An 11-year-old girl with reflex sympathetic dystrophy successfully treated by thoracoscopic sympathectomy

Abstract We report on an 11-year-old girl with reflex sympathetic dystrophy (RSD) complaining of severe pain in her right upper extremity. Oral administration of narcotics or non-steroid anti-inflammatory drugs gave no relief in pain. Thoracoscopic electrocauterization of the thoracic sympathetic ganglion at the level of T3 was performed 3 months after the start of symptoms, and brought complete resolution of pain.     

Second and third malignant solid tumor in a girl with ovarian Sertoli-Leydig tumor

We report a Sertoli-Leydig cell (SLC) tumor of the right ovary in a 10-year-old girl, which was dealt with surgical removal. Three months after resection, she presented with a new episode of acute abdomen because of an abdominal mass, which histologically was compatible with an undifferentiated embryonal rhabdomyosarcoma. Chemotherapy, according to SIOP-??? 89 protocol, was administered additionally to radiotherapy (3,960 cGy). Three years after completing treatment, the patient developed a painful swelling at her left upper arm. The diagnosis was Ewing sarcoma of the humerus, which was confirmed by identification of the typical 11; 22 translocation on cytogenetic and molecular analysis of the tumor tissue. The patient died 14 months from Ewing diagnosis due to progressive disease.     

Reflex sympathetic dystrophy in children: treatment with transcutaneous electric nerve stimulation

During the past 6 years, ten children with reflex sympathetic dystrophy were treated. Pain in an extremity was the initial complaint in all patients. The pain was unilateral in 90% of the patients; upper and lower extremities were affected with equal frequency. Tenderness to palpation, extreme hyperesthesia, and dysesthesia were other dominant features. All patients had some evidence of autonomic nervous system dysfunction in the affected extremity (swelling, color change, decreased temperature, and/or hyperhidrosis). The median duration of symptoms prior to referral and diagnosis was 5 months. All children were treated as outpatients with a transcutaneous electric nerve stimulator and home-based physical therapy. With this regimen, seven patients had complete remission within 2 months. Two other patients improved with transcutaneous electric nerve stimulation therapy, and one patient had no response to transcutaneous electric nerve stimulation. Reflex sympathetic dystrophy is frequently underdiagnosed in children. Increased awareness of this syndrome is important because accurate diagnosis is crucial and transcutaneous electric nerve stimulation offers a safe, simple, and effective outpatient therapy for reflex sympathetic dystrophy in children.     

Pseudo-tumoral osteomyelitis. Immunologic study and etiopathogenic approach. Apropos of 2 cases]

Two cases of pseudo-tumoral osteomyelitis are reported. The first concerns a 12 year-old boy who presented with pain of the left knee during 8 months and, later on, with swelling of the upper extremity of the left leg, without fever or local inflammatory signs. The radiological aspect of condensation with a filling defect and "chimney" crossing the cartilage led to osteotomy. Local bacteriological samplings were normal. The second case concerns a 11 year-old boy who, after having complained from pain of the right wrist during 2 weeks, presented with swelling and on X-ray films a picture of metaphyso-epiphyseal lysis and an aspect of sequestrum in its center. There was no biological sign of inflammation. Evolution was favorable after antibiotic treatment and immobilization. In both cases, an immunological study showed an activation syndrome of the immune system with increased serum IL-1, IL-2 receptors and class II antigen receptors on the surface of T cells, suggesting a previous immunization of both children towards staphylococcus and, thereby, the subacute nature of evolution.     

Case of adolescent with Paget-Schroetter syndrome and underlying thrombophilia due to an elevated lipoprotein (A)

Upper extremity deep vein thrombosis (UEDVT) is a rare disorder in children and is most often associated with a central venous catheter (CVC), cancer, or an underlying thrombophilia. In adults, repetitive or strenuous upper extremity activity has been linked to mechanical compression of the thoracic outlet resulting in subclavian vein thrombosis, also known as Paget-Schroetter Syndrome (PSS). We present the case of a 14 year old female who was found to have an UEDVT consistent with PSS. On subsequent thrombophila screening, she was noted to have a significantly elevated lipoprotein (a) level of 83 (normal: <30 mg/dL). She underwent 3 months of anticoagulation therapy prior to her first rib resection and in follow-up was noted to have complete patency of her subclavian vein. This case illustrates the necessity for thrombophilia screening in the pediatric patient with UEDVT despite evidence of anatomic abnormality or mechanical venous compression.     

Proteus syndrome

Abstract: This female Asian (Malay) baby had clinical features of Proteus syndrome. She had a large right facial lipolymphangioma with hyperpigmentation of the overlying skin. There was a smaller lymphangioma over the left side of her neck with excess nuchal folds, macrodactyly and bilateral talipes equinovarus. Despite the extensive hemifacial swelling, there was no evidence of upper respiratory tract obstruction. Generalized seizures developed on the sixth day of life which were controlled with phenobarbital. The lymphangiomas were excised without recurrence.     

Treatment of superior vena cava syndrome with recombinant tissue plasminogen activator in a sickle cell patient undergoing bone marrow transplantation

Superior vena cava (SVC) syndrome is a well-recognized clinical entity seen with mediastinal malignancies and intraluminal venous thrombosis. The role of recombinant tissue plasminogen activator (rt-PA) in the resolution of SVC syndrome caused by thrombosis in the bone marrow transplant settings has not been described. The authors report a case of SVC syndrome with good clinical response in a 16-year-old female with sickle cell disease undergoing an allogeneic bone marrow transplant (BMT) from her HLA identical sibling. Shortly after her transplant, she was found to have significant facial edema and swelling above the neck. Concomitantly, her renal function deteriorated with progressive elevation of serum urea nitrogen and creatinine levels, requiring the use of continuous veno-venous hemofiltration. An upper extremity venogram showed complete SVC obstruction (type III) with apparent inferior reflux into the azygos system. rt-PA was started at a dose of (0.5 mg/kg/day) for 2 days. There was a dramatic resolution of her symptoms, including significant improvement in renal function with increase in urine output. A repeat venogram showed free flow from the distal tip of the central line consistent with a patent superior vena cava. There was no evidence of any bleeding manifestations with rt-PA. This report highlights the usefulness of rt-PA as a treatment modality for SVC syndrome in the BMT settings.     

TREATMENT OF SUPERIOR VENA CAVA SYNDROME WITH RECOMBINANT TISSUE PLASMINOGEN ACTIVATOR IN A SICKLE CELL PATIENT UNDERGOING BONE MARROW TRANSPLANTATION

Superior vena cava (SVC) syndrome is a well-recognized clinical entity seen with mediastinal malignancies and intraluminal venous thrombosis. The role of recombinant tissue plasminogen activator (rt-PA) in the resolution of SVC syndrome caused by thrombosis in the bone marrow transplant settings has not been described. The authors report a case of SVC syndrome with good clinical response in a 16-year-old female with sickle cell disease undergoing an allogeneic bone marrow transplant (BMT) from her HLA identical sibling. Shortly after her transplant, she was found to have significant facial edema and swelling above the neck. Concomitantly, her renal function deteriorated with progressive elevation of serum urea nitrogen and creatinine levels, requiring the use of continuous veno-venous hemofiltration. An upper extremity venogram showed complete SVC obstruction (type III) with apparent inferior reflux into the azygos system. rt-PA was started at a dose of (0.5 mg/kg/day) for 2 days. There was a dramatic resolution of her symptoms, including significant improvement in renal function with increase in urine output. A repeat venogram showed free flow from the distal tip of the central line consistent with a patent superior vena cava. There was no evidence of any bleeding manifestations with rt-PA. This report highlights the usefulness of rt-PA as a treatment modality for SVC syndrome in the BMT settings.     

A lateral cervical lipomyelomeningocele associated with diplomyelia

We report a case of a neonate with complex spinal dysraphism and associated anomalies of the axial skeleton. Based on the clinical presentation and radiographic findings, and recent advances in the understanding of human embryogenesis, we formulate a hypothesis that such a presentation is the result of failure of normal gastrulation. A 1-day-old male neonate presented with multiple right-sided anomalies, including hypoplastic right face and decreased movement of the right upper extremity. Radiographic studies demonstrated absent right cervical hemivertebrae, right cervical lipomyelomeningocele, and cervical diplomyelia with right hemicord terminating in a blind pouch. Anterior and posterior cervical and thoracic fusion with instrumentation was performed at the age of 3 years, and on follow-up the patient had improvement in right upper extremity strength. Complex spinal dysraphism is a pathological process that occurs during different stages in human development. We describe a case involving a rare lateral lipomyelomeningocele in the cervical-thoracic area.     

Post-thrombotic syndrome is uncommon in childhood cancer survivors

Deep vein thrombosis occurs in up to 50% of children with tunneled central venous catheters (CVCs). CVC-related deep vein thrombosis involving the upper extremity is usually asymptomatic but can result in post-thrombotic syndrome (swelling, pain, skin changes, and functional impairment). In a cohort of childhood cancer survivors evaluated clinically a mean of 7.5 +/- 2.8 years after completion of therapy who previously had CVCs in place for a median 15.5 months, none of 50 patients (95% CI = 0% to 6%) had these features diagnostic of post-thrombotic syndrome. Five patients had arm circumference 3% to 5% greater ipsilateral to the prior CVC.     

Cutis marmorata telangiectatica congenita: an unusual cause of lower extremity hypoplasia.

Cutis marmorata telangiectatica congenita is a relatively benign, rare cutaneous disorder with various manifestations. A seven-year-old girl, who presented with extremity hypoplasia, had the characteristic reticular, patchy, blue-pink cutaneous lesions, which became more prominent with exposure to cold temperatures. She had 4.8 cm shortening of her right lower extremity, which was also thinner than on the left side. The patient did not have skin atrophy, ulcers, glaucoma or macrocephaly. She is being followed for a future extremity lengthening procedure.     

Prediction rules for selective radiographic assessment of extremity injuries in children and adolescents

To assess the potential for selective use of roentgenography in evaluating extremity injuries, prediction rules were developed based on prospective observations for 617 injured children and adolescents examined in our Emergency Department (phase 1) and tested on 601 examined 1 year later (phase 2). Logit analysis produced best-fitting statistical models for phase 1 data with significant (P less than 0.05) direct effects of gross signs, point tenderness, activity not routine, swelling moderate or severe, time from injury less than 6 hours, and pain with motion for upper extremity injuries; and, for lower extremity injuries, not knee injury, activity not routine, point tenderness, and foot injury. Prediction rules developed in phase 1 performed equally well when tested on phase 2 injuries. Data from both phases were combined, therefore, in analysis that produced risk estimates. For all injury types (ie, for injuries with all possible combinations of presence or absence of these findings), risk for fracture was derived. For upper extremity injuries, with a threshold risk for fracture of 20% used to select specific injury types for roentgenography, prediction rule outcomes were 18.1% of roentgenograms avoided and 5.3% of fractures missed. For lower extremity injuries, using a threshold risk of 10% to select injury types for roentgenography, outcomes were 25.8% of roentgenograms avoided and 5.3% of fractures missed. Alternative prediction rules allowed still greater roentgenogram avoidance, although missed fractures also increased. Risk of adverse functional outcome from missed fractures appeared small. Annual national cost savings from the elimination of 18.1% of upper and 25.8% of lower extremity roentgenographic evaluations was estimated at $103 million.     

Cheilitis granulomatosa in a child]

Granulomatous cheilitis is a rare disorder characterized by intermittent swelling of one or both lips, which may become persistent. We report a case occurring in a child, which improved with minocycline treatment. CASE REPORT: An eight year-old girl was seen for the swelling of her upper lip, which persisted for more than one year. There was no history of applied irritants, local trauma or atopy. On examination, the upper lip was swollen, indurated, erythematous and fissured with a bilateral perleche. The tongue and gums were normal. There was no facial palsy. The girl was in good health and did not take any drugs. Blood investigations were normal. Patch tests were negative. Antibiotic treatment or local steroids provided only temporary improvement. A biopsy showed extravascular epithelioid and gigantocellular non-caseating granulomas with lymphoid infiltrates. There was no sign of sarcoidosis or Crohn's disease. Treatment with hydroxychloroquine for three months, then with oral metronidazole, did not improve the symptoms. Minocycline (100 mg/d) was effective. CONCLUSION: Granulomatous cheilitis should be considered in children as well and differentiated from infectious, allergic or factitious dermatoses. Granulomatous cheilitis can be associated with Crohn's disease or sarcoidosis, and could precede these disorders from several months or years, thus requiring a long-term follow-up.     

Retroperitoneal lipoblastoma involving the right common iliac artery and vein.

A case of benign lipoblastoma of the retroperitoneum in a 12-month-old boy, presenting as a huge abdominal mass and right lower extremity swelling, was reported. Surgery revealed involvement of the right common iliac artery and vein by lipoblastoma, requiring the sacrifice of both vessels for complete removal of the tumour. Vascular continuity of both vessels was re-established by a saphenous vein graft interposition. Involvement of a great artery by benign lipoblastoma has not been reported before.     

Benign monomelic amyotrophy in a 7-year-old girl with proximal upper limb involvement: case report

Monomelic amyotrophy (MMA) is a benign motor neuron disease characterized by neurogenic amyotrophy, which usually affects one of the upper or lower extremities. Progression is slow and symptoms are clinically stable. Symptoms are seen in the second or third decades of life. In this study, we present a seven-year-old girl who was diagnosed and directed to the Physiotherapy Department at the age of 5 years and had unilateral proximal upper limb involvement. Family history of the case was recorded. Neurologic evaluation was performed. Range of joint motion, muscle shortness and strength, posture, extremity lengths, gait, timed performance, arm function, and motor and mental maturation were assessed. The physiotherapy program was designed progressively as strengthening and resistive exercises. Motor and mental developmental milestones were normal. There was no limitation in active or passive motion of all joints. She had more flexible joints, scapula alata, asymmetry between shoulder levels, and weakness on proximal muscles of the right upper extremity. In the follow-up assessment at eight months, there was no asymmetry between shoulder levels and scapular symmetry began to improve. Female gender and involvement restricted to one proximal upper limb are rare in the literature. This patient demonstrates the positive effects of physical therapy with early diagnosis of MMA. The rapid recovery of muscle weakness shows the importance of strengthening and resistive exercises applied to specific muscles in the treatment.     

Concurrent Langerhans cell histiocytosis and nephroblastoma

Both Langerhans cell histiocytosis (LCH) and nephroblastoma are rare in children. We report herein the first case of a patient with both diseases concurrently. A 2‐year‐old female presented with bone pain and swelling of the right humerus. As a result of the local incision biopsy, she was diagnosed as LCH. A nephroblastoma of the left kidney was discovered during her staging work‐up. After complete resection of the nephroblastoma, she received standard chemoradiotherapy for nephroblastoma. She is alive without relapse 14 months after initial presentation. Pediatr Blood Cancer 2009;52:662–664. © 2009 Wiley‐Liss, Inc.     

Increasing the accuracy of lung perfusion scintigraphy in children with bidirectional Glenn circulation

Background

In children who have undergone a bidirectional Glenn procedure without antegrade or additional pulmonary blood flow, we have often noted a discrepancy between apparent lung perfusion on scintigraphy and superior vena cava angiography when evaluating right and left pulmonary blood flow. We found a tendency for radionuclide, tracer 99mTc-MAA, when administered through a single upper extremity vein, to preferentially accumulate in the ipsilateral lung.

Objective

In the present study, we examined whether the ratio of right-to-left pulmonary flow varied when 99mTc-MAA was administered via either the right upper or the left upper extremity vein.

Materials and methods

We studied six children (median age 1.3?±?0.23 years) who underwent a bidirectional Glenn before total cavopulmonary connection. Five children who underwent biventricular repair served as a control. Perfusion scintigraphy using 99mTc-labeled macroaggregated albumin (99mTc-MAA) was performed in all children. First, we injected radionuclide via the right upper extremity and calculated the pulmonary accumulation in both lungs (R-image). Second, we injected the same dose of radionuclide via the left upper extremity and calculated the pulmonary accumulation (B-image), which represented the resulting administration via both upper extremities. The lung accumulation that resulted from radionuclide administration via the left upper extremity (L-image) was determined by subtracting the R-image from the B-image. We evaluated the right-to-total pulmonary blood flow ratio (radionuclide accumulation in right lung / radionuclide accumulation in both lungs) in the R-, L- and B-images.

Results

The right-to-total pulmonary blood flow ratios in the R-, L- and B-images were 815?±?15.3%, 39.8?±?11.7% and 61.3?±?11.8%, respectively, and there were significant differences among the three images (P?<?0.01). On the other hand, in the control group, the right-to-total pulmonary blood flow ratios in the R-, L- and B-images were 59.3?±?22.4%, 57.8?±?26.4% and 58.8?±?23.7%, respectively, and there was no significant difference.

Conclusion

In children with bidirectional Glenn circulation without antegrade or additional pulmonary blood flow, the venous blood of each arm tends to flow into the ipsilateral lung. The administration of radionuclide via both arms is important for accurate evaluation of lung perfusion scintigraphy in children who have undergone a bidirectional Glenn procedure.     

Dermatophyte infection of the eyelid in a neonate

A 25-day-old neonate had redness and swelling on her left upper eyelid. Examination revealed a 16 x 10-mm yellow, crusted, ring-like rash on the left upper eyelid. Microscopic examination of a skin scraping on 10% potassium hydroxide revealed Microsporium canis. The patient was treated successfully with topical miconazole.     

Septic pulmonary embolism in a child

Septic pulmonary embolism (PE) is a diagnosis seldom considered in children. A 15 year old girl presented with fever, extremity swelling and pain for 5 days. Chest radiograph revealed a large, round density in the right lung and consolidation areas in peripheral portions of both lungs. Computed tomography showed bilateral multiple round and cavitary nodules in peripheral portions of both lungs, pleural effusion in the right lung and hypodense round lesion (8 cm by 4 cm) in the superior segment of right lower lobe, characteristic of PE. Cefotaxime and clindamycin were administered. By the 5th hour, the patient passed away. Staphylococcus aureus was isolated from blood, pleural fluid and tracheal aspirate cultures.     

Endobronchial tuberculosis complicated with Staphylococcus aureus pneumonia and empyema in a child

Childhood tuberculosis might have unusual clinical presentation. A seven-year-old female patient was admitted with fever and pleural effusion. Her pneumonia resolved following 21-day treatment period. An atelectatic appearance remained on the right middle zone in her chest X-ray. Tuberculin skin test showed 13 mm induration. Triple drug antituberculosis treatment was started. Since atelectasis persisted on her follow-up radiograph one month later, bronchoscopy was performed which revealed a hemorrhagic polypoid mass occluding the right upper lobe anterior segment orifice. Surgical removal was performed by right upper lobectomy. The pathological diagnosis was necrotizing granulomatous infection suggesting tuberculosis. The patient has been well on follow-up after completing a nine-month course of antituberculous treatment.