Meckel’s Diverticulum in Children: A Monocentric Experience and Mini-Review of Literature

Vitelline duct anomalies (VDA, including Meckel’s diverticulum (MD)) result from failed embryologic obliteration. This study aimed for characteristics in symptomatic versus asymptomatic VDA, analyzing clinico-laboratory data from 73 children, aged 1 day to 17 years, treated at a tertiary Pediatric Surgery Institution from 2002–2017. A male preponderance was obtained (ratio 3.6:1). MD accounted for 85% of VDA. Incidence of symptomatic VDA decreased with older age. Leading symptoms were intestinal obstruction and hemorrhage. Mucosal heterotopia (present in 39% of symptomatic MD) was associated with anemia and lowered CRP-levels. On ROC-analysis, hemoglobin < 8.6 g/dL, CRP < 0.6 mg/dL and MD distance to ileocecal valve >40 cm were predictors of ectopic tissue in symptomatic MD. Our data confirmed known characteristics as male preponderance, declined incidence of symptomatic cases with age and predominance of gastric ectopia in symptomatic MD. Moreover, anemia and prolonged distance of MD to ileocecal valve were predictors of ectopic mucosa in symptomatic MD.

Pulmonary choristoma in a new-born calf with multiple cranial and nervous malformations

ABSTRACT: Multiple congenital malformations can occur concomitantly in several species since the anomaly in one organ may lead directly to the malformation of another. Additionally, the etiology is not always clarified. Choristoma refers to an ectopic tissue that is histologically normal in an abnormal location. A case of pulmonary and nodal choristoma associated with cranioschisis, hydrocephalus, and syringomyelia in a new-born calf is reported here. Clinically, the calf had a mass in the frontal region of the head associated with local bone deformation. At necropsy, there was cranioschisis in the junction of the frontal bones and a 14 × 10 × 7 cm mass, grossly consistent with well-differentiated pulmonary tissue covered by skin, covering the opening between the frontal bones, and extending into the cranial cavity, leading to local cerebral compression. With the pulmonary choristoma, there was a well-differentiated lymphoid tissue. Additionally, in the central nervous system, there was severe hydrocephalus involving lateral ventricles and multiple areas of syringomyelia in the spinal cord.

Multifaceted control of E-cadherin dynamics by the Adaptor Protein Complex 1 during epithelial morphogenesis

Intracellular trafficking regulates the distribution of transmembrane proteins including the key determinants of epithelial polarity and adhesion. The Adaptor Protein 1 (AP-1) complex is the key regulator of vesicle sorting, which binds many specific cargos. We examined roles of the AP-1 complex in epithelial morphogenesis, using the Drosophila wing as a paradigm. We found that AP-1 knockdown leads to ectopic tissue folding, which is consistent with the observed defects in integrin targeting to the basal cell-extracellular matrix adhesion sites. This occurs concurrently with an integrin-independent induction of cell death, which counteracts elevated proliferation and prevents hyperplasia. We discovered a distinct pool of AP-1, which localizes at the subapical Adherens Junctions. Upon AP-1 knockdown, E-cadherin is hyperinternalized from these junctions and becomes enriched at the Golgi and recycling endosomes. We then provide evidence that E-cadherin hyperinternalization acts upstream of cell death in a potential tumour-suppressive mechanism. Simultaneously, cells compensate for elevated internalization of E-cadherin by increasing its expression to maintain cell-cell adhesion.

Rare presentation of ectopic thymoma

Thymoma is the most common tumor of the anterior mediastinum with an incidence of 0.15 cases per 100,000. Ninety percent of all thymoma occur in the anterior mediastinum, with the remainder occurring in the neck or other mediastinal areas. Ectopic cervical thymoma (ECT) is an extremely rare tumor that originates from ectopic tissue and is caused by the aberrant migration of the embryonic thymus. ECTs are often confused with thyroid or parathyroid swellings due to their anatomical positioning. Intrathyroid epithelial thymoma is a rare tumour comprising about 0.08% of all primary thyroid malignancies. It is low-grade thyroid carcinoma with squamous cell differentiation whose overall survival rate was found to be 71%. Here we presented a case of 43 year old female, came with swelling in front of the neck since many years. Ultrasound neck and fine needle aspiration cytology (FNAC) was performed. Patient underwent total thyroidectomy and specimen obtained was sent for histopathological examination. It revealed ectopic intrathyroid thymoma.

COLONIC DUPLICATIONS, COMPLICATED BY ACUTE INTESTINAL OBSTRUCTION IN A 9-YEAR-OLD GIRL

Gastrointestinal tract (GIT) duplication is a rare congenital anomaly with an incidence of about 1 in 4,500 newborns. Duplication of the intestine, as a rule, is diagnosed at 2–3 years, however, cases of this pathology being detected in patients of any age are described. Duplication of GIT can be asymptomatic or be detected when massive intestinal bleeding, intestinal perforation, obstruction due to intussusception or volvulus, and even malignancy in the doubled section occur. The characteristic signs of a duplication of the intestine are a well-developed muscular membrane, a general blood supply to the duplication with a portion of the supporting intestine, the presence of a mucous membrane that coincides with the supporting segment, in a third of cases, ectopic tissue is found. Our article describes the staged treatment of a patient with total tubular colon duplication complicated by acute intestinal obstruction.

Triple ectopic thyroid-a rare case report

The ectopic tissue of the thyroid gland along the descent of thyroglossal duct is a rare congenital aberration with or without the presence of normal thyroid gland. Single or dual ectopic thyroid has been reported. Three ectopic focuses at different locations are extremely rare. We present a rare case report of twenty years old female who presented with swelling over the anterior aspect of the neck mimicking thyroglossal cyst. Up on imaging evaluation ectopic thyroid at three different levels were diagnosed by computed tomography (CT) neck. Hence surgery was avoided and patient was managed with thyroxine supplement and follow up.

COUP-TFI specifies the medial entorhinal cortex identity and induces differential cell adhesion to determine the integrity of its boundary with neocortex

Development of cortical regions with precise, sharp, and regular boundaries is essential for physiological function. However, little is known of the mechanisms ensuring these features. Here, we show that determination of the boundary between neocortex and medial entorhinal cortex (MEC), two abutting cortical regions generated from the same progenitor lineage, relies on COUP-TFI (chicken ovalbumin upstream promoter–transcription factor I), a patterning transcription factor with graded expression in cortical progenitors. In contrast with the classical paradigm, we found that increased COUP-TFI expression expands MEC, creating protrusions and disconnected ectopic tissue. We further developed a mathematical model that predicts that neuronal specification and differential cell affinity contribute to the emergence of an instability region and boundary sharpness. Correspondingly, we demonstrated that high expression of COUP-TFI induces MEC cell fate and protocadherin 19 expression. Thus, we conclude that a sharp boundary requires a subtle interplay between patterning transcription factors and differential cell affinity.

ABDOMEN AGUDO DE ETIOLOGÍA INUSUAL: DIVERTÍCULO DE MECKEL PERFORADO POR UNA ESPINA DE PESCADO.

Meckel's diverticulum is the most common congenital malformation of the digestive tract; It has a prevalence that ranges from 0.3% - 2.9% in the general population according to different publications, its incidence is higher in males with a 1.5-4: 1 ratio. In general, they present an asymptomatic course throughout life, but it is estimated that 4.2% will have some associated complication, and 3% will require hospitalization and surgery. A 37-year-old male patient who presented with nonspecific abdominal pain, the physical examination revealed a painful abdomen in the mesogastrium and right lower quadrant, without signs of peritoneal irritation. Laboratory studies without alterations; as there was no improvement in the abdominal condition, it was complemented with a computed tomographic study, which showed a hyperdense linear image in the distal ileum in relation to a probable foreign body. A surgical resolution was decided using a laparoscopic approach; as laparoscopy equipment was not available, an infraumbilical laparotomy was performed. A foreign body was identified in relation to a fishbone that crosses Meckel's diverticulum at 80 cm from the ileocecal valve. A diverticulectomy with linear stapler was performed without complications, the histopathology reported Meckel's diverticulum without evidence of ectopic tissue, and he was discharged after 48 hours. Conclusions: the presence of a Meckel diverticulum as an etiology of an acute abdomen should be taken into account and be part of the differential diagnosis of pain in the right lower quadrant; foreign body perforation being rare.

The Activation of ROS/NF-κB/MMP-9 Pathway Promotes Calcium-Induced Kidney Crystal Deposition

Idiopathic hypercalciuria is an important risk factor for the formation of calcium-containing kidney stones. Matrix metalloproteinase-9 (MMP-9) is closely related to cell and tissue remodeling and is involved in ectopic tissue calcification. However, little is known about its role in kidney stone formation. In this study, we found that the expression of MMP-9 and that of osteoblastic-related proteins was increased in normal rat kidney epithelial-like (NRK-52E) cells following treatment with a high concentration of calcium, while the knockout or overexpression of MMP-9 could, respectively, significantly inhibit or upregulate the expression of osteoblastic-related proteins and calcium crystal deposition. In addition, apoptosis and calcium crystal deposition were significantly reduced in Sprague–Dawley rats with 1,25(OH)2D3-induced hypercalciuria following MMP-9 inhibitor I treatment. Furthermore, inhibiting reactive oxygen species (ROS) production or the nuclear factor kappa-light-chain-enhancer of activated B cell (NF-κB) pathway significantly reduced calcium-induced MMP-9 expression and calcium crystal deposition. In summary, our results suggested that a high calcium concentration promotes epithelial–osteoblastic transformation and calcium crystal deposition in renal tubule cells by regulating the ROS/NF-κB/MMP-9 axis and identified a novel role for MMP-9 in regulating calcium-induced calcium crystal deposition in renal tubules.

Lateral aberrant thyroid having papillary carcinoma: a case report

Aberrant thyroid is a mass of tissue having the structure of a pathological thyroid gland situated at some definite distance from the normal thyroid gland. In all the variants of carcinoma of thyroid the papillary variant is the most common. Predominantly seen in females ranging in the age group of 25-45 years. It is often well differentiated, slow growing and localised. Here we report a case of a 35-year-old lady with a swelling in the right anterior triangle of the neck with normal thyroid gland. Histopathological report was suggestive of papillary carcinoma of thyroid. The origin of lateral ectopic thyroid tissue is not fully understood and controversial. The lateral localisation is a rare entity and debated extensively in the literature. Though rare, the possibility of an ectopic thyroid carcinoma must always be considered by the surgeon in cases of a pathological mass in the neck. This case report demonstrates that a normal thyroid gland on clinical examination does not exclude the presence of thyroid carcinoma in an ectopic tissue.