A rare cause of neonatal hypercalcemia: Neonatal severe primary hyperparathyroidism: A case report and review of the literature.

INTRODUCTION: Neonatal severe primary hyperthyroidism is an extremely rare disorder that occurs in the first six months of life. Early recognition and prompt surgical intervention are of vital importance for survival and to avoid neurological sequel. Hypotonia, lethargy, respiratory distress, and growth and developmental delay occur in association with elevated serum parathormone levels and hypercalcemia (Gannon et al., 2014). Definitive therapy involves total parathyroidectomy. CASE PRESENTATION: We are presenting a patient with Neonatal severe primary hyperparathyroidism, who successfully underwent total parathyroidectomy. The patient had been followed up with medical therapy until he was seven months old, with no adequate clinical response to medical therapy. Parathormone levels rapidly declined following total parathyroidectomy, and the parathormone level fell to zero after removal of the ectopic tissue with a second surgery, and the patient was discharged with full recovery. DISCUSSION: Sestamibi scintigraphy might not always show an ectopic parathyroid gland. In such conditions, confirmation of parathyroid glands excised with total parathyroidectomy by frozen biopsy is not sufficient to terminate surgery. Intraoperative parathormone monitoring is particularly important at this point. Persistently elevated parathormone levels should suggest a remnant parathyroid tissue at the surgical site or an ectopic parathyroid gland that needs to be excised. CONCLUSION: Neonatal severe primary hyperparathyroidism is a life-threatening condition. Early surgery is life-saving in cases in whom medical therapy fails to control the disease.

Transvers testicular ectopia: A case report and literature review.

INTRODUCTION: Transverse testicular ectopia (TTE) is a rare anomaly characterized by the presence of both testicles in the same hemiscrotum or inguinal region. The most common clinical findings of TTE are unilateral nonpalpable testis in the scrotum and inguinal hernia on the side of palpable testis in the scrotum. It should be kept in mind that TTE may coexist with Persistent Mullerian Duct Syndrome (PMDS). Therefore, appropriate treatment should be performed considering PMDS. PRESENTATION OF CASE: Type 2 transverse testicular ectopia was diagnosed in one patient who was operated with bilateral undescended testis. He was treated with transseptal orchiopexy and excision of mullerian structures. DISCUSSION: In case of TTE with PMDS, optimal surgical approach with orchiopexy and excision of Müllerian duct is necessary. Intraoperative aggressive dissection of vas deferens and testicular vessels should be avoided in TTE patients. They should be closely followed because of the increased risk of malignant transformation in the postoperative period. CONCLUSION: An investigation of transvers testicular ectopia should be performed in all nonpalpable undescended testis anomalies.

Comparison of Single-Port Percutaneous Extraperitoneal Repair and Three-Port Mini-Laparoscopic Repair for Pediatric Inguinal Hernia.

AIM: Laparoscopy has been widely used in surgical practice in pediatric age, and many techniques for laparoscopic hernia repair have been described till now. In this study, we compared two laparoscopic techniques performed by two surgeons; each surgeon practicing only one of the two techniques. MATERIALS AND METHODS: A retrospective analysis was performed on the surgical charts, enrolling 71 patients with uncomplicated inguinal hernia. Patients were divided into two groups according to the type of surgery: (Group A, 24 patients aged 2 months-8 years) laparoscopic percutaneous internal ring suturing technique and (Group B, 47 patients aged 35 days-12 years) three-port mini-laparoscopic technique. The hernia sac was ligated at the level of internal ring, using nonabsorbable 4/0-3/0 suture. Any unexpected contralateral opening was repaired in the same manner for both groups. Follow-up period was 4 months-2 years and 9 months-8 years, respectively. Operative time and complications were analyzed. RESULTS: Operation time (19.58 ± 7.06 minutes versus 35.87 ± 10.34 minutes, P < .001) was shorter in the percutaneous repair group. However, when subdivided by unilateral and bilateral presentation, only unilateral operative time was shorter compared to three-port group. There were no recurrences in Group A, while two recurrences occurred in Group B during the learning curve period. A contralateral opening accompanied the presenting unilateral hernia in 3 cases for Group A and 16 for Group B. One patient had to be converted open resulting from epigastric vessel injury, and postop hydrocele formation was seen in another in Group A. No intraoperative complications were seen in Group B. CONCLUSION: The overall experience shows that laparoscopic repair is a reliable approach regardless of the chosen technique. Percutaneous repair seems to be a less invasive method with shorter operative time, but it is not free of complications according to this series.

The Role of Doctors and Patients in Appendicitis Perforation.

In this study, we aimed to determine factors that cause appendix perforations and to identify the role of physicians and patients in contributing to the cause of these perforations. This study was conducted between April 2010 and May 2015 and included 64 patients with perforated appendicitis. Patients' medical records were examined for factors that might have contributed to perforation, and the roles of patients and physicians in perforation appendicitis were evaluated. The perforation rate of patients with appendicitis was 16.0 per cent. The average duration from symptom onset to hospital admission was 4.4 days (29 patients were admitted to hospital within two days, 35 were admitted later). In total, 38 patients had visited a different hospital before admission. Furthermore, six out of 26 patients who had not visited any other hospital had consumed analgesics. Factors contributing to appendix perforation included misdiagnosis at the patient's initial visit (56.0%), delayed admission to hospital (11.0%), and use of analgesics (9.0%). The cause of perforation was mostly physician-related in children and adults, and patient-related in older adults.

Smith-lemli-opitz syndrome: a case with annular pancreas.

Smith-Lemli-Opitz syndrome is an autosomal recessive disease of cholesterol metabolism. It is a multiple malformation syndrome with typical dysmorphic features such as bitemporal narrowing, ptosis, epicanthus, microcephaly, micrognathia, and cardiovascular, skeletal, urogenital, and gastrointestinal anomalies. This report presents a typical case of Smith-Lemli-Opitz syndrome with annular pancreas which is an unreported gastrointestinal abnormality.

Importance of the ultrasonography in diagnosis of ileal duplication cyst.

Gastrointestinal duplication cysts are rare congenital anomalies that can be seen in anywhere of the gastrointestinal system from the mouth to the anus. These are prenatally diagnosed through antenatal ultrasonography. However, attention must be paid since these formations might be confused with ovarian or mesenteric cysts. Our patient, who had been diagnosed with ovarian cyst on the ultrasonography performed in another center and with mesenteric cyst based on the abdominal MRI carried out at fifth month of life, presented to our clinic with the only complaint of constipation at 9th month of life. The diagnosis was set through double wall appearance of duplication cyst on the abdominal ultrasonography. The patient's cyst was resected.

Cross ectopic multicystic dysplastic kidney with ureterocele in nonectopic site.

Crossed renal ectopy (CRE) is the second most common fusion anomaly of the kidney, with an incidence of 1 in 7000 autopsies; it comes in second after horseshoe kidney. Crossed renal ectopy is associated with an ectopic ureter and generally an ectopic kidney fused with a normal kidney. A 7-month-old boy who had left-to-right crossed non-fused renal ectopy and multicystic renal dysplasia with ureterocele in nonectopic kidney was reported in English language literature. In this article, we present the first case of CRE where surgical intervention has been performed.

Preoperative sonography of nonreducible inguinal masses in girls.

PURPOSE: Inguinal hernia is one of the most common surgical pathologies in childhood. Any of the abdominal organs can slide into the hernial sac and become incarcerated there. In girls, the fallopian tubes, ovaries, uterus, and-rarely-ovarian cysts can form the sliding component of an inguinal hernia. The aim of this study was to investigate the diagnostic value of preoperative sonographic examination in girls with nonreducible inguinal masses. METHODS: Nine girls ranging in age from 2 months to 8 years who were admitted to our clinic with nonreducible inguinal masses were included in the study. All patients underwent sonographic examination followed by surgery on the day of admission. RESULTS: A definitive diagnosis was obtained in 6 patients on preoperative sonographic evaluation, whereas 3 patients were misdiagnosed. One patient was diagnosed sonographically as having lymphadenopathy, but surgery revealed an ovarian cyst sliding into the hernial sac. A second patient was found to have an infected lymph node at surgery instead of a strangulated bowel loop as diagnosed on sonographic examination. In the third patient, the preoperative sonographic diagnosis was an ovarian cyst in the hernia sac, but surgery revealed a cyst of the canal of Nuck. CONCLUSION: Inguinal masses in young girls must be carefully evaluated, because the sonographic preoperative diagnosis may be misleading.

Minimal access surgical repair of Morgagni hernia: the fate of the unresected hernia sac.

Treatment of retrosternal diaphragmatic (Morgagni) hernia is composed of a simple surgical closure of the retrosternal opening either conventionally by open abdominal or thoracic approaches, or more recently, by using minimal access surgery (MAS). Clinical experience using the latter approach is very limited in children. Removal of the hernia sac is a controversial issue, since the sac is said to carry the risk of cyst formation or show spontaneous resolution. This issue represents a 7-year-old boy with Morgagni hernia that was successfully repaired by the MAS approach. The hernia sac was not resected because of adhesion to the adjacent tissues. The patient was readmitted with fluid accumulation within the remaining sac 2 months after the operation. A complete spontaneous resolution was observed within 2 months under conservative follow-up. To our knowledge, the temporary complication described in this paper has not been published in the literature.

Esophageal muscle cell interaction with biopolymers.

BACKGROUND: The in vitro interactions of esophageal smooth muscle cells (SMCs) with synthetic absorbable polymers were tested and artificial muscle tissues harvested from subcutaneous implantation were examined. MATERIAL/METHODS: Esophageal tissue samples from adult and fetal (25-day gestational age) rabbits were cut into small pieces and cultured in Dulbecco's Modified Eagle Medium supplemented with 10% fetal bovine serum. Growing cells were identified as SMCs by immunostaining for anti-actin and anti-myosin antibodies. Equal volumes of agar gel and medium were mixed and used for 3-D culture. 5x10(5) cells and 1 mg polyglycolic acid (PGA) and poly-lactide-co-glycolide acid (PLGA) fibers were seeded in six-well tissue culture plates. On days 2 and 7 growing cells were counted by a hemocytometer and cell-polymer interactions were evaluated with light microscopy. Adult and fetal SMCs were seeded onto the PGA and PLGA scaffolds, cultivated for two weeks, and implanted subcutaneously on the backs of the rabbits. Cell-polymer implants were retrieved after four weeks and muscle formation was evaluated histologically and immunohistochemically. RESULTS: Growing cells stained positive for actin and myosin proteins. Cell-polymer interactions were poor after 24 hours, whereas intensive attachment to the fibers was detected 48 hours following cultivation. Both fiber materials supported cell proliferation. PLGA scaffolds improved muscle formation more efficiently than PGA, and fetal and adult SMCs showed similar mass quality. CONCLUSIONS: Scaffolds are important as cell-carrying vehicles, and material-cell interactions should be tested before application. A 3-D culture prepared with agar gel and medium is practical for testing material toxicity.

Thoracoscopic diaphragm plication in children and indications for conversion to open thoracotomy.

Thoracoscopic plication of the diaphragm has been recently described as an alternative treatment for eventration. It is considered to be much less traumatic than the conventional method. We attempted thoracoscopic diaphragm plication on three patients. Two patients were treated successfully by the minimally invasive technique and were discharged from hospital on the second postoperative day. In the third case, the presence of a mobile intrathoracic kidney due to previous diaphragmatic hernia repair necessitated conversion to open thoracotomy. This patient was discharged on postoperative day six following an uneventful recovery. All patients are well and asymptomatic on followup. We advocate thoracoscopic diaphragm plication in children as a safe procedure with less morbidity and excellent cosmetic results.

Fluoroscopy-guided laparoscopy in the management of intraabdominal foreign body.

Appendicitis or perforation as a result of intraluminal foreign body is uncommon. The method of diagnosis and proper approach for an elective appendectomy in an otherwise silent intraluminal foreign body is under dispute. A laparoscopic exploration under intraoperative fluoroscopy guidance was performed in a mentally retarded 9-year-old boy with asymptomatic foreign bodies in the right lower abdominal quadrant. The authors recommend laparoscopic appendectomy, assisted by intraoperative fluoroscopy for patients with pointed foreign bodies in the appendix.

Expression of cell adhesion molecules in the adriamycin-induced esophageal atresia rat model.

Cell adhesion molecules are well-known membrane glycoproteins widely expressed during embryonic development that play a crucial role in cell division, migration and differentiation. We investigated the cell-matrix relationship using N-CAM and pan-cadherin adhesion molecules in the adriamycin-induced esophageal atresia (EA) rat model in the hope of finding a clue to the mechanisms of this unique anomaly.Time-mated pregnant Sprague-Dawley rats were given either saline or adriamycin on days 8 and 9 of gestation. Embryos were harvested on the 18th day of gestation. Esophageal specimens obtained from adriamycin-exposed embryos with (EA+) or without esophageal atresia (EA-) and from saline-exposed embryos were immunostained with N-CAM and pan-cadherin primary antisera. The esophageal specimens from control and EA- groups revealed similar immunostaining properties: weak N-CAM and pan-cadherin immunoreactivity. In contrast, the EA+ group showed intense immunoreactivity. Our study demonstrated an increased synthesis of N-CAM and pan-cadherin in the epithelial cells of the atretic esophagus and trachea. These results suggest that embryonic cell-cell and cell-matrix interactions may play a crucial role in the development of adriamycin-induced EA.

Isolation and culture of adult and fetal rabbit bladder smooth muscle cells and their interaction with biopolymers.

PURPOSE: The aim of this study was to test the feasibility of isolation and culture of adult and fetal rabbit bladder smooth muscle cells (SMCs) and comparison of their interactions with different types of biodegradable biopolymers in cell culture. METHODS: Bladder SMCs isolated from adult and fetus rabbits were identified by immunostaining for smooth muscle alpha-actin and myosin. Growth kinetics of SMCs were estimated using population doubling time (PDT) and thymidine labeling index (TLI). Poly (D, L-lactide-co-glycolide; PLGA) copolymers were synthesized at 85:15 and 75:25 monomer ratios. The porous scaffolds prepared from these polymers were seeded with SMCs. The study compared the effectiveness of adsorbing fibronectin and fetal calf serum (FCS) on these biopolymers. The cells grown on these polymers were quantified using a neutral red uptake assay. RESULTS: Over 90% of the 2 cell populations stained positive for SMC marker proteins. Fetal SMCs were seen to emerge from the tissue after 3 to 4 days, whereas adult SMCs were seen after 5 to 6 days. However, estimated PDT of fetal and adult SMCs was 85.2 and 54.5 hours, respectively, and TLI of adult SMCs was also higher than with fetal SMCs. Proliferation on 75:25 PLGA was better than for 85:15 and for both biopolymers; adsorption of FCS significantly affected cell attachment relative to fibronectin. CONCLUSIONS: Although fetal SMCs were shown to emerge from explants early after seeding onto dishes, doubling time and S-phase fraction of adult bladder SMCs were markedly higher than of fetal derived cells. Adsorption of serum proteins significantly enhances the attachment of both fetal and adult SMCs to biopolymers.