Case Presentation PK1 조 :: 조재완

Chief complaint For colostomy repair 이 O 원 M/7mo Via OPD

Patient’s illness 상환 7 개월된 남아로 출생시 37+1wks, 49.5cm, 2.94kg 이었으며 C-sec A/S 9/10 출생 환아임. 출생 당시 항문 - 직장기형을 동반한 multiple anomaly 로 본원 응급실 통해 내원하였으며 08 년 07 월 colostomy, 08 년 11 월 Pena OP 시행 받은 바 있고, 이번에 colostomy repair 위해 내원

Past history via EM  Abd. Trans lat. : Initial anus imperforate Foot X-ray : Lt. 배열이상, Rt – oligodactyly Hand X-ray : Lt. polydactyly, Rt. Syndactyly Abd U/S : –inf. Location of rectum –R/O fistula of urethara-rectum –Kidney, bladder normal config. Spine U/S : thickened filum terminale Chromosome analysis : normal karyote PS consult : polydactyly, oligodactyly, F/U after 1yr 6mo OP : colostomy, loop sigmoid

via OPD  Anal atresia & stenosis with recto-urethral fistula VCUG : vesicourethral reflux both (Rt.II, Lt. IV) Distal colostomy : imperforated anus with recto-periurethral fistula OP : : Pena OP

Family & Society histoy FHx : none Vaccination Hx –DTPx3, 소아마비 x3, 간염백신 x3, MMR, BCG

Physical exam V/S –HR : 124 –RR : 48 –BT : 36.8 Height : 76 cm (75%) Weight : 9.5 kg (75%)

Diagnosis Anal atresia & stenosis with recto-urethral fistula S/P colostomy (080716) S/P Pena OP (081127)

Plan Colostomy repair OP day : OP name : Colostomy repair

Progress note [POD #0] Subject : 열이 나요. Object : –HR : 154 회 / 분 –RR : 42 회 / 분 –BT : 38.8 ℃ –I/O : 1000/1033 (L-tube 0) Assessment : –Anal atresia & stenosis with recto-urethral fistula –S/P 3 stage OP –R/O Transfusional reacation –R/O Pul. atelecasis Plan : Chest percussion, CXR, Suspen sup.

Progress note [POD #1] Subject : 방귀 뀌었고 변도 봤어요. 자꾸 보채요. Object : –HR : 138 회 / 분 –RR : 40 회 / 분 –BT : 38 ℃ –I/O : 1300/1034 (L-tube 8 brownish color) –Gas out : + –Defication : 1 회 Assessment : –Anal atresia & stenosis with recto-urethral fistula –S/P 3 stage OP Plan : Pechidine, CXR

Progress note [POD #2] Subject : 많이 좋아졌어요. Object : –HR : 120 회 / 분 –RR : 40 회 / 분 –BT : 37 ℃ –I/O : 1200/990 (L-tube 0) –Defication : 3 회 Assessment : –Anal atresia & stenosis with recto-urethral fistula –S/P 3 stage OP Plan : PD & UR Follow up, L-tube remove

Book review Anorectal malformation

Epidemiology Incidence : 1 in 4000~5000 birth Male : Female = 56 : 44 Associated syndrome –VATER or VACTERL syndrome (Vertebral, Anal, Cardiac, Tracheo-esophageal, Renal and Limb anomaly) –Townes-Brocks syndrome –Currarino triad –FG syndrome –Cat-eye syndrome –Lowe syndrome

Classification Wingspread classification FemaleMale Anorectal agenesis with rectovaginal fistula without fistula Rectal atresia High Anorectal agenesis with rectoprostatic urethral fistula without fistula Rectal atresia Rectovestibular fistula Rectovaginal fistula Anal agenesis without fistula Intermediate Rectobular urethral fistula Anal agenesis without fistula Anovestibular fistula Anocutaneous fistula Anal stenosis Low Anocutaneous fistula Anal stenosis Cloaca Rare anomlay Rare anomaly

Classification Pena classification MaleFemale Low defects : Cutaneous fistula, anal stenosis, anal membrane, and ‘bucket handle’ malformation Rectourethral bulbar fistula Rectourethral prostatic fistula Rectovesical (bladder neck) fistula Anorectal agenesis without fistula Rectal atresia and stenosis Cutaneous (perineal) fistula Vesibular fistula Vaginal fistula Anorectal agenesis without fistula Rectal atresia and stenosis Persistent cloaca

Diagnosis Anatomy –Location of anorectum –Location of fistula Another anomaly Wangensteen invertogram Cross-table lateral x-ray Urine anlalysis Chest X-ray Abdominal & Pelvic X-ray Abdominal & Pelvic U/S VCUG Distal loopogram or dital colostogram CT & MRI

Treatment  Usually requires immediate surgerysurgery to open a passage for faeces M/ Low anomaly (at birth) –Cut-back anoplasty F/ Low anomaly (after 2~3mo) –Cut-back anoplasty or Jump-back anoplasty Intermediate & High anomaly 1. At birth : Colostomy 2. 3~6 mo : Posterior sagittal anorectoplasty, Pena OP 3. After 2~3 mo from Pena OP : Colostomy repair

Prognosis With a high lesion –Uncontrolling bowel function –Constipation With a low lesion –Good bowel control –Constipation Poor outcome for continence and constipation –Further surgery - Angle between the anus and the rectum Remove that dilated segment Malone stoma –An antegrade enema for appendix to the skin