Sphincter saving anorectoplasty (SSARP) for the reconstruction of Anorectal malformations

<p>Abstract</p> <p>Background</p> <p>This report describes a new technique of sphincter saving anorectoplasty (SSARP) for the repair of anorectal malformations (ARM).</p> <p>Methods</p> <p>Twenty six males with high ARM were treated with SSARP. Preoperative localization of the center of the muscle complex is facilitated using real time sonography and computed tomography. A soft guide wire is inserted under image control which serves as the route for final pull through of bowel. The operative technique consists of a subcoccygeal approach to dissect the blind rectal pouch. The separation of the rectum from the fistulous communication followed by pull through of the bowel is performed through the same incision. The skin or the levators in the midline posteriorly are not divided. Postoperative anorectal function as assessed by clinical Wingspread scoring was judged as excellent, good, fair and poor. Older patients were examined for sensations of touch, pain, heat and cold in the circumanal skin and the perineum. Electromyography (EMG) was done to assess preoperative and postoperative integrity of external anal sphincter (EAS).</p> <p>Results</p> <p>The patients were separated in 2 groups. The first group, Group I (n = 10), were newborns in whom SSARP was performed as a primary procedure. The second group, Group II (n = 16), were children who underwent an initial colostomy followed by delayed SSARP. There were no operative complications. The follow up ranged from 4 months to 18 months. Group I patients have symmetric anal contraction to stimulation and strong squeeze on digital rectal examination with an average number of bowel movements per day was 3–5. In group II the rate of excellent and good scores was 81% (13/16). All patients have an appropriate size anus and regular bowel actions. There has been no rectal prolapse, or anal stricture. EAS activity and perineal proprioception were preserved postoperatively. Follow up computed tomogram showed central placement the pull through bowel in between the muscle complex.</p> <p>Conclusion</p> <p>The technique of SSARP allows safe and anatomical reconstruction in a significant proportion of patients with ARM's without the need to divide the levator plate and muscle complex. It preserves all the components contributing to superior faecal continence, and avoids the potential complications associated with the open posterior sagittal approach.</p

Anorectal malformations

Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage, are often associated with other anomalies, and have a poor functional prognosis. The surgical approach to repairing these defects changed dramatically in 1980 with the introduction of the posterior sagittal approach, which allowed surgeons to view the anatomy of these defects clearly, to repair them under direct vision, and to learn about the complex anatomic arrangement of the junction of rectum and genitourinary tract. Better imaging techniques, and a better knowledge of the anatomy and physiology of the pelvic structures at birth have refined diagnosis and initial management, and the analysis of large series of patients allows better prediction of associated anomalies and functional prognosis. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. With early diagnosis, management of associated anomalies and efficient meticulous surgical repair, patients have the best chance for a good functional outcome. Fecal and urinary incontinence can occur even with an excellent anatomic repair, due mainly to associated problems such as a poorly developed sacrum, deficient nerve supply, and spinal cord anomalies. For these patients, an effective bowel management program, including enema and dietary restrictions has been devised to improve their quality of life

Abordagem ventilatória protetora no tratamento da hérnia diafragmática congênita Gentle ventilatory approach for the treatment of congenital diaphragmatic hernia

OBJETIVO: Descrever a evolução de recém-nascidos com diagnóstico de hérnia diafragmática congênita admitidos na Unidade de Terapia Intensiva Neonatal de um hospital privado de nível terciário, no qual aplicou-se uma estratégia ventilatória protetora. MÉTODOS: Coorte histórica com análise de prontuários de pacientes portadores de hérnia diafragmática congênita, admitidos de junho de 2001 a julho de 2006. Avaliaram-se dados referentes ao recém-nascido (índices prognósticos antenatais, peso ao nascimento, idade gestacional, sexo), dados da reanimação e estabililização pré-operatória, cuidados pós-operatórios e taxa de sobrevida. RESULTADOS: Oito neonatos tiveram diagnóstico de hérnia diafragmática congênita. O peso variou entre 2,38 e 3,45kg e a idade gestacional, entre 36 e 39 semanas; cinco deles eram do sexo masculino. Todos foram intubados em sala de parto até o final do primeiro minuto de vida. A correção cirúrgica ocorreu entre o segundo e o sexto dias de vida e, em quatro pacientes, houve necessidade do uso de patch. Uma estratégia ventilatória protetora foi utilizada em seis neonatos, com dados gasométricos visando PaO2 pré-ductal normal e tolerando-se hipercapnia (PaCO2 50 a 60mmHg). A extubação ocorreu entre o primeiro e o 12ºdias do pós-operatório, com exceção de um paciente. Seis recém-nascidos receberam alta, em média, com 30 dias de vida (19 a 55 dias). A sobrevida foi de 75%. CONCLUSÕES: A sistematização do cuidado de pacientes com hérnia diafragmática congênita pode garantir, em nosso meio, uma sobrevida comparável aos principais centros mundiais que lidam com a doença.<br>OBJECTIVE: To describe the clinical evolution of newborns with congenital diaphragmatic hernia admitted to neoretal Intensive Care Unit of a tertiary private hospital and treated with a gentle ventilatory approach. METHODS: Analysis of charts of patients born between June 2001 and July 2006. The following data were collected: birth weight, gestational age, sex, delivery room procedures, pre and post-surgery parameters and survival rate. RESULTS: Eight newborns with diagnosis of congenital diaphragmatic hernia were included. They presented birth weight from 2.38 to 3.45kg, gestational age between 36 and 39 weeks; five of them were males. All infants were intubated at delivery within the first minute of life. The surgery was performed between the 2nd and the 6th days of life, and a patch was needed in four patients. A "gentle" ventilation strategy was used in six infants, targeting normal pre-ductal PaO2 and allowing hypercapnia (PaCO2 between 50 and 60mmHg). The extubation occurred between the 1st and 12th day after surgery, except for one infant who died. Six newborns were discharged with an average post-natal age of 30 days (19 to 55 days). The survival rate was 75%. CONCLUSIONS: The systematic care of infants with diagnosis of congenital diaphragmatic hernia can assure a survival rate comparable to reference centers