if, i'ediatr. 45 : 269, 1978

NEONATAL SURGICAL EMERGENCIES (2)--EMERGENCIES WITHOUT RESPIRATORY EMBARR&~GMENT* K. YADAV

Chandigarh Neonatal surgery is based mainly on

the correction of congenital abnormalities. |t~ success depends on early accurate diagnosis, transportation to the specialist paedlatric centre and skilled postoperative management. I n the present report, I shall discuss tire m a n a g e m e n t of some of the t~eo~atal emergencie~ ~,ithout respiratory tl~barr~ssment such as abdominal wall t[.~fec,ts, small and large bowel obstrtmtion tie..,(,Fable I)

General Management As tile local medical ;,ractitioner i* the flr*t to ste the neonate ~.',.ith a congenital .defect, it is in~portant to make him aware ~at an early attd ne.tr a c e - r a t e diagnods, transportatJt,n and early specialist treatment be arranged to save such babies. There are certain pte-transportation precautions he has to undertake and also to make the parents understand fully the basic pathology prior to departure. We may consider the baby with gastroschisis, where the diagnosis is obvious at birth. T h e defect is situated on the right side of the xxormally attached umbilical cord (Plate I, Fig. 1). The gut i~ with*mr nnv coveting, due to prolonged intrauterine exposure to amniotic fluid, From the Department of Paediatric Surgery, P~e,tgrttduate ln.,.titute of lktedical Education and .P~e~rch, Chandigar h. R~eprint$ to DE. K. Yadav, Lecturer in Paediatric Sgxgery, P.G.I, Chandlgarh, India. Received o n Jut're IS, 1978.

thus the he.;.~.~,cd bowel is oedematous, fore-shortened and m a t t e d together by a fibrinous m e m b r a n e ; in addition the intestinal circulation m a y be impaired. Here t h e p r i m a r y t r e a t m e n t -is to cover the exposed bowel with w a r m saline soaked sponges or simply to wrap it in a plastic bag to prevent h y p o t h e r m i a . Since this is an emergency, urgent transportation ;o a specialist centre is done. Neonatal intestinal ob,truetion at times is difficult to diagnose. Though the classical clinical features mentioned are bile stained vomiting, abdominal distension and failure to pass meconium, th?y are not al.~ays present, and there are many pttfdlls in reaching the diagnosis. Persistent vomiting, whether bile stained or not, tbllowing every feed, leading to loss of weight, should be considered doubtful unless proven otherwise. T h e first pitfall in diagnosis is absence of distension of the a b d o m e n [for e x a m p l e with duodenal and jejunal obstruction). Secondly, the neonate m a y pass m e c o n i u m in a r.ormal manner. W h e n the obstruction is incomplete (for example, malrotation or Hirschsprung's disease) there m a y be even diarrhoea. Thus, uittess i m m e d i a t e a b d o m i n a l X - r a y e x a m i n a t i o n Is arranged, the diagnosis of neonatal duodenal Plain X-rays of

obstruction is delayed. the a b d o m e n , supine

and upright anteroposterior, should be obtained and m a y be diagnostic (Plate I, Pig. 2).

270

VoL. 45, No. 3l~

INDIAN JOURNAL OF PEDIATRICS

T a b l e 1. Neonatal surgical emergencies without

respirator~ embarrat~ment,

A,

Ga.,tro~ntestinal problem.,: 1.

Neonatal small a.

Duodenal

bowel Obstruction obstruction--atre.,ia -- stenosi~ --diaphragm .... m e m b r a n e

b. Intestinal obstruction atrefia or stenosis

c. Malrotation midgut

and

volvulus

of

d. Meconium {leus e. Hirschsprung's disease f. M e c o n i u m plug g. I m p e r f o r a t e anus b. Gastrointestinal

perforation

i. Neonatal necrotising enteroeolitis

B.

Skin defects Gastroschisis Exomphalos Meningomyelocoele Sacrococcygeal t e r a t o m a Cleft lip

C.

Abdominal masses a.

Benign renal disease -- Polycystic kidneys - - Hydronephrosis

b.

Neonatal turnouts - - ~ r i l m s tumours -- Neuroblastoma -- Teratoma ~ E m b r y o n i e sarcoma - - I n t e s t i n a l duplication

cyst

Following the diagnosis, the case reterred to the paediatric centre for specll~ irwestigations. ~ .The treatment prior departure is simply to decompress thtt bowel by passing a Ryte's tube and keepin it open in the polythene bag. Neonate most of the time do not require fluid unle they are severely dehydrated (as in complete duodenal obstructi0nl due to persisten vomiting for a long time a,ld here the doctor is required to supplement ahe neonate with fluid as advised (Yadav 1976 1978). T h e parents are advised not to feed the .baby till it reaches the centre and the procedure is properly explained to them so that they agree with the t r e a t m e n t o[ the specialist. T h e importance of keeping the child w a r m during transportation must be emphasised. This can be done by covering him with clothes and using hot water bottles during travel. The diagnosis o f l n t e s t i n a l ob3truction in cases where the lower small bowel or colon is involved can be m a d e by carrying out specific investigations, r u t h as a gastrografin enema. This also acts as t r e a t m e n t in relieving the obstruction due to a meconium plug or m e c o n i u m ileus in uncomplicated cases. A further definite diagnosis of Hirschsprung's disease can be done when the neonate is fit, by taking a rectal biopsy for the presence of ganglion cells. T h e upper gastro-intestinal tract study is also done after aspiration of the stomach t,, localise the site of obstruction. Though this study excludes duodenal atresia it still does not rule out the presence of duodenal $tenosis or m a l r o t a t i o n etc. Here the gastrografin study is helpflfl. The only precantion undertaken is to have a good intravenous

y A D A V - - NEONATAL SURGICAL It,MEROENCIES

drip going prior to and during tile procedure, to prevent severe dehydration due to a sudden shift of fluid to the lumen of the gut resulting in sodium depletion; and saline is given to tile baby to protect him from dehydration prior to, during and even after a gastrografin enema. Neonatal Intestinal Obstruction The incidence of intestinal obstruction in neonates is about 1 in 1500 babies (Nixes 1966). T h e obstruction occurs due to a late intra-uterine vascular catastrophe such as volvulus, intussusception or strangulation o~f.a loop of bowel within the umbilical cord, resulting in atresia or ttenosis of the intestines. Some cases may be due to failure ofcanalisation of the solid stage m early embryonic life. Abnormalities of rotation and fixation of the bowel al~o t~-oduce intestinal obstruc*ion, either by volvulus of the midgut around a very narrow attachment of the root of the mesentery at the site of origin of the superior mesenteric arter 7 or duodenal obstruction due to congenital adhesion bands. Some neonates with cystic fibrosis presefft with meconium ileus. Here the meconium is abnormal due to abnormal mucous secretion resulting in obstruction of the distai ileum by inspissated meconium. In Hirschsprung's disease, a segment o f the bowel for a variable distance u p w a r d from the anal canal is deprived of ganglion cells, therefore, tl~e segment is incapable of co-ordinated peristalsis. (a) Intestinal atresia and stenosis. In intestinal atresia the aim is to restore the continuity by direct anastomosis. The dilated portion of t h e bowel proximal to the atresia is unable to function efficiently, so,this should always be resected. Kesection

271 can be done in both jejunal and ileal atresia or stenosis cases and the continuity is maintained by end to end or end to back anastomosis. In duodenal atresia, a side to side duodenoduodenostomy or duodenojeiunostomy is done. In this ease a decompressing gastrostomy tube is placed and a transpyloric feeding tube is usually passed across the anastomosis in anticipation of prolonged stasi.~ in the dilated unresectable proximal duodenum Colonic atresia is rare and it~ treatment is often carried out with a preliminary colostomy. (b) Malrotation of the intestine. T h e surgical treatment of malrotation consi3u of a complete and meticulous division of the adhesions both between the intestinal loops and between the intestines and parteta 1 peritoneum. The caecum is then mobilised, and after completion and ensuring ,hat there is no residual kinking, the intestines are returned to the abdominal cavity, with colon and caecum on the left and small bowel on the right. I n addition, appendicectomy is always carried out. I f clinical signs of intestinal strangulation are present, a volvulus of the midgut is one possibility. The volvulus is untwisted and nonviable bowel is resected. (c) Hirschsprung's disease. Though the definite treatment of Hirschsprung's disease is excision o f the aganglionic segment of the bowel and pull-through of normal bowel into the perimeum, yet this operation is not done in the neonatal period. T h e regime untaken by Ehrenpreis (1955)is, .firstly, to decompress the bowel by rectocolonic saline irrigations. I f the initial treatment is successful stage colostomy is not done by some surgeons unless the parents are unable to cope with tile patient at home, as life threatening enterocolitis can complicate

272

tmn^t~ JOURNAL (,V PROIA'I'aIf}S

unexpected cases Nowadays, most surgeons establish a preliminary colostomy proximal to the aganglionic segment as soon as the diagnosis of Hirschspurng's di~ea.~e i~ made. Some (Gross 1948) ~dvise resection of the abnormal distal bowel down to the rectum at the initial laparotomy in order to redue, e the incidence o f emerocolitis after colostomy. I n eontres where tt pathologist is not available, surgeons perform a transverse COlon colostomy, because in 98% of cases ganglion calls are present; only in 2% of eases, o n e gets a long segment with Hirschsprung's ~ disease and ileostomy is required. (d) M e c o m u m ileus The most commonly used procedure" for .,neconi::m ileu., i.- that described by Bishop and Koop (IE87), in which after resection of the grossly distended ileal segment, the distal end of the divided segment of bowel is brought cut as an ileostomy and the proximal loop is anastomosed to the side of the distal loop approximately 5 cm. from the ~ ileostomy. Postoperatively, gastrografin is instilled into tthe ileostomy to liquify residual meconium fo relieve the obstruction. Though the operation is simple, the mortality even in good centres is over 50 per cent. In 1963, attempts were m a d e to release the obstruction by giving a gastrografin enema. This is indicated in the absence o f complications such as votvulus, gangrene, perforation and peritonitis. T h e gastrografin enema is done under fluoroscopic control. The gastrografin is instilled into the colon until the dilated bowel above the obstruction is outlined. I n successful cases,,the inspissated meconium is passed shortly after the study and intestinal obstruction subsides. Since gastrografin is hypersonic

VoI.,, 4.5, No and causes an osmotic flow of fluid into th lumen of the bowel and the detergt,~ Tween 80 also inc'reases the peristalslO hence the continuous infizsion of saline essential during the procedure. T h e resUI! with gastrografin have been proved to b successful, and those who survive the:.neont~ period have a good prognosis with respect to cystic fibrosis because along with the bOwel obstruction the prophylactic treatment for pulmonary infection was alto started at an early age. B.

Skin Defects of the

Abdomeu

Gastroschisis. In this condition there n a full thickness defect of the ~ante'r~or, abdominal wall resulting in evisceration ol the intestines. The defect is considerd to be due to failure of ingrowth of the lumbar myotomes into the laleral fold of the anterior abdomihal wall (Moor and Stores 1953) rather than from rupture of an intrauterine exomphalos (Collins et al 1970)! The treatment is urgent because delay results in increased mortality and morbidity from hypothermia and bacterial peritonitis. T h e , ~operation is undertaken with an aim to cover the herniated viscera. Gastrostomy is performed in order to provide adequate postoperative gastric decompression. Very often the gangrenous or nonviable bowel repuires resection. The use of an extra abdominal silastic pouch sutured to the fall thickness defect with gradual reduction of the viscera every other day to achieve complete repair in 8 to 10 days is done (Schuster 1967). The preferred method of staging is to create a visceral hernia by enlarging the defect vertically (Gross 1948) or transversely (Savage etaI. 1971) with repair of the skin only. The technique of Savage and

INDIAN JOURNAL

PI.ATE

O F PI~DIATRIC$ .

ii'i.+?~,:i,:,?~:= ~.; :.)..+. . . . . . .

+,

.... ;~'+;'~"/':";~' :":~Z

, ~r

Fig. 1.

.i~~, '-

~'

-. +

: :

;

Gastrmchisis

Fig. 2.

YADAV--NEONATAL SURGICAL EMERGENCIES.

Hirsclasprung's disease

I

PLATE II

INDIAN JOURNAL OP PEDIATRICs

Fig 1. Left carotid angiogram--arteria] phase. (Case !).

Fig. 2.

Left carotid angiogram-venous phase. (Case 1 ).

MALIK ET AL.~M[DDLE CEREBRAL ARTERY OCCLUSION IN YOUNG CHILDREN.

ADAV~NI?,ONATAI. SURGICAL EMEROF~NGIES D~,ey (1971)

provides superior release of

i ~ t r a . a b d o m i n a l ten.~ion and produces a ventral il~J,,ia which is easIqy controlled and repaired at a later stage. Following operations i n t r a v e n o u s fluids are continued till the jntest|nal f u n c t i o n returns, I n t r a v e n o u s h y , : . , ..... ~ n t a t i o n is r e q u i r e d if p a r a l y t i c iletts pertu,,, continues l t J t m o r e t h a n 7 days. Thi~ isju.~t to provide calories to, p r e v e n t malnutrition, Gastro$chisis has a p o o r prognosis; the m e rtality rate in g o o d p a e d i a t r i c c e n t r e s in Western c o u n t r i e s is o v e r 50 per cent, T h e high ~aortnlity r a t e is d u e to h y p o t h e r m i a injection|, c o m p r e s s i o n effect of tight a b d o m i n a l wall closure and prolonged postoperative p arJl~t!c ileus. I f the n e o n a t e survives, the d ~ t e l o p m e n t is n o r m a l as a s s o c i a t e d anomallei are u n c o m m o n ( T o u l o u k i a n et al. 1971). Conclusion

T h e aggressive t r e a t m e n t of n e o n a t a l surgical e m e r g e n c i e s is justified, if the survivorJ Are to d e v e l o p into n o r m a l individuals. So the policy of selection of cases for those to be, t r e a t e d in the n e o n a t a l period, is neeel~ry. I n s o m e abnormalities, such as, m e n i n g o m y e l o c o e l e , there is always resi-

27~ dual m e n t a l or physical handle,W, and the parents should be m a d e a w a r e of this prior to (reatment. Refer.nces

Bishop, H.E., Keep, C.E. (IRK;7). Management 6~ mecoM~,m ileum, Ann. SurI. 145.410. Collltls, D.L., $chumacher, A.E. (1970). Omph~lo. cpele ruptured before birth. Prec. Patdtat. Surg. Gen&r. Melbourne, 1, 2t. Ehrenpreis, T. (i955). Hitchspruttg't disease in the neonatal period. A,ch. Dis. Childh. 30, 8. Gross, R.E. (19~t8), New method for surgical treatment of large omphalocoeles. ,$'urger~.24, 2"/7, Moore, T.C., Stokes G.E. (19521. Gastto"s~hidJ. Report .or" two cases treated by modtfication of gross op*rati0n for omphalocoe!e~ - Surger5, 39, 112. Nixon, H.H., O'Donnell, B. (1986..), . T h e Essentials of Paediatric Surgery. 2nd editton. Hctncmann Mr books, London. Savage, J.P., Davey, R.B. (1971). The treatment ofgast~'bschisis. 9ft. ?Ptdiafr. Slti[. 6'. 1'48." Schus'ter, S:R. (1967)0 A new method for stag~rd repair of large omphalocoeles. Surg. Gynae. Obatet. 125,837. Touloukian, R l . , Schackman, T.J. (1971). Gastrointe~tinM function and radiographic appearance following galtroschisis repair. ~. P~diat,. Sutg. 6, 427. Yadav, K.(1977), Fluid and electrolyte therapy in surgical neonates. Technique of "admiQistratiofit. Indian .]. PediaO. '14. 189. ~'adav K. (1978). Fluid and electrolyte the~'apu in surgical neonates. Actual estimation. In the Press.