The Influence of the Q-Angle and Muscle Strength on Idiopathic Anterior Knee Pain in Adolescents.

Background and Objectives: Idiopathic anterior knee pain is a common condition in adolescents and is mostly of unknown cause. The aim of this study was to examine the influence of the Q-angle and muscle strength on idiopathic anterior knee pain. Materials and Methods: Seventy-one adolescents (41 females and 30 males) diagnosed with anterior knee pain were included in this prospective study. The extensor strength in the knee joint and the Q-angle were monitored. The healthy extremity was used as a control. The Student's paired sample t-test was applied for testing the difference. Statistical significance was set at 0.05. Results: There was no statistically significant difference in the Q-angle value between the idiopathic AKP and the healthy extremity (p > 0.05) within the entire sample. A statistically significant higher Q-angle of the idiopathic AKP knee (p < 0.05) was obtained in the female subgroup. No statistically significant difference (p > 0.05) was found in the male subgroup. Within the male subgroup, the strength of the extensors within the knee joint of the healthy extremity had statistically significant higher values than the strength of these muscles in the affected extremity (p < 0.05). Conclusion: A greater Q-angle is a risk factor linked to anterior knee pain within the female population. Decreased muscle strength of knee joint extensors is a risk factor linked to anterior knee pain in both sex subgroups.

Necrotizing Fasciitis-Severe Complication of Bullous Pemphigoid: A Systematic Review, Risk Factors, and Treatment Challenges.

Background and objectives: Bullous pemphigoid (BP), the most common subepidermal autoimmune skin blistering disease (AIBD) has an estimated annual incidence of 2.4 to 42.8 new cases per million in different populations, designating it an orphan disease. Characterized by disruption of the skin barrier combined with therapy-induced immunosuppression, BP could pose a risk for skin and soft tissue infections (SSTI). Necrotizing fasciitis (NF) is a rare necrotizing skin and soft tissue infection, with a prevalence of 0.40 cases per 100,000 to 15.5 cases per 100,000 population, often associated with immunosuppression. Low incidences of NF and BP classify them both as rare diseases, possibly contributing to the false inability of making a significant correlation between the two. Here, we present a systematic review of the existing literature related to the ways these two diseases correlate. Materials and methods: This systematic review was conducted according to the PRISMA guidelines. The literature review was conducted using PubMed (MEDLINE), Google Scholar, and SCOPUS databases. The primary outcome was prevalence of NF in BP patients, while the secondary outcome was prevalence and mortality of SSTI in BP patients. Due to the scarcity of data, case reports were also included. Results: A total of 13 studies were included, six case reports of BP complicated by NF with six retrospective studies and one randomized multicenter trial of SSTIs in BP patients. Conclusions: Loss of skin integrity, immunosuppressive therapy, and comorbidities commonly related to BP patients are risk factors for necrotizing fasciitis. Evidence of their significant correlation is emerging, and further studies are deemed necessary for the development of BP-specific diagnostic and treatment protocols.

Acute hemorrhagic edema of infancy - is it really a mild, benign disease?

BACKGROUND: Acute hemorrhagic edema of infancy (AHEI) is a rare vasculitis, which presents dramatically as palpable purpuric skin lesions on the limb, face and auricles, with swelling of these parts and low-grade fever, in children up to 2 years of age. To date, approximately 400 cases have been described in the literature. The etiology remains mostly unknown. With or without treatment, AHEI goes to spontaneous recovery within 1-3 weeks, usually without any complications. To our knowledge, compartment syndrome as complication of AHEI has only been reported in one case. We present an unusual case of AHEI with serious complications due to compartment syndrome of the right-hand fingers. CASE: A 16-month-old male child presented with fever and sudden appearance and rapid spread of palpable, painless, non-itching ecchymotic hematomas on the thigh, cheeks, earlobes, forearms, dorsum of hands and feet, with mild edema of these regions. Complete systemic examination and all vital parameters were normal for age. There was no history of bleeding disorders in the family. Except low hemoglobin on complete blood count and increased D-dimer values, all other laboratory investigations were in the normal range. Changes on the right forearm and hand expanded on almost the entire dorsal side and all surfaces of the fingers, with pronounced swelling and formation of bullous lesions, which were spreading and cracking. Skin biopsy confirmed nonspecific small-vessel vasculitis. That required the use of Methylprednisolone, low-molecularweight heparin, antibiotics and debridement of necrotic eschar, with necrectomy of the affected fingers. CONCLUSIONS: Early recognition of AHEI is important to avoid unnecessary investigation and therapy. On the other hand, our reported case warns that unexpected complications may occur.

Congenital prepubic urachal-cutaneous fistula.

Congenital prepubic fistula is a very rare congenital anomaly that manifests as a punctiform opening in the midline of the pubic region. According to Campbell, congenital prepubic fistulae are duplicates of the dorsal urethra. Recent investigations have shown that this problem may have a complex etiology. We report a case of a congenital prepubic fistula in 2-month old female neonate, which manifested as a simple punctiform opening in the pubic region, with purulent secretion that resulted from applying pressure to the region. The surgical treatment was successful, and the infant had an uneventful postoperative course of healing.

Gastric tube esophageal reconstruction in children with esophageal atresia and caustic stricture Study of clinical value based on 25 single-center. Centre experience.

AIM: The aim of this study was estimation of clinical value of gastric tube esophagoplasty in children based on one single center experience. METHODS: Forty-nine patients with diagnosis of EA (n=22) and caustic esophageal injury (n=27) had undergone reverse gastric tube esophagoplasty during 25 years at our institution. Almost all EA patients received initially gastrostomy and cervical esophagostomy. Majority of CS patients had initially repeated dilatation therapy. Demographics, preoperative features (initial condition, initial treatment and nutritional status) and postoperative features (leakage, stenosis, nutritional status and disease-specific symptoms) were retrospectively reviewed and analyzed. Mean follow up time was 8.2 years. RESULTS: Of 49 patients, 2 patients died and gastric tube failed in one patient. The most common complications were anastomotic leak (52%) and anastomotic stricture (47,8%). Disease-specific symptoms (dysphagia and reflux symptoms) with important clinical manifestations were observed in 10.87% of patients. Preoperatively, all patients were eutrophic. One year after operation 96% of patients stayed eutrophic and two EA patients were malnourished. CONCLUSION: The most common complications such as anastomotic leak and anastomotic stricture may be considered benign. Postoperative nutrition, as good indicator of the procedure's adequacy, for the majority of patients is satisfactory. Gastric tube esophagoplasty is an appropriate alternative for esophageal replacement in children. KEY WORDS: Children, Gastric tube, Esophagoplasty.

Nine toes; Mirror Foot Deformity.

Mirror foot is a very rare congenital anomaly, with only a few papers presenting definitive treatment for this entity. There are limited management recommendations. Most cases are treated before walking age. In our case, there were no associated developmental defects of the leg. The child underwent complex rays resection with medial foot reconstruction. After 7.5 years of followup, definitive surgical treatment was performed with satisfactory cosmetic and functional outcome.

Sirolimus for the Treatment of a Massive Capillary-Lymphatico-Venous Malformation: A Case Report.

Management of patients with complex vascular anomalies (VAs) is often associated with significant morbidity and mortality because of the lack of effective treatment modalities that may lead to significant improvement of the disease and/or healing. Recently, reports of treatment of patients with complex VAs with sirolimus revealed encouraging results. Sirolimus inhibits the mammalian target of rapamycin, which acts as a master switch of numerous cellular processes. We report a successful use of sirolimus for the treatment of a patient with a complex CLVM of the trunk and the right lower extremity believed to be untreatable. Our patient had 44 hospitalizations during the 10-year period, with various unsuccessful treatments and continuous deterioration of his clinical condition, ending up in a wheelchair. His condition reversed to normal everyday activities 9 months after initiation of sirolimus therapy. We conclude that sirolimus is a very promising therapeutic option for children with complex VAs of capillary-lymphatico-venous type.

Unilateral galactocele in a male infant.

INTRODUCTION: Galactocele, generally occuring in young women during or after lactation, is an extremely rare cause of breast enlargement in infants and children of exclusively male gender. Only 26 cases have been published so far, including two our cases. CASE REPORT: We described unilateral, cystic, breast enlargement, without any endocrinologic and other abnormalities in a 29-month-old boy. A typical clinical and histopathologic presentation of galactocele was followed with a complete excision. CONCLUSION: This was a 27th well documented case of galactocele in a male infant with typical clinical and histopathologic presentation. There are several hypotheses regarding etiology of the lesion, but it is likely to be multifactorial. Because of its extreme rarity, there are some difficulties in differential diagnosis and treatment options of galactocele in male infants.

Analysis of Prognostic Factors and Mortality in Children with Esophageal Atresia.

OBJECTIVE: The survival rate in newborns with congenital esophageal atresia (EA) is about 85-90 %, and it raises over 95 % in the developed countries. The survival rate in developing countries is much lower and the authors describe their experience with 60 newborns. METHODS: Medical records of 60 newborns (40 boys and 20 girls) with congenital EA were reviewed for the prognostic factors and mortality. RESULTS: The birth weight, mean Apgar score (AS) value, gestational age and birth-operative treatment time had significant influence on the mortality of treated patients (p < 0.05). Thirty five percent newborns had aspirational pneumonia at the moment of hospitalization and 86.7 % of them were operated during the first 48 h. The presence of associated anomalies considerably affected the death rate of treated patients (p < 0.05). The incidence of postoperative complications was similar to those in developed countries but the total mortality was higher (28.3 %); sepsis being the main cause of mortality. The postoperative complications and sepsis significantly influenced the mortality of patients (p < 0.05). CONCLUSIONS: Total mortality in newborns with EA was high; sepsis being the most frequent cause of death. The high total mortality was also caused by prematurity, delay in diagnosis, increased incidence of the aspiration pneumonia and shortage of qualified nurses.

Cervical chondrocutaneous branchial remnants--report of 17 cases.

BACKGROUND/PURPOSE: Cervical chondrocutaneous branchial remnants are congenital, benign and rare neck masses. These anomalies are limited in the literature, reported mostly as case reports. Cervical chondrocutaneous branchial remnant is always present at birth, and the lesion is usually unilateral. Understanding and treatment of cervical chondrocutaneous branchial remnants requires knowledge of the related embryology. METHODS: From January 2005 to December 2008, 17 patients with mean age of 32 months (range from 2 months to 15 years) with CCBRs were treated at the Division of Pediatric Surgery, Department of Plastic and Reconstructive Surgery and Burns at the Institute for Mother and Child Health Care, Belgrade, Serbia. The following objections were recorded: sex, lesion side, surgical data, associated malformations and pathohistology findings. RESULTS: There were 7 females and 10 males, 4 with bilateral presences. Five children had associated anomalies, as follows: vesicoureteral reflux, atrial and ventricular septal defect, ventricular septal defect, branchiootorenal syndrome and preauricular sinus. There was a positive family history in one patient. Fifteen patients (88%) were treated with complete surgical excision and no connections with deep underlying structures of the neck were found. There were no complications at surgery. No recurrence was found during follow-up. Histopathology analysis revealed both, hyaline and elastic cartilage. CONCLUSION: Cervical chondrocutaneous branchial remnants are rare anomalies arising from branchial arch, probably originally from remnants of first or second arch. Surgical excision is the treatment of choice. From our experience, we suggest surgical treatment early in childhood because of esthetic reason, simplicity of the intervention and low complication rate. Also, the abdominal ultrasound and cardiac examination is recommended because of associated anomalies.

Duplication cyst with esophageal mucosa at the floor of the mouth: a case report.

Intraoral localization of alimentary tract duplication is extremely rare. We report a duplication cyst at the floor of the mouth, lined by esophageal epithelium and treated successfully by surgical excision. To our knowledge, this is the third case of intraoral enteric duplication cysts lined with esophageal epithelium reported in the literature.

Diffuse neonatal hemangiomatosis treatment with cyclophosphamide: a case report.

We present 3-month-old male infant with diffuse neonatal hemangiomatosis. There were 63 cutaneous hemangiomas over the scalp, face, trunk, and extremities. Computed tomography scan revealed the presence of hemangiomas in the liver and kidneys; laryngobronchoscopy identified the presence of hemangioma in tracheobronchial tree. The child had symptoms of heart failure therefore digitals and diuretics were administrated. Thyroid functions were normal. Treatment with corticosteroids, in dose of 3 mg/kg/d intravenously, was initiated. As there was no significant clinical improvement, cyclophosphamide was administrated. He received 4 courses, 10 days apart. Each course consisted of 10 mg/kg/d of cyclophosphamide and 10 mg/kg/d of mesna for 4 consecutive days. After 4 cycles of cyclophosphamide, the liver was notably decreased in size and the cardiac failure was resolved. Magnetic resonance imaging of the abdomen revealed the marked decrease in size of the liver hemangioma. After 3 years of follow-up the child is well developed, fully recovered, without cardiologic or respiratory problems.

Circular excision and purse-string suture technique in the management of facial hemangiomas.

Hemangiomas are the most common soft tissue tumors in infancy. Treatment options for hemangiomas are numerous. The scar after lenticular excision and linear closure is often unacceptable. Localized cutaneous infantile hemangioma acts like a tissue expander. Therefore, circular excision and purse-string suture technique are applicable for hemangioma at any stage. For 2-year period we have operated 14 patients with hemangioma using circular excision and purse-string suture technique. In eight patients hemangioma was localized in the head and neck region. Two infants with large hemangioma of the face are presented in this article. One patient had hemangioma with ulceration resistant to conservative treatment. Second patient had giant hemangioma, which caused partial disturbation of visual axis. We applied circular excision and purse-string suture technique in both patients. The results were good, without any postoperative complications.