Primary Corrective Surgery Research Articles

Airway obstruction following palatoplasty of a patient with sleep disturbance

Airway obstruction following palatoplasty of a patient with sleep disturbance

Improvement of Continence with Reoperation in Selected Patients After Surgery for Anorectal Malformation

Fecal incontinence is a serious complication after repair of anorectal malformations. We investigated whether reoperation can improve fecal continence. Medical records of 41 patients (40 children and one adult; 26 male and 15 female) who underwent reoperation after previous reconstruction of an anorectal malformation were reviewed for outcomes of bowel function. Type of primary corrective surgery performed, therapeutic measures, results of physical examination and barium enema, and reoperation procedures were evaluated. A questionnaire was administered to assess stool behaviour and level of continence at follow-up three or more years after secondary operation. Secondary operations in males comprised posterior sagittal anorectoplasty (PSARP) in 16 patients, PSARP with antegrade continent enema in one patient, antegrade continent enema alone in 6, anoplasty in one, rectosigmoid resection in 1, and definitive colostomy in 1 patient. Secondary operations in females included PSARP alone in 4 patients, PSARP with total urogenital mobilization in 4, PSARP with vaginoplasty in 2, PSARP with vaginoplasty and antegrade continent enema in 2, and PSARP with vaginourethroplasty in 3. Of 41 patients 18 (44 percent) were continent at follow-up, 21 (51 percent) were clean with use of enemas, diet, or drug therapy. One patient had a definitive colostomy. One died after kidney transplantation. Surgery is a good option for improving incontinence in selected patients previously operated for anorectal malformations. Posterior sagittal anorectoplasty is advocated to improve bowel control. Antegrade continent enema is a reliable therapeutic option to maintain clean patients with fecal incontinence.

Wound Problems in Surgery for Talipes Equinovarus

The traditional treatment for talipes equinovarus (TEV) has been open surgery in many units around the world. The complication rates for primary corrective surgery are relatively small but may affect outcome significantly. The complications of surgery in relapsed TEV are more frequent than for primary surgery, and problems relating to wound healing present difficult challenges to the treating surgeon. An overview of the approaches to management of this still challenging condition are presented, and the factors involved in wound healing problems and strategies for their prevention and treatment are presented. In particular, the belated but rapid spread worldwide of the Ponseti nonoperative approach to treating talipes is the most important factor in reducing the rates of surgery and therefore the complications that may ensue.

Lower Lid Epiblepharon as the Presenting Feature of Spontaneous Dural Carotid Cavernous Fistula in an Asian Patient

Lower lid epiblepharon is a relatively common problem among young Asian patients. Occasionally, it may be secondary to underlying orbital pathology. We report a 33-year-old Chinese patient with a dural carotid cavernous fistula who presented with an ipsilateral lower lid epiblepharon in the absence of any other significant signs. After a recurrence following primary corrective lid surgery, she was investigated further because of mild ipsilateral proptosis. CT-scans of the orbit revealed an enlarged superior ophthalmic vein and a dural carotid cavernous fistula was confirmed on angiography. Subsequent embolisation of the fistula led to resolution of the proptosis as well as the epiblepharon. We believe that extension of orbital fat to the inferior tarsal border in Asian eyelids together with a poorly fixed anterior lamella contributes to the occurrence of epiblepharon when the Asian orbit becomes congested. The new onset of such features in the Asian patient should lead one to suspect underlying orbital pathology, even in the absence of other, more classic signs.

The outcome of the older (≥100 days) infant with biliary atresia

Background There is a detrimental effect of increasing age on the results of the Kasai portoenterostomy for biliary atresia (BA), and some centers routinely advocate primary liver transplantation for the older infant, irrespective of other criteria. This perception that such infants are indeed irretrievable was tested by retrospective analysis. Methods All infants who had undergone surgery for BA during the period 1980 through 2000 aged ≥100 days were reviewed. Actuarial survival was calculated using 2 end-points (death and transplantation). A retrospective review of their ultrasonography (n = 12) and preoperative liver histology (n = 22) was also undertaken to ascertain possible predictive criteria. Results A total of 422 infants had BA diagnosed during this period, of which 35 (8.2%) were ≥100 days at surgery (median [interquartile range], 133 [range, 108 to 180] days). Surgery included portoenterostomy (n = 26), hepaticojejunostomy (n = 7), and a resection and end-to-end anastomosis (n = 1). A laparotomy only was performed in 1. Five- and 10-year actuarial survival rate with native liver was 45% and 40%, respectively. Currently, 12 (35%) patients are alive with their native liver (8 are anicteric), 9 (28%) have undergone transplantation, and 13 have died. Although there were some survival advantages for types 1 or 2 BA and “noncirrhosis” at time of surgery, neither reached statistical significance. Individual histologic features (eg, degrees of fibrosis, giant cell transformation, bile duct destruction) in the retrospective review of available material were not discriminatory. The finding of a “heterogeneous” parenchyma on ultrasonography was predictive of poor outcome but lacked sensitivity. Conclusions The potential for reasonable medium-term survival is present in about one third of infants 100 days or older coming to primary corrective surgery. In the absence of accurate discrimination, the authors continue to favor this option rather than subject all to transplant simply on the basis of age.

Functional and radiographic outcomes after surgery for adult scoliosis using third-generation instrumentation techniques.

Retrospective radiograph and chart review of 28 patients with adult idiopathic scoliosis undergoing primary corrective surgery. Clinical and radiographic parameters were assessed before surgery, after surgery, and at a 2-year follow-up assessment. A self-perceived outcome questionnaire was administered to the study patients at a minimum 2-year follow-up assessment. To assess patient outcomes after surgery for adult scoliosis using traditional radiographic parameters along with a self-perceived outcomes questionnaire. The clinical and radiographic results and the outcomes for the surgical treatment of adult idiopathic scoliosis have not been established in the literature with respect to the use of modern third-generation instrumentation techniques. Most studies reviewing the surgical treatment of adult idiopathic scoliosis look primarily at Harrington instrumentation techniques. Records and radiographs were reviewed retrospectively for all the patients (n = 54) undergoing primary corrective surgery for adult idiopathic scoliosis between December 30, 1994 and December 30, 1997. Of the 54 patients reviewed, 28 (52%) met the following inclusion criteria: age exceeding 20 years, primary surgery, fusion above the sacrum, availability of medical records along with preoperative, postoperative, and 2-year follow-up radiographs. Additionally, a self-perceived outcomes questionnaire was administered to these patients at a minimum 2-year follow-up assessment. All the patients were women (28/28). The indications for surgery were pain and progression in 54% (15/28) and pain in 29% (8/28) of the patients. The average preoperative major curve measurement was 65 degrees (range, 38-98 degrees ). The average postoperative major curve measurement was 24 degrees (range, 5-59 degrees ), for a correction of 64%. The average follow-up curve measurement was 27 degrees (range, 3-60 degrees ), for a correction of 61%. Whereas 71% of the cases were anteroposterior, 29% were posterior only. There was one intraoperative complication among the 28 patients and four postoperative complications in 3 of the 28 patients. The self-perceived outcome questionnaires were available for 83% (23/28) of the patients. Definite or probable relief of symptoms was reported in 74%(17/23). Improved ability to sleep was reported in 61% (14/23), and ability to return to their usual job was reported in 57% (13/23). Satisfaction with the results of surgery was reported in 87% (20/23). Surgery for adult idiopathic scoliosis using third-generation instrumentation techniques provides significant clinical improvement, scoliosis correction, maintenance of sagittal alignment, and patient satisfaction, with an acceptable complication rate.

Surgical management of congenital heart disease in the 1990s.

To provide a broad overview of recent trends in surgical management of congenital heart disease. Review of recent literature. Major centers for surgical management of congenital heart disease. Analysis of important recent clinical publications. None. Definitive, corrective surgery was previously reserved for children beyond infancy. Recognition of the complications associated with palliative operations as well as the realization that neonates and infants did not fare worse than older children during open heart surgery provided the impetus for primary corrective surgery at an earlier age. Advances in our understanding of perioperative care, improved diagnostic capability, and increasing experience with corrective surgery in association with several different congenital heart lesions has resulted in an established therapeutic approach to surgical management of congenital heart disease in neonates and infants.

Electrophysiological studies of puborectalis and external anal sphincter in incontinent children with corrected high ano-rectal anomalies

Thirty incontinent patients with corrected high ano-rectal anomalies were studied using concentric needle electromyography (EMG) ‘sphincter mapping’. The findings were correlated with the ability to excite the external anal sphincter by transrectal pudendal nerve stimulation in 13 cases. EMG evidence of a functional external anal sphincter was found in 22 patients, but this was either to one side of or completely separate from the surgically created anal orifice in 17 cases. No activity could be detected in 8 cases without complex anomalies, a much higher incidence than in a previous series of children examined pre-operatively. This suggests damage to the sphincter during primary corrective surgery. These combined neurophysiological techniques are useful in the assessment of incontinent children with corrected high ano-rectal anomalies, even though striated sphincter function is not the sole consideration in establishing continence.

Management of Hirschsprung's disease. The definitive operation: which, when, why and how.

Analysing the results of 34 infants operated upon from 01.01.77 to 12.31.82, the authors advocate primary corrective surgery without colostomy in the first 3 months of life. Medical treatment is based on total parenteral nutrition (TPN) and enemas. Surgery using any of the three major techniques (Swenson, Soave and Duhamel) is performed after 1 month of oral feeding and maternal care. Results with a follow-up ranging up to 6 years showed excellent defaecation control and short duration of hospitalization.

Transposition of the great arteries with intact ventricular septum and patent ductus arteriosus

At the time of initial balloon atrial septostomy a patent ductus arteriosus was found with angiography in 39 of 81 infants with transposition of the great arteries with intact ventricular septum. By angiographic criteria the ductus shunt was considered small in 21 infants and large and significant in 18. In contrast to the usual clinical presentation of neonates with transposition and intact ventricular septum, 12 of these 18 infants with a significant patent ductus arteriosus had only slight cyanosis and 8 presented with tachypnea out of proportion to the degree of cyanosis. Ten of the 18 infants had no continuous murmur, bounding pulses, mid-diastolic rumble or differential cyanosis. Clinically occult narrowing or closure occurred, presumably gradually and relatively late, in six infants. Acute early narrowing or closure, spontaneous (six infants) or surgically produced (three infants), occurred usually within the 1st month of life and was associated with a marked decrease in arterial oxygen saturation in eight infants, often with a rapid clinical deterioration. Persistence of a large patent ductus arteriosus for several months appears to be associated with an increased incidence of early pulmonary vascular disease. Therapeutic considerations for the infant with a large patent ductus arteriosus after initial balloon atrial septostomy include: (1) careful initial follow-up of the infant in clinically stable condition in case the ductus arteriosus should acutely narrow or close and the patient require urgent palliative or corrective surgery; (2) urgent early closure of the ductus in the infant with overt left heart failure with concurrent atrial septectomy or preferably primary corrective surgery; and (3) elective closure of a persistent significant patent ductus arteriosus before age 4 months with concurrent corrective surgery in the infant in clinically stable condition.

The surgical treatment of congenital webbing (pterygium) of the popliteal area.

We describe a case of congenital popliteal pterygium in conjunction with other specific congenital malformations. This syndrome has been called the quadruple syndrome. The literature is reviewed. A rationale for more aggressive treatment of this deformity is discussed. One child who has undergone major multiple nerve grafts as a part of the primary corrective surgery is reported. Her result has been pleasing to the patient, the parents, and the surgeons.

Systemic hypertension and polycythemia in tetralogy of Fallot.

In our series of 111 cases of tetralogy of Fallot, the result revealed that cases with high Hb-content was accompanied more frequently by hypertension. However, their causes and effect relationship could not be clarified and more complicated mechanism was imagined to be acting.From this series, these conditions of high Hb-content, hypertension and positive urinary protein will be estimated to occur in a patient at about the age of 10 years with tetralogy of Fallot, but for surgical point of view, such conditions do not contraindicate primary corrective surgery for the disease. Palliative operations are also found to be preferable to improve these conditions. In patients of an advanced age with hypertension, careful examinations including renal biopsy will be helpfu 1 to make the final decision for surgery.

The roentgen spectrum in persistent common atrioventricular canal.

With the advent of corrective surgery in congenital heart disease, the differential diagnosis of cardiac abnormalities has become a matter of vital importance to the clinician and the radiologist. The group of lesions classified as persistent common atrioventricular canal defects or endocardial cushion defects represent a broad spectrum of clinical and anatomic abnormalities. In addition, especially with respect to the surgical results, the prognosis depends upon the actual anatomic and functional severity of the anomaly. A brief survey of the literature indicates a marked difference in prognosis between patients with an ostium primum defect in association with a cleft mitral valve and those with an ostium primum defect, a common atrioventricular canal, and a significant ventricular septal defect (Table I). Operative mortality in the first group varies between 7.5 and 22 per cent in several reported series (3–5, 8). If patients in the second group are subjected to primary corrective surgery, however, operative mortality may be as high as 67 per cent. It thus becomes extremely important to determine as accurately as possible the exact anatomic abnormality prior to surgical intervention. Classification We have subdivided our cases in a manner corresponding to the classification of Wakaiand Edwards (10), as follows (Table II): I. Partial form: An ostium primum type of interatrial septal defect in association with a cleft in the anterior leaflet of the mitral valve. Slight shortening of the septal leaflet of the tricuspid valve may be present. Usually there is no ventricular septal defect, although occasionally one which is small and functionally insignificant may be encountered. II. Intermediate form: An ostium primum interatrial septal defect with cleft mitral and tricuspid valves. Small interventricular septal defects are usual, but their size is much smaller than in the complete form. III. Complete form: A continuous cleft through both the anterior mitral and septal tricuspid leaflets in association with an ostium primum interatrial septal defect and a functionally significant ventricular septal defect. IV. Complete or partial forms: Associated pulmonic stenosis. Clinical Material We have seen 43 patients with persistent common atrioventricular canal defects, and in 33 of these, roentgen examinations were adequate. The patients ranged in age from two weeks to fifty-four years (Table III). Plain and Findings The diagnosis of persistent atrioventricular canal is ordinarily made at the time of the clinical examination, including electrocardiography. The electrocardiogram is usually quite characteristic, demonstrating evidence of left axis deviation in association with an rSR or right ventricular hypertrophy pattern in the right precordial leads (2, 7).