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Hisprung Disease Ns. Eky Madyaning Nastiti, S.Kep

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  • Hisprung DiseaseNs. Eky Madyaning Nastiti, S.Kep

  • Hirschsprungs disease is the most common cause of lower intestinal obstruction in neonates.Hirschsprungs disease (aganglionic megacolon) is a congenital anomaly caused by migratory failure of neural crest cells leading to abnormal innervations of the bowel (plexus aurbach)The defect begins in the internal anal sphincter and extends proximally for a variable length of gut.

  • Incidence & EtiologyINCIDENCE : 1\5000 live birth newborn 70-80% is boys. (M / F 4: 1 )Less common in blacks

  • PATHOPHYSIOLOGYThe fundamental pathology in HD is the absence of ganglion cells in the submucosal and intermuscular nerve plexuses and is associated with an increase in the nerve fibers in the affected segment .That aganglionic segment usually involves the terminal intestine, i.e. the rectum or rectosigmoid. The aganglionic segment may, however, include the entire large bowel and even small bowel.The gross pathologic feature of HD is a dilated proximal intestine with gradual or abrupt transition to normal calibrated distal intestine . The TZ is typically funnel like or cone shaped .The colon proximal to the aganglionic segment, in an effort to overcome the partial obstruction, becomes distended and its wall markedly thickened because of muscle hypertrophyThe degree of hypertrophy and dilatation depends upon the duration and degree of obstruction and thus, indirectly to the age of the patient.

  • TYPES.

    Congenital : This type is the commonest one .Etiology of the disease is still unknown.but Genetic factors are now identified.10% of cases have familial history, especially those with long segment disease.Acquired : Degeneration of the ganglions may occur due to:-Vascular causes like after pullthrough procedure due to ischemia & tension. - Non vascular causes like Trypanosoma (chaga's disease). Vit B1 def. Chronic infection ( TB.).

  • ASSOCIATED ANOMALIES

    HD is usually a solitary anomaly in a full term, otherwise healthy infantAssociated anomalies do occur in nearly 20% of cases urogenital system (11%)cardiovascular system (6%) gastrointestinal system (6%), with 8% having various other malformations

    Prematurity is reported in as many as 10% of those children with HD Trisomy 21 occurs in approximately 5% of cases

  • CLINICAL PRESENTATIONS :Failure to pass meconium in the 1st 24h of life98% of neonates pass meconium in the first 24 hours of age. Any newborn who fails to pass meconium in the first 24-48 hours of life should be evaluated for possible Hirschsprung's disease.Neonatal Intestinal obstructionsymptoms include bilious vomiting, abdominal distension and refusal to feed.Recurrent Enterocolitis mainly in the 1st three months of life.TOXIC MEGACOLON :Fever. Abdominal distension.Bile stained vomitous. Explosive diarrhoea.Dehydration.Shock.Spontanous perforation occurs in 3%,specially if long segment aganglionosis.Chronic constipation patients may have chronic constipation in response to changes in feeding. And may have Growth retardation. Multiple fecal masses on abdominal examination.

  • AnamnesisPada neonatus :1.Mekonium keluar terlambat, > 24 jam2.Tidak dapat buang air besar dalam waktu 24-48 jam setelah lahir3.Perut cembung dan tegang4.Muntah5.Feses encerPada anak :1.Konstipasi kronis2.Failure to thrive (gagal tumbuh)3.Berat badan tidak bertambah4.Nafsu makan tidak ada (anoreksia)Pemeriksaan FisikPada inspeksi abdomen terlihat perut cembung atau membuncit seluruhnya, didapatkan perut lunak hingga tegang pada palpasi, bising usus melemah atau jarang. Pada pemeriksaan colok dubur terasa ujung jari terjepit lumen rektum yang sempit dan sewaktu jari ditarik keluar maka feses akan menyemprot keluar dalam jumlah yang banyak dan kemudian kembung pada perut menghilang untuk sementara.

  • Management :

    Manegement of HD differs according to the presentation form and clinical situation of the patients:Acute I.O. : if the patient presents with acute intestinal obstruction in the early life the management will beresuscitation ,NGT , NPOIVF ,Antibiotics ,Rectal tube,irrigations .The initial treatment requires performing a "leveling" colostomy in the most distal colon with ganglion cells present. This requires exploration with multiple seromuscular biopsies of the colon wall to determine the exact extend of the aganglionosis. The colostomy is placed above the transition zone. Placement of the colostomy in an area of aganglionosis will lead to persistent obstructionWhen the patient becomes stable, then the definitive treatment will be planned.Chronic constipation :laxative saline enema.Work up to establish the diagnosisthen the definitive treatment will be planned

  • Definitive procedures:

    Once the child has reached an adequate size and age (6-12 months; 20 pounds or more), a formal pull-through procedure is done Open surgery :There are many surgical options for Pull-through operation. All aiming at resection of aganglionic segment and anastomosing the two normal ganglionic ends. They give excellent result in 90%.a.swenson.b.soave. c.Rehbein.d. Duhamel.e. Boley's. LAPAROSCOPY .

    TEPT transanal endorectal pullthrough (without laparotomy )

  • Tindakan SwensonTindakan Duhamel