Experimental Studies of Latissimus Dorsi Detrusor Myoplasty for Bladder Acontractility: A Systematic Review.

Current therapies that allow patients with bladder acontractility to void are limited. The standard therapy is clean intermittent catheterization. Latissimus dorsi detrusor myoplasty (LDDM) has been shown to provide functional contraction and allow patients with bladder acontractility to void voluntarily. Our goal was to summarize experimental studies of LDDM. We hypothesized that experimental studies would show that latissimus dorsi muscle (LDM) flaps for detrusor myoplasty have superior outcomes when compared with other types of flaps. On January 17, 2020, we conducted a systematic review of the PubMed/MEDLINE, Cochrane Clinical Answers, Cochrane Central Register of Controlled Trials, and EMBASE databases, without time frame limitations, to identify articles on the use of LDDM. We excluded studies that investigated other treatments. Of 54 articles identified by the search, three fulfilled the eligibility criteria. A total of 24 dogs underwent procedures and were evaluated with a maximum follow-up of 9 months. Three types of procedures were performed: LDM in situ reconfiguration, LDM myoplasty, and augmentation cystoplasty after supratrigonal cystectomy. Electrical stimulation, cystography, urodynamic and hydrodynamic measurements, and microscopic examinations were performed. Innervated LDM flaps transferred to the bladder were able to contract and promote voiding in response to electrical stimulation. Experimental studies have shown the feasibility of LDDM in canine models. Although no comparison groups were included, innervated LDM flap transferred to the bladder showed promising results regarding contraction capable of voiding.

Floppy eyelid syndrome "plasty" procedure: Employment of a periosteal transposition flap for surgery of floppy eyelid syndrome.

IMPORTANCE: Floppy eyelid syndrome "plasty" (FESplasty) is a surgical technique that addresses underlying superior tarsal plate and lateral canthal instability in floppy eyelid syndrome (FES) and aims to restore normal anatomical and physiological function to the upper eyelid. BACKGROUND: To describe the use of FESplasty in the surgical management of FES, and to report outcomes in an initial patient cohort. DESIGN: Retrospective study. PARTICIPANTS: Seven patients (nine eyelids) with FES undergoing FESplasty. METHODS: A single surgeon (G.W.) performed all procedures. FESplasty utilizes a periosteal flap based at the inferolateral orbital rim, and applied to the anterior surface of the upper tarsal plate. It is combined with a titrated shortening procedure of the upper eyelid. Patient demographics, comorbidities and ocular symptoms and signs were recorded preoperatively. Pre- and postoperative upper eyelid distractibility were graded and documented. MAIN OUTCOME MEASURES: Postoperative improvement in upper eyelid distractibility and symptomatology, operative complications and FES recurrence. RESULTS: FES symptoms and upper eyelid laxity improved at last follow-up (average 24 weeks) in all patients, with no FES recurrences after a maximum follow-up of 36 weeks. One patient, in whom FESplasty exacerbated his pre-existing aponeurotic ptosis, required definitive ptosis surgery subsequently. There was one case of postoperative wound infection. Obstructive sleep apnoea was present in four of the seven patients. The remaining three patients were awaiting assessment. CONCLUSIONS AND RELEVANCE: FESplasty is likely to confer long-term effective stabilization of the lateral canthal tendon, lateral commissure and superior tarsal plate. Anatomical and functional results appeared to have been successfully achieved.

Anesthetic Management of a Patient With Emanuel Syndrome.

Emanuel syndrome is associated with supernumerary chromosome, which consists of the extra genetic material from chromosome 11 and 22. The frequency of this syndrome has been reported as 1 in 110,000. It is a rare anomaly associated with multiple systemic malformations such as micrognathia and congenital heart disease. In addition, patients with Emanuel syndrome may have seizure disorders. We experienced anesthetic management of a patient with Emanuel syndrome who underwent palatoplasty. This patient had received tracheotomy due to micrognathia. In addition, he had atrial septal defect, mild pulmonary artery stenosis, and cleft palate. Palatoplasty was performed without any complication during anesthesia. Close attention was directed to cardiac function, seizure, and airway management.

Usefulness of Noninvasive Positive-pressure Ventilation During Surgery of Flaccid Neuromuscular Scoliosis.

STUDY DESIGN: This is a retrospective study. OBJECTIVE: To assess the effects of noninvasive positive-pressure ventilation (NIPPV) through evaluations of outcomes and incidences of postoperative pulmonary complications among patients with flaccid neuromuscular scoliosis for pulmonary support in the perioperative periods. BACKGROUND DATA: There is no report on the effects of NIPPV on neuromuscular scoliosis patient during the perioperative periods. METHODS: We retrospectively reviewed 73 patients diagnosed with neuromuscular scoliosis who underwent staged anterior and posterior spinal surgery and instrumentations for deformity correction from 2003 to 2010. A total of 73 patients were divided depending on whether they had received NIPPV treatment or not during the perioperative period. Twenty-eight patients who received NIPPV for respiratory support and 45 patients with no mechanical ventilation were compared according to age, sex, body mass index, number of fusion levels, and end-tidal pressure of CO(2) and forced vital capacity values. The incidence of pulmonary complications associated with either group (pneumonia, atelectasis, pneumothorax, prolonged ventilator support, and postoperative tracheostomy) was then evaluated. RESULTS: In between the 2 groups, the forced vital capacity (41% vs. 64%, P<0.0001) were observed to be significantly decreased with the use of NIPPV. End-tidal pressure of CO(2) was not statistically different between the 2 groups. Although statistically not significant, patients in the non-NIPPV group had a higher incidence of pulmonary complications (38% vs. 21%, P=0.1584). None of the aforementioned patients required tracheostomy. In addition, no other mortality or neurological complications were noted postoperatively. CONCLUSIONS: There is a definite advantage of using NIPPV, because the incidence of postoperative pulmonary complications and the need for tracheostomy in patients with severely decreased pulmonary function are not increased from the use of NIPPV.

Anesthetic management of pediatric patients with Emanuel syndrome.

Emanuel syndrome is a rare anomaly associated with multiple systemic malformations. We present two cases involving pediatric patients with Emanuel syndrome. The first patient presented with micrognathia and had patent ductus arteriosus and a single kidney. The patient was difficult to intubate with McGRATH(®) but was successfully intubated with an Airtraq(®) device. The second patient did not present with micrognathia and was not difficult to intubate. A thorough examination of the heart, kidney, and spinal cord is important when planning the anesthetic management of patients with Emanuel syndrome. Moreover, adequate preparation for a difficult airway is essential, and the Airtraq(®) device may be useful for intubating patients with Emanuel syndrome with micrognathia.

Surgical treatment for kyphoscoliosis in Cohen syndrome.

Cohen syndrome is a very rare disease. Complication by spinal deformity has been reported, but management and surgery for spinal deformity in Cohen syndrome has not been previously described. The objective of this study was to examine the outcome of surgical treatment for kyphoscoliosis of Cohen syndrome with a literature review. The patient was a 14-year-old male with the characteristics of Cohen syndrome: truncal obesity, mental retardation, arachnodactyly, microcephalia, and a facial malformation. Scoliosis was conservatively treated with a brace at 13 years of age, but the spinal deformity rapidly progressed within a year. Plain radiographs before surgery showed scoliosis of 47 degrees (T5-T11) and 79 degrees (T11-L3), and kyphosis of 86 degrees (T7-L1). One-stage anteroposterior corrective fusion of T4-L3 was scheduled after 2-week Halo traction. Postoperative respiratory management was carefully performed because of Cohen syndrome-associated facial malformation, obesity, and reduced muscle tonus. Respiration was managed with intubation until the following day and no respiratory problems occurred. After surgery, thoracolumbar scoliosis was 28 degrees (correction rate: 65%). Kyphosis was markedly improved from 86 degrees to 20 degrees, achieving a favorable balance of the trunk. The outcome is favorable at 6.5 years after surgery. In conclusion, Cohen syndrome is often complicated by spinal deformity, particularly kyphosis, that is likely to progress even in adulthood. In our patient, spinal deformity progressed within a short period, even with brace treatment. Surgery should be required before progression to the severe spinal deformity with careful attention to general anesthesia.

Floppy eyelid associated with Down syndrome.

Eyelid laxity due to Down Syndrome may be better classified under the rubric of lax eyelid conditions.

Management of floppy eyelid associated with Down's syndrome: a case report.

PURPOSE: To report a case of floppy eyelid associated with Down's syndrome in a 2-year-old child. CASE REPORT: A 2-year-old child associated with Down's syndrome presented with a history of eversion of both upper lids during sleep and while crying since birth. There was no history of sleep apnea and no mechanical stimuli over eyelids. Examination under anesthesia revealed gross laxity with thick and rubbery tarsi in both the upper and lower lids. Vascularized corneal scar was present in both eyes. The laxity in the upper eyelids was corrected by pentagonal wedge resection and in the lower lids by lateral tarsal sling. No recurrence was observed up to 6-month follow-up. CONCLUSION: Although floppy eyelid is more common in middle-aged obese patients, it should be carefully looked for children particularly while dealing with Down's syndrome.

Functional detrusor myoplasty for bladder acontractility: long-term results.

PURPOSE: We present the long-term results of a multicenter investigation with latissimus dorsi detrusor myoplasty in patients with bladder acontractility caused by lower motor neuron lesion in whom there is no treatment alternative other than lifelong clean intermittent catheterization. MATERIALS AND METHODS: From 2001 to 2008, 24 patients (median age 39 years, range 13 to 63, 16 males, 8 females) requiring complete clean intermittent catheterization 4 to 7 times daily underwent latissimus dorsi detrusor myoplasty at 4 centers worldwide. Before the procedure patients were on clean intermittent catheterization for a median of 55 months (range 17 to 195). Median followup was 46 months (range 8 to 89) and was performed by urodynamics and measurement of post-void residual urine volume. Bladder contractility index was calculated. The t test was used for statistical analysis. RESULTS: Of the 24 patients 17 (71%) gained complete spontaneous voiding with a mean post-void residual urine volume of 25 ml (range 0 to 100). Mean bladder contractility index increased from 20.1 ± 7.6 to 176.2 ± 25.4 (p <0.001). In 3 patients (13%) the frequency of clean intermittent catheterization was reduced to 2 to 4 times daily with a mean post-void residual urine volume of 200 ml (range 150 to 250). Mean bladder contractility index was 12.0 ± 7.2 preoperatively and 68.7 ± 28.1 postoperatively (p = 0.12). Recurrent urinary tract infections (defined as the presence of clinical symptoms such as dysuria and fever, and microbiological evidence of germs) ceased in 21 of 23 patients (91%, mean preoperatively 8 per year). Four patients (17%) required clean intermittent catheterization with the same frequency as before the procedure (mean bladder contractility index preoperatively 22.5 ± 10.3 and postoperatively 26.0 ± 12.3, p = 0.83). No chronic pain at the donor site or vesicoureteral reflux was observed in any patient. CONCLUSIONS: The results of this multicenter analysis demonstrate that latissimus dorsi detrusor myoplasty is an effective alternative to clean intermittent catheterization in a select group of patients with neurogenic bladder acontractility.

Acquired progressive hypotonia in infancy: consider compressive cervical myelopathy.

UNLABELLED: This case report presents a rare cause of progressive hypotonia due to a congenital bony defect of the atlas in a 2-month-old girl. The patient was initially referred to the paediatric department with feeding problems. Within days after admission she developed progressive hypotonia and showed decreased reflexes of the upper extremities. Magnetic resonance imaging showed compressive myelopathy at the level of vertebra C1. After laminectomy of the C1-vertebra the neurological symptoms resolved. CONCLUSION: Most anomalies of the vertebras are asymptomatic. Only symptomatic anomalies with compression and neurological symptoms need surgery.

Basicranial diplomyelia: an extension of the split cord malformation theory. Case report.

Basicranial diastematomyelia is an extremely rare congenital disorder. A review of the literature indicates only one reported case of basicranial diastematomyelia in which an osseous peg divided the brainstem in two. The authors present the first reported case of basicranial diplomyelia split by a fibrous band and correlate its pathogenesis with that of split cord malformation (SCM). The patient described in the present report had a fibrous stalk dividing the brainstem, and therefore the condition was categorized as a diplomyelia, or SCM Type II. Because the occipital dermatomes behave similarly to the spinal dermatomes early in development, they may be subject to the same embryonic error that results in SCM. The authors propose that the mechanism leading to SCM is the same as that found in basicranial split malformations and that the theory explaining it be modified to include the posterior fossa.

Down syndrome in children: the role of the orthopaedic surgeon.

Down syndrome, the result of trisomy of chromosome 21, is one of the most common chromosomal abnormalities. Patients have a characteristic facial appearance, variable levels of intelligence and self-care skills, and a variety of associated medical conditions. Orthopaedic manifestations occur frequently; most are related to hypotonia, joint hypermobility, and ligamentous laxity. Atlanto-occipital and atlantoaxial hypermobility, as well as bony anomalies of the cervical spine, can produce atlanto-occipital and cervical instability. Methods of screening for this instability, particularly with regard to participation in sports, are a subject of controversy. Scoliosis, hip instability, slipped capital femoral epiphysis, patellar instability, and foot deformities are other musculoskeletal conditions found in patients with Down syndrome that can be challenging for the orthopaedic surgeon to treat.

Tracheostomy in neurologically compromised paediatric patients: role of starplasty.

OBJECTIVES: Starplasty tracheostomy is an alternative to traditional tracheostomy. This paper reviews neurologically compromised paediatric patients with tracheostomies and discusses the role of starplasty tracheostomy. METHOD: A retrospective review was conducted of paediatric patients with a neurological disorder who underwent tracheostomy between 1997 and 2011. RESULTS: Forty-eight patients, with an average age of 7.3 years, were identified. The most common indications for tracheostomy were: ventilator dependence (39.6 per cent), an inability to tolerate secretions or recurrent aspiration pneumonia (33.3 per cent), and upper respiratory obstruction or hypotonia (12.5 per cent). The most common underlying neurological diagnosis was cerebral palsy. There were no early complications. Eighteen (43 per cent) of 42 patients with follow up experienced at least 1 delayed complication. Only 12 patients (28.6 per cent) were decannulated. CONCLUSION: Patients with primary neurological diagnoses have low rates of decannulation; starplasty tracheostomy should be considered for these patients. Patients with seizure disorder or acute neurological injury tended to have a higher short-term decannulation rate; traditional tracheostomy is recommended in these patients.

The effect of retropubic urethropexy on detrusor stability.

A group of 86 women with genuine stress incontinence who underwent retropubic urethropexy were evaluated with both pre- and postoperative urodynamics. Twenty of these 86 women (23.3%) also had unstable detrusors preoperatively. Eleven of these 20 women (55%) had stable detrusors after retropubic urethropexy. Five of the 66 patients (7.6%) who had stable detrusors preoperatively were found to have unstable detrusors on postoperative urethrocystometry. The overall cure rate for women with detrusor instability and genuine stress incontinence was only 30%. Analysis of symptoms, previous anti-incontinence procedures, age, parity, and cystometric parameters revealed no differences between those women who had stable detrusors after retropubic urethropexy and those who remained unstable. Similarly, patients whose bladders became unstable after retropubic urethropexy could not be distinguished from those who remained stable. Patients undergoing retropubic urethropexy should understand the possibility that the operation may cause urinary incontinence due to detrusor instability even if it cures their genuine stress incontinence, and that if they have both genuine stress incontinence and detrusor instability, their chances for an operative cure of both conditions are low.

New concept in bladder remodeling.

During the past five years a surgical procedure for rehabilitation of partially decompensated large-capacity bladders has been developed and utilized in 9 children and 2 adult patients. The patients presented with detrusor muscle failure. The key steps of this procedure are (1) creation of laterally-based mucosa-free detrusor pedicle flap, (2) reduction of bladder size and wrapping the flap around the body of the bladder thus doubling the muscle bulk, and (3) mobilization and extraperitonealization of the greater omentum to the reconstruction site. The indications, rationale, and surgical technique are presented. The gratifying results, as gauged by urodynamic testing, suggest that detrusor remodeling is a worthwhile procedure for a highly select group of patients.

Direct cyclopexy after persistent hypotony as a complication after cyclodialysis and goniotomy.

The surgical technique of direct cyclopexy, which has been performed on 3 patients suffering on persistent hypotony after cyclodialysis and goniotomy, is reported. The extension of the cyclodialysis reached from 60 to 90 degrees, the persistence of hypotony from 12 to 36 months, and the postoperative follow-up from 10 to 24 months.

Refixation of the ciliary body after traumatic cyclodialysis.

In 10 eyes a direct refixation of the ciliary muscle to the scleral sporn with many 10 X 0 nylon sutures very close to each other has been performed after a traumatic cyclodialysis. In all 10 eyes the intraocular pressure could be normalized between 12 and 18 mm Hg, the visual acuity recovered in all cases and the signs of ocular hypotony disappeared at the anterior and posterior segment. The follow-up ranged from 6 months to 4 1/2 years. The extent of cyclodialysis ranged from 30 to 120 degrees. In 5 eyes the direct cyclopexy was performed within 10 days after the traumatic cyclodialysis and in 5 eyes after 2-3 months, which was not important for the visual recovery. Intraoperatively no complications were seen and postoperatively in only 4 eyes a temporary increase of intraocular pressure was noted.

Tendon transfers in the paralytic hip.

We analyzed the results of transfers of the iliopsoas or external oblique muscles performed to augment the abductor power of the hips in 149 patients with flaccid paralysis of the hips. More than 800 supplemental procedures were performed on the lower extremities of these patients. Twenty-three of the thirty-eight hips that had been subluxated or dislocated became stable in the reduced position after the transfer operation. Every hip that had been stable before the transfer remained stable, and thirty-seven patients who had been brace-dependent became brace-free. We concluded that transfers of the iliopsoas or external oblique muscles are useful adjuncts in the treatment of paralytic disorders of the hips.