Case Reports in Pediatrics The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. Orthosis Effects on the Gait of a Child with Infantile Tibia Vara Thu, 21 May 2015 16:03:26 +0000 Infantile tibia vara (ITV) is an acquired form of tibial deformity associated with tibial varus and internal torsion. As there is currently insufficient data available on the effects of orthotics on gait parameters, this study aimed to document the influence of orthosis on walking. A male infant with bilateral tibia vara used orthoses for five months. Gait evaluations were performed pre- and posttreatment for both legs. The kinematic parameters were collected by using a motion analysis system. The orthotic design principle was used to correct the femur and tibia. Posttreatment gait parameters were improved compared to pretreatment parameters. After 5 months, there was remarkable change in the stance-phase degrees of frontal plane hip joint abduction and knee joint varus. We found that orthoses were an effective treatment for the infantile tibia vara gait characteristics in this patient. Full-time use of single, upright knee-ankle-foot orthosis with a drop lock knee joint and application of corrective forces at five points along the full length of the limb were effective. Serap Alsancak and Senem Guner Copyright © 2015 Serap Alsancak and Senem Guner. All rights reserved. Clinical and Imaging Features of a Congenital Midline Cervical Cleft in a Neonate: A Rare Anomaly Thu, 21 May 2015 13:01:48 +0000 Congenital midline cervical cleft (CMCC) is a rare congenital anomaly. CMCC and its complications and treatment have been well described in ENT, dermatology, and pediatric surgery literature. However, to our knowledge, the imaging work-up has not been reported in the literature thus far. We present a case of CMCC in a neonate with description of clinical presentation and imaging features. Rachelle Goldfisher, Pritish Bawa, Zachary Ibrahim, and John Amodio Copyright © 2015 Rachelle Goldfisher et al. All rights reserved. Chronic Lipoid Pneumonia in a 9-Year-Old Child Revealed by Recurrent Chest Pain Thu, 21 May 2015 11:56:18 +0000 Lipoid pneumonia in children is a rare disorder due to accumulation of fatty oily material in the alveoli and usually associated with an underlying condition. In absence of obvious context, diagnosis remains difficult with nonspecific clinical and radiological features. We report the first case of voluntary chronic aspiration of olive oil responsible for exogenous lipoid pneumonia, in a previously healthy 9-year-old boy. Clinical presentation was atypical; LP was revealed by isolated chest pain. We discuss radiological and bronchial alveolar lavage characteristics suggestive of lipoid pneumonia. Conclusion. Lipoid pneumonia is a disease to be reminded of in children, which can occur with original findings in terms of etiology and clinical presentation. A. Hochart, C. Thumerelle, L. Petyt, C. Mordacq, and A. Deschildre Copyright © 2015 A. Hochart et al. All rights reserved. Rahnella aquatilis Sepsis in a Premature Newborn Mon, 18 May 2015 09:38:31 +0000 Rahnella aquatilis is an infrequently isolated Gram-negative rod within the Enterobacteriaceae family. The organism’s natural habitat is water. The organism is rarely isolated from clinical specimens and it seldom causes infection in immunocompetent individuals. Here we present a one-month-old boy who was born prematurely at 27th week of gestation by cesarean section with a birth weight of 730 g. He developed sepsis caused by Rahnella aquatilis during the treatment for ventilator associated pneumonia due to Stenotrophomonas maltophilia with ciprofloxacin. He was successfully treated with a combination of amikacin plus meropenem. Although R. aquatilis is one of the saprophyticus organisms, it may cause life-threatening infection in newborn. Canan Kuzdan, Ahmet Soysal, Hülya Özdemir, Şenay Coşkun, İpek Akman, Hülya Bilgen, Eren Özek, and Mustafa Bakır Copyright © 2015 Canan Kuzdan et al. All rights reserved. Molar Tooth Sign with Deranged Liver Function Tests: An Indian Case with COACH Syndrome Sun, 17 May 2015 09:51:51 +0000 We report the first genetically proven case of COACH syndrome from the Indian subcontinent in a 6-year-old girl who presented with typical features of Joubert syndrome along with hepatic involvement. Mutation analysis revealed compound heterozygous missense mutation in the known gene TMEM67 (also called MKS3). Rama Krishna Sanjeev, Seema Kapoor, Manisha Goyal, Rajiv Kapur, and Joseph Gerard Gleeson Copyright © 2015 Rama Krishna Sanjeev et al. All rights reserved. Acute Kidney Injury Complicated Epstein-Barr Virus Infection in Infancy Mon, 04 May 2015 13:14:32 +0000 Infectious mononucleosis is an acute lymphoproliferative disorder caused by the Epstein-Barr virus (EBV) and seen most commonly in children and young adults. Clinical presentation of the disease is characterized by fever, tonsillopharyngitis, lymphadenopathy, and hepatosplenomegaly, whereas serological findings of this benign disorder include positive heterophilic antibody formation (transient increase in heterophilic antibodies) and prominence of hematological lymphocytosis of more than 10% of atypical lymphocytes. An EBV infection is usually asymptomatic in childhood, but acute kidney injury can be a rare complication during its course. Most cases recover from the disease completely. Early recognition of EBV infection and estimation of its complication are important for its prognosis. In light of previous literature, we discuss the case evaluated as an EBV infection complicated by acute kidney injury in early childhood and results of tubulointerstitial nephritis shown on a renal biopsy that was later diagnosed as an EBV infection by serological examination. Gamze Ozgurhan, Mustafa Ozcetin, Aysel Vehapoglu, Zeynep Karakaya, and Fatih Aygun Copyright © 2015 Gamze Ozgurhan et al. All rights reserved. Hyperinsulinemic Hypoglycaemia in a Turner Syndrome with Ring (X) Wed, 29 Apr 2015 11:29:11 +0000 Hyperinsulinemic hypoglycaemia (HH) is a group of clinically, genetically, and morphologically heterogeneous disorders characterized by dysregulation of insulin secretion by pancreatic beta cells. HH can either be congenital genetic hyperinsulinism or associated with metabolic disorder and syndromic condition. Early identification and meticulous management of these patients is vital to prevent neurological insult. There are only three reported cases of HH associated with a mosaic, r(X) Turner syndrome. We report the four cases of an infant with a mosaic r(X) Turner genotype and HH responsive to diazoxide therapy. Michela Cappella, Vanna Graziani, Antonella Pragliola, Alberto Sensi, Khalid Hussain, Claudia Muratori, and Federico Marchetti Copyright © 2015 Michela Cappella et al. All rights reserved. NIV-Helmet in Severe Hypoxemic Acute Respiratory Failure Mon, 27 Apr 2015 13:35:50 +0000 Noninvasive ventilation (NIV) is a method to be applied in acute respiratory failure, given the possibility of avoiding tracheal intubation and conventional ventilation. A previous healthy 5-month-old boy developed low-grade intermittent fever, flu-like symptoms, and dry cough for 3 days. On admission, he showed severe respiratory distress with SpO2/FiO2 ratio of 94. Subsequent evaluation identified an RSV infection complicated with an increase of inflammatory parameters (reactive C protein 15 mg/dL). Within the first hour after NIV-helmet CPAP SpO2/FiO2 ratio increased to 157. This sustained improvement allowed the continuing of this strategy. After 102 h, he was disconnected from the helmet CPAP device. The NIV use in severe hypoxemic acute respiratory failure should be carefully monitored as the absence of clinical improvement has a predictive value in the need to resume to intubation and mechanical ventilation. We emphasize that SpO2/FiO2 ratio is a valuable monitoring instrument. Helmet interface use represents a more comfortable alternative for providing ventilatory support, particularly to small infants, which constitute a sensitive group within pediatric patients. Joana Martins, P. Nunes, C. Silvestre, C. Abadesso, H. Loureiro, and H. Almeida Copyright © 2015 Joana Martins et al. All rights reserved. Two Mutations in Surfactant Protein C Gene Associated with Neonatal Respiratory Distress Mon, 27 Apr 2015 13:04:59 +0000 Multiple mutations of surfactant genes causing surfactant dysfunction have been described. Surfactant protein C (SP-C) deficiency is associated with variable clinical manifestations ranging from neonatal respiratory distress syndrome to lethal lung disease. We present an extremely low birth weight male infant with an unusual course of respiratory distress syndrome associated with two mutations in the SFTPC gene: C43-7G>A and 12T>A. He required mechanical ventilation for 26 days and was treated with 5 subsequent doses of surfactant with temporary and short-term efficacy. He was discharged at 37 weeks of postconceptional age without any respiratory support. During the first 16 months of life he developed five respiratory infections that did not require hospitalization. Conclusion. This mild course in our patient with two mutations is peculiar because the outcome in patients with a single SFTPC mutation is usually poor. Anna Tarocco, Elisa Ballardini, Maria Raffaella Contiero, Giampaolo Garani, and Silvia Fanaro Copyright © 2015 Anna Tarocco et al. All rights reserved. An Interesting Fistula Tract Presenting with Recurrent Gluteal Abscess: Instructive Case Sun, 05 Apr 2015 08:45:17 +0000 A fistula extending from the gluteus to penis is an extremely rare entity. In this paper, we have highlighted novel variant of congenital penile to gluteal fistula complicated with gluteal and penoscrotal abscess in a previously healthy boy. A fistulous tract extending from the gluteus to penis has been shown by fistulogram. Bleomycin has been used in fistula tract with successful results in our patient. Gulsum Iclal Bayhan, Ozge Metin, Burak Ardicli, Ayse Karaman, and Gonul Tanir Copyright © 2015 Gulsum Iclal Bayhan et al. All rights reserved. Ectopic Lingual Thyroid Sun, 29 Mar 2015 11:12:37 +0000 Ectopy of the thyroid gland is an abnormal embryological development. Its occurrence in children is rare. In this study, we report the case of a 12-year-old girl that presented with dysphagia and nocturnal dyspnea. Magnetic resonance imaging confirmed the presence of a lingual thyroid. Thyroid scintigraphy showed intense and elective uptake of radiotracer at the base of the tongue. Hormonal tests revealed hypothyroidism. Treatment consisted of opotherapy based on levothyroxine. Evolution has been favourable and the patient showed significant improvement with reduction of the dyspnea and the dysphagia and normalization of thyroid hormone tests. Khaled Khamassi, Habib Jaafoura, Fahmi Masmoudi, Rim Lahiani, Lobna Bougacha, and Mamia Ben Salah Copyright © 2015 Khaled Khamassi et al. All rights reserved. Empyema Necessitans Complicating Pleural Effusion Associated with Proteus Species Infection: A Diagnostic Dilemma Sun, 29 Mar 2015 09:33:08 +0000 Background. Empyema necessitans, a rare complication of pleural effusion, could result in significant morbidity and mortality in children. It is characterized by the dissection of pus through the soft tissues and the skin of the chest wall. Mycobacterium tuberculosis and Actinomyces israelii are common causes but Gram negative bacilli could be a rare cause. However, there were challenges in differentiating between Mycobacterium tuberculosis and nontuberculous empyema in a resource poor setting like ours. We report a child with pleural effusion and empyema necessitans secondary to Proteus spp. infection. Methods. We describe a 12-year-old child with empyema necessitans complicating pleural effusion and highlight management challenges. Results. This case was treated with quinolones, antituberculous drugs, chest tube drainage, and nutritional rehabilitation. Conclusion. Empyema necessitatis is a rare condition that can be caused by Gram negative bacterial pathogens like Proteus species. M. S. Yauba, H. Ahmed, I. A. Imoudu, M. O. Yusuf, and H. U. Makarfi Copyright © 2015 M. S. Yauba et al. All rights reserved. Spontaneous Duodenal Perforation as a Complication of Kawasaki Disease Thu, 26 Mar 2015 12:25:59 +0000 Kawasaki disease is generally known as a systemic vasculitis that often concerns doctors due to its serious cardiac complications; however, other visceral organs may get involved as well. Surgical manifestations of the intestinal tract in Kawasaki disease are rare. In this report, we describe the case of a 2.5-year-old boy with typical Kawasaki disease who presented with GI bleeding and surgical abdomen. The diagnosis of duodenal perforation was confirmed. Kambiz Masoumi, Arash Forouzan, Hossein Saidi, Hazhir Javaherizadeh, Ali Khavanin, and Mohammad Bahadoram Copyright © 2015 Kambiz Masoumi et al. All rights reserved. Thoracic Block Technique Associated with Positive End-Expiratory Pressure in Reversing Atelectasis Thu, 26 Mar 2015 07:29:45 +0000 A preschool four-year-old male patient had been admitted to the Mandaqui Hospital with a diagnosis of lobar pneumonia, pleural effusion, and right lung atelectasis. Treatment consisted of antibiotics and physiotherapy sessions, using a technique described in the literature as Insufflation Technique to Reverse Atelectasis (ITRA), which consists of a thoracic block of healthy lung tissue, leaving only the atelectasis area free, associated with the use of invasive or noninvasive mechanical ventilation with positive airway pressure for reversal of atelectasis. Two physiotherapy sessions were conducted daily. The sessions lasted 20 minutes and were fractionated into four series of five minutes each. Each series bilateral thoracic block was performed for 20 seconds with a pause lasting for the same time. Associated with the thoracic block, a continuous positive airways pressure was used using a facial mask and 7 cm H2O PEEP provided via CPAP. Conclusion. ITRA technique was effective in reversing atelectasis in this patient. Luciana Carnevalli Pereira, Ana Paula de Souza Netto, Fernanda Cordeiro da Silva, Silvana Alves Pereira, and Cristiane Aparecida Moran Copyright © 2015 Luciana Carnevalli Pereira et al. All rights reserved. Treatment for Retinopathy of Prematurity in an Infant with Adenoviral Conjunctivitis Thu, 19 Mar 2015 11:43:26 +0000 Retinopathy of prematurity (ROP) has been a major problematic disorder during childhood. Laser photocoagulation (LPC) has been proven to be effective in most of the ROP cases. Adenoviral conjunctivitis (AVC) is responsible for epidemics among adult and pediatric population. It has also been reported to be a cause of outbreaks in neonatal intensive care units (NICU) several times. We herein demonstrate a case with AVC who underwent LPC for ROP. And we discuss the treatment methodology in such cases. Murat Gunay, Gokhan Celik, and Rahim Con Copyright © 2015 Murat Gunay et al. All rights reserved. Gingival Bleeding of a High-Flow Mandibular Arteriovenous Malformation in a Child with 8-Year Follow-Up Tue, 17 Mar 2015 09:07:29 +0000 Intraosseous arteriovenous malformations (AVMs) in the head and neck region are uncommon. There are several types and they can have a wide range of clinical presentations. Depending on the blood flow through the AVM, the treatment may be challenging for the attending team and may lead to life-threatening hemorrhages. A clinical case report is presented. A 9-year-old girl, seen for gingival bleeding during oral hygiene, was found to have a high-flow AVM located within and around the mandible. Two-stage treatment consisted of intra-arterial embolization followed by intraoral injection of a sclerosing agent 8 weeks later. At the 8-year follow-up, imaging study showed no evidence of recurrent lesion inside or outside the bone. The final outcome is a correct occlusion with a symmetric facial result. This case shows that conservative treatment may be the first treatment option mostly in children. Arteriography and transcortical injection were enough to control the AVM. Elvira Ferrés-Amat, Jordi Prats-Armengol, Isabel Maura-Solivellas, Eduard Ferrés-Amat, Javier Mareque-Bueno, and Eduard Ferrés-Padró Copyright © 2015 Elvira Ferrés-Amat et al. All rights reserved. Prolonged Ileus in an Infant Presenting with Primary Congenital Hypothyroidism Tue, 17 Mar 2015 08:55:37 +0000 Congenital hypothyroidism (CH) is the most common endocrine disorder affecting the newborn. Universal newborn screening (NBS) has virtually eliminated the static encephalopathy and devastating neurodevelopmental syndrome known as cretinism. This report describes the presentation of an infant referred by the primary pediatrician to our hospital at 12 days of age for confirmatory testing after the NBS was consistent with CH. The infant had hypoglycemia secondary to lethargy and poor feeding and required transfer to the neonatal intensive care unit for worsening abdominal distension despite normalization of serum thyroid function tests following hormone replacement. In particular, the recalcitrant ileus and secondary bowel obstruction resulted in an additional diagnostic workup and lengthened hospital day. Our report highlights the acute gastrointestinal consequences of hypothyroidism despite evidence of effective treatment. We believe that the preclinical detection and immediate therapy for CH have lessened the prevalence of this presentation in general practice, and hence practitioners are less likely to be familiar with its natural history and management. Caroline Chua, Shilpa Gurnurkar, Yahdira Rodriguez-Prado, and Victoria Niklas Copyright © 2015 Caroline Chua et al. All rights reserved. Therapeutic Hypothermia and Out-of-Hospital Cardiac Arrest in a Child with Hypertrophic Obstructive Cardiomyopathy Mon, 16 Mar 2015 14:10:09 +0000 Neurologic outcomes following pediatric cardiac arrest are consistently poor. Early initiation of cardiopulmonary resuscitation has been shown to have positive effects on both survival to hospital discharge, and improved neurological outcomes after cardiac arrest. Additionally, the use of therapeutic hypothermia may improve survival in pediatric cardiac arrest patients admitted to the intensive care unit. We report a child with congenital hypertrophic obstructive cardiomyopathy and an out-of-hospital cardiac arrest, in whom the early initiation of effective prolonged cardiopulmonary resuscitation and subsequent administration of therapeutic hypothermia contributed to a positive outcome with no gross neurologic sequelae. Continuing efforts should be made to promote and employ high-quality cardiopulmonary resuscitation, which likely contributed to the positive outcome of this case. Further research will be necessary to develop and solidify national guidelines for the implementation of therapeutic hypothermia in selected subpopulations of children with OHCA. Nancy Spurkeland, Gregory Bennett, Chandran Alexander, Dennis Chang, and Gary Ceneviva Copyright © 2015 Nancy Spurkeland et al. All rights reserved. Costello Syndrome with Severe Nodulocystic Acne: Unexpected Significant Improvement of Acanthosis Nigricans after Oral Isotretinoin Treatment Sat, 28 Feb 2015 12:58:43 +0000 We report the case of 17-year-old female diagnosed with Costello syndrome. Genetic testing provided a proof with G12S mutation in the HRAS gene since 3 years of age with a presentation of severe nodulocystic acne on her face. After 2 months of oral isotretinoin treatment, improvement in her acne was observed. Interestingly, an unexpected significant improvement of acanthosis nigricans on her neck and dorsum of her hands was found as well. We present this case as a successful treatment option by using oral isotretinoin for the treatment of acanthosis nigricans in Costello syndrome patients. Leelawadee Sriboonnark, Harleen Arora, Leyre Falto-Aizpurua, Sonal Choudhary, and Elizabeth Alvarez Connelly Copyright © 2015 Leelawadee Sriboonnark et al. All rights reserved. Pulmonary Epithelioid Hemangioendothelioma in a Patient with Crohn’s Disease Sat, 28 Feb 2015 11:09:02 +0000 Pulmonary epithelioid hemangioendothelioma (PEH) is a rare neoplasm, largely unresponsive to chemotherapeutic medications, and with varied prognosis. Imaging on computerized tomography may demonstrate perivascular nodules, but diagnosis is ultimately made on biopsy with immunohistochemical analysis. Here we describe a case of PEH in a 14-year-old male with Crohn’s disease, which, to our knowledge, has not previously been described in the literature. Nanda Ramchandar and Henry A. Wojtczak Copyright © 2015 Nanda Ramchandar and Henry A. Wojtczak. All rights reserved. Corrigendum to “Gitelman Syndrome in a School Boy Who Presented with Generalized Convulsion and Had a R642H/R642W Mutation in the SLC12A3 Gene” Sat, 28 Feb 2015 07:14:33 +0000 Shigeru Makino, Toshihiro Tajima, Jun Shinozuka, Aki Ikumi, Hitoshi Awaguni, Shin-ichiro Tanaka, Rikken Maruyama, and Shinsaku Imashuku Copyright © 2015 Shigeru Makino et al. All rights reserved. Systemic Steroid Treatment for Severe Expanding Pneumococcal Pneumonia Thu, 26 Feb 2015 09:17:24 +0000 The treatment of bacterial community-acquired pneumonia (CAP) is based on appropriate antibiotic therapy and supportive care such as intravenous fluids and supplemental oxygen. There is no available data regarding the use of steroids in CAP in children. We present an unusual case of a child with severe respiratory distress, on the brink of mechanical ventilation, due to a rapidly expanding pneumococcal pneumonia. The administration of systemic steroids resulted in a dramatic response with rapid improvement of clinical and radiological abnormalities followed by improvement of laboratory abnormalities. This case report should raise the awareness of the potential benefits of steroids in the treatment of severe pneumonia in children. Prospective randomized trials are needed to confirm the efficacy of steroids in this setting and to determine which patients would benefit most from this. Eran Lavi, David Shoseyov, Natalia Simanovsky, and Rebecca Brooks Copyright © 2015 Eran Lavi et al. All rights reserved. Subacute Sclerosing Panencephalitis in a Child with Recurrent Febrile Seizures Tue, 24 Feb 2015 08:15:12 +0000 Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis. Ayşe Kartal, Ayşegül Neşe Çıtak Kurt, Tuğba Hirfanoğlu, Kürşad Aydın, and Ayşe Serdaroğlu Copyright © 2015 Ayşe Kartal et al. All rights reserved. Successful Treatment with Mycophenolate Mofetil and Tacrolimus in Juvenile Severe Lupus Nephritis Tue, 17 Feb 2015 12:39:54 +0000 Lupus nephritis (LN) of juvenile onset often has severe disease presentation. Despite aggressive induction therapy, up to 20% of patients with LN are resistant to initial therapy and up to 44% suffer a renal relapse. However, there is no consensus on an appropriate therapeutic regimen for refractory LN. We report a 13-year-old girl with recurrent LN who was not taking her medications. At age of 11 years, she was diagnosed with LN classified as International Society of Nephrology/Renal Pathology Society (ISN/RPS) class IV G (A) + V. She was treated with prednisolone and MMF after nine methylprednisolone pulses. Nineteen months later, she was admitted to the hospital with generalized edema. Her symptoms were nephrotic syndrome and acute renal dysfunction. She received three methylprednisolone pulses for 3 days, followed by oral prednisolone and MMF. Twenty-seven days after the three methylprednisolone pulses, her acute renal dysfunction was improved, but the nephrotic syndrome was not improved. A second biopsy showed diffuse lupus nephritis classified as the predominant finding of ISN/RPS class V. We added tacrolimus to the MMF. Four months after adding tacrolimus, the nephrotic syndrome improved. We conclude that adding tacrolimus to the treatment regimen for LN resistant to MMF is effective. Tomoo Kise, Hiroshi Yoshimura, Shigeru Fukuyama, and Masatsugu Uehara Copyright © 2015 Tomoo Kise et al. All rights reserved. Multicentric Castleman’s Disease in a Child Revealed by Chronic Diarrhea Mon, 09 Feb 2015 06:29:31 +0000 Multicentric Castleman’s disease is a rare benign and unexplained lymphoproliferative disorder that is extremely uncommon in children. It presents with fever, systemic symptoms, generalized lymphadenopathy, and laboratory markers of inflammation. Its treatment is not standardized and its prognosis is poor. We report a novel case of multicentric Castleman’s disease in a 13-year-old girl who had presented with chronic diarrhea as the only initial presenting symptom. The diagnosis of celiac or inflammatory bowel diseases was suspected, but two and a half years later, the diagnosis of multicentric Castleman’s disease was brought following the appearance of abdominal mass whose biopsy revealed Castleman’s disease in the plasma cell form. The outcome was favorable after treatment by corticosteroid, chemotherapy, and surgery. The occurrence of diarrhea as the initial symptom of multicentric Castleman’s disease without lymph node involvement is very rare. This case report underlines the diagnostic difficulties and the long interval between onset and diagnosis when diarrhea occurs first. Sarra Benmiloud, Sana Chaouki, Samir Atmani, and Moustapha Hida Copyright © 2015 Sarra Benmiloud et al. All rights reserved. Congenital Portosystemic Shunt: Our Experience Mon, 02 Feb 2015 09:15:36 +0000 Introduction. Congenital portosystemic venous malformations are rare abnormalities in which the portal blood drains into a systemic vein and which are characterized by extreme clinical variability. Case Presentations. The authors present two case reports of a congenital extrahepatic portosystemic shunt (Type II). In the first patient, apparently nonspecific symptoms, such as headache and fatigue, proved to be secondary to hypoglycemic episodes related to the presence of a portosystemic shunt, later confirmed on imaging. During portal vein angiography, endovascular embolization of the portocaval fistula achieved occlusion of the anomalous venous tract. In the second patient, affected by Down’s syndrome, the diagnosis of a portosystemic malformation was made by routine ultrasonography, performed to rule out concurrent congenital anomalies. Because of the absence of symptoms, we chose to observe this patient. Conclusions. These two case reports demonstrate the clinical heterogeneity of this malformation and the need for a multidisciplinary approach. As part of a proper workup, clinical evaluation must always be followed by radiographic diagnosis. Tiziana Timpanaro, Stefano Passanisi, Alessandra Sauna, Claudia Trombatore, Monica Pennisi, Giuseppe Petrillo, Pierluigi Smilari, and Filippo Greco Copyright © 2015 Tiziana Timpanaro et al. All rights reserved. Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge Thu, 29 Jan 2015 08:52:04 +0000 Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-month-old female child with failure to thrive and recurrent respiratory tract infections since 8 months of age. Physical examination was normal except for pulmonary auscultation with bilateral crackles and wheezes. Laboratory tests revealed elevated erythrocyte sedimentation rate, and elevated serum IgG and IgG4 with polyclonal hypergammaglobulinemia. Thoracic CT and MRI showed multiple mediastinal lymphadenopathies and a nodular posterior mediastinal mass in right paratracheal location with bronchial compression. Initial fine needle aspiration biopsy was compatible with reactive lymphadenopathy but after clinical worsening a thoracoscopic partial resection of the mass was performed and tissue biopsy revealed lymphoplasmacytic infiltrate and increased number of IgG4-positive plasma cells and a ratio of IgG4/IgG positive cells above 40%. Glucocorticoids therapy was started with symptomatic improvement, reduction in the size of the mass, and decrease of serum IgG4 levels after 6 weeks. There are very few reports of IgG4-RD in children. Long-term follow-up is necessary to monitor relapses and additional organ involvement. Susana Corujeira, Catarina Ferraz, Teresa Nunes, Elsa Fonseca, and Luísa Guedes Vaz Copyright © 2015 Susana Corujeira et al. All rights reserved. Separation of the Proximal Humeral Epiphysis in the Newborn: Rapid Diagnosis with Ultrasonography Wed, 28 Jan 2015 11:49:06 +0000 Separation of the proximal humeral epiphysis (SPHE) is a well-known occurrence and may occur secondary to trauma, infection, and nonaccidental trauma. Since most newborns do not have the proximal humeral epiphysis ossified at birth, the diagnosis may be difficult to make on routine radiographs. Ultrasonography of the shoulder in the newborn is rapid, noninvasive, and nonionizing imaging techniques which can diagnose SPHE. In this report, we describe and emphasize the diagnostic utility of state-of-the-art ultrasonography for the diagnosis of SPHE. Rachelle Goldfisher and John Amodio Copyright © 2015 Rachelle Goldfisher and John Amodio. All rights reserved. A Case of Battery Ingestion in a Pediatric Patient: What Is Its Importance? Tue, 27 Jan 2015 11:57:52 +0000 This is a case of a two-year-old boy who has been suffering from food regurgitation and frequent vomiting over the past seven months which were progressively worsening with time. He was initially diagnosed with gastroesophageal reflux disease and treated accordingly but responded only minimally. Investigations and interventional procedures including a chest X-ray showed a metallic round object in the upper esophagus consistent with a button battery which was removed via a thoracotomy after an esophagoscopy was not successful. This child would not have developed such serious complications and would not have required major surgery had the foreign body been identified and removed early on. Elie Alam, Marc Mourad, Samir Akel, and Usamah Hadi Copyright © 2015 Elie Alam et al. All rights reserved. Bilateral Symmetrical Herpes Zoster in an Immunocompetent 15-Year-Old Adolescent Boy Tue, 27 Jan 2015 08:31:45 +0000 Herpes zoster is uncommon in immunocompetent children. The bilateral symmetrical occurrence of herpes zoster lesions is extremely rare. We report a 15-year-old immunocompetent Chinese adolescent boy who developed bilateral symmetrical herpes zoster lesions. To our knowledge, the occurrence of bilateral symmetrical herpes zoster lesions in an immunocompetent individual has not been reported in the pediatric literature. Alexander K. C. Leung and Benjamin Barankin Copyright © 2015 Alexander K. C. Leung and Benjamin Barankin. All rights reserved.