SURGICAL EMERGENCIES IN THE NEWBORN

SURGICAL EMERGENCIES IN THE NEWBORN
TRACY L. MCTIERNAN, MA, CPNP DIVISION OF PEDIATRIC SURGERY NYU LANGONE MEDICAL CENTER

WHAT ARE WE TALKING ABOUT?
Congenital Diaphragmatic Hernia Esophageal Atresia Congenital Intestinal Obstruction Duodenal Atresia Ileal and Jejunal Atresia Meconium Ileus Malrotation and Volvulus Hirschsprung’s Disease Imperforate Anus Abdominal Wall Defects Omphalocele Gastroschisis

Congenital Diaphragmatic Hernia
Incidence Estimated to be between 1/2000 to 5000 live births 1/3 of infants are stillborn Males > females in live births Approximately 80% are on the left side Occurrence risk in a 1st degree relative ~2%

Etiology Associated Anomalies Cause of CDH is unknown
Increasing evidence that it may be related to exposure to environmental factors Associated Anomalies Incidence ranges from 10-50% Skeletal defects ~32% Cardiac anomalies ~24% Tracheobronchial tree ~18% Insecticides and drugs such as phenmetrazine, thalidomide, quinine, cadmium, lead and nitrofen

Pathology In most cases the defect is established by gestational week 12 Classic left sided CDH features a 2-4 cm posterolateral defect Herniated contents often include the left lobe of the liver, the spleen, and almost the entire GI tract Long term compression of the developing fetal lungs results in pulmonary maldevelopment and lung hypoplasia All the major bronchial buds are present in the CDH lung but the number of bronchial branches is greatly reduced Alveolar development is also severely affected

Pathology Pulmonary vascular bed is abnormal, resulting in increased pulmonary vascular resistance Increasing hypoxia results in increasing right-to left shunting leading to severe and progressive respiratory failure

Diagnosis Prenatal ultrasound is accurate in 40-90% of cases
Mean gestational age is 24 weeks with some discovered as early as 11 weeks Polyhydramnios is present in up to 80% of cases After birth the respiratory symptoms are determined by the degree of pulmonary hypoplasia and reactive pulmonary hypertension

Diagnosis Most severely affected infants develop respiratory distress at birth Majority demonstrate symptoms within the first 24 hours Infants will have scaphoid abdomen and symmetrically distended chest Tracheal deviation away from the defect Confirmed by plain chest radiograph which demonstrates loops of bowel in the chest

Management Key to consider that CDH is a physiologic emergency NOT a surgical emergency Early surgery further decreases lung compliance The post birth transition of vascular and pulmonary function is prolonged in CDH In theory, delayed surgery provides additional time for this transition to occur resulting in a more stable infant Infants should be intubated and an nasogastric tube passed Ventilation by mask or Ambu bag is contraindicated May be managed on a conventional ventilator or a high frequency ventilator

Management Key to success is currently thought to be gentle ventilation with permissive hypercapnea to reduce barotrauma Preductal pulseoximetry should be monitored Metabolic acid base disturbances should be corrected with fluid management or bicarbonate administration ECMO in severe cases - but often NOT necessary

Surgical Management Timing of surgery is dependent on the infants condition, ideally anywhere from 5 days to 2 weeks of age Infant’s ventilator settings are improving and being weaned Surgical approach is usually through a subcostal incision. Laparoscopic and thoracoscopic techniques can also be used Abdominal viscera are gently reduced into the abdominal cavity Defect is closed primarily or utilizing a patch Abdominal wall is closed if possible avoiding increased intra-abdominal pressure

Outcomes Survival rates as high as 80-90% are being reached with current treatment modalities High variation in survival rates among institutions represent different treatment strategies Associated anomalies such as congenital heart disease remain a significant risk factor for poor outcome

Esophageal Atresia and Tracheoesophageal Fistula
Incidence EA and TEF are relatively common congenital anomalies 1 in 4500 live births in the US Male=Female 0.5% - 2% increase risk in newborns with one affected sibling Risk increases to 20% if more than one sibling is affected

Esophageal atresia rarely occurs as an isolated congenital anomaly.
Classification Esophageal atresia rarely occurs as an isolated congenital anomaly. Esophageal atresia alone is due to failure of the recanalization of the esophagus during the 8th week of gestation Gross Classification

85% are type C (Distal TEF)
7% are type A (Pure atresia) 4% are type E ( H-type fistula) Proximal fistula is the least common

Associated Anomalies VACTERL: vertebral, anal, cardiac, TE, renal, limb
Congenital heart disease is associated with higher mortality VSD is most common ASD Tetralogy of Fallot PDA Echocardiogram – important to determine position of aortic arch

Associated Anomalies Vertebral and radial anomalies will be seen on x-ray Sonogram of the kidneys Physical Exam of the anus

Clinical Features Prenatally
Predictive value of prenatal ultrasound ~20-40% Polyhydramnios (2/3 cases) Small or absent stomach bubble Postnatally- Most infants are symptomatic within the first few hours of life Excessive salivation Regurgitation of first feed Cyanosis with/without feeds Respiratory distress Inability to pass a catheter into the stomach Gastric distention (with distal fistula)

Diagnosis Failure to pass NG tube (not Type E)
CXR- tube coiled in upper esophagus “Pouchogram” with air Distal air on AXR confirms the presence of a fistula H-type fistulas are often diagnosed later H-type fistulas may present with recurrent pneumonia

Diagnosis Confirm with AP chest x-ray that demonstrates the catheter curled in the upper esophageal pouch. Abdominal XR can help distinguish esophageal blind pouch (no gastric air) from distal TEF (gastric air)

Attempt to pass catheter into stomach. Cannot pass more than 10-15 cm.
Diagnosis Attempt to pass catheter into stomach. Cannot pass more than cm.

Diagnosis When diagnosis is uncertain or proximal TEF is suspected, a small amount of water-soluble contrast material can be injected into the esophageal pouch under fluoroscopic guidance (must remove contrast material immediately to avoid regurgitation and aspiration)

Proximal Esophageal Atresia
Diagnosis Proximal Esophageal Atresia

Diagnosis- Type E “H type” TEF
This newborn had feeding difficulty and respiratory distress after birth. A chest x-ray is shown above. The oro-gastric tube tip is consistent with a location in the right lower lobe bronchus. There is consolidation of the right lung. There is gas in the intestinal tract. The findings are consistent with a double tracheo-esophageal fistula or an H-type fistula. Exploration and surgical correction demonstrated a double tracheo-esophageal fistula. Patients with H- Type TEFs may present early if the defect is large, with coughing and choking associated with feeding as the milk is aspirated through the fistula. However, smaller defects of this type may not be symptomatic in the newborn period. Delay in diagnosis ranged from 26 days to 4 years.

Management Preoperatively Minimize complications from aspiration
Suction blind pouch continuously with Replogle tube NPO/TPN Upright position of child Early surgery for short gap atresia Long gap atresia may be delayed up to 6-12 weeks Long gap atresia should have gastrostomy tube placed for enteral nutrition

Management Surgery Surgical ligation of the fistula
Primary anastomosis of the esophageal segments Primary repair may not be possible if the distance between esophageal segments is long. Staged procedures have been performed that include elongation of the esophagus with circular myotomy, interposition of the colon, and gastric transposition

To repair esophageal atresia, a thoracotomy incision is made (A)
To repair esophageal atresia, a thoracotomy incision is made (A). The proximal and distal esophageal segments are identified (B). The distal fistula is transected (C) and anastomosed to the upper esophageal pouch (D). (With H-type fistula a cervical approach can be used in most cases)

Post Operative Management
Adequate fluid resuscitation Wean ventilator No deep tracheal suctioning Avoid bag/mask ventilation No pacifiers/sucking Chest tube remains in place for approximately one week Replogle tube (10 Fr) remains in place +/- one week Esophagram 1 week post op No reintubation

Infants with increased risk of morbidity and mortality
Outcome PROGNOSIS 85% to 95% survival Infants with increased risk of morbidity and mortality Low birth weight (>1500) Major CHD Severe associated anomalies Ventilator dependency Long gap atresia

Other Associated Issues
Outcome Complications Anastomotic Leak Esophageal stricture Recurrent fistula Other Associated Issues Tracheomalacia Disturbed peristalsis and delayed gastric emptying are common “Seal Bark” cough Gastroesophageal reflux and aspiration

Congenital Intestinal Obstruction
Intestinal Atresia Duodenal atresia Ileal and jejunal atresia Meconium Ileus Malrotation and Volvulus Hirschspung’s Disease Imperforate Anus

Duodenal Atresia Incidence
Most common site of neonatal intestinal obstruction 1 in 6,000 to 10,000 live births 75% of stenoses and 40% of atresias are found in Duodenum Multiple atresias in 15% of cases 50% pts are LBW and premature

Etiology No specific genetic abnormality
Increase incidence in siblings Has been shown to occur in several generations of a family Association with Trisomy 21 Suggestive of a genetic link

Associated Anomalies Down Syndrome 28% Annular pancreas 23%
Congenital heart disease 23% Malrotation 20% Esophageal atresia/TEF 9% Genitourinary 8% Anorectal 4% Other bowel atresia 4% None 45%

Pathology Type I: The most common type is formed by a membrane composed of mucosa and submucosa and obstructs the lumen. A variation is the windsock deformity. Type II: The atretic ends of the duodenum are connected by a fibrous cord. Type III: Complete separation of the atretic segments. Most biliary duct anomalies are associated with this type.

Diagnosis Prenatally Postnatally Diagnosed in 32-57% of patients
Dilated stomach bubble apparent by 3rd trimester Polyhydramnios in 32-59% of cases Postnatally Symptoms usually appear within the first 24 hours Recognition of partial obstruction can be delayed Repeated bilious emesis is characteristic – 85% Bilious emesis is a surgical emergency until proven otherwise Nonbilious emesis is present when the atresia is above the level where the bile duct enters the duodenum (papilla of Vater)

Diagnosis Radiologic studies
Plain radiograph of the abdomen will generally confirm the diagnosis with a finding of the “double bubble sign Upper GI series or barium enema may be helpful to differentiate from midgut volvulus

Management Replogle tube (10 Fr) Intravenous fluid resuscitation
Electrolyte correction PICC line may be considered If midgut volvulus has been ruled out, surgical correction is not urgent Surgery performed is a duodenoduodenostomy or duodenojejunostomy Can be performed laparoscopically

Post-operative Management
NPO Replogle tube (10 Fr) IV fluids /hyperalimentation No feeds until return of bowel function

Complications Early postoperative complications can be related to:
Prematurity Coexisting congenital anomalies Parenteral nutrition Anastomotic obstruction/stenosis Anastomotic leak Adhesions Prolonged feeding intolerance Duodenal dysmotility

Outcomes Current survival rates are 90-95%
Higher mortality rates are associated with prematurity and multiple congenital anomalies Postoperative complications are reported to be 14-18%

Jejunoileal Atresia Incidence
Has been reported to be as high as 1/3000 live births in the US Wide variation in the reported incidence Males = females 1/3 to ¼ of infants are low birth weight Higher incidence in African-American infants Increased risk with maternal use of pseudoephedrine alone and in combination with acetaminophen Increased in mothers with migraine headaches receiving ergotamine tartrate and caffeine

Etiology Cause is unknown
Most likely associated with a late intrauterine mesenteric vascular catastrophe Has been associated with volvulus, intussusception, internal hernia and constriction of the mesentery in a tight gastroschisis or omphalocele

Associated anomalies Gastroschisis/Omphalocele Ascites Cystic fibrosis
Malrotation and volvulus Genitourinary

Pathology Equally distributed between the jejunum (51%) and the ileum (49%) Atresia is usually single (>90%) Multiple atresias more often involve the proximal jejunum Currently 5 classifications

Type I: Mucosal atresia with intact bowel and mesenetery
TypeII: Blind ends separated by a fibrous cord Type III(a): Blind ends separated by a V-shape mesenteric defect Type III(b): Apple-peel atresia Type IV: Multiple atresias (string of sausages)

Diagnosis Clinical Signs Radiologic Studies
Polyhydramnios - more commonly seen in proximal atresias Bilious emesis – SURGICAL EMERGENCY UNTIL PROVEN OTHERWISE Abdominal distension Jaundice Failure to pass meconium Radiologic Studies Supine and erect abdominal films Contrast enema or UGI 3 purposes of the contrast enema: 1) distinguish between small and large bowel, 2) to look for microcolon, 3) to locate the position of the cecum

Management Replogle tube (10 Fr) to continuous suction
Intravenous fluids Electrolyte correction PICC line placement Operative procedure depends on defect May require multiple anastomoses May require ostomy May require tapering of proximally dilated intestine

Postoperative Management
IV fluid resuscitation NPO Replogle (10 Fr) to suction Clear liquids with return of bowel function Advance to formula- medium chain triglyceride or casein hydrolysate formulas should be offered Malabsorption and diarrhea are common in infants: With short bowel length In whom the ileocecal valve has been resected With multiple atresias Apple peel atresia

Outcomes Overall survival rates for jejunoileal atresia are reported to range from 80-90% Most common cause of early death Pneumonia Peritonitis Sepsis Postoperative complications Intestinal obstruction at the anastomosis Anastomotic leak

Outcomes Factors affecting morbidity and mortality
Associated anomalies Short bowel syndrome Prematurity Respiratory distress

Meconium Ileus Incidence Almost always associated with cystic fibrosis
Reported to be the presenting symptom in 15-20% of cases Incidence is reported to be 30-40% in all patients with cystic fibrosis

Pathology Simple meconium ileus
Small intestine mucous glands produce overly thick secretions Meconium formed is abnormally viscid, sticky and adherent Proximal ileum dilated with thick, sticky meconium Distal ileum and colon are collapsed and obstructed by thickly packed round mucous plugs – rabbit pellets

Complicated meconium ileus
In utero dilated proximal intestine volvulizes Early in gestation may cause one or more atresias Late in gestation infants may present with perforation -> meconium peritonitis

Associated Anomalies Cystic fibrosis

Diagnosis Prenatally Postnatally Prenatal history of cystic fibrosis
Polyhydramnios is reported in ~20% of mothers – more common finding in complicated meconium ileus Postnatally Intestinal obstruction is evident hours after birth -> abdominal distention -> bilious emesis -> failure to pass meconium within 48 hours Complicated meconium ileus presents at or shortly after birth -> severe abdominal distension -> abdominal wall may be red and inflamed -> extremely ill appearing infant

Radiologic Studies – Simple MI
Plain radiographs Varying sized loops of distended bowel Absence of air fluid levels Soap bubble appearance particularly in the right lower quadrant

Contrast Enema Water soluble contrast Microcolon
Pellet-like meconium when contrast is refluxed into the terminal ileum Curative in 30% of patients

Radiologic studies – Complicated MI
Plain film Areas of calcification (calcified meconium) Large dense mass with a rim of calcification – cystic meconium peritonitis Intraperitoneal calcifications can occur within 4 days of perforation Contrast enema may not be necessary

Management – Simple MI Aggressive fluid resuscitation
Naso/orogastric tube to suction Hyperosmolar contrast enema (Gastrografin) May be curative in 30-60% of patients Goal of surgery is to completely evacuate the obstructing plugs and meconium Enterotomy and irrigation Intestinal resection and primary anastomosis Ostomy may be necessary

Management – Complictated MI
Typically a much sicker infant Aggressive fluid resuscitation Electrolyte correction Findings at surgery dictate the procedure More likely to have an ostomy

Outcomes Current 5 year survival is approaching 100%
72% survival at 10 years Current operative mortality is reported to be 10-20% Current life expectancy for patients with cystic fibrosis is 35 years Complications are related to the CF Most common gastrointestinal problem in patients with CF is distal intestinal obstruction syndrome ¼ of patients will develop gallstones Intussusception occurs in 1% Rectal prolapse develops in 1/3 to 1/5 of patients Colonic strictures secondary to high dose oral enzyme therapy Intestinal obstruction syndrome may be a result of steatorrhea or noncompliance with oral enzyme therapy

Intestinal Malrotation and Volvulus
Incidence Occurs in 1/500 live births Male to female ration is 2:1 in neonatal presentation No sexual predilection in patients over 1 year As many as 40% present within the first week 50% present by 1 month 75% present by 1 year

Etiology Rotation and fixation of the intestine takes place during the first 3 months of gestation As rotation begins, the intestine moves outside the abdomen At 10 weeks of gestastional age the intestine returns to the abdomen Normal mesenteric attachment extends from the ligament of Treitz to the cecum Ascending and descending colon are fixed retroperitoneally

Etiology Malrotation is when the normal process of rotation is arrested or deviated at various stages Anomalous fixation may also occur Dense fibrous bands extending from the cecum and right colon across the duodenum to the retroperitoneum may form – Ladd’s Bands

Pathology Midgut may be supported by a narrow pedicle that contains the entire blood supply Narrow pedicle predisposes the bowel to a clockwise twisting from the duodenum to the transverse colon Distension and peristalsis may initiate torsion of the intestine on the pedicle - Volvulus Acute midgut volvulus occurs when the blood supply to the midgut is disrupted by the torsion Vascular obstruction and necrosis of the complete midgut develops rapidly Ladd’s Bands can cause a mechanical duodenal obstruction without vascular compromise

Associated Anomalies Are found in 30 to 60% of patients
Malrotation is almost always associated with Diaphragmatic hernia Omphalocele/gastroschisis Prune belly syndrome Can be associated with Duodenal atresia Ileal atresia Meconium ileus Congenital heart disease Imperforate anus Annular pancreas Biliary atresia

Diagnosis Clinical symptoms Radiologic studies
Most frequent symptom is bilious emesis Pain and irritability in the toddler or older child Abdomen is soft and non-tender to palpation initially Becomes distended and tender with strangulation of bowel Stool may be bloodstained Radiologic studies Abdominal radiographs may show a dilated duodenem with a fluid level, however can be read as normal in 20% of cases Barium enema is unreliable – position of cecum varies

Diagnosis Radiologic studies
Upper GI series with small bowel follow through is the most reliable – 96% specificity in one report Dilated duodenum with a typical corkscrew appearance Absence of Ligament of Treitz Small bowel on right side of abdomen UGI may be misleading if duodenal obstruction is complete (also seen with duodenal atresia)

Management Once the diagnosis is made on a symptomatic child, the patient should be taken to the operating room immediately Ladd’s procedure is performed Derotation of the bowel Division of the Ladd’s bands if present Widening the small bowel mesentary by lysis of congenital adhesions Return the bowel to a position of nonrotation Appendectomy

Management If ischemic bowel is present every attempt to preserve bowel length should be made Bowel is not surgically fixed into position Procedure may also be done laparoscopically

Outcomes Mortality rate with midgut volvulus is at least 15%
Return of bowel function is dependent upon duration of obstruction and extent of compromise High incidence of short gut syndrome Recurrent obstructions are rare Higher incidence of intussusception post op than with laparotomies for other reasons ~3%

Hirschsprung’s Disease
Incidence Ranges from 1/4400 to 1/7000 live births Male to female ratio 4:1 Long segment disease ratio approaches 1:1 but may actually favor females Incidence increases to 6% for siblings of affected children Brothers and sons of affected females have the greatest risk (24% and 29%, respectively)

Etiology Cause of Hirschsprung’s Disease is unknown
Features of the disease are suggestive of a complex pattern of inheritance RET gene mutation was found in~ 35% of sporadic cases and 49% of familial cases

Associated Anomalies Trisomy 21 has been reported in 4-16% of cases
Large and small bowel atresia Anorectal atresia Ondine’s curse

Pathology Absence of ganglion cells in the distal intestine is the hallmark of the disease Ganglion cells are absent in the submucosal plexus and intermuscular plexus Rectosigmoid region in ~80% of cases Aganglionosis is almost always continuous distally Long segment disease has been reported 11-26% of cases Total colonic 3-12% Small bowel 0.4-3%

Diagnosis Clinical findings Delayed passage of meconium within the first 48 hours of life 95 % of full term infants stool within the first 24 hours Remainder will stool by 48 hours This history may be absent in 6 to 42% of patients Constipation Abdominal distension Failure to thrive Hirschsprung’s Disease should be considered in any child who has a history of constipation dating back to the newborn period

Diagnosis Radiologic studies Supine and positional abdominal radiographs - distended loops with paucity of air in the rectum Contrast Enema with water soluble contrast – classic finding is a narrow spastic distal segment with a dilated proximal segment Point of caliber change or transition zone is the key radiographic finding Diagnostic accuracy of contrast enema has been reported to be 76% to 92% In any infant with a suspected diagnosis of Hirschspung’s Disease no rectal stimulation of any kind should be performed

Diagnosis Rectal biopsy is the gold standard May be done at the bedside Accuracy of suction rectal biopsy has been reported as high as 99.7% Major problem is inadequate specimen Full thickness rectal biopsy may be preformed in the operating room

Management Primary pull-through procedure Transanal approach for normal segment disease Intraoperative biopsies are performed to confirm the transition zone Procedure may be performed with laparoscopic assistance Older children may require a colostomy first with the definitive procedure performed several weeks later

Outcome Early Complications Intestinal obstruction 8-13% Wound infection 10-15% Anorectal stenosis 10-20% Anastomotic leak 2% Late Complications Incontinence 3-8% - greatest impact on quality of life Constipation reported to range from 6-30% Hirschsprung’s enterocolitis Major cause of morbidity and mortality Incidence ranges from 20-30% Cause is unknown but some feel it is related to persistent state of colonic and small bowel stasis which leads to bacterial overgrowth

Outcome Hirschsprung’s enterocolitis Manifested by explosive diarrhea, abdominal distension and fever Treatment is with aggressive washout with a rectal tube, IV antibiotics or oral metronidazole Quality of life is reasonably good and significantly better when compared to patients with other anorectal malformations

Imperforate Anus Incidence
Average incidence worldwide is believed to be 1 in 5000 live births There is a slight male preponderance Males are twice as likely to have high imperforate anus Some families have a genetic predisposition Most common in females – rectovestibular fistula Most common in males – rectourethral fistula Imperforate anus without fistula 5% of patients and half of these patients have Down’s syndrome Cause is unknown

Associated Anomalies VACTERL syndrome 50-60% of patients will have one or more anomalies ~ 1/3 will have congenital heart disease ASD PDA Tetrology of Fallot VSD Gastrointestinal Tracheoesophageal abnormalities 10% Duodenal obstruction 1-2%

Associated Anomalies Spinal and vertebral 1/3 to ½ of patients Tethered cord is most common Spinal lipomas Syringomelia Myelomemingocele Hemivertebrae Scoliosis Butterfly vertebrae Hemisacrum

Associated Anomalies Genitourinary Incidence ranges from 1/3 to 1/2 of patients Incidence increases with increasing complexity of malformation Vesicoureteral reflux is most common Renal agnenesis and dysplasia Cryptochidism 3-19% Hypospadias 5% Gynecologic Hydrocolpos in the newborn period Mullarian anomalies leading to menstrual difficulties Uterine abnormalities Vaginal anomalies

Deformities are divided into high, intermediate and low
Pathology Deformities are divided into high, intermediate and low Classification depends on how far the rectum is descended into the pelvis and whether there is a fistula Patients will have varying degrees of striated muscle development from normal appearing to no muscle at all Majority of patients are born without an anal canal and, consequently, can not discriminate between liquid and gas after surgical repair Patients will have a spectrum of rectosigmoid motility disorders which can lead to constipation issues Fistula in girls to the vagina, boys the urinary tract

Infants should be given sufficient time to pass meconium
Diagnosis All suspected cases of anorectal malformation require a thorough perineal examination Infants should be given sufficient time to pass meconium Two positional radiographs may be helpful Invertograms are not useful Xrays can help determine the degree of obstruction and possible location of the rectum

Figure 341-3 Imperforate anus in females. A, Vestibular fistula
Figure Imperforate anus in females. A, Vestibular fistula. B, Cloaca. (From Peña A: Atlas of Surgical Management of Anorectal Malformations. Springer-Verlag, New York, 1989, pp 50, 60.)

Figure Imperforate anus in males. A, Low lesions. B, High lesions. (From Peña A: Atlas of Surgical Management of Anorectal Malformations. Springer-Verlag, New York, 1989, pp 7, 26.)

Management IV fluids Replogle tube (10 Fr) Workup for VATER syndrome Echocardiogram Dedicated spinal films/spinal ultrasound Renal ultrasound

Single perineal orifice
Newborn Female Perineal Inspection Single perineal orifice Cloaca No visible fistula Perineal fistula Vestibular fistula Rectum below coccyx High rectum Anoplasty or Dilatations Colostomy Primary repair Colostomy Adapted from Pena, A, Levitt, M: Anorectal Malformations. In Grossfeld, J, O’Neill, J, et al (ed) Pediatric Surgery. 6th Ed.

Perineal Inspection Perineal fistula Anoplasty Newborn Male
Rectal gas below coccyx No associated anomalies Perineal fistula Rectal gas above coccyx Associated defects Abnormal sacrum Flat bottom Anoplasty PSARP +/- colostomy Colostomy Adapted from Pena, A, Levitt, M: Anorectal Malformations. In Grossfeld, J, O’Neill, J, et al (ed) Pediatric Surgery. 6th Ed.

Management Operative repair Anoplasty for perineal fistulas Posterior Sagittal Anorectoplasty - PSARP Electrical stimulation of the perineum to evaluate sphincter contraction Midline sagittal incision dividing the sphincter mechanism into 2 equal parts Important nerves and vessels do not cross the midline Colostomy with delayed PSARP for high imperforate anus

Management Postoperatively Oral feeding may begin when the infant is awake enough Antibiotics for at least 24 hours Rectourethral fistula – foley cathether for 7 days Rectal dilation program is begun ~ 2 weeks post op Colostomy, if present, is closed when anal caliber is adequate (~12Fr Hegar dilator in a 1-4 month old) Diligent perianal care – protective barrier cream Multiple stools initially

Outcomes Complications Wound infections and dehiscence can be catastrophic Rectal strictures Vaginal strictures Rectal mucosal prolapse Urologic injuries in males Constipation -> megarectosigmoid -> overflow pseudoincontinence Studies suggest that 75% of patients achieve good bowel control and continence

Outcomes Incontinence is generally divided into 2 groups 1. Constipation – aggressive bowel irrigation is suggested 2. Increased motility leading to diarrhea – constipating diet, medication to decrease bowel motility and colonic irrigation is suggested May require a bowel management program to achieve continence Appendicostomy for retrograde enemas may be helpful Late toilet training

Abdominal Wall Defects Omphalocele
Incidence 1-2.5 per 5000 live births Male preponderance Second most common abdominal wall defect

Etiology Cause is unknown Rare reports occurring in families No specific gene has been identified

Associated Anomalies 30-70% will have associated anomalies Congenital heart disease – up to 45% VSD ASD Ectopia Cordis Tricuspid Atresia Coarctation of the Aorta Persistent Pulmonary Hypertension of the Newborn Chromosomal Abnormalities – up to 40% Beckwith-Wiedemann syndrome Down’s Syndrome Trisomy 13-15 Trisomy 16-18

Associated Anomalies Musculoskelatal abnormalities Neural tube defects GERD – up to 43% in one study Cryptorchidism – 33% Macrosomia

Pathology Central abdominal wall defect Usually greater than 4cm in diameter Covered by a translucent sac unless ruptured inutero Muscles of the abdominal wall are normal Sac usually contains the liver, midgut and frequently the spleen and gonad All have malrotation Infants are usually full term

Diagnosis Usually antenatally by ultrasound In a European study sensitivity of ultrasound was 75% Usually detected at 18 weeks +/- 6 weeks

May be delivered vaginally Placed on mechanical ventilation
Management May be delivered vaginally Placed on mechanical ventilation Replogle (10 Fr) tube should be placed Intravenous fluids Prophylactic antibiotics Cardiology evaluation and echocardiogram – this should not delay surgery Surgery Small defects may be closed primarily More likely a staged procedure with the placement of a silo Silo is reduced daily if tolerated at the bedside – ideally reduced in 7 days Operating room for final closure Prolonged reduction will have separation of the prosthesis from the abdominal wall

Contents of omphalocele after sac is removed

Management Post Operative Care IV fluids to maintain adequate urine output Wean ventilator after final closure Replogle tube (10 Fr) NPO until return of bowel function Return of bowel function may be delayed TPN

Outcomes Post operative complications Hypovolemia – very high fluid requirements Tight closure -> ventilatory compromise, decreased venous return and low cardiac output, oliguria Metabolic acidosis as a result of kinking of the hepatic veins from reduction of the liver Survival rates range from 70-95% Mortality is often related to associated anomalies

Abdominal Wall Defects Gastroschisis
Incidence 2-4.9 per 10,000 live births Male preponderance Most common abdominal wall defect

Etiology Cause is unknown, but may be related to fetal distress Infants are more likely to be premature Term babies are more likely to be SGA

Associated Anomalies Much less common than with omphalocele Intestinal atresia may be seen in 10-15% GERD ~ 16% Undescended testicle ~15% Malrotation is always present

Pathology Small abdominal wall defect: usually 2-4cm Occurs adjacent to the umbilicus almost always to the right Abdominal muscles are normal There is no sac Midgut is herniated through the defect Matted bowel loops may be present at birth Exposed bowel may appear normal at birth but quickly becomes matted with fibrinous exudate such that individual loops can not be distinguished

Diagnosis Antenatally by ultrasound Sensitivity has been reported to be 83% Detected at 20 weeks +/- 7 weeks

May be delivered vaginally
Management May be delivered vaginally Cover exposed bowel with warm saline moistened sterile dressing Replogle tube (10 Fr) Support of the premature infant Monitor for fluid and heat loss Should be taken to the operating room ASAP The faster the bowel can be reduced the more likely primary closure can be achieved If reduction and primary closure can not be achieved a silo is placed Post operatively requires adequate fluid resuscitation Faster surgery means less bowel edema and less exudate

Management Post operatively Wean ventilator after final closure Replogle tube (10 Fr) NPO until return of bowel function May be more prolonged than with omphalocele TPN

Outcomes Post operative complications Hypovolemia Closure too tight -> ventilatory compromise, decreased venous return and low cardiac output, oliguria Survival rate is ~90% Complications related to prematurity responsible for most morbidity and mortality Primary fascial closure has been reported in up to 80%; some recommend silos in all cases Median time to feeding ~30 days with a range reported of 5-60 days Atresias are repaired several weeks after intestinal reduction and fascial closure Associated atresia may require more time to feeds

In Summary BILIOUS EMESIS IS VOLVULUS UNTIL PROVEN OTHERWISE
History and plain films will guide sequence of further studies Remember the associated anomalies

References Boloker, J., Bateman, D., Wung, J., Stolar, C. Congenital Diaphragmatic Hernia in 120 Infants Treated Conservatively With Permissive Hypercapnea/Spontaneous Respiration/Elective Repair. Journal of Pediatric Surgery 2002; 37: Dalla Vechia, L.K.,MD; Grosfeld, J.L., MD; West, K,. MD; Rescoria, F., MD; Scherer, L., MD; Engum, S., MD. Intestinal Atresia and Stenosis. Arch Surg. 1998;133: Grosfeld, J., MD; O’Neill, J., MD; Fonkalsrud, E. MD; Coran, A., MD (eds). Pediatric Surgery. 6th ed. Philadelphia, PA: Mosby-Elsevier; 2006. Lewis, N., MBBS, FRCS; Glick, P., MD, MBA. Duodenal Atresia and Stenosis, Surgical Perspective. eMedicine from WebMD Millar, A., Rode, H., Cywes, S. Malrotation and Volvulus in Infancy and Childhood. Seminars in Pediatric Surgery 2003;12: Parish, A., MD, Hately, R., MD. Intestinal Malrotation. Emedicine Reid, J., MD. Midgut Volvulus. Emedicine Rowe, M., O’Neill, J., Grosfeld, J., Fonkalsrud, E., Coran, A. (eds). Essentials of Pediatric Surgery. St. Louis, MI: Mosby-Year Book, Inc.; 1995. University of Michigan Department of Surgery. Meconium Ileus.

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