Diastematomyelia in congenital scoliosis: a report of two cases.

INTRODUCTION: Even if diastematomyelia is a rare condition, it always has to be identified in case of diagnosing and treating a case of congenital scoliosis. The consequence of missing the diagnosis of such a malformation may be devastating to the involved patient. This paper wants once again to make aware the physicians of the eventual presence of a spinal dysraphic malformation when dealing with a congenital spinal malformation. METHODS: The presence of diastematomyelia was noticed in two of our cases, one with congenital scoliosis and another with congenital kyphoscoliosis. First of all in these cases, we performed the resection of the bony septum, followed by spinal fusion in a single-stage surgical procedure. We noticed no complications during and after surgery related to the resection of the bony septum. RESULTS: In both patients, we obtained partial correction and stabilization of the congenital spinal malformation after a safe excision of the bony septum. CONCLUSIONS: Diastematomyelia is a rare condition. It has to be taken into consideration when dealing with a congenital scoliosis. The first step in the surgical procedure has to be the resection of the diastematomyelic septum. In case of a scoliosis ranging up to 30° and not presenting a progressive potential, the expectative-evaluation attitude is a correct one.

An unusual case of congenital scoliosis associated with rib agenesis in the upper part of the concavity treated by VEPTR vertebra to vertebra.

BACKGROUND CONTEXT: Rib agenesis in congenital scoliosis is rarely encountered, and its disposal in the application area of the proximal vertical expandable prosthetic titanium rib (VEPTR) module is a challenge to the orthopedic surgeon. PURPOSE: To present a case in which known treatment methods in early-onset scoliosis were not possible to apply. STUDY DESIGN: Case report. METHODS: A patient aged 1 year and 10 months, presenting a congenital scoliosis with the following characteristics: left T3 hemisegmented hemivertebra, T5-T6-T7 hemivertebral segment, T9, T10 trapezoidal vertebrae, right side I-IV rib agenesis with T1-T2-T4 hemivertebral hypoplasia (T3 agenesis) and bilateral XIIth rib agenesis, and V-VI and VII-VIII-IX fused ribs on the right side. We applied a standard VEPTR in a new construct, vertebra to vertebra. RESULTS: The VEPTR vertebra to vertebra proved to be an efficient and stabile construct after 1.5 years of follow-up and three device distractions in a row. The curve corrected from 100 to 58 Cobb degrees. CONCLUSIONS: We believe that the vertebra-to-vertebra construct with eventual modifications may be a solution in the treatment of early-onset scoliosis needing surgery, which associate rib agenesis in the area where the proximal module has to be applied.

Upper and lower limb length equalization: diagnosis, limb lengthening and curtailment, epiphysiodesis.

INTRODUCTION: This article contains notions and guidelines derived from the current therapeutic approach used in the Pediatric Orthopedics Clinic of "M.S. Curie" Hospital, Bucharest. The purpose of this work is to illustrate the clinic's experience theoretically and by means of images. METHODS: This article is based on 25 years of clinical experience (1986-2011), gained at Mangalia Regional Hospital and the Pediatric Orthopedics Clinic of "M.S. Curie" Hospital in Bucharest, having treated more than 250 such cases. In post-traumatic shortenings with associated soft tissue injuries we used double leveled corticotomies and lengthening. The lengthening rarely exceeded 10 cm, especially for congenital deformities and repeated lengthening. From 2001 onwards, all lengthening operations included the intramedullary implantation of one or two TEN rods, with the purpose of reducing the time to fixator removal and to shrink the healing index. In the last five years we frequently used minimally invasive osteotomies after the placement of TEN implants, achieving the separation of a small fragment that sites itself between the major fragments. The lengthening rate was 1 mm per day, broken down in four steps (0.25 mm every 6 hours). For difficult cases, such as congenital pseudarthroses or the presence of scar tissue around the osteotomy site, we recommended 0.75 mm of lengthening per day (0.25 mm every 8 hours). For congenital pseudarthroses we used controlled epiphysiolysis and bone transport. For inequalities ranging 3 to 5 cm we used temporary epiphysiodesis, initially with staples, and subsequently with "8", "H" and "I" plates. Limb shortening followed by locked intramedullary fixation was reserved for those patients who did not follow through with the evaluation program and who could not benefit from temporary epiphysiodesis. RESULTS: The amount of lengthening per segment varied between 3 and 17 cm. The longest staged lengthening measured 20 cm, in two stages, and the greatest overall lengthening was 25 cm for an entire lower limb. An appropriate stabilization, followed by the adequate choice of osteotomy site and the postoperative weight loading of the limb ensured a quick and qualitative healing process. COMPLICATIONS: Less than half of the patients suffered complications, most of them being minor ones. Pseudarthroses have been treated by compaction of the site, followed by distraction, and/or the injection of BMP (Bone Morphogenic Protein). We saw no complications after epiphysiodesis or limb shortening. CONCLUSIONS: Limb lengthening procedures up to 5 cm lead to rapid consolidation and minimal complications. Lengthenings exceeding 5 cm require a good psychological preparation and careful monitoring. In lengthenings more than 10 cm, a faster rate of consolidation requires a double corticotomy, the use of intramedullary fixation and the immobilization of adjacent joints. In Lobstein's disease, good results can be obtained by the use of an Ilizarov external fixator. Restoring limb length equality by temporary epiphysiodesis, around the age of 10-12, is the least aggressive method and is very effective. Limb shortening by segmental resection should become obsolete.

The therapeutic attitude in distal radial Salter and Harris type I and II fractures in children.

INTRODUCTION: Salter Harris Fractures type, especially type I and II are treated by orthopedic reduction in the emergency room or operating room, under general anesthesia, followed by plaster immobilization. Neglected or incorrectly treated fractures, leading to malunion and radiocarpal subluxations which require surgical procedure. PURPOSE: This paper proposes to evaluate the correctly applied orthopedic treatment and the expose of an original surgical technique in case of neglected and incorrectly treated fractures, leading to mal-unions and impediments in the radiocarpal mobility and aesthetics. MATERIAL AND METHOD: we studied a group of 238 children with Salter Harris fractures type I and II, treated in "M.S. Curie" Emergency Hospital for Children, Bucharest. Out of the studied group, 200 children were treated by orthopedic reduction and immobilization in a plaster device. Malunions present within 38 children due to neglected or mistreated fractures, underwent open reduction with internal osteosynthesis by a technique that avoids violating the growth cartilage. This technique involves making an internal fixation with the radial joint surface in a normal position. RESULTS: Children receiving proper orthopedic reduction and immobilization in plaster device, 200 patients, were cured after 30-45 days of immobilization, depending on age and joint mobility which were within normal range. The 38 children with malunions underwent surgery to rectify the position of the radial joint surface. Postoperative results were good, proper position of the radiocarpal joint were made during the surgical procedure. The intemal fixation is ensured by a transepiphyseal wire and after 30 days of immobilization in a plaster device the patients started the recovery treatment. Radiocarpal joint mobility returned to normal after a variable period of 3 to 6 months, depending on the patient's age. CONCLUSIONS: Salter Harris I and II fractures are absolute indication for orthopedic treatment, in a matter of emergency, preferably in the operating room under general anesthesia. Verification is necessary between the 7th and 14th day after orthopedic reduction, to avoid malunions. Malunited fractures require surgical intervention after a special technique, avoiding damage to the growth cartilage and radial epiphysis.

Malignant bone tumors: therapeutical strategies related to localization.

Modular concept of reconstruction in malignant bone tumors in children and adolescents is trying to solve a complex problem in order to replace a certain bone segment of various sizes or joint, fully adjustable and fully aware ofmorpho-functional features related to the child's age. Given the high frequency of malignant bone tumors in children, occupying the third place in osteoarticular pathology, after injuries and malformations, due to the progress made in terms of knowledge and identifying certain factors (genetical, biological, immunological, etc.) and the increasing life expectancy of these sick children, paediatric orthopedics should offer the possibility of reconstruction of the resected segment. One of the basic concerns in this regard is modular endoprosthetic reconstruction of the resected area, adapted to each case and each bone or osteoarticular segment. Amputation is no longer the only option in the modern treatment in children and adolescent bone malignancies, being often replaced with increasing size piece resection and reconstruction with large massive cortical bone grafts or modular endoprosthetic replacement.

Diagnosis and complications of renovascular hypertension in children: literature data and clinical observations.

INTRODUCTION: Renovascular hypertension in children is a very rare illness. It occurs as a result of the imbalance between hypotensive and hypertensive systems. Renal ischaemia (95% of the cases) and the shortening of hipotensive factors (5% of the cases) are responsible for the production mechanism of renovascular hypertension in children. In order to make an early diagnosis regarding the renovascular hypertension in all children suffering from renovascular illnesses, blood pressure must be taken correctly and repeatedly. MATERIALS AND METHODS: This paper is a case study on 19 children with renovascular hypertension, aged between 2 and 15 years old. Most cases were divided into two groups: subjects aged 4-7 years old and subjects aged 8-12 years old. Each group represents 34.2% of all cases. The diagnosis of renovascular hypertension in those 19 children was established after correctly taking the blood pressure and comparing it with the normal values for each age. Hypertension was diagnosed before knowing its cause in 8 neglected cases. The blood pressure was repeatedly taken in the other 11 children suffering from renovascular illnesses and the diagnosis of hypertension was early established when blood pressure values increased. Previously, blood pressure was normal in these 11 cases. RESULTS: The etiopathogenical diagnosis showed parenchymal diseases in 12 cases--63.1%. Seven patients suffered from renovascular lesions--36.9%. Laboratory exams, radiology, imagistic exams, arteriography and scintigraphy were steps taken in order to establish the etiopathogenical diagnosis. These exams showed the next direct causes of renovascular hypertension: bilateral chronic pyelonephritis in 4 cases--21.4%, hydronephrosis in 3 cases--16.2%, congenital renal hypoplasia in 2 cases--10.4% and doubled kidney in 2 cases--10.4%. The other 8 cases presented acute glomerulonephritis, unilateral renal agenesis, horseshoe kidney, unilateral pyelonephritis, renal artery agenesis, renal trauma, renal abcess and Wilms tumor, one case of each illness--5.2%. The major complications were: retinopathy, chronic renal failure and stroke. CONCLUSIONS: Laboratory data are just a hint in diagnosing renovascular hypertension. However, radiology, imagistic exams, arteriography and scintigraphy are compulsory in the renourinary status and etiopathogenical diagnosis.

[Renovascular hypertension: symptoms and signs. Clinical study on 20 patients]. / Hipertensiunea arteriala de origine renala: simptome si manifestari. Studiu clinic pe 20 pacienti.

OBJECTIVES: symptoms in renovascular hypertension can be wrong interpreted, which leads to a late diagnostic, after discover the determination disease. MATERIALS AND METHODS: the study on the renovascular hypertension was made on 20 patients, aged between 2 and 36 years old, diagnosed with reno-ureteral malformations, pyelonephritis, reno-ureteral reflux and renal trauma as a determination disease, leads to manifestation types that guide the diagnostic: neuro-psychiatric signs, weight loss, renal signs and digestive signs. Beginning from these signs the arterial hypertension was diagnosed and the investigations determined the causes. RESULTS: Periodic postoperative evaluation at 3 months, during a period between 4 months and 7 years, individualised 4 evolutional clinical types: AHT with lumbar pain, AHT with no clinical signs, AHT with ophthalmologic signs and AHT with encephalitis like signs. CONCLUSIONS: symptoms in renovascular hypertension don't have pathognomonic clues and the identified signs, one type or all together, enforce the evaluation or even the monitoring of the arterial tension at least 30 days. If the values exceed the normal, complex investigations will be made in order to determine the specific cause of the AHT.

Osteogenesis imperfecta: diagnosis and treatment.

Osteogenesis imperfecta is a heritable disorder characterized by extremely fragile bones, blue sclerae, dentinogenesis imperfecta, hearing loss, and scoliosis. In 1979, Sillence classified the condition into four types based on genetic and clinical criteria. Three more classifications have subsequently been added. Diagnosis of osteogenesis imperfecta may be done prenatally (in severe cases), clinically, radiographically, or via biochemical or genetic examination. Medical treatment consists of bisphosphonate use, even in patients younger than age 2 years. Surgical treatment consists of internal splinting of long bones. Research is currently being done on the use of smart intramedullary rods (ie, composed of nitinol shape-memory alloy) for correction of bone deformity and on the use of bone marrow transplantation to increase osteoblast density, thereby reducing fracture frequency.

Reno-ureteral diseases inducing hypertension in children.

UNLABELLED: Revealing the cause of renal hypertension is a major objective in medical practice. A series of investigations are required in order to elucidate the primary disease and then the treatment surgical and/or medical. The transitory hypertension of adolescent is not discussed in this paper. Measuring the blood pressure in children is often neglected and the evaluation of the hypertension is performed after the diagnosis of the renovascular disease. AIMS: The classification of renovascular hypertension in children and the statistical evaluation of its etiology in order to reveal the most frequent renovascular disease are a topical work of several authors. A guide of the most frequent causes of hypertension in children edited after the studies of several authors may lead to a complete classification and describe a complexed concept regarding diagnosis, evaluation and treatment of the patients. MATERIALS AND METHODS: This paper displays an analytic study on 19 patients, aged between 2 and 15 years, diagnosed with hypertension and presenting signs and symptoms of hypertension. Four of the 19 patients needed medical treatment, 12, surgical treatment, and for three patients the treatment was mixed. RESULTS: The renal hypertension was determined by aberrant renal arteries in four cases, by pyelonephritis in four cases, by reno-pyeloureteral duplication with congenital megaureter in two cases and by transversal renal rupture, renal agenesis, horse shoe kidney, glomerulonephritis and Wilms tumor, in one case each. In our study, the parenchymal diseases predominated (12 cases of 19-63.1%) over the 7 cases with renovascular lesions--36.9%. Among the parenchymal diseases seven are unilateral, six reno-ureteral malformations and a pyelonephritis, and five are bilateral, four pyelonephritis and a glomerulonephritis.Those seven cases of renovascular lesions include six indirect lesions (three hydronephrosis, one Wilms tumor, one renal abscess, one renal trauma) and one case of congenital vascular anomaly without stenosis (renal agenesis). CONCLUSIONS: All reno-ureteral diseases included in Ursea-Ionescu-Târgoviste classification can induce hypertension but renovascular hypertension does not appear in all cases. The diagnosis of the reno-ureteral diseases inducing hypertension in children permits the treatment of hypertension and reno-ureteral illness. The analytic study of diagnostic and treatment methods of different diseases and authors may select the most efficient methods and orientate towards a new therapeutical concept and/or evaluation system. The treatment of the reno-ureteral diseases inducing hypertension avoids the development of some serious complications: the retinopathy, the chronic renal failure, the cerebrovascular accidents.

Osteoarticular reconstructive surgery in malignant bone tumors: the importance of external fixators.

This paper is a retrospective study on 8 patients admitted and treated in Paediatric Surgery and Orthopaedics Clinic of "M. S. Curie" Hospital Bucharest between 1997 and 2007. The patients with malignant bone tumors (table 1.) were studied by sex, tumor type, location, age at the moment of diagnosis, age at the moment of the last evaluation, type of surgery, external fixator implanted, complications, results and survival period. We also considered for each patient the extent of the tumor to diaphysis, soft tissue involvement, involvement of physis and epiphyseal invasion, articular extent, vessels and nerves invasion, presence of metastases and local skin invasion. The certain diagnosis was based on pathological anatomy exam, because clinical and imagistic data were not decisive in each case. There were studied only those patients who received external fixators, the only method to achieve oncological safe resection and osteoarticular recontruction. We used monoplanar or circular fixators, in adjustable or mixed mountings. The postoperative complications were not fatal. The survival period has been between 6 months and 18 years. Only two patients, who have survived 6 months and respectively 18 months, were not able to return to prior activities. The other six were reinserted in social activities. Nowadays, there is made a great effort to save the affected limbs. The conservative treatment is preferred to the amputation, which is being used in very few cases. The development of reconstructive bone surgery is sustained by the possibility to delineate the tumor by diagnosis based on imaging and by the possibility to use modern preoperative and postoperative chemotherapy and radiotherapy. Limb conservation was possible only in aggressive benign tumors up to 1970. Since then the same treatment was preferred also in malignant bone tumors, because the relapse appeared as frequent as in cases with amputation but the physical and psychological comfort made the patients to accept it readily. The goal of malignant bone tumors treatment is to save the life of the patient, to preserve the affected limb, to maintain the length and function of the limb. Oncologic surgery consists of "en bloc" tumor resection followed by bone reconstruction or modular prosthetic replacement. Modular prosthetic replacement leads to the loss of at least one growing cartilage. The use of radiotherapy in some cases may also affect other growing cartilages, leading to limb length discrepancies.

Symptomatology and exploration methods of pulmonary tumours in children.

The paediatric, thoracic and genetic surgeons are still highly interested in the appearance of primitive and metastasised pulmonary tumours in new-born children and babies. Once the human genome was decoded, the studies for individualising the genes responsible for the appearance of pulmonary malignant tumours or with other localization have been intensified. The diagnosis within the first 15 weeks of pregnancy will allow the parents to make a decision regarding the future evolution. Genetic surgery will eliminate in the future the drama of pregnancy discontinuity by the possibility of replacing the genes responsible with the growth of these tumours. At present, the symptomatology allows detecting the tumour at approximately 9-12 months since its appearance, and Rx/anatomopathological exploration can confirm or deny the diagnosis, offering information on its primitive or metastasised, benign or malignant, operable or non-operable character.

Acute osteomyelitis--special cases with particularities related to specific locations.

In the clinical practice of paediatric orthopaedic surgery, there are a few cases of acute osteomyelitis which are not characterized by general and local infectious syndrome, making the diagnosis difficult and leading to severe complications. The specific locations, seldom affected, are the spine, pubis, ilium, ischium, sacrum and coccyx. Comparing with other regions, spinal osteomyelitis is less frequent (1:80). It affects not only children, sometimes aged under one year, but also adults of 40-50 years old. The infection may be located in the vertebral body or vertebral arch. Osteomyelitis of the pubis is diagnosed too late if based only on clinical data. The bone abscess is frequently located in the horizontal branch. During the first three days of evolution, the patient has an acute infectious syndrome and tenderness in pelvic area. Osteomyelitis of the ilium is the most frequent osteomyelitis of the hip bones. The abscess may diffuse anteriorly in the Scarpa triangle or may infiltrate the lesser pelvis. The lack of local signs may lead to serious errors, which may determine severe complications, including death. The infection of the ischium is extremely rare. The local infectious syndrome becomes apparent late and consists of gluteal and perineal swelling. Most frequently, the osteomyelitis of the sacrum is also diagnosed late and clinically appears like septicemia, meningitis or peritonitis. Being situated close to the skin, the osteomyelitis of the coccyx is easily diagnosed, but it appears only exceptionally.

Current opinions regarding the spinal cord ischemia syndrome.

Spinal cord ischemia syndrome (SCIS) is a serious complication which may occur after either internal or surgical diseases in newborn, young children, teenagers and adults; it is also followed by paraplegia. The onset is acute in 95% of cases. In the other cases the onset may be subacute, developing within one week, or it may be chronic, with slow, progressive development, within a few months to a year. The etiology and pathogenesis of this syndrome raises the interest of many medical fields, such as anatomy, physiology, internal medicine, surgical and imaging specialties. In current medical practice the role of spinal arteriography in diagnosing spinal cord ischemia is essential. Arteriography reveals obstructive lesions in the emerging area of the lumber artery, located between T8 and T12 in 85% of patients. Usually, after diagnosing this syndrome, it may be very difficult to reveal the underlying disease and it may require several investigations such as normal and 3D CT scans, SCIS, cerebral or myelic densitometries. This condition may be caused by metabolic congenital or acquired diseases, infectious vascular diseases, osteoporosis: it may also occur after general or peridural anaesthesia or surgical procedures such as spine surgery, neuro- and cardio-vascular surgery, vertebral and myelic trauma and so on. Treatment for this syndrome will be conducted with respect to the underlying disease. Prognosis may depend on patient's age and it is usually difficult to estimate due to the impossibility of determining the type and extent of the medullary lesion (axonotmesis, neurotmesis or other lesions).

Renovascular hypertension in children: symptoms and manifestations. A 19 patients clinical study.

UNLABELLED: The symptomatology in renovascular hypertension in children may be sometimes misunderstood, leading to the establishment of a late diagnosis, even after the inducing disease is diagnozed. METHODS: The main types of manifestations on which the diagonsis was established: neuropsychiatric, weight loss, renal and digestive manifestations were studied in 19 patients, age ranged 2 to 15 years old in which the main causes diagnosed were reno-ureteral malformations, pyelonephritis, reno-ureteral reflux and renal trauma. Starting from these observations the diagnosis of arterial hypertension was established and the further investigations have shown the real causes of arterial hypertension noticed. RESULTS: The periodic postoperative evaluation performed every three months over a peri6d ranging from 4 months to 7 years individualized 4 clinical evolutive types: Arterial Hypertension with lumbar pain, without any clinical manifestation, with ophthalmologic manifestations and with encephalopathic manifestations. CONCLUSIONS: Symptomatology in renovascular hypertension has no pathognomonic indices and the observed manifestations being single or all together impose the evaluation of blood pressure or even more, the monitoring of it over a long period, for at least 30 days. If the values of blood pressure are greater than the normal accepted ones, more complex investigations are required in order to establish a certain etiology of the hypertensive status.

Von Recklinghausen's disease--elements of clinical practice.

Neurofibromatosis is a condition described for the first time by Von Recklingahausen in 1882. This disease is one of the most common genetic disorders and is characterized by multiple tumors of the central and peripheral nervous system, brown macules on the skin, bone deformities, and by vascular and visceral disorders. Despite extensive basic science research, the diagnosis is still based largely on clinical criteria, which often present gradually during childhood.

Discal hernia in children and teenagers: medical, surgical and recovery treatment.

Lumbar disc hernia represents a rare situation for the physician. The first intervention in disc hernia was performed during the '40. The rate of surgery needing lumbar hernia is about 1-2%. Lumbar disc hernia in children and teenagers has 4 main causes: familial history, trauma, congenital malformation of the spine and disc degeneration. The symptoms in young patients are dominated by local or ischiadic irradiated pain, but neurological discrepancies rarely occur.