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Case Presentation and Discussion on Imperforate Anus

Marlou O. Padua, MD


General Data:  M. I., 3 day old, male

Chief Complaint:  Absent anal opening

History of Present Illness:

    Born to a 22 y.o. G1P1 via NSD at home assisted by a ‘hilot’

    24th HOL               (-) passage of meconium

                                (-) urine output

                                (+) vomiting

                                 noted absent anal opening

                                 brought to Ospital ng Muntinlupa

                                         - OGT

                                         - Invertogram done

                                         - Ampicillin, Gentamycin

     78th HOL                 transferred to OMMC

Maternal History: unremarkable

Physical Examination:

Gen. Survey: asleep, comfortable

Vital Signs:  CR: 118/min          RR: 34/min             T:37.1 oC

HEENT: normocephalic, patent non-bulging ant. & posterior fontanelles, pink palpebral conjunctivae, anicteric sclerae, supple neck, OGT inserted

Chest/Lungs: symmetrical chest expansion, no retractions, clear breath sounds

CVS: adynamic precordium, normal rate, regular rhythm, no murmur

Abdomen:  globular, dry umbilical stump, hypoactive bowel sounds, soft

Genitalia: undescended testes R

Perineum:  (-) fistula, (+) midline groove, (+) prominent anal dimple

 

Invertogram:

 

 

 

 

 

 

 

 

 

Salient Features:

•  Newborn, male

•  (-) anal opening

•  (-) perineal fistula

•  Invertogram result

 

Clinical Diagnosis:

Primary Clinical Diagnosis

Imperforate Anus, High Lying

90% Certainty

Secondary Clinical Diagnosis

Imperforate Anus, Low Lying

10%  Certainty

 

Algorithm:

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Paraclinical Diagnostic Procedure:

Is there a need for a further paraclinical diagnostic procedure?

No, since the degree of certainty for my primary diagnosis is already 90%.

 

Paraclinical Diagnostic Options:

  Benefit Risk

Cost

Availability
Invertogram will differentiate low from high lying imperforate anus exposure to radiation Php 300
Cross-table Lateral, Prone position with Pelvis elevated will differentiate low from high lying imperforate anus exposure to radiation Php 300

 

Pre-Treatment Diagnosis:

Primary Clinical Diagnosis

Imperforate Anus, High Lying

90% Certainty

Secondary Clinical Diagnosis

Imperforate Anus, Low Lying

10%  Certainty

 

Goals of Treatment:

•   Decompress the bowel obstruction by diverting the flow thru a colostomy.

•   Create a temporary outlet of feces while prior to anoplasty

•   Provides protection during the healing of subsequent total repair (anoplasty).

 

Treatment Options:

  Benefit Risk

Cost

Availability

Sigmoid Colostomy

provide decompression

relatively short segment of defunctonalized distal colon

easier mechanical cleansing of distal colon prior to definitive repair

wound infection

necrosis colostomy

Php 4,000
Transverse Colostomy

provide decompression

unformed stool leading to mashing of  skin Php 4,000
Minimal PSARP

provide decompression

one stage procedure

damage to nerve seminal vesicles and prostate Php 4,000

 

Plan of Operation:

 Diverting Sigmoid Colostomy

 

Pre-operative Preparation:

•  Informed consent obtained

     -  Explain the diagnosis and proposed treatment

     -  Explain the need to perform colostomy               

•  Provide psychosocial support to allay fear and anxiety of parents

      -  Questions regarding survival, prognosis and life with a colostomy

•   Preoperative Preparation

       -  Hydration : D10 0.3 NaCl 60cc X 6h

       -  Thermoregulation 

        -  Antiobiotics

                Ampicillin 125mg TIV q12

                Gentamycin 12mg TIV OD

        -  Decompression

        -  OGT inserted

•  Screening for other health problems

        -  CXR

 

Intra-operative Management:

•   Position of patient - Supine

•   Incision

 

 

 

 

 

•  Sigmoid Colostomy

 1.                                              2.

 

 

 

 

3.                                                4.

 

 

 

 

 

•   Meconium evacuated

•  Serosal tear repaired

•   Hemostasis check

 • Correct count

  

Intra-operative Findings:

•  Well formed midline groove with prominent anal dimple with no bulge.  No fistula noted on the perineal area. 

 •  On doing LLQ transverse incision, sigmoid noted to be dilated (5cm widest diameter) containing meconium with serosal tear

 

Post-operative Diagnosis:  Imperforate Anus, High Lying

 

Post-operative Care:

•  Maintain on NPO/OGT

 •  IVF continued

•  Ampicillin and Gentamycin continued,  Metronidazole 18mg TIV q12 started

•   Thermoregulation

•   1st POD- (+) colostomy output

•   2nd POD - started breast feeding

•   3rd POD - transferred to Pedia Ward for nutritional build-up

 

Follow-up Plan:

•   Daily follow up of colostomy for signs of infection

•   Other wok-ups - Ultrasound of KUB,  2D Echo

•   Parents advised colostomy care and scheduled definitive management after 8 weeks

 

Outcome:

•   Resolution of the obstruction due to imperforate anus

•   Live patient

•   No complications

•   Satisfied patient

•   No medico-legal suit

 

Discussion:

 Types of Anorectal Malformation

 •   Classification of Anorectal Malformation in Male

  Malformation

Colostomy Required

Male

Cutaneous Fistula

No
 

Rectourethral Fistula

        Bulbar

        Prostatic

 

Yes

Yes

  Rectovesical Fistula

Yes

 

Anorectal Agenesis w/o Fistula

Yes

 

 

Rectal Atresia

Yes

 

 

 

 

 

 

 

                Perineal Fistula                             Rectourethrobulbar Fistula

 

 

 

 

 

 

       Rectourethroprostatic Fistula                     Rectobladder neck Fistula

 

Anorectal Agenesis w/o fistula – rectum ends 1-2 cm from perineal skin

          -  50% of cases seen in Down’s syndrome

          -  90% of Down syndrome w/ imperforate anus suffer from this defect

 

Rectal Atresia – extremely unusual

          -  Lumen of rectum totally (atresia) or partially (stenosis) interrupted

          -  Lower portion represented by small anal canal, 1 to 2 cm deep

 

Treatment

High Lying (>1cm bowel skin distance) –  Diverting Colostomy

Low  Lying (<1cm bowel skin distance) – Perineal Anoplasty

 

Questions:

 

Answer Key:

 

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Last modified: 10/31/04