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Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia.

Two children with incidentally-diagnosed ectopic pancreatic tissue in the jejunum at surgery for extrahepatic biliary atresia (EHBA) and choledochal cyst (CC) are reported. No case has been reported in the literature describing the association of a CC with ectopic pancreas, and only one case of EHBA associated with ectopic pancreas has been reported. We believe that incidentally-detected ectopic pancreatic tissue should be excised, even though the patient is symptom-free, in order to prevent the risk of serious complications due to either the mass effect or the potential for acute pancreatitis, cystic degeneration, or malignant transformation at a later date.

Biliary Atresia↗

Dual ectopic thyroid.

Ectopic thyroid is an uncommon embryological aberration characterized by the presence of thyroid tissue in a site other than in its usual pre-tracheal region. It occurs along the path of descent of the developing thyroid primodium from the foramen cecum. It most commonly presents itself as a lingual thyroid and is the only thyroid in 70% of all cases. It is extremely rare for two ectopic foci of thyroid tissue to be present simultaneously. The authors report a recent case of dual ectopic thyroid present in the lingual and infrahyoid areas with no thyroid tissue in the pre-tracheal area in a 15-year-old girl. The patient had originally been scheduled for surgery under the impression of thyroglossal duct cyst; however, a pre-operative computed tomography scan of neck and thyroid scans revealed the presence of dual ectopic thyroid, thus preventing unnecessary surgery. Therefore, thyroid scan, along with either neck CT or neck ultrasonogram, should be performed routinely to avoid unnecessary surgery if the clinical picture is at all compatible with thyroid ectopia.

Adolescent↗

Ectopic bone formation around the poly-L-lactide screw head in rotational acetabular osteotomy for hip dysplasia.

Ectopic bone formation around the poly-L-lactide (PLLA) screw head in eccentric rotational acetabular osteotomy for hip dysplasia was investigated. A total of 174 hips in 165 patients with hip dysplasia were consecutively treated with eccentric rotational acetabular osteotomy. Average age at the time of operation was 37 years. Acetabular fragments of 123 patients (132 hips) were fixed by Kirschner wires (K-wire group), and 42 hips in 42 patients were fixed with PLLA screws (PLLA group). There was no statistically significant difference between the backgrounds of the two groups. All patients were evaluated clinically and radiologically. In the K-wire group, ectopic bone formation of class 2 was observed in only 1 hip. In the PLLA group, ectopic bone formation of class 3 in 1 hip and class 2 in 3 hips was observed around the screw head 3 months postoperatively, and all hips but 1 showed class 2 at final follow-up. One hip with class 3 at 1 year developed marked reduction of range of motion, and this patient complained of moderate hip pain and stiffness. PLLA screws significantly enhanced ectopic bone formation around the screw head in eccentric rotational acetabular osteotomy.

Absorbable Implants↗

Effect of sodium channel blocker (mexiletine) on pathological ectopic firing pattern in a rat chronic constriction nerve injury model.

We studied the efficacy of mexiletine as a sodium channel blocker for neuropathic pain by investigating the effect of mexiletine on the pathological ectopic firing pattern in a chronic constriction nerve injury (CCI) model. The experiment was conducted with 60 male Wistar rats. The CCI model was created by loosely ligating the sciatic nerve. After breeding 7 days, the frequency and pattern of ectopic firing antidromically recorded from the sural nerve and the amplitude of antidromic sensory nerve-evoked potential were analyzed. The CCI rats were given an intravenous injection of normal saline and mexiletine (5 or 15 mg/kg). Mexiletine significantly suppressed spontaneous firing frequency, an on-off firing pattern that consisted of cyclic bursting spikes and ectopic firing generation under the hypoxic condition. Mexiletine did not influence the amplitude of A-delta component in the antidromic sensory nerve-evoked potential. Mexiletine suppressed ectopic firing by blocking activity of the abnormal sodium channel at the nerve-injured site and dorsal root ganglion without blocking nerve conduction. This study suggests that mexiletine is useful for treating neuropathic pain in peripheral neuropathy.

Animals↗

Ectopic cervical thymus in an infant.

We report a case of accessory cervical thymus presenting as a unilateral neck mass in a 2-month-old boy. Ultrasonography (US) showed a mass isoechogenic to muscle in the left neck. Computed tomography (CT) revealed a well-defined, mildly enhanced mass located anterior to the sternocleidomastoid muscle, anterolateral to the carotid sheath, and posterior to the submandibular gland. On magnetic resonance imaging (MRI), the mass was isointense to muscle on T1-weighted images and hyperintense to muscle on T2-weighted images. Diffusion-weighted images showed relatively low apparent diffusion coefficient (ADC) values, and the mass was slightly enhanced after administration of contrast material. We suspected ectopic thymus, but we could not exclude the possibility of a malignant lesion. Therefore, the tumor was surgically resected. The histological diagnosis was ectopic cervical thymus. Ectopic thymus should be included in the differential diagnosis of a submandibular or cervical mass in infants. US and MRI can provide useful information for the diagnosis of ectopic cervical thymus.

Biomarkers, Tumor↗

Is prolactin secreted ectopically?

The ectopic secretion of prolactin by neoplasms has been reported only rarely although in one preliminary report it has been found to be present commonly in tumor extracts. To ascertain whether this is due to a true rarity of prolactin-secreting tumors or to the fact that prolactin hypersecretion causes few notable clinical symptoms in patients with cancer, we measured prolactin levels by radioimmunoassay in the serums of 215 patients who had a variety of malignancies. Prolactin levels were elevated (greater than 25 ng/ml) in 15 patients. In 12 of these 15 patients, the elevations could be explained by the use of phenothiazine or opiates, or prior irradiation to the chest wall or head. Additional serum samples showed clearly normal prolactin levels in one of the patients. Of the entire series of 215 patients, therefore, only two patients (1 percent) had modestly elevated prolactin levels without having known causes for hyperprolactinemia: one patient with squamous cell carcinoma of the lung (prolactin 34.1 ng/ml) and one patient with breast cancer (prolactin 45.4 ng/ml). Even in these two patients the hyperprolactinemia could as likely be due to stimulation of afferent nerves in the chest wall as to ectopic secretion by the tumors. There is no clear evidence in our study or in the literature that prolactin is secreted ectopically; this is in sharp contrast to many studies showing the high frequencies of the ectopic secretion of a number of other hormones. Why this is so is unknown.

Humans↗

Pregnancy-specific beta-1 glycoprotein in ectopic pregnancy.

PSBG and hCG were estimated by radioimmunoassays in the serum of 19 consecutive patients undergoing abdominal surgery for suspected ectopic pregnancy. Ectopic pregnancy was verified at operation in 14 cases, and the other five patients had a hemorrhagic cyst of the corpus luteum. Demonstrable PSBG and hCG were found in all patients with ectopic pregnancy. One of them had taken bromocriptine for infertility, and in serial blood samples taken during the luteal phase both PSBG and hCG were identified on day 24 of the fertile cycle. One of the five patients with a hemorrhagic cyst of the corpus luteum showed detectable PSBG and hCG in several serum samples taken before operation. This patient had experienced legal abortion 3 months ago, and the presence of PSBG and hCG in her serum may represent prolonged trophoblastic activity after pregnancy. Our results show that PSBG appears in the serum very early in pregnancy. However, some obscure positive results may be encountered, and this must be appreciated when these markers are applied for clinical use. Since the PSBG and hCG tests are likely to have different sources of error, PSBG estimation may become a useful adjunct to the hCG test in the detection of trophoblastic activity in suspected ectopic pregnancy.

Adolescent↗

Meckel's diverticulum: value of ectopic tissue.

Cases of symptomatic Meckel's diverticulum treated surgically on an emergency basis during the last decade are reviewed. A series of 18 patients were divided into two groups depending on the presence or absence of ectopic tissue in the diverticulum. Group 1 consisted of five patients (28 percent) without ectopic tissue, and Group 2 consisted of 13 patients (72 percent) with ectopic tissue (8 gastric ectopia, 2 pancreatic ectopia, and 3 both gastric and pancreatic ectopia). Previous clinical records related to Meckel's diverticulum were found for 54 percent of the patients in Group 2 and for none of those in Group 1. The most common acute manifestations were intestinal occlusion (seven patients), digestive hemorrhage (five patients), and peritonitis (three patients). The postoperative course was 7 days in Group 1 and 15 days in Group 2 regardless of the surgical technique used. We conclude that the presence of ectopic tissue in patients with Meckel's diverticulum seems to be the main risk for occurrence of an acute nonmechanical complication. This complication appears more frequently and with more severity in young patients.

Adult↗

Presence of tyrosine-hydroxylase activity in anterior pituitary adenomas and ectopic anterior pituitaries in male rats.

There is evidence for local regulatory effects of dopamine (DA) and norepinephrine (NE) on the release of prolactin (PRL) and other hormones from the anterior pituitaries transplanted to an ectopic site. In order to further explore these suspected regulatory mechanisms and to determine if they may exist also in pituitary tumors, we have examined the activity of tyrosine-hydroxylase (TH) in ectopic pituitaries and in hyperplastic pituitaries of estrogen-treated rats. Adult male rats with a pituitary graft in each kidney and sham-operated controls were examined 5 weeks after surgery. Rats implanted for 3 months with Silastic capsules containing diethylstilbestrol (DES) or with empty capsules were used 10 weeks after the capsules were removed. TH activity in ectopic anterior pituitaries of grafted rats was significantly higher than that measured in the eutopic anterior pituitaries of control animals. Similarly, TH activity was significantly higher in DES-induced pituitary tumors than in control pituitaries. These data support the possible existence of local catecholaminergic mechanism(s) that could be modulating PRL secretion from pituitary tumors and from ectopic pituitaries.

Adenoma↗

Alteration of pectoral fin nerves following ablation of fin buds and by ectopic fin buds in the Japanese medaka fish.

The role of the pectoral fin bud for outgrowth by fin axons was assessed by ablation of pectoral fin buds and by transplantation of fin buds to ectopic sites in the embryos of the Japanese medaka fish (Oryzias latipes). Normally nerves from segments 1-4 (S1-4) and less frequently the S5 nerve converged at the base of the fin bud by extending toward the fin bud on the ventral surface of the axial muscles (H. Okamoto and J. Y. Kuwada, 1991, Dev. Biol. 146). Following ablation of the fin bud before motor growth cones have begun to extend laterally, nerves in S1-5 followed a trajectory down the middle of each segment parallel to the borders of the metamerically arranged axial muscles rather than converging. This trajectory was similar to that of more posterior segmental nerves which do not converge toward the fin bud. When fin buds were transplanted to more posterior segments, nerves from S1-5 often changed their trajectories and extended to the base of ectopic buds. Furthermore, motor nerves from segments posterior to S5, which normally do not innervate the fin bud, also extended to the ectopic fin bud. When faced with both the host and ectopic fin bud, motor nerves extended to either fin bud or branched and extended to both fin buds. These results demonstrate that the early fin bud is necessary for correct outgrowth of fin nerves and suggest that the fin bud normally attracts fin nerves to its base. One possible mechanism for the attraction of motor growth cones by the fin bud is a long distance cue emitted by the fin bud.

Animals↗

Ectopic umbilical liver in conjunction with biliary atresia: uncommon association.

The case of a neonate with an ectopic liver in the umbilicus in conjunction with biliary atresia in the liver proper and an ectopic pancreas in the jejunum is reported. Following excision of the ectopic umbilical liver and Kasai type 1 hepatic portoenterostomy, bile fistula originating in the anterior inferior area of the right lobe of the liver was a complication, and it was successfully treated by construction of hepaticojejunostomy. No similar case has been reported in English language literature. It is of particular interest that the pathological features of the ectopic liver and the liver proper are quite similar.

Biliary Atresia↗

Single-system ectopic ureters: a review of 12 cases.

Eight boys and 4 girls with single-system ectopic ureters were seen during a 15-year period. The abnormality was bilateral in 4 patients and the majority had major associated urological and other abnormalities. Eight ectopic ureters were reimplanted successfully into the bladder and 4 nonfunctioning kidneys were removed, as were 2 "blind" ectopic ureters. One patient died during the neonatal period and the others have been followed from 1 to 15 years. Renal function was noted to be normal in 10 patients and 1 has mild renal failure. Bladder function was normal in 8 patients. Early surgery should be undertaken in patients with single-system ectopic ureters. Stabilization of the upper urinary tract can be anticipated and satisfactory bladder function can also be expected in the majority of cases.

Child↗

Regulation of GH and TSH release from hyperplastic and ectopic pituitaries: effects of dopamine in vitro.

This study was undertaken to examine the consequences of prolonged removal of the pituitary from hypothalamic control and of estrogen-induced pituitary tumors on the susceptibility of GH and TSH release to regulatory influences of dopamine (DA). Adult male Fischer 344 rats were treated with transplants of female anterior pituitaries under the renal capsule or with Silastic capsules containing diethylstilbestrol (DES). Capsules with DES remained in place until the animals were killed (DES-IN) or were removed 7 weeks prior to sacrificing the rats (DES-OUT). Both pituitary grafts and DES caused the expected elevation in plasma prolactin and suppression of plasma GH and TSH levels. Basal GH release in vitro was not affected by exposure to DES in vivo but was reduced by transplantation of the pituitary to an ectopic site. Treatment with DA in vitro suppressed GH release from the in situ pituitaries of control, DES treated and grafted rats but increased GH release from the ectopic pituitaries. Basal release of TSH in vitro was reduced in the pituitaries of DES-IN and DES-OUT animals but was not affected by the presence of pituitary transplants. No detectable TSH was released from the ectopic pituitaries in the absence of DA. DA decreased TSH release from the pituitaries of control, DES-OUT and DES-IN rats but not from the in situ pituitaries of grafted rats. In contrast, DA produced an increase in TSH release from ectopic pituitaries. These results demonstrate that somatotrophs and thyrotrophs removed from the hypothalamic influences on subjected to direct and indirect effects of DES exhibit abnormal responses to DA. We suspect that prolonged absence of normal pituitary control leads to the development of regulatory mechanism of pituitary hormone release which are different from those operating under physiological conditions.

Animals↗

Serum antibodies to Chlamydia trachomatis in women with ectopic pregnancy, normal pregnancy or salpingitis.

Serum antichlamydial antibodies were studied in three groups. Group A included 49 women with recent ectopic pregnancy, group B included 56 women with normal first trimester pregnancy, and group C included 20 women with a first episode of acute salpingitis. In the ectopic pregnancy group, a significantly higher percentage of women had IgG antichlamydial antibodies than in the normal pregnancy group (75.5% vs. 46.4%, P less than 0.001). Women with acute salpingitis had the same prevalence of IgG antibodies as patients who had an ectopic pregnancy (60% vs. 75.5%, P less than 0.10). In the ectopic pregnancy group, the women had a significantly higher geometric mean titer (GMT) of antichlamydial IgG antibodies than women with an intra-uterine pregnancy (70.4% vs. 27.3%, P less than 0.001). The results suggest that there is a positive relation between prior Chlamydia trachomatis infection and risk for tubal pregnancy.

Acute Disease↗

Simultaneous ectopic pregnancy with intra-uterine gestation after in vitro fertilization and embryo transfer.

A case of combined intra-uterine and tubal ectopic pregnancy is described following in vitro fertilization and the transfer of two four-cell and one two-cell embryos. This phenomenon is known to be related to ovarian stimulation by gonadotropin therapy, and there is an increased risk with tubal disease. Techniques applied at the time of embryo transfer, the use of culture medium with 50% fetal cord serum to convey the embryos to the uterus, the catheterization method, and the position of the patient during transfer are presented. The risk of multiple pregnancies and combined intra-uterine and ectopic gestations increases with numbers of transfers and large volume of transfer medium. We would therefore recommend that after IVF-ET treatment in women with tubal disease, intensive care should be taken in the early follow-up period to rule out the possibility of ectopic pregnancy. In this case, a viable ongoing intra-uterine pregnancy was confirmed after surgery for right ampullary ectopic pregnancy. And a 2,925 g male in excellent condition was delivered by Cesarean section without complications.

Adult↗

High incidence of ectopic nucleolar organizer regions in human testicular tumors.

Eight primary testicular germ cell tumors, one teratocarcinoma cell line, and one Leydig cell tumor were studied to determine the importance of modifications of the nucleolar organizer regions (NORs) in human testicular tumors. Cytogenetic analysis after silver staining showed active ectopic NORs in two primary embryonal carcinomas (EC) in the cell line and in single cells of each of two seminomas (S). In one EC, an ectopic NOR was localized to chromosomal region 1q4; the others were on unidentified rearranged chromosomes. All tumors in which ectopic NORs were observed were hyperdiploid and possessed marker chromosomes typical of human germ cell tumors. Quantitative DNA analysis was performed on three tumors: a teratocarcinoma (TC) and the Leydig cell tumor, which had provided no analyzable mitoses, and a seminoma which was cytogenetically diploid. In all three cases, the major populations were hyperdiploid. The results, in combination with those of an earlier study, provide evidence that active ectopic NORs are common in human testicular tumors.

Aneuploidy↗

Ectopic cartilage in subglottic stenosis: hamartoma or reaction to trauma?

In an experimental study in growing rabbits an endolaryngeal injury to the subglottis resulted in the development of a stenosis due to the formation of scar tissue containing ectopic cartilage. For comparison, biopsies taken from the subglottic stenosis in 8 children were studied histologically. In 6 cases ectopic cartilage was observed; all patients had a history of endotracheal intubation. In 3 children the diagnosis hamartoma was made. In the remaining 3 cases the formation of ectopic cartilage might have been a direct reaction to the endolaryngeal intubation. The observations suggest that the formation of ectopic cartilage in acquired subglottic stenosis is not always due to a developmental aberration such as a hamartoma.

Animals↗

Ectopic sensory discharges and paresthesiae in patients with disorders of peripheral nerves, dorsal roots and dorsal columns.

Ectopically generated and antidromically conducted nerve impulses were recorded in 5 patients with tungsten microelectrodes inserted into skin nerve fascicles. All patients had mainly positive sensory symptoms and reported paresthesiae which could be provoked by different maneuvers which suggested increased mechanosensitivity of the primary sensory neurons at different anatomic levels. Ectopic multiunit nerve activity correlating in intensity and time course to the positive sensory symptoms was recorded: when Tinel's sign was elicited in a patient with entrapment of the ulnar nerve at the elbow, when paresthesiae were provoked by elevation of the arm in a patient with symptoms consistent with a thoracic outlet syndrome, when paresthesiae were evoked by straining during chin-chest maneuver in a patient with an S1 syndrome due to a herniated lumbar disc, when a painful Lasegue's sign occurred during the straight-leg raising test in a patient with an S1 syndrome due to root fibrosis, and when Lhermitte's sign was elicited by neck flexion in a patient with multiple sclerosis. The sites for the ectopic impulse generation in these cases are suggested to be peripheral nerve, brachial plexus, dorsal root or dorsal root ganglion and dorsal columns. The paresthesiae were non-painful except in the patient with Lasegue's sign and the ectopic impulses were probably recorded from large myelinated afferent fibers.

Adult↗