these events was completely minimized since the neonatal screening of congenital hypothyroidism, also by the common practice in adults of serum TSH determinations. Congenital and acquired hormonal deficits are now early detected and correct with suppletive doses of levothyroxine. Oral expression of hyperthyroidism is poor, even if thyrotoxicosis is sometimes revealed by increased thirst. Caution is required for dental care, mainly due to changes in the hemostatic functions. Finally, a genetic predisposition to the medullary thyroid cancer may be revealed by an oral and neuromatosis. Radioisotopic therapy of thyroid cancers is likely to alter the salivation.

Ectopic orbital meningioma: Fact or fiction?

Primary intraorbital ectopic meningiomas are rare and their existence remains controversial. We present a 30-year-old female with painless, non-axial proptosis and a palpable superomedial mass. The MRI demonstrated that the mass had no optic nerve sheath or sphenoid wing involvement and was initially reported to have no intracranial extension. The patient was initially thought to have an ectopic orbital meningioma. Subsequent multidisciplinary team (MDT) consultation and further specialist review of the MRI revealed a subtle dural tail connecting to an enhancing mass in the olfactory groove. Biopsy revealed a WHO Grade 1 transitional meningioma with an infiltrative pattern. We argue that some previously reported cases of ectopic meningioma may lack the requisite imaging to discover the primary disease. Our report highlights the importance of MRI in this group of patients and the role of a skull-base MDT with specialist neuroradiology input to determine the true origin and extent of these extradural orbital meningiomas.

Hydronephrosis Secondary to an Ectopic Decidual Reaction in the Urinary Bladder.

A 28-year-old pregnant woman at 19 weeks gestation presented with dysuria as well as lower abdominal and left flank pain. Imaging revealed left-sided hydronephrosis and a mass invading the posterior bladder wall. Management included placement of a left nephrostomy tube and transurethral resection of ~25% of the mass. Microscopy showed an ectopic decidual reaction within the muscularis propria. The patient improved symptomatically and continued prenatal care. Complete resolution of her ureteral obstruction was demonstrated during the postpartum period. Ectopic decidual reactions involving the urinary bladder are extremely rare, and ureteral obstruction secondary to this phenomenon has not yet been reported.

Ectopic immature renal tissue in gubernaculum associated with undescended testis of a 1-year-old child: A case report.

RATIONALE: Ectopic immature renal tissue (EIRT) is extremely rare in congenital malformations. Moreover, the fundamental pathogenesis of EIRT is still unclear and controversial. PATIENT CONCERNS: The right scrotum of a 1-year-old man was found empty for a period of 1 month. B-ultrasonography revealed normal bilateral kidneys and a hypoechoic nodule in the right groin. DIAGNOSES: Based on B-ultrasonography, surgery and pathological examination, we concluded a case of abnormally located and EIRT in the inguinal canal. INTERVENTIONS: After pathological diagnosis, the patient was not treated with drugs. OUTCOMES: One year after the operation, the patient recovered. LESSONS: EIRT in gubernaculum is extremely rare. Because of the potential risk of malignant transformation, it is necessary to diagnose and treat it early.

Esophageal Inlet Patch: An Under-Recognized Cause of Symptoms in Children.

OBJECTIVES: To determine the incidence of inlet patch (IP) and to assess the clinical and pathological features, role of the diagnostic workup in treatment decision making, efficacy of medical and endoscopic therapy, and natural history in a pediatric population. STUDY DESIGN: Consecutive patients aged <18 years (n = 1000) undergoing esophagogastroduodenoscopy were enrolled prospectively. Biopsy specimens were obtained from IPs and the proximal and distal esophagus, stomach, and duodenum. Multichannel intraluminal impedance and pH monitoring (MII-pH) was performed in all symptomatic patients. Symptomatic patients were treated with proton pump inhibitors for 8 weeks, and IP ablation by argon plasma coagulation (APC) was performed in unresponsive patients. RESULTS: The endoscopic incidence of IP was 6.3%, with a cumulative missing rate of 5.8%. Thirty-five of the 63 patients (56%) were asymptomatic, 11 (17%) had symptoms clearly related to the underlying digestive disorder, and 17 (27%) had chronic IP-related symptoms. MII-pH was positive in 10 of the 28 symptomatic patients. All 17 patients with IP-related symptoms were unresponsive to proton pump inhibitors and were treated with APC, and all had achieved complete remission by the 3-year follow-up. Patients with underlying disorders were successfully treated with medical therapy, and asymptomatic patients remained symptom-free, with no endoscopic or histological changes seen at the 3-year follow-up. CONCLUSION: IP is an under-recognized cause of symptoms in children with unexplained esophageal and respiratory symptoms. MII-pH and bioptic sampling are needed to exclude entities mimicking IP symptoms and to direct therapy. APC is safe and effective for treating IP-related symptoms.

Ectopic Foot Nails: Clinical and Dermoscopic Features, Treatment and Outcome in 20 Cases.

BACKGROUND: Ectopic nail (EN) is an extremely rare condition in which the small nail apparatus grows outside the normal nail unit of the hand and fingertips. Limited data can be found in the literature regarding ectopic nails of the foot and toes (EFN). OBJECTIVE: The study aimed at characterizing the clinical, therapeutic and dermoscopic EFN by a comparison with and differentiation from EN observed on the hands. METHODS: Data on EFN collected during 2004-2014 were analysed. A literature search was performed to evaluate articles for review points on the topic. RESULTS: Characteristics of shape, development, ultrasound aspects, dermoscopy and the response to treatment of 22 EFN were reported. Results from our study were compared with cases reported in the literature. CONCLUSION: In this article, new aspects on EFN from the clinical-morphological and dermoscopic points of view are reported; these may facilitate the diagnosis of an anomaly even without a histological examination.

Multiple thyroid nodules in the lung: metastasis or ectopia?

BACKGROUND: Intrapulmonary thyroid tissue with no malignant history of the thyroid gland is extremely rare. Usually, it is interpreted as ectopic thyroid tissue. Here we describe a case of bilateral pulmonary thyroid nodules with a history of multinodular thyroid goiter. HISTORY: A 37-year-old female had recurrent multinodular thyroid goiter and showed bilateral pulmonary nodules on CT scan. Video-assisted thoracic surgery (VATS) was performed for the largest nodule biopsy. Pathological and molecular examinations were done after biopsy, and both were shown the characters of benign thyroid tissues. To eliminate the possibility of thyroid carcinoma metastases, total thyroidectomy with modified radical neck dissection was performed, and there were no malignant pathological findings. After surgery, this patient accepted adjuvant radiometabolic treatment for ablation of the remaining intrapulmonary nodules. Her thyroglobulin level decreased to an undetectable level, and she has currently survived for 24 months after surgery. CLINICAL SIGNIFICANCE: In this case, pulmonary ectopic thyroid and metastasizing thyroid carcinoma should both be considered, but the metastatic pattern and benign pathological characters were inconsistent with any of the corresponding diagnosis. Ultimately, this patient accepted postoperative treatment of thyroid carcinoma metastasis. CONCLUSIONS: This is a rare thyroid disease with malignant behavior but no pathological evidence. Careful diagnosis and postoprative follow-up should be carried out whenever such nodules are encountered in clinical practice. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1255194331453728 .