Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinically obvious reasons why children with neurological impairment (NMI) may be more severely affected in case of a viral respiratory tract infection include reduced vital capacity due to muscular weakness or spastic scoliosis, disturbed clearance of respiratory excretions (weak coughing and dysphagia), inability to comply actively with physiotherapeutic interventions, recurrent micro-aspirations (gastroesophageal reflux disease, vomiting related to coughing), a history of frequent exposure to antibiotics and health care institutions, colonization with resistant pathogens, impaired immunologic defence mechanisms due to severe malnutrition and cachexia, and early clinical deterioration in case of high fever with metabolic acidosis and hypercapnia, and maybe associated seizures or febrile convulsions.Data from the literature suggests that in all children with NMI, who have to be hospitalized with severe clinical deterioration due to an airway infection, at least one specimen of nasopharyngeal secretions should be sent as soon as possible to a virologic laboratory to detect viral pathogens. Children with severe NMI and those mechanically ventilated for other reasons being hospitalized during the RSV season must be strictly protected against nosocomial RSV infection by means of standard and droplet precautions. Finally, children with severe NMI and age below 24 months of life should receive passive immunization with palivizumab following international recommendations.
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PMID:Respiratory syncytial virus infection in children with neuromuscular impairment. 2226 88

We report the case of a 14-year-old boy with pectus excavatum and mild scoliosis. The patient underwent a minimally invasive Nuss repair with excellent cosmetic result. He returned 3 weeks post-operatively with bilious emesis and a 3.6-kg weight loss (5.5% of total body weight). Radiographic evaluation was consistent with the diagnosis of superior mesenteric artery syndrome and the child ultimately required nasojejunal feedings. Following return to his baseline weight, he was transitioned to oral feedings and has done well in follow-up. This is the first report of SMA syndrome following minimally invasive pectus excavatum repair.
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PMID:Superior mesenteric artery syndrome after minimally invasive correction of pectus excavatum: impact of post-operative weight loss. 2316 12

A 28-year-old male with scoliosis presented with complaints of dyspnea and vomiting. His medical history revealed a mediastinal ganglioneuroma resection at the age of 2. After the surgery, he had not been followed up until his admission to our hospital. Computed tomography and MRI showed severe scoliosis of the thoracic spine and a paravertebral mass extending from the upper thoracic level to the level of renal arteries. Based on its radiological findings and the patient's history, the tumor was considered to be a recurrent ganglioneuroma. Paravertebral ganglioneuromas may cause progressive scoliosis, and a careful examination for patients with progressive scoliosis is mandatory.
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PMID:Radiologic findings of thoracic scoliosis due to giant ganglioneuroma. 2331 87

Corrective surgery for scoliosis is an extensive procedure with well-known problems of postoperative pain control. Additional problems with nausea, vomiting, ileus, and sedation can result in delayed mobilization and a prolonged inpatient hospital stay. At our institution, a multimodal approach to pain management has been used to successfully address these issues. The use of intravenous acetaminophen has been a helpful adjunct to our armamentarium of pain medication in this patient population. We present an illustrative case of our use of multimodal analgesia beginning intraoperatively and continuing during the acute inpatient postoperative period.
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PMID:Multimodal pain management after spinal surgery for adolescent idiopathic scoliosis. 2337 74

Superior mesenteric artery (SMA) syndrome is a rare but potentially fatal complication following spinal fusion for scoliosis. The aims of our study were to identify clinical features and evaluate their importance in SMA syndrome following posterior spinal fusion (PSF) in adolescent idiopathic scoliosis (AIS) patients and to recommend a simple approach for the recognition and management of SMA syndrome. This is an IRB-approved study. Three patients in our series and 11 patients from other published case reports who underwent PSF for adolescent idiopathic scoliosis were included in our study. Patient demographics, clinical presentation, investigations, and management were recorded and analyzed. In this combined series of 14 patients (mean age 14.8 years), the main presenting clinical features included vomiting of any kind (92.9%), abdominal pain/tenderness (57.1%), abdominal distension (42.9%), bilious vomiting (35.7%), and hypoactive bowel sounds (28.6%). Most patients presented within 2 weeks of surgery (71.4%). In total, 50% of patients presented with both vomiting (of any kind) and abdominal tenderness. The number of presenting symptoms appeared to be directly related to the severity of SMA syndrome. Our second patient had intermittent vomiting on postoperative day (POD) 3, which was initially considered as insignificant; she was discharged on POD 9 and readmitted for recurrent vomiting, during which SMA syndrome was diagnosed. Our first patient presented in the classical manner. Our third patient had bilious vomiting after discharge that started on POD 13 and increased in frequency until readmission on POD 27. Vomiting and abdominal pain are nonspecific symptoms following PSF; differentiating between SMA syndrome and postoperative ileus can be challenging. The highest index of suspicion applies to patients who present within the first week with symptoms of vomiting and abdominal pain. We propose an algorithm for the management of SMA syndrome, which includes a focused clinical assessment to evaluate for intestinal obstruction, followed by an abdominal radiography and barium contrast study if clinical assessment is positive. An early referral to general surgery should be considered especially for high-risk patients (BMI<5% percentile, sagittal kyphosis). Early diagnosis of SMA syndrome allows for early intervention, reducing the likelihood of future complications and need for surgery.
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PMID:Superior mesenteric artery syndrome following surgery for adolescent idiopathic scoliosis: a case series, review of the literature, and an algorithm for management. 2468 92

De novo monoallelic variants in NFIX cause two distinct syndromes. Whole gene deletions, nonsense variants and missense variants affecting the DNA-binding domain have been seen in association with a Sotos-like phenotype that we propose is referred to as Malan syndrome. Frameshift and splice-site variants thought to avoid nonsense-mediated RNA decay have been seen in Marshall-Smith syndrome. We report six additional patients with Malan syndrome and de novo NFIX deletions or sequence variants and review the 20 patients now reported. The phenotype is characterised by moderate postnatal overgrowth and macrocephaly. Median height and head circumference in childhood are 2.0 and 2.3 standard deviations (SD) above the mean, respectively. There is overlap of the facial phenotype with NSD1-positive Sotos syndrome in some cases including a prominent forehead, high anterior hairline, downslanting palpebral fissures and prominent chin. Neonatal feeding difficulties and/or hypotonia have been reported in 30% of patients. Developmental delay/learning disability have been reported in all cases and are typically moderate. Ocular phenotypes are common, including strabismus (65%), nystagmus (25% ) and optic disc pallor/hypoplasia (25%). Other recurrent features include pectus excavatum (40%) and scoliosis (25%). Eight reported patients have a deletion also encompassing CACNA1A, haploinsufficiency of which causes episodic ataxia type 2 or familial hemiplegic migraine. One previous case had episodic ataxia and one case we report has had cyclical vomiting responsive to pizotifen. In individuals with this contiguous gene deletion syndrome, awareness of possible later neurological manifestations is important, although their penetrance is not yet clear.
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PMID:Malan syndrome: Sotos-like overgrowth with de novo NFIX sequence variants and deletions in six new patients and a review of the literature. 2511 28

We present the case of a 12-year-old girl who underwent an uncomplicated posterior spinal fusion with instrumentation for scoliosis and who later developed nausea, emesis, and abdominal pain. We discuss the epidemiology, prevalence, anatomic findings, symptoms, diagnostic tests, and clinical management, including nonsurgical and surgical therapies, of superior mesenteric artery syndrome (SMAS), a rare condition. Over a 2-week period, the patient developed an uncommon type of bowel obstruction likely related to her initial thin body habitus, correction of her deformity, and weight loss after surgery. The patient returned to the operating room for placement of a Stamm gastrostomy feeding tube with insertion of a transgastric-jejunal (G-J) feeding tube. The patient had the G-J feeding tube in place for approximately 6 weeks to augment her enteral nutrition. As she gained weight, her duodenal emptying improved, and she gradually transitioned to normal oral intake. She has done well since the G-J feeding tube was removed. Posterior spinal fusion for adolescent idiopathic scoliosis is a relatively common procedure, and SMAS is a rare condition. However, in the case of an asthenic adolescent with postoperative weight loss, intestinal obstruction can develop. When planning operative spinal correction in scoliosis patients who have a low body mass index at the time of surgery and who have increased thoracic stiffness, be alert for signs and symptoms of SMAS. This rare complication can develop, and timely diagnosis and medical management will decrease morbidity and shorten the length of time needed for nutritional rehabilitation.
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PMID:A Rare Cause of Postoperative Abdominal Pain in a Spinal Fusion Patient. 2637 64

Compression and obstruction of the duodenum can occur after surgical correction of spinal scoliosis. We report a case of 15-year-old girl who developed superior mesenteric artery syndrome (SMAS) following scoliosis surgery. On the 4th postoperative day, the patient complained of nausea and vomiting, which was considered as side effects of opioids as she was treated with intravenous fentanyl infusion with patient-controlled analgesia (PCA) device. Nasogastric tube was placed and background infusion rate of the PCA was tapered. On the 5th postoperative day, fentanyl infusion was stopped, but she complained of persistent nausea and vomiting. Barium upper gastrointestinal series and abdominal echography revealed compression in the third portion of the duodenum between the superior mesenteric artery and aorta on the 7th postoperative day. She responded to conservative treatment (nutritional and fluid supplementation), which lasted about two weeks. She was discharged home on the 51st postoperative day. SMAS is rare but sometimes carries serious complications. Vomiting following scoliosis surgery should be examined thoroughly including the possibility of SMAS, especially during postoperative pain management with opioids (i. e., IV-PCA with fentanyl). Early diagnosis and institution of appropriate conservative therapy is essential to reduce the likelihood of future complications and avoid the need for surgery.
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PMID:[Superior Mesenteric Artery Syndrome following Scoliosis Surgery during Intravenous Patient Controlled Analgesia (IV-PCA) with Fentanyl: A Case Report]. 2700 94

Superior mesenteric artery syndrome is a rare complication occurring after surgical treatment for scoliosis. Surgical correction of the scoliotic spine produces vertical traction on the mesenteric artery and the narrowing of the aortomesenteric angle, hence the compression of the third part of the duodenum causing the syndrome. We here report the case of a young girl with an early form of superior mesenteric artery syndrome secondary to surgical correction of idiopathic scoliosis. The patient underwent posterior spinal fusion for idiopathic scoliosis. On the third postoperative day she experienced uncontrollable vomiting associated with no evacuation of faeces and no passing of flatus. Urgent abdominal CT scan was performed which allowed the diagnosis of superior mesenteric artery syndrome. Treatment consisted in the rest of the digestive tract associated with early parenteral nutrition and correction of fluid and electrolyte imbalances. Lacking evidence of clinical improvement, surgical indication was posed. The evolution was favourable with an uneventful postoperative recovery and the resumption of adequate food intake on the fourth postoperative day. The patient was discharged on the seventh postoperative day. Contributing factors are young age, long-limbed morphotype with BMI below 18. CT scan shows an important gastric dilatation associated with complete halt of the third part of the duodenum. The treatment is multidisciplinary, medical (first-line treatment) and surgical (if medical treatment do not help). A better knowledge of the predictors of medical treatment failure would reduce the length of hospital stay.
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PMID:[Early form of Wilkie's syndrome: a rare complication of scoliosis surgery, about a case and review of the literature]. 2829 53

Superior mesenteric artery (SMA) syndrome is a rare and potentially life-threatening complication of scoliosis surgery. The anatomical relationship of the duodenum and the superior mesenteric artery, the correction of angular deformity of the spine, and the normal adolescent growth spurt all contribute to the condition. We report the case of a 14-year-old boy who had a history of idiopathic scoliosis and presented with bilious vomiting that had persisted for 7 days after posterior T9-L4 fusion with instrumentation. After an unremarkable immediate postoperative course, on postoperative day 19 the patient presented to the emergency department with abdominal pain, nausea, and vomiting. Unrelenting brown vomitus, abdominal pain, and a 20-lb weight loss were noted. A series of upper gastrointestinal radiographs confirmed a diagnosis of SMA syndrome. A nasojejunal tube was placed, and nutritional rehabilitation was optimized. We highlight this case for its rarity and emphasize the importance of maintaining a high index of suspicion when evaluating a child who has had spinal deformity correction and presents with postoperative gastrointestinal complaints. Early recognition of the nonspecific symptoms of abdominal pain, abdominal distension, bilious or projectile vomiting, hypoactive bowel sounds, and anorexia plays a key role in post-scoliosis surgery and is crucial in preventing the severe morbidity and mortality associated with SMA syndrome.
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PMID:Superior Mesenteric Artery Syndrome as a Complication of Scoliosis Surgery. 2843 98


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