CASE REPORTS

Fetal Midgut Volvulus Presenting at Term

By Christopher A. Crisera, Howard B. Ginsburg, and George K. Gittes
New York, New York

This report describes a rare case of intrauterine midgut particularly fortunate that the volvulus occurred at a point in
volvulus that presented at term. The pregnancy was uncom- gestation when she was mature enough to tolerate birth and
plicated. There were no signs of fetal distress or polyhydram- surgery. This case is also the first demonstration that volvu-
nios, and the child was delivered vaginally. This patient had lus can present with abdominal distension in the immediate
the unusual presentation in that at the time of delivery, the newborn period.
patient was distended and acidotic. She immediately re- J Pediatr Surg 34:1280-1281. Copyright o 7999 by W. B.
quired an extensive resection of gangrenous small bowel. Saunders Company.
Comparing this case to the 10 other cases of fetal intestinal
volvulus that have been reported, it seems this child was INDEX WORDS: Midgut volvuIus.

M ALROTATION with midgut volvulus is a poten-

tially life-threatening intraabdominal anomaly
frequently encountered by pediatric surgeons. Midgut
nasogastric
demonstrated
follows:
tube yielded bilious aspirate. An abdominal
a paucity of distal bowel gas. Laboratory
white blood cell count, 28,OOO/pL; hematocrit
radiograph
values were as
level, 37%:
platelets, 217.OOO/pL; room air arterial blood gas pH, 7.36; Pacoz, 37:
volvulus most commonly presents in the first year of life, Paoz. 71; HCOa, 21. base excess, 4.6.
especially in the first month. la2Infants affected before the The child underwent emergent laparotomy within 6 hours of delivery.
age of 1 year have a relatively greater morbidity and which showed malrotation with midgut volvulus causing hemorrhagic
necrosis of the small bowel. The volvulus was reduced. Much of the
mortality than older patients.3%4Only 10 cases of midgut
jejunum and ileum were resected leaving 16 cm of healthy-appearing
volvulus occurring prenatally have been reported in the proximal jejunum and 17 cm of distal ileum. A proximal jejunostomy
literature, and, in 4 of these cases, the child survived. We and an ileal mucous fistula were created.
recently encountered an unusual case in which the midgut Postoperatively, the child recovered quite well. She received 10 days
volvulus occurred perinatally, presenting immediately of antibiotics and was extubated uneventfully on postoperative day 2.
She required parenteral nutrition until postoperative day 13, at which
after birth with abdominal distension. Although the child
time enteral feeding was initiated. She was gradually completely
required resection of a significant amount of small bowel, weaned from parenteral nutrition. Three weeks after her operation. her
she survived and has continued to do well without jejunostomy and mucous fistula were studied vra contrast radiography.
parenteral nutrition. No strtctures were evident, and she underwent takedown of her
jejunostomy 5 weeks after her original operation. She recovered
CASE REPORT uneventfully from this operation and was discharged home at 9 weeks of
age and has continued to thrive at home.
A female infant was born at 37 weeks’ gestation via a spontaneous
vaginal delivery. Her mother was a healthy G2PO 32-year-old woman
wtth no past medtcal history. The pregnancy was uncomplicated. DISCUSSION
Significantly. there was no hrstory of polyhydramnios. The Apgar scores
were 7 and 9 at 1 and 5 minutes. respectively. Her vital signs at the time Ten cases of prenatal intestinal volvulus have been
of delivery were as follows: temperature, 99.2; blood pressure, 59/36: reported in the literature. Two cases had hydrops fetalis
heart rate. 160: respiratory rate, 36. Physical examination showed a and died.5.6 Four of the cases were reports of necropsies
tense, distended abdomen that was tender to palpation diffusely. There from spontaneous abortions between 19 and 22 weeks’
was bluish discoloration over the abdominal wall, and a small amount of
gestation in which the intraabdominal findings were
clotted blood was passed per rectum during the examination. A
discovered incidentally.’ Of the four surviving fetuses,
two manifested signs of distress at 34 weeks and were
From the Division ofPediatric Surgery, Department of Surgevy New delivered via emergent cesarean section.8.9After immedi-
York University Medical Center; New York, NZ ate resuscitation and diagnostic radiographs, the infants
Address reprint requests to George K. Gittes, MD. Laboratory of underwent life-saving surgery for resection of the in-
Developmental Biology and Repail; Department of Surgery, Division of
volved necrotic small bowel. Another case described
Pediatric Surge?, New York University Medical Center: 530 First Ave,
Suite IOU: New York, NY 10016. diagnosis of fetal intestinal obstruction and volvulus by
Copyright o 1999 by WB. Saunders Company ultrasound scan.‘O This fetus survived after cesarean
0022.3468/99/3408-0020$03.00/O section delivery at 33 weeks and immediate surgery.

1280 Journal of Pediatric Surgery, Vol 34, No 8 (August), 1999: pp 1280-l 281
FETAL MIDGUT VOLVULUS AT TERM 1281

Finally, one reported case describes spontaneous delivery
at 30 weeks of a fetus with a history of polyhydramnios
and neonatal abdominal distension.* This child also
survived after resection of gangrenous small bowel from
volvulus.
From these previously reported cases, we might try to
generate a list of prenatal signs suggestive of in utero
intestinal volvulus. A number of the affected fetuses were
diagnosed with polyhydramnios, which certainly is con-
sistent with intestinal volvulus causing complete obstruc-
tion. One of the reports suggested that ultrasound scan
may be a useful tool in helping to make this rare
diagnosis.‘0 In that report, a static abdominal mass with
dilated intestinal loops was visualized. A number of
reviews of malrotation and midgut volvulus have cited a
high association of its occurrence with other congenital
anomalies (omphalocele, gastroschisis, congenital dia-
phragmatic hernia, and trisomies).4.11 Furthermore, sev-
eral of the case reports cited above describe general signs
of distress, particularly decreased fetal motion and poor
heart rate variability. Interestingly. none of these prospec-
tive signs was noted in the current case. This patient had
an uncomplicated prenatal course with a normal spontane-
ous vaginal delivery at full term. She was clearly no less
ill than the previously reported patients that delivered
prematurely, requiring an extensive resection of gangre-
nous small bowel. The absence of a reliable set of
prenatal signs certainly underscores the acute nature of
the onset of this affliction.
It is interesting to consider the similarities between the
current patient and the survivors in the other reported
cases of in utero intestinal volvulus. The amount of
intestine that is compromised by the volvulus as well as
the timing of the event with respect to gestational age
appear to be important variables. Some investigators
have stipulated that small segmental volvuluses occur-
ring early in gestation may be a cause of intestinal
atresias seen in neonates.‘v8 The previously reported cases
of prenatal volvulus would suggest that a midgut volvu-
lus occurring during this same time frame might be an
adequate insult to compromise the viability of the fetus
and cause miscarriage. These reports also suggest that a
similarly large insult occurring in the third trimester will
induce premature labor. A relationship between prenatal
midgut volvulus and preterm delivery has been postu-
lated previously.7J2 It has been suggested that acute fetal
stress may activate adrenal and hypothalamic hormones,
which may induce premature uterine activity and birth. It
would seem, then, that the critical factor predicting the
survivability of a fetus with a substantial intestinal
volvulus is its maturity. In the current case, the child was
simply fortunate that when her bowel was compromised,
she was mature enough to tolerate delivery as well as
major abdominal surgery.

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