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Congenital lymphedema of the external genitalia is a rare, disfiguring disorder. We describe here a case of a 3-year-old male with primary foreskin lymphedema persisting since birth. A compact, heterogenous swelling of the foreskin's distal third was observed, inhibiting preputial retraction (phimosis). Right lower extremity lymphedema was also observed in this case, while no further abnormalities were found. Surgery was performed, maintaining the foreskin, producing an excellent result with no recurrence at 10-month follow up.

We present an extremely rare case of inguinal hernia coexisting with testicular ectopia in a child. Male infant 9.5 month old presented with an empty scrotum and the ipsilateral intravaginal testis lying in a high iliac crest position. When crying a moving right inguinal bulge appeared on clinical examination. This grew bigger in moments of increased abdominal pressure and seemed to move upwards towards the right ileac crest. No abdominal wall defect could be palpated. At operation a large hernia sac fixed in the area of the right iliac crest was identified. Adjacent was the fixation point of the gubernaculum and the testis was found in an ectopic location. We removed the large sac after separating the vas and vessels and the testis and we strengthened the dorsal inguinal wall and fixed the testis in a subdartos scrotal pouch. No postoperative complications happened. An undescended testis may present as an iliac crest ectopy, coexisting with moving inguinal hernia. In our case we propose that the higher position of the aponeurosis of the external oblique in combination with ectopia of gubernacular fixation in the ipsilateral scrotum may have caused the ectopic fixation of the sac in the ipsilateral inguinal crest.

Diphallia is an uncommon deformity, with glans duplication being its rarest form. We discuss here a case of a 6-year-old boy who presented due to excess prepuce malformation that, on examination, revealed a "penile nodule" that proved to be glans duplication. Our patient had only one urethra present and no congenital anomalies. Surgical uneventful penile reconstruction involving radiofrequency ablation (RFA) resolved the issue.

Sperm cell granuloma, also termed epididymitis nodosa, is a form of chronic epididymitis that occurs secondary to inflammation, trauma, and/or a vasectomy. We present a rare such case that to our knowledge is unique in pediatric literature: a 13-year-old boy presented with clinical and sonographic findings of epididymo-orchitis. At first, the boy was admitted for a short course of conservative treatment and was discharged. Symptoms deteriorated, he was readmitted and surgery revealed a non-twisted testis, with normal vas and vessels that ended in a necrotic testicular parenchyma. Histology showed a case of epididymitis nodosa and ruptured sperm granuloma that caused vascular compression, thrombosis and testicular necrosis. Epididymitis nodosa should be considered in cases of testicular swelling when clinical course is unusual and conservative treatment fails.

We describe an asymptomatic case of PJS in a six-year-old boy with café au lait spots in several parts of his body, a large gastroduodenal polyp, two polyps near the ampulla of Vater, and another in the jejunum. This patient shows some unique aspects of PJS. No other such large gastric polyp in a Peutz-Jeghers child is reported in the literature. The large size of the gastric polyp with lack of symptoms is unusual and poses a unique challenge in terms of management and surgical resection.

Background: Although circumcision for phimosis in children is a minor surgical procedure, it is followed by pain and carries the risk of increased postoperative anxiety. This study examined predictive factors of postoperative pain and anxiety in children undergoing circumcision. Methods: We conducted a prospective cohort study of children scheduled for elective circumcision. Circumcision was performed applying one of the following surgical techniques: sutureless prepuceplasty (SP), preputial plasty technique (PP), and conventional circumcision (CC). Demographics and base-line clinical characteristics were collected, and assessment of the level of preoperative anxiety was performed. Subsequently, a statistical model was designed in order to examine predictive factors of postoperative pain and postoperative anxiety. Assessment of postoperative pain was performed using the Faces Pain Scale (FPS). The Post Hospitalization Behavior Questionnaire study was used to assess negative behavioral manifestations. Results: A total of 301 children with a mean age of 7.56 ± 2.61 years were included in the study. Predictive factors of postoperative pain measured with the FPS included a) the type of surgical technique, b) the absence of siblings, and c) the presence of postoperative complications. Predictive factors of postoperative anxiety included a) the type of surgical technique, b) the level of education of mothers, c) the presence of preoperative anxiety, and d) a history of previous surgery. Conclusions: Although our study was not without its limitations, it expands current knowledge by adding new predictive factors of postoperative pain and postoperative anxiety. Clearly, further randomized controlled studies are needed to confirm its results. (Korean J Pain 2015; 28: 244-253)

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder caused by germline mutations in the STK11 tumor suppressor gene. PJS patients face a cumulative cancer risk as high as 93% for all sites combined. The present study reports the spectrum of STK11 mutations in eight families with clinical diagnosis of PJS, summarizes the clinical characteristics of sixteen mutation carriers and launches a National Registry for PJS in Greece. STK11 loss-of-function (LoF) mutations were detected in 87.5% of index patients. Carriers presented with their first manifestation at a median age of 24.9 years, while early-onset breast cancer was the most frequent malignancy observed, highlighting the need for breast surveillance. Out of the deleterious STK11 mutations identified, two were novel: c.375_376delGT and c.676_679dupAACG, with 57.2% of these potentially occurring de novo. Using all available clinical and genetic data, the National Registry for Greek PJS was established in an attempt to better characterize the syndrome and raise awareness among patients and clinicians (available at https://www.peutzjeghersgreece.org). This is the first comprehensive genetic analysis and clinical characterization of Greek PJS patients, where a high incidence of breast cancer was observed and the first attempt to centralize all data in a National Registry.

Background: Alimentary tract duplications (ATDs) are rare congenital anomalies of the gut tube, seen mainly in neonates and infants. Their presentations are often mimicking other conditions, thus posing a diagnostic challenge. Surgical treatment is required in all cases. Objective: The aim of this study is to present our experience in the diagnosis and management of this condition. Subjects and Method: We, retrospectively, reviewed 7 duplications in 7 patients and analyzed sex, age, clinical presentation, location, complications, diagnostic work-up, surgical methods and post-operative course. Encountered diagnostic and surgical difficulties were also reviewed in two extremely rare cases. Results: Patients’ age varied between 2 months and 10 years. All duplications were single. Six of them were intra-abdominal and one thoracoabdominal Three ADTs were asymptomatic and discovered during routine X-ray imaging. One ADT involving the cecum was mimicking appendicitis and complicated by recurrent intussusceptions. The thoracoabdominal one proved a surgical challenge as it was a completely isolated ATD. All patients underwent surgery without postoperative complications. We conclude that despite their rarity, ATDs require a high level of clinical suspicion, especially if they are presented as thoracic masses. Appropriate diagnostic investigation of the pediatric patients is always necessary to avoid delay in diagnosis.

We report a rare case of cutaneous hybrid tumor composed of epidermal cyst and cystic pilomatricoma that presented in an unusual way in a child. A 14-year-old teen girl presented with a skin lesion on the inner side of the left arm, approximately at the level of the elbow. The lesion was initially observed 5 months before and was continuously increasing in size during that time resembling other benign or malignant cutaneous tumors.

Acute appendicitis is the most common cause of the right lower quadrant acute abdominal pain in children. Some other conditions including cecal epiploica appendix torsion, can simulate acute abdomen. Epiploica appendix torsion usually occurs in the sigmoid colon and rarely in the cecum of adult males. In children, this entity is extremely rare and may represent a diagnostic and therapeutic dilemma. We report a case of an 8-year-old Greek girl, presented with signs and symptoms mimicking acute abdomen. Our patient is the younger one among the other four with cecal epiploica appendix torsion that had been reported in the literature.

Introduction Lower Spigelian hernia is a very rare entity. The clinical findings are similar to those of inguinal hernias and in many cases may be misdiagnosed. In the literature, only a few references to this entity have been reported in children. To the best of our knowledge, this is the first case report of a lower Spigelian hernia in a child who presented with an acute painful scrotum. Case presentation We discuss the case of a 6-year-old Greek boy who presented to our emergency department complaining of severe pain in the left inguinal area and scrotum. The acute painful swelling started suddenly, without any obvious cause. The initial diagnosis was incarcerated inguinal hernia which was reduced with difficulty. Five days later, the patient still experienced mild pain during palpation and he was operated on. During the operation, a large lower Spigelian hernia was revealed and reconstructed. Conclusion Although Spigelian hernias are rare in children and difficult to diagnose, physicians should be aware of them and include them in the differential diagnosis.

Objective Circumcision has been the traditional method of choice in the treatment of boys with phimosis. Recently, several published studies worldwide have focused their interest on more conservative approaches in management of this condition. These studies advocate the use of topical steroids in the phimotic foreskin. We aimed to investigate the efficacy and safety of a medium potency corticosteroid in boys with different types of prepuce retractabilty. Patients and methods A prospective study was performed involving boys referred to our hospital for possible phimosis between January 2004 and February 2008. All were treated initially with fluticasone proprionate 0.05% for a period of 4–8 weeks. Patients were reassessed after 6 months of follow up. Results A total of 1185 boys with a diagnosis of phimosis were treated with fluticasone proprionate 0.05%. Successful results were achieved in 1079 (91.1%) patients including boys with mild balanitis xerotica obliterans. No side effects were noticed. Conclusion Our results show that fluticasone proprionate 0.05%, a mild potent corticosteroid, is effective and safe in the treatment of boys with different types of phimosis.

The mastoid process originates from a recent phylogenetic formation and is currently considered as an evolution of the supper-structures bones. The aim of this study was the detailed description of the anatomic elements of the mastoid and the temporal bone in a large Greek adult corpse material. In total, 298 temporal bones from 149 cadaverous (74 male and 75 female) were examined from 1973 through 1993. Concerning the shape of the mastoid apex 94.0% were normal, while the rest presented several variations No statistically significant difference was revealed between genders. The diameters and axis of the temporal bone presented an independent correlation with gender in a multiple regression model. This prescription may add in easier surgical approach by adding new morphological details directly from the corpse material.

Traditional methods for bile culturing may miss a large number of underlying bacterial infections that could lead to acute or chronic cholecystitis.to evaluate possible differences regarding the site of material collection and thus to detect the most suitable sample site for gallbladder culture.A cohort of 137 patients with symptomatic cholelithiasis was enrolled. After surgical excision of the gallbladder, bile cultures were separately performed from fundus, body and neck. Identification of bacteria as well as computation of mean bacterial concentrations were performed with standard microbiological techniques. Wilcoxon's paired and Chi-square tests were used for comparison between continuous and discrete parameters, respectively.Thirty-one patients (22.6%) demonstrated at least one positive culture sample. Positivity was 31/31 (100.0%) in neck samples, 20/31 (64.5%) in body and 13/31 (41.9%) in fundus samples (P0.001). The microorganisms identified were Escherichia coli (14 cases) and Enterococcus faecalis (10 cases), followed by Staphylococcus aureus (3 cases), Pseudomonas aeruginosa, Enterococcus faecium, Enterobacter aerogenes and Enterobacter cloacae (1 case each). Mean bacterial concentrations in positive samples derived from the neck (272.2 +/- 187.5) were higher (P0.01) when compared to those derived from both the body (38.2 +/- 28.7) and the fundus (12.5 +/- 11.3). Mean bacterial concentrations in positive samples derived from the body were higher (P0.01) than those derived from the fundus.The neck of the gallbladder hosts the biggest bacterial load in comparison with the body and the fundus. This difference might be attributed to the presence of Rokitansky-Aschoff sinuses, which is the main histological characteristic of the region.

We report the case of a 3-yr-old boy who presented an acute right hydrocele. A rapid scrotal swelling under tension developed the first hours and the child complained for discomfort especially during palpation of the scrotum. Three days later, surgical exploration revealed an incomplete torsion of a communicated and pedunculated peritoneal sac arising from the tunica vaginalis testis. The present case report represents the eleventh report of torsion of processus vaginalis saccular protrusion in the literature, being unique due to painless hematocele.

Introduction Popliteal artery aneurysms are the most common peripheral arterial aneurysms, and are frequently bilateral. Acute limb ischemia, rupture and compression phenomena can complicate these aneurysms when the diameter exceeds 2 cm. Case Presentation We report an 82-year-old male patient with two giant femoropopliteal aneurysms, 10.5 and 8.5 cm diameters, managed in our institution. Both aneurysms were resected and a polytetrafluoroethylene (PTFE) femoropopliteal interposition graft was placed successfully. Management and literature review are discussed. Conclusion We believe this is the first report in the medical literature of bilateral giant femoropopliteal aneurysms.

Introduction Hepatic abscesses have been reported as a rare complication of diverticulitis of the bowel. This complication is recognized more commonly at the time of the diagnosis of diverticulitis, or ruptured diverticula, but also can be diagnosed prior to surgery, or postoperatively. Case presentation This report describes a man who developed an hepatic abscess within a simple hepatic cyst, two months after operation for ruptured diverticula of the sigmoid colon. The abscess was drained surgically and the patient made a complete recovery. Conclusion The development of an hepatic abscess in a pre-existing hepatic cyst, secondary to diverticulitis, is a rare complication. A high degree of clinical suspicion is required for immediate diagnosis and treatment.

The abdominal compartment syndrome (ACS) is a potentially fatal entity that occurs as a result of an acute increase in intra-abdominal pressure (IAP). The authors report on a girl with a giant ovarian cystic mass, and clinical signs of ACS and intracranial hypertension (ΙΗ). The possible mechanism of IH secondary to ACS is discussed.

Background Gastroparesis is defined as an inhibition of the gastric motility associated with delayed gastric emptying, which is mainly presented with acute dyspepsia. Gastroptosis is the downward displacement of the stomach. Case Report We report a rare case of secondary gastroparesis due to gastroptosis in an 11-year-old female child. The patient complained for bulking mass in the left lower quadrate presented a week ago, which was mimicking a large abdominal hernia. The laboratory and radiological exams revealed an excessive gastroptosis associated with gastroparesis. We searched the literature but we failed to find other cases with idiopathic gastroptosis in a child. The patient was treated conservatively and six months after the initial diagnosis and treatment the patient does not complains for dyspepsia. Conclusion Gastroparesis associated with gastroptosis is a rare entity that can be treated conservatively with acceptable results.

Carcinoids are the most common tumours of the appendix. These tumours show prevalence in white children. The clinical presentation of the appendiceal carcinoids is similar to that of acute appendicitis, although in many cases the tumour is diagnosed incidentally during an operation. The diagnosis should be confirmed histologically. The prognosis in patients with local disease is excellent. In small lesions isolated appendicectomy is considered as the most appropriate treatment, while in larger lesions right colectomy should be performed. We report a case of a carcinoid tumour in the tip of the appendix of a thirteen year old girl which was diagnosed intraoperatively. The patient received isolated appendicectomy due to the small size of the lesion. Ten years after the operation there is no evidence of recurrence or metastases, and the patient is considered free of disease.

Introduction Crohn's disease (CD) can affect any part of the alimentary tract from the mouth to the anus, with most common site being the terminal ileum. Case presentation A child suffering from undiagnosed Crohn disease (CD), presented with an acute abdominal obstruction due to a large pseudopolyp in the jejunoileal area. At laparotomy, a jejunoileal segment of 45 cm, containing multiple areas of damage to the small intestine, was excised and a primary end – to – end anastomosis was performed. Conclusion The coexistence of an intestinal pseudopolyp with undiagnosed Crohn's disease may be the cause of acute abdominal obstruction in children.

Background A new technique for the treatment of children's phimosis is presented that minimizes the repairing time, the postoperative complications and maintains the physical foreskin appearance intact. Methods Eightyseven children with phimosis were treated with this new developed technique, between 2003 and 2005. Sutureless prepuceplasty creates a permanent surgical extension of the close prepuce. Stretching and retraction of phimotic foreskin reveals a tight prepuce ring that is cutting in its dorsal surface longitudinally. Rarely triple symmetric incisions in the preputial outlet are necessary. The foreskin is loose and moves absolutely free in bilateral courses. The wounds are healing by second intention. Antisepsis, steroids and Elicina cream, (which contains allantoin, collagen, elastin, glycolic acid and vitamins A, D, and E) should apply daily, for twenty to thirty days. Results The foreskin is moving in centripetal or efferent courses absolutely loosely, painlessly and bloodlessly. The mean time of follow-up was 27 months (one to four years). No complications were observed. Conclusion Sutureless prepuceplasty may present an acceptable alternative in children's phimosis reconstruction.

We report an unusual case of an 11-year-old Greek girl with complicated acute appendicitis. The pelvic plastron that had been formatted secondary to appendix perforation was mimicking appendiceal intussusception in the preoperative ultrasound and computed tomography images. Although acute complicated appendicitis and appendiceal intussusception may represent possible causes of acute abdomen no similar cases have reported in the literature.