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CLINICAL
CASE ANALYSIS
WORKSHEET

Submitted by
Submitted to

Date Presented: ___________________


Date Submitted: ___________________
____ Semester SY ___________

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TABLE OF CONTENTS

Page
COVER PAGE
Introduction (Includes the Background and Rationale of the analysis) . . . . . . . . . . . .
Scenario (if presented in a virtual progressive scenario, write the summary) . . . . . . .
Phenomenon (Series of incidents leading to the occurrence
of the main (health) problem) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Concept Map (brief but concise graphical presentation of the phenomena) . . . . . . .
Learning Objectives (SMART; includes the main parts of the Clinical
Case Analysis Worksheet; Nursing Process – Approached) . . . . . . . . . . . . . .
Clinical Case Analysis Worksheet
Patient’s Personal Data
I. Family Background . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
II. Developmental Data . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
III. Chief Complaints . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
IV. Health History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
V. Complete Diagnosis of the case chosen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
a. Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
b. Symptomatology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
c. Anatomy and Physiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
d. Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
VI. Medical Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
a. Laboratory Interpretation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
b. Drug Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
VII. Nursing Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
a. Nursing Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
b. Nursing Care Plan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
VIII. Evaluation and Implication of the case to: . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
a. Nursing Practice (What might the case mean for other nurses?) . . . . . . . .
b. Nursing Education (What might the case contribute to education).. . . . . .
c. Nursing Theory (applicable nursing theory in the care of the case) . . . . . .
d. Nursing Research (any related issues that may need investigation) . . . . .
IX. Recommendations/Referrals/ Follow – ups . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
X. Journal Reading Related to the Case (EBP Readings). . . . . . . . . . . . . . . . . . . . . .
XI. REFERENCES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
APPENDIX (Any relevant documentation as long as it will
Not violate the Intellectual Property Rights) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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INTRODUCTION
Intestinal malrotation refers to any variation in this rotation and fixation of the gastrointestinal tract
during development. Interruption of typical intestinal rotation and fixation during fetal development can
occur at wide range of locations and this leads to various acute and chronic presentations of disease. The
first reports of intestinal malrotation were based on surgical and autopsy findings and occurred prior to
1900; however the first description of the embryologic intestinal malrotation and fixation was not
published until 1898. In 1923, Dott was the first to describe the relationship between embryologic
intestinal rotation and surgical treatment. In 1936, William E. Ladd wrote the classic article on treatment
of malrotation. His surgical approach, now known as the Ladd procedure that remains the cornerstone of
practice today.

Intestinal malrotation is the most common disease in pediatric patients than in adults. In the United
States, about one of every 500 children is born with intestinal malrotation. Although intestinal
malrotation can occur in older children (or even adults), up to 90% of patients are diagnosed by age one
and many within the first week after birth. A small minority of people who have intestinal malrotation
never experience symptoms, and sometimes live their whole lives without being diagnosed. While many
boys with intestinal malrotation develop symptoms earlier and this occurs equally in boys and girls.

In the Philippines, congenital anomalies rank among the top 20 causes of death across the life span and
are already the third leading cause of death in the infancy period (Philippine Department of Health,
1996). Despite the magnitude of the problem, no formal systematic registration of birth defects has been
done in the country up until 1999. Various attempts have been made by different study groups to gather
data but there was never a formal effort to consolidate the information and establish a centralized
registry. The intestinal malrotation is a rare case in the Philippines and an unusual type of congenital
deformities.

This clinical case analysis will provide a descriptive and detailed information regarding to the clinical
case scenario of the patient named Martine, a 10 year old female who was brought by her parents for
consultation of complaining tummy ache, upon assessing she was lethargic and her growth is slowed.
The doctor diagnosed it as an intestinal malrotation- volvulus. This intestinal malrotation is a life
threatening that is why it needed an immediate surgery.

The rationale of this case analysis is to analyze, discuss and evaluate the clinical case occurring to the
patient

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SCENARIO

Shaun: Does that hurt? Yes. Does that hurt? Yes. Does that hurt? Jerry: How much are we paying for
this? She has a tummy ache. - That's what they do... they ache. - Shh. She's just trying to get out of
school. Again. Where do you think she learned that from? You don't think she hears you calling in sick
to work right before you go golfing? I like school. Me too. What did you eat for dinner last night?
Cereal. - Jerry: I thought you made meatloaf. - I did. And it got cold while we were waiting for you. I
want D-dimer, lactate, and amylase... What tests are you ordering? D-dimer, lactate, and amylase. - For
a tummy ache? Hmm? - Yes. I'm concerned it may be... Shall we talk outside? Cecile: I told you
something was wrong. You're happy she might be sick? I'm happy you're wrong. Yeah. Let's talk
outside. ♪ Have they been bickering like that the whole time? I didn't notice. They aren't sick. - Yeah,
they're what we call a vector. - Hmm? A carrier. A cause of disease. - You mean the parents? - Yeah.
That little girl has a tummy ache because mommy and daddy won't stop fighting. This isn't a medical
issue. Send them home. Could be intestinal malrotation, which could quickly become fatal. And every
patient in this hospital could have malaria, but that doesn't mean we're gonna go around testing for every
condition we think they could have. For example, that MRI you ordered on the guy with the ear
infection? Nice call, genius. Thank you. I was being sarcastic. It's normal. He's healthy. Send him home,
too. Why are you smiling? Because you're right. So you thought he was making a mistake and didn't say
anything? Just stood by watching, taking notes while he wasted everybody's time? Is that your job? In
my experience, doctors don't listen to nurses. And they only talk to us to lecture us when they figure we
screwed something up. ♪ From now on, you don't run any tests you don't have to run. How do I know if
a test is needed until after I run it? ♪ She'll tell you. Today, she's your boss. Nurse Fryday: Is that the girl
with the bickering parents? Didn't we send her home four hours ago? I sent a girl home today. How
often do people develop stomach issues because of their brain? Based on my personal experience? -
Every single day. - [Elevator dings] I want D-dimer, lactate, and amylase for a -year-old girl, Martine
LaDuff. She might have a tummy ache caused by stress. Or she might not. Everything is within normal
range. Hmm. Slightly elevated lactate and amylase, and she's very small. Normal should be lower.
Normal should be lower. What do you think she has? Thank you. [Knocking on door] W-What the hell?
It... it's after : . I ran tests. The results were ambiguous. I think Martine has intestinal malrotation and
that a volvulus has occurred. Ambiguous tests told you this. It's a genetic condition. The symptoms are
very similar to stress. [Exhales sharply] Call us in the morning. Okay, she may not be alive in the
morning. Does your boss know you're here? No, I think he'd be upset with me for being here. [Exhales
sharply] I am not waking my daughter up in the middle of a school night because of some freak. And
you don't need to call me in the morning, because I'll be calling your boss in the morning. ♪ [Knocking
on door] Were you being sarcastic? - How hard is it to get rid of someone. - I told him... You're right.
I'm weird. Part of my weirdness is that I perseverate. That means I keep thinking about things. So I will
keep knocking on your door until I know Martine is okay. ♪ Martine. Martine. Martine. [Loudly]
Martine, you need to wake up! [Gasps] - Honey? - [Normal voice] Martine? - She vomited. - I-I can't
wake her! - I'll call . - N-No. There's no time. - Do you have a car? - Yeah. Her pulse is too weak to
perfuse her organs. What? What does that... ♪ Jerry! Please hurry. [Engine revs] Watch your back!
Shaun: Patient is a -year-old female. She has bradycardia with hypovolemic shock. - She needs oxygen,
IV adrenaline... - Go! ...and a liter of saline. - Wide open, please! - Man: Coming through! ♪ Her pulse is
better. She needs an ultrasound. [Monitor beeps] There. The small bowel is twisted around the superior
mesenteric artery. Martine needs surgery immediately. We need to confirm with Dr. Melendez. N-No.
Dr. Melendez is in surgery. Part of Martine's bowel is dying - and killing her with it. - No. Is this the
O.R. scheduler? Yes, this is Dr. Murphy. Prepare an O.R. for surgery. Ten blade.

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PHENOMENON
At the ER:
-Martine was brought by her parents for consultation due to tummy ache
-The attending physician suspected intestinal malrotation- volvulus
-He ordered D-Dimer lactase amylase
-The head of the attending physician questions his order and advised to send the patient home instead.

At the station:
-The attending physician was looking for patient’s laboratory results but it’s not in yet

At the laboratory:
-The attending physician requested for D-Dimer lactate amylase result and found out it is slightly
elevated.

At the patient’s home:


-The attending physician rushed into Martine’s house and insisted to see Martine’s condition
-Martine was confinrd to bed but unconscious and has vomited already

Back to the ER:


-Weak pulse, bradycardic with signs of hypovolemic shock
-The physician ordered IV adrenaline and liter of saline
-Ultrasound revealed that small bowel is twisted around the superior mesenteric artery

At the OR:
-For immediate surgery

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CONCEPT MAP

Weak pulse
Martine, 10 year old girl
Tummy ache brought by her parents Bradycardic
at hospital for
consultation Hypovolemic shock

At the hospital, the nurse Needed immediate


assess for: surgery

 Family assessment
 Client Assessment
 Physical Assessment

Upon laboratory, test done:

 D-dimer Lactate Amylase (slightly


elevated)
 Ultrasound (small bowel twisted)

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LEARNING OBJECTIVES
At the completion of this case, the learners will be able to comprehensively appreciate the
management of intestinal malrotation-volvulus.
Specifically, the learners will be able to:
a. define intestinal malrotation volvulus
b. collect the patient’s data, family background and developmental data
c. discuss the concept map and phenomenon
d. explain diagnosis and health problem
e. discuss medical management, laboratory interpretation and drug study
f. make appropriate nursing management, nursing diagnosis and nursing care plan
g. evaluate and provide implications
h. give recommendation/referral/follow-ups
i. discuss journal reading related to case

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CLINICAL CASE ANALYSIS WORKSHEET


CONCEPT

PATIENT’S PERSONAL DATA:_


Name (optional): Martine Age: 10 year old Sex: M Civil Status: Single Religion:N/A
Address: N/A

I. FAMILY BACKGROUND:

Occupation: Student
Number of Siblings/Children: N/A
Other Relevant Data: N/A

II. DEVELOPMENTAL DATA: Specify the Stage.


(Based on Havighurst’s and Erikson’s Life and Developmental Task/Psycho-Social).
In developmental task theory, Erikson identified six stages of psychosocial development and the
stage that fits in our case is the Industry versus Inferiority since the patient is just a 10 year old female
wherein the child is in school age, so called stage of latency. According to Erikson this stage is vital in
developing self-confidence. During school and other social activities, children receive praise and
attention for performing various task. In our patient’s life basing on the developmental stage, the child
has intestinal malrotation and she will think this as her fault of having this abnormality wherein her level
of confidence will drop and will think she is a failure.
In Havighurst developmental theory wherein he divided the growth and development into six (6)
stages, our patient is belong to the Middle Childhood (6 to 12 year old). In our patients case, she may
feel emotional of having such disease since in the middle childhood stage, a child’s transition from total
physical and psychological dependency to self-sufficiency and independence occurs gradually that is
why parent’s should understand and aware of it.

Reference: Cherry, K (April 2020). Industry vs. Inferiority in Psychosocial Development.About, inc.
(Dotdash). https://www.verywellmind.com/industry-versus-inferiority-2795736#

CHIEF COMPLAINTS: Tummy ache

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III. HEALTH HISTORY:


Past Illness/Surgery: None

Inclusive Period of Hospitalization: unknown


Present Illness: Small bowel twisted around superior mesentery artery

IV. COMPLETE DIAGNOSIS OF THE CASE CHOSEN


a. Definition (at least 2 Definition with Bibliography)

1. Intestinal malrotation – is a developmental abnormality of the midgut (i.e the portion


of the intestine between the duodenojejunal flexure and the middle of transverse colon).

Reference: Mohan, H.2010.Textbook of Pathophysiology.6th edition. Jaypee Brothers


Medical Publisher (P) LTD. Pg.561.

2. Volvulus- is the twisting of loop of intestine upon itself through 180 degree or more. This
leads to obstruction of the intestine as well as cutting off the blood supply to the affected
loop. The usual causes are bands and adhesions and long mesenteric attachment.

Reference: Mohan, H.2010.Textbook of Pathophysiology.6th edition. Jaypee Brothers


Medical Publisher (P) LTD. Pg.563.

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b. Etiology
List all the Basic Etiology Actual Etiology on the Patient Rationale (Include the reference as endnote)

Intestinal malrotation

 Gastroschisis  Occurs which allows the intestine and other organs


extends outside the body, usually to the right side of
belly button. Robert A. hoekelman, primary pediat
care, third edition, chapter 109, page 847.

 Crohn’s disease  Due to inflammation of the digestive tract, which c


lead to abdominal pain, severe diarrhea, fatig
heat loss and malnutrition. Catherine E. Burns, Ar
M. Dunn et, al. , SAUNDRES Elsevier, Pedia
primary care unit four, fourth edition, chapter
gastrointestinal disorders, page 813
 Diverticulitis  Due to low fiber diet is the main cultprit in diverticulo
Robert A. Hoekelman, primary pediatric care, th
edition, chapter 109, page 850.

 Trichobezoar  Condition causing acute abdomen in children. Robert


hoekelman, primary pediatric care, third editi
chapter 109, page 850
 Meckel diverticulum  Due to viral infection such as rotavirus or noroviru
Catherine E. Burns, Ardys M. Dunn et, al. , SAUNDR
Elsevier, Pediatric primary care unit four, fourth editi
chapter 32 gastrointestinal disorders, page 813.

 During early development of the baby in the moth


Volvulus womb, nerve cells stop growing towards the end o
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child’s bowels. Catherine E. Burns, Ardys M. Dunn et, a


SAUNDRES Elsevier, Pediatric primary care unit fo
fourth edition, chapter 32 gastrointestinal disorders, p
Long mesenteric 812.

When the nerve cells in the colon don’t form complet


 Hirschsprung disease because nerve in the colon control the muscle constractio
that move food through the bowel. Robert A. hoekelm
primary pediatric care, third edition, chapter 109, page 8

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c. Symptomatology

Actual
List all the Basic
Symptomatology on Rationale(Include the reference as endnote)
Symptomatology
the Patient
 Vomiting green √  Due to swollen abdomen that’s tender to the touch. Catherine E. Burns, Ardys M. Dunn et, al. ,
digestive fluid (bile) SAUNDRES Elsevier, Pediatric primary care unit four, fourth edition, chapter 32 gastrointestinal
disorders, page, 815.
 Stomach pain √  due to anal fissure or allergic colitis, Meckel’s diverticulitis or polyps. Catherine E. Burns, Ardys M.
Dunn et, al. , SAUNDRES Elsevier, Pediatric primary care unit four, fourth edition, chapter 32
gastrointestinal disorders . page, 813.
 Swollen belly  Due to a lack of protein in the diet, affects the balance and distribution of fluids in the body and often
leads to swollen belly. Pediatric primary Catherine E. Burns, Ardys M. Dunn et, al. , SAUNDRES
Elsevier, Pediatric primary care unit four, fourth edition, chapter 32 gastrointestinal disorders, page 814.
 Diarrhea  Due to infection from viruses like rotavirus, bacteria like salmonella and rarely, parasites like giardia.
Pediatric primary care author, Catherine E. Burns, Ardys M. Dunn et, al. unit four, chapter 32
gastrointestinal disorders, page 813.
 Constipation  Due to intestinal blockage. Pediatric primary care author, Catherine E. Burns, Ardys M. Dunn et, al. unit
four, chapter 32 gastrointestinal disorders, page,811.
 Rectal bleeding  Happen when passing a large or hard stool, which stretches the lining of the anus until it tears. Catherine
E. Burns, Ardys M. Dunn et, al. , SAUNDRES Elsevier, Pediatric primary care unit four, fourth edition,
chapter 32 gastrointestinal disorders, page 810.
 Due to eating disorders. Catherine E. Burns, Ardys M. Dunn et, al. , SAUNDRES Elsevier, Pediatric
 Poor appetite primary care unit four, fourth edition, chapter 32 gastrointestinal disorders, page 812.

 Fever  When the body’s temperature is higher than normal because of an infection. Ardys M. Dunn et, al. ,
SAUNDRES Elsevier, Pediatric primary care unit four, fourth edition, chapter 32 gastrointestinal
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disorders, page 813.

 Due to adrenal insufficiency and increased intracranial pressure, inherited arrhythmias and
 Bradycardia  cardiomyopathy. Homoud MK. Sinus bradycardia. http://www.uptodate.com/home. Accessed Dec. 28, 2016.

 Decrease blood volume caused by blood loss, which leads to reduced cardiac output and adequate tissue
 Hypovolemic shock perfusion. Hypovolemia shock - Riley MR. (2015). Maternal cardiovascular and hemodynamic

adaptations to pregnancy.uptodate.com/contents/maternal-cardiovascular-and-hemodynamic-adaptations.

 Delay occurs when a child’s isn’t growing at the normal rate for their age. Grimberg A, et al. (2017).

 Slow growth Guidelines for growth hormone and insulin-like growth factor-I treatment in children and adolescents.
DOI:10.1159/000452150

 Colicky pain  Due to blockage that keeps food and liquid from passing through the body. Robert A. hoekelman,
primary pediatric care, third edition, chapter 109, page 847.

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XII. Anatomy and Physiology (Organ or System Involved)

The digestive system is made up of organs that are important for digesting food and liquids.
These include the mouth, pharynx (throat), esophagus, stomach, small intestine, large intestine, rectum,
and anus. The digestive system also includes the salivary glands, liver, gallbladder, and pancreas, which
make digestive juices and enzymes that help with digestion.

Salivary glands – The salivary glands produce saliva, which keeps the mouth and other parts of the
digestive system moist. It also helps break down carbohydrates (with salivary amylase, formerly known
as ptyalin) and lubricates the passage of food down from the oro-pharynx to the esophagus to
the stomach

Esophagus –  is a muscular tube connecting the throat (pharynx) with the stomach. The esophagus is
about 8 inches long, and is lined by moist pink tissue called mucosa

Stomach – is a hollow organ, or "container," that holds food while it is being mixed with stomach enzymes

Large intestine – The long, tube-like organ that is connected to the small intestine at one end and the
anus at the other

Small intestine – The small intestine connects the stomach and the colon. It includes the duodenum,
jejunum, and ileum.

Liver – makes a digestive juice called bile that helps digest fats and some vitamins

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Gallbladder – a small pouch that sits just under the liver. The gallbladder stores bile produced by the
liver

Pancreas - Is a long, flat gland that lies in the abdomen behind the stomach

Anus – The anus is the last part of the digestive tract. It is a 2-inch long canal consisting of the pelvic
floor muscles and the two anal sphincters (internal and external)

Infrieor vena cava – carries deoxygenated blood from the lower body to the heart

Superior mesenteric – the superior mesenteric artery (SMA) is a primary source of blood from the
heart for many organs of the midgut, all of which are associated with the digestive system

Ascending colon – is the beginning part of the colon. It is usually located on the right side of the body,
extending from the cecum upward.

Adhesion – Abdominal adhesions are bands of scar-like tissue that form inside your abdomen.

Obstruction – Intestinal obstruction is a blockage that keeps food or liquid from passing through your
small intestine or large intestine (colon)

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The digestive system is made up of the digestive tract - a series of hollow organs joined in a
long, twisting tube from the mouth to the anus - and other organs that help the body break down and
absorb food. Organs that make up the digestive tract are the mouth, esophagus, stomach, small intestine,
large intestine - also called the colon - rectum, and anus. Each part of your digestive system helps to
move food and liquid through your GI tract, break food and liquid into smaller parts, or both. Once
foods are broken into small enough parts, your body can absorb and move the nutrients to where they
are needed. Each organ of the digestive system has an important role in digestion. We can say that the
digestive system and it's part are functioning properly if it is no problem inside, each part are in correct
location, the person doesn’t experience any pain in the abdomen or any symptoms that can cause poor
digestion of food. The normal anatomical abdomen formation are divided into three segment first is
foregut, midgut, and hindgut from the abdominal cavity, the large intestine are made of the following
part cecum, colon, the colon consist of four part the ascending colon, transvers colon, descending colon,
sigmoid colon and the rectum.

Intestinal malrotation: the intestine actually form outside the of the body of the baby the
abdomen is not completely closed and about ten week of gestation about two and half month into the
pregnancy the intestine is start to return back into the abdomen and that when we have normal rotation
where the intestine drape itself around the major blood vessels that supplies that call the superior
mesenteric artery if the no rmal rotation doesn’t occur properly then the situation called intestinal
malrotation, when it come to fixing the rotation of intestine it occurs actually more commonly done
we’re are aware of it’s only as certain of patient who become a symptomatic so there are plenty of child
with malrotation that are completely not causing any problem and they are not even aware they have it
so happen it about 0.5% of the papulation so five out of thousand people if you thought that pyloric
stenosis are very common in boys other than girls.

Volvulus: The term volvulus actually come from the latin word Volver which means to roll so evolved
ulis is an obstruction caused by a loop of the intestine that rolls or twist around itself and it surrounding
mesenteric which is the tissue that attaches the intestine to back wall of the abdomen.

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e. Pathophysiology (Schematic Diagram as applied to your patient)

INTESTINAL MALROTATION

 Age
 Environmental factors  Sex
 Dietary habits  Cultural background
 lifestyle  Familial history of disease
(genetic)

Disruption of the normal embryologic development of the bowel

NONROTATION INCOMPLETE ROTATION


INCOMPLETE FIXATION

Arrest in stage I
development that Arrest in stage II Formation of hernia
usually occurs between development that usually pouches
5-10 weeks gestation occurs at 10 weeks gestation

Mesentery on the right and


Duodenal junction did not lie Duodenal obstruction left colon and duodenum
inferiorly to the left of SMA do not become fixed to the
(Superior mesenteric artery) retroperitoneum
Mesenteric base
narrowing

Cecum did not lie in the right


lower quadrant Descending mesocolon
attachment remain
Internal herniation may unfixed
occur if duodenojejunal
Mesentery forms a narrow loop doesn’t rotate
base as the gut lengthens on 19
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Narrow base is prone to The small intestine will be


clockwise twisting Small bowels in the mesentery pushed out to unsupported
leading to midgut will be trap in the large bowel area as it migrates to left upper
volvulus quadrant

Creation of mesocolic Creation of left mesocolic


hernia (paraduodenal hernia with possible
hernia) entrapment and strangulation
of the bowel

 Vomiting
 Bloody stools
 Lethargic
 Pain in abdomen
 Abdominal
distention

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Intestinal malrotation occurs due to disruption of the normal embryologic development of the
bowel. Understanding of normal abdominal development aids in the understanding of the etiology of the
clinical findings seen with malrotation. In the normal embryologic development of the alimentary tract
the alimentary tract develops from the embryologic foregut, midgut and hindgut. Normal rotation takes
place around the superior mesenteric artery as the axis. It is described by referring to two ends of the
alimentary canal, the proximal duodenojejunal loop and the distal cecocolic loop, and is usually divided
into three (3) stages. Both loops make a total of 270 degree in rotation during normal development and
these loops start in vertical plane parallel to the SMA and end in a horizontal plane.

The development of intestine of the fetus has three stages. In the stage I it occurs between
5-10 weeks’ gestation, a period of physiologic herniation of the bowel into the base of the
umbilical cord. The duodenojejunal loop begins superior to the SMA at a 90 degree position and
rotates 180 degree, the loop is to the anatomical right of SMA, and by 270 degree it is beneath
the SMA. The cecocolic loop begins beneath the SMA at 270 degree. It rotates 90 degree in a
counterclockwise manner and ends at the anatomical left of the SMA at 0 degree position. Both
loops maintain these positions until the bowel returns to the abdominal cavity. Also during this
period, the midgut lengthens along the SMA, and as rotation continues a broad pedicle is formed
at the base of the mesentery and this broad base protects against midgut volvulus. During stage
II, it occurs at 10 weeks gestation, the period in which the bowel returns to the abdominal cavity
and as it return the duodenojejunal loop rotates an additional 90 degree position. The cecocolic
loop turns 180 degree more as it reenters the abdominal cavity. This turn places it to the
anatomical right of SMA, a 180 degree position. During stage III, last from 11 weeks gestation
until term. It involves the descent of the cecum to the right lower quadrant and fixation of the
mesenteries.

Since those are the normal development of gut in fetal development, the following are
what will happen if there is intestinal malrotat ion. In this case, it has what we call nonrotation
wherein the arrest happened at stage I resulting in nonrotation. Subsequently the duodenojejunal
junction does not lie inferiorly and to the left of the SMA, and the cecum does not lie in the right
lower quadrant. The mesentery in turn forms a narrow base as the gut lengthens on the SMA
without rotation and this narrow base is prone to clockwise twisting leading to midgut volvulus.
The width of the base of the mesentery is different in each patient, and not every patient with
nonrotation develops midgut volvulus.
In incomplete rotation, the arrest start at stage II that results in incomplete rotation and is
most likely to result in duodenal obstruction. Typically, pretoneal bands running from the
misplaced cecum to the mesentery compress the third portion of the duodenum. Depending on

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how much rotation was completed prior to arrest, the mesenteric base may be narrow and again,
midgut volvulus can occur. Internal herniations may also occur with incomplete rotation if the
duodenojejunal loop does not rotate but the cecocolic loop does rotate. This may trap most of the
small bowel in the mesentery of the large bowel creating a right mesocolic hernia.
During incomplete fixation, the potential hernia pouches form when the mesentery of the
right and left colon and the duodenum do not become fixed to the retroperitoneum. If the
descending mesocolon between the inferior mesenteric vein and the posterior parietal attachment
remains unfixed, the smack intestine may push out through the unsupported area as it migrates to
the left upper quadrant. This creates a left mesocolic hernia with possible entrapment and
strangulation of the bowel. If the cecum remains unfixed, the volvulus of the terminal ileum,
cecum and proximal ascending colon may occur. These three has the same signs and symptoms
that leads to vomiting, bloody stool, lethargic, pain in abdomen and swelling.

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V. MEDICAL MANAGEMENT
a. Laboratory Interpretation

Basic Diagnostic
Procedures Required Purpose / Rationale Result of the Patient Clinical Significance Implication to Nursing
with Normal Values
Abnormal:  If small bowel is twisted in SMA  Orient the patient about the
the tissue in that area of intestine procedure
Abdominal Due to suspicion of intestinal Small bowel twisted around
can die  Instruct not to eat food and drinks
ultrasound malrotation superior mesenteric artery
 If normal, the SMA provides blood for 8 to 12 hours before ultrasound
supply to the majority of small  Assist the patient to change clothes
intestine into hospital gown
 Assist the sonographer to put a small
amount of warm gel to patients
abdomen
 Attend patient’s concern if there is
any.

 Orient the patient about the


 Normal D-dimer is 250 nanograms procedure
D-dimer Lactate administered upon palpating Slightly elevated per milliliter.
Amylase patient’s abdomen and due to  Positive/High D-Dimer is 500
suspicion of intestinal malrotation  Ensure that the patient has had the
nanograms per milliliter or higher appropriate preparation like a
indicates that there might be special diet or fasting.
significant blood clot formation
and break down in the body  Nurse must be aware of normal and
 Slightly elevated means you have abnormal ranges of the test done in
clotting disorder. order to understand the significance
of the result

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b. Drug Study (All drugs Indicated for the Patient’s Illness


Dosage and
Generic Name Pharmacologic Effects / Indication and
Classification Route of Side Effects Nursing Responsibilities
(Brand Name) Mechanism of Action Contraindication
Administration
Indication ;epinephrine  Difficulty
Drug ordered: acts quickly to improve breathing  Check patients HR, pulse
IV Adrenaline breathing, stimulate the rate and O2sat and
0.1 mg ,kg dose Through its action on alpha heart, raise a dropping administer oxygen as
E Alpha-and beta 0.1 ml kg dose of receptors epinephrine blood pressure ,reverse prescribed.
P adrenergic a 0.1 mg ml induces increased vascular hives and reduce swelling  Dizziness
I agonists solution .IV OR smooth muscle contraction. of the face, lips,and throat.  Ensure patients safety at all
N I0; May repeat Other significant effects Contraindication; there are times by securing the side
E every 3 to 5 include increased heart no absolute rails or instruct patient not
P minutes rate .myocardial contraindications against to go alone outside if
H contractility, and renin using epinephrine. Some  Nausea feeling dizzy
R release via beta -7 receptors relative contraindications and
I include hypersensitivity to Vomiting  Instruct patient not to give
N sympathomimetic food immediately
E drugs,closed –angle
glaucoma ,anesthesia with
halothane another unique
contraindications to be
aware of is
catecholaminergic
polymorphic ventricular
tachycardia.

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Dosage and
Generic Name Pharmacologic Effects / Indication and
Classification Route of Side Effects Nursing Responsibilities
(Brand Name) Mechanism of Action Contraindication
Administration
Moderately
Drug ordered: saline 2-10 Extracellular fluid  Nausea and
IV saline primary 0.45% sodium Sodium and chloride major replacement (e.g., vomiting  Ensure patient’s safety at all
drainage water chloride electrolytes of the fluid dehydration times by raising side rails.
and ground injection, USP compartment outside of ,hypovolemia,  Swelling in
S water Contains 4.5 gL cells {I,e extracellular} ,hemorrhage ,sepsis) hands, ankles  Instruct S.O not to give food
O sodium Work together to control or feet immediately after vomiting
D Highly saline chloride .as extracellular volume and Treatment of
I 10-25 directed by a blood ,disturbance in metabolic alkalosis in  Assess patient’s Heart rate,
U secondary physician dosage sodium concentrations in the presence of fluids  Difficulty of pulse rate and O2sat and
M drainage water is dependent the extracellular fluid are loss. breathing administer oxygen as
and ground upon the age associated with disorders of prescribed
C water weight and water balance. Mild sodium  severe
H condition of the depletion irritation
L Very highly patient.  Provide a non-pharmalogical
O saline 25-45 Contraindication: management such as music
R very saline Sodium chloride therapy, imagery.
I ground water 0.9% is
D Intravenous contraindicated in
E Brine >45 patients with
seawater congestive heart
failure ,severe renal
impairement
,conditions of sodium
retention ,oedema
,liver cirrhosis and
irrigation during
electrosurgical
procedures

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VI. NURSING MANAGEMENT


a. Nursing Diagnosis (Write five according to priority needs.)

1. Acute pain related to disruption of the normal embryologic development of the bowel
secondary to intestinal mal-rotation.

2. Risk for fluid volume deficit related to active fluid loss as evidenced vomiting secondary
to intestinal mal-rotation.

3. Imbalanced nutrition less than body requirement related to inability to absorbed or


metabolize foods as evidenced by intestinal blockage or twisting secondary to intestinal
mal-rotation.

4. Activity intolerance related to pain secondary to intestinal mal-rotation.

5. Risk for bleeding related to gastrointestinal disorder as evidenced by bloody stools


secondary to intestinal mal-rotation.

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b. Nursing Care Plan (Develop 3 NCP from the 5 Nursing Diagnosis)

Nursing Objective of Nursing Actions with Rationale


Date / Cues Needs Evaluation
Diagnosis Care (@ least 5 nursing interventions)
SD: S Acute pain After 8hrs span of care  Foresee the need of pain relief. GOAL MET:
“She has tummy ache and a related to the patient will be able Rationale: Early intervention may decrease the total amount of After 8 hours span
little blood on her poop” as A disruption of the to: analgesic required. of care the patient
verbalized by the mother normal  Describe is able to display
F embryologic satisfactory pain  Provide rest periods to promote relief, sleep and improved well
development of control at a level relaxation. being such as
OD: E the bowel less than 3-4 on a Rationale: Pain may result in fatigue, which may result in baseline levels and
 Bloody stool secondary to rating scale of 0 exaggerated pain. A peaceful and quiet environment may no signs of pain
 Abdominal distention T intestinal mal- to 10 facilitate rest. noted.
(swelling upon rotation  Patient displays
palpation) Y improvement in  Determine appropriate pain relief method. GOAL
 Facial mask of pain mood and coping Rationale: patients with acute pain should be given a nonopoid PARTIALLY
noted  Display improved analgesic around the clock unless contraindicated. MET:
 Lethargic Rationale: well-being such After 8hrs span of
Sudden onset of as baseline levels  Provide non-pharmalogical method such as imagery, care the patient is
pain is commonly for pulse and destruction techniques, music therapy. able to describe
associated with respiration Rationale: to lessen the stress, tension and subsequently satisfactory pain
perforation of the decreasing the pain. control at a level 2
gastrointestinal on a rating scale of
tract  Provide analgesics as ordered, evaluating the 0 to 10
effectiveness and inspecting for any signs and
symptoms of adverse effects. GOAL NOT MET:
After 8 hours span
Rationale: Effectiveness of pain medications must be evaluated of care the patient
individually to absorb and metabolized differently by patients. is not able to
Analgesics may cause mild- severe side effects display
improvement of
mood, coping
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Nursing Objective of
Date / Cues Needs Nursing Responsibilities Evaluation
Diagnosis Care
SD: P After 8 hours span of care  Aid the patient if he or she is unable to eat with GOAL MET:
“She vomited. I H the patient is: assistance and encourage family or SO to assist  After 8 hours span of
can’t wake her up” Y Risk for fluid volume  Normovolemic as with feedings as necessary. care the patient able
as verbalized by S deficit related to evidenced by urine Rationale: dehydrated patients may be weak and unable to display signs of
mother I active fluid loss as output greater than to meet prescribed intake independently. normovolemia as
O evidenced by vomiting 30 ml/hour and evidenced by urine
OD: L secondary to intestinal normal skin turgor  Administer parenteral fluids as prescribed. output greater than
 Bradycardic O mal-rotation Consider the need of an IV fluid challenge with 30 ml/hour and
 Paleness G  Patient immediate infusion of fluids for patients with normal skin turgor
noted I demonstrate abnormal vital sign. GOAL PARTIALLY MET:
 Decrease C Rationale: one of the lifestyle changes to Rationales: Fluids are necessary to maintain hydration  After 8 hours span of
skin turgor A common sources of avoid progression status. Determination of type and amount of fluid to be care the patient is able
 Dry mucous L fluid loss are the GI of dehydration replaced and infusion rates will vary depending on to verbalized
membrane N tract which leads to clinical status awareness of
noted E vomiting  Patient verbalizes causative factors and
 Lethargic E awareness of  Assist the physician with insertion of central behaviors essential to
 Hypotensiv D causative factors venous line and arterial line, as indicated. correct fluid volume
e S and behaviors Rationales: it allows fluid to be infused centrally and for deficit.
 Decreased essential to correct monitoring of CVP and fluid status.
urine W fluid volume deficit GOAL NOT MET:
output (20 A  Adapt modification to their current practices After 8 hours span of care the
ml/hr) T Rationales: accepting patients of family’s preferences patient not able to Patient
E shows respect to their culture demonstrate lifestyle
R changes to avoid progression
of dehydration

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Nursing Objective of Nursing Actions with Rationale


Date / Cues Needs Evaluation
Diagnosis Care (@ least 5 nursing interventions)
SD: P Imbalanced After 8hours span of GOAL MET:
“her appetite H nutrition less care both patient and  Set appropriate short term and long term goals. After 8hours span of care
decreases Y than body parents will be able Rationale: patients may lose concern in addressing this dilemma the patient is able to
because of S requirement to: without realistic short term goals. verbalized and demonstrate
abdominal I related to selection of foods or meals
discomfort” as O inability to  Verbalizes  Consider six small nutrient dense meals instead of three larger in accomplishing a
verbalized by L absorb or and meals daily to lessen the feeling of fullness. termination of imbalanced
mother O metabolize demonstrate Rationale: eating small frequent meals lessen the feeling of nutrition
G foods as selection of fullness and decreases the stimulus to vomit.
OD: I evidenced by foods or GOAL PARTIALLY MET:
 Stunted C intestinal meals that  Determine time of day when the patient’s appetite is at peak. After 8hours span of care
growth A blockage or will Offer highest calorie meal at that time. the patient is able to
 Paleness L twisting accomplish a Rationale: giving meals is more effective when appetite is at its identify short term goals
noted N secondary to termination peak but needed a further
 Weakness E intestinal mal- of imbalanced teachings for long term
noted E rotation nutrition  Give TPN (total parenteral nutrition) as prescribed goals
 Lack of D  Identify short Rationale: TPNs are essential in reinforcing nutrition if a patient
appetite S term goals is unable to eat or swallow or having episodes of vomiting GOAL NOT MET:
 Irritabilit Rationale: and long term After 8hours span of care
y patient usually goals  Instruct patient to increase fluid intake as prescribed. the patient is not able to
FOOD consumes foods  Patients weighs within 10% of ideal
that is not weighs within Rationales: oral fluid replacement is indicated for mild fluid body weight
enough to 10% of ideal deficit and is a cost-effective method for replacement treatment.
sustain body weight
nutritional body
requirement

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VII. EVALUATION AND IMPLICATION OF THE CASE TO:


a. Nursing Practice

The study will provide the students nurses with sufficient knowledge and skills towards
the management of patient having intestinal malrotation-volvulus. It will help the nursing students
as well as to provide a higher quality of care to patients with the same condition. The ideas and
learnings given by this case study will help nursing students to identify all the basic needs of every
patient having the same problem and also this will formulate effective nursing intervention right
away.

b. Nursing Education

The professional development of the nursing profession requires a well-defined nurse role.
Stated goals of professional programs for nursing do not include the entire body of tacit
knowledge. The overall development requires recognition of professional status together with a
clear and well-defined role. To equip the learners the following examples are given to
appreciate the rationale of basic interventions necessary to maintain the standards of care. For
sudden pain abdomen advice to avoid carbonate beverages, avoid chewing gum or sucking
candies, avoid drinking through straw or sipping surface of hot beverages. If irritable bowel
syndrome occur, and emotional stress decreases go for health check-up. Also educating how to
demonstrate ostomy care including wound cleaning if colostomy is done, high standard of
personal hygiene at home and environmental sanitation.

c. Nursing Theory

A learning theory is a logical framework describing, explaining or predicting how


people learn. Whether used singly or in combination, learning theories have much to offer
the practice of health care. Interestingly, health professionals including nurses must
demonstrate that they regularly use sound methods and a clear rationale in their education
efforts, patient and client interactions, staff management, training, continuing education and
health promotion programs. The uncertainty in illness theory by Merle Mishel is suited in
this case analysis because it addresses the process that occurs when a person lives with
unremitting or uncertainty found in chronic illness or in illness with a potential for
reoccurrence. Since intestinal malrotation is a genetic abnormality it cannot be prevented,
parents support is needed since they are the one who can understand their childs situation.
Self organization, probabilistic thinking and formation of a new life perspective is
important prior to life experience, physiological status, social resources and health care
provider.

d. Nursing Research

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As nurses continue to quest for new knowledge. It is very important to emphasize


evidence-based practice since we need to have a basis in everything we work on in order to have a
reliable and accurate source. More research to explore means more learning to appreciate.

VIII. RECOMMENDATION/REFERRALS/FOLLOW-UPS
Health education is focused on the parents such as:
1. Provide reliable basis information about intestinal malrotation-volvulus

2. Encourage to ask questions and clarifications regarding this diagnosis to


assess level of understanding

3. Explain the signs and symptoms with emphasis on their language used to
elicit cooperation

4. Listen to their issues and concerns regarding care of their patient at home

5. Discuss the importance of medication, diet and overall treatment


compliance

6. Emphasis assistance needed and its availability to motivate full


participation

7. Acknowledge limitations but stress the importance of follow-up check-up


and be open to referrals as needed

8. Give examples on how to promote patient’s safety at home and in school.

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IX. JOURNAL READINGS RELATED TO THE CASE


(Should be an Evidence-Based Practice (EBP) Reading: Attach the photocopy of
your journal reading)
Summary
Congenital intestinal malrotation is mainly detected in childhood and caused by incomplete
rotation and fixation of the intestines providing the prerequisites for life-threatening volvulus of the
midgut. Volvulus is more common in the younger age group. Intestinal congenital malrotation
should be recognized as a reason for abdominal pain also in adults which has also been emphasized
in a recent population based study by Coe et al. It describe a substantial number of symptomatic
patients being diagnosed in mature age often after several years of suffering. Malrotation may
present with alarming symptoms, causing life-threatening conditions which in one case resulted in
death due to short bowel syndrome. It also shows that young adults have a tendency towards more
severe symptoms requiring emergency treatment. Symptomatic malrotation occurs in both children
and the adult population. Improving awareness and an accurately performed CT scan can reveal the
malformation and enable surgical treatment. In the worst case scenario, malrotation may develop
into a midgut volvulus with torsion causing high risk of ischemia and necrosis of the parts of the
intestine supplied by the superior mesenteric artery. This life-threatening condition is well known
among pediatric surgeons and is always considered when physicians treat critically ill infants with
abdominal symptoms and unknown diagnoses. Ladd’s procedure relieved most patients from their
severe complaints even when a history of several years of suffering existed.

Reaction
This journal provides us a guideline to our case that gives more information to insight and
to enlighten our case analysis. The most symptoms of intestinal malrotation are swollen abdomen
that's tender to the touch, diarrhea and/or bloody poop (or sometimes no poop at all), fussiness or
crying in pain, with nothing seeming to help, rapid heart rate and breathing, little or no pee because
of fluid loss, and fever. Vomiting is another symptom of malrotation, and it can help the doctor
determine where the obstruction is. Vomiting that happens soon after the baby starts to cry often
means the blockage is in the small intestine; delayed vomiting usually means it's in the large
intestine. The vomit may contain bile (which is yellow or green) or may resemble feces. Based on
this article, mainly detected in childhood and caused by incomplete rotation and fixation of the
intestines providing the prerequisites for life-threatening volvulus of the midgut. And treating
significant malrotation almost always requires surgery. The timing and urgency will depend on the
child's condition. If there is already a volvulus, surgery must be done right away to prevent damage
to the bowel. Any child with bowel obstruction will need to be hospitalized. We do hope that this

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kind of case will be determine in order to provide an awareness in our community especially on
adolescents.
Reference:

DOI 10.1186/s40064-016-1842-0

RESEARCH Open Access

Congenital intestinal malrotation in adolescent and adult patients: a 12-year clinical


and radiological survey
Britt Husberg1,2,3,4, Karin Salehi5,6, Trevor Peters7, Ulf Gunnarsson8, Margareta Michanek3,4,
Agneta Nordenskjöld5,6 and Karin Strigård8*

Background a similar but secondary incomplete rotation


In congenital intestinal malrotation an and fixation of the intestines (Torres and
impaired embryological development of the Ziegler 1993).
gut causes incomplete rotation and fixation of The inadequate fixation of the bowel
the intestines to the abdominal wall (Dott alongside remaining embryonic fibrous
1923). The fulfillment of the third embryonic adhesions, the Ladd’s bands (Ladd 1932,
rotation includes the traversing of the 1936), may give rise to a variety of intestinal
duodenum to the left side of the abdomen, malfunction. In the worst case scenario,
forming the ligaments of Treitz, and the malrotation may develop into a midgut
migration of the ileo-caecal junction to the volvulus with torsion causing high risk of
lower right abdominal quadrant. The fixation ischemia and necrosis of the parts of the
of the full-length bowel is complete during intestine supplied by the superior mesenteric
the twelfth week (Penco et al. 2007). artery. This life-threatening condition is well
Congenital malformations such as known among pediatric surgeons and is
diaphragmatic hernia, omphalocele or always considered when physicians treat
gastroschisis are associated with critically ill infants with abdominal symptoms
and unknown diagnoses.
Malrotation has primarily been diagnosed in
*Correspondence: karin.strigard@umu.se early childhood, with estimated onset of
8
Department of Surgical and Perioperative symptoms during the first year of life in 90 %
Sciences, Umeå University Hospital, 901 87 of the cases (Vaos and Misiakos
Umeå, Sweden
Full list of author information is available at the end of the article

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© 2016 Husberg et al. This article is distributed under the terms of the
Creative Commons Attribution 4.0 International License
(http://creativecommons.org/licenses/by/4.0/), which permits unrestricted
use, distribution, and reproduction in any medium, provided you give appropriate credit to the
original author(s) and the source, provide a link to the Creative Commons license, and indicate if
changes were made.
2010; Pickhardt and Bhalla 2002; Stewart et al. Medical charts
1976). There are recent reports of manifestation All medical records were evaluated with regards
later in life, both as emergency conditions or to symptoms, surgical procedures, previous
more chronic gastrointestinal symptoms (Penco disorders and outcomes. For analysis of
et al. 2007; Pickhardt and Bhalla 2002; Nehra differences according to age, the patients were
and Goldstein 2011). The exact incidence of divided into three groups (15–20, 21–50, 51–67
intestinal malrotation is thus still difficult to years). Radiological diagnostics
determine. It was earlier described to be To establish the degree of malrotation, the
approximately 0.2 % (Stewart et al. 1976; radiologist identified the position of the
Donnellan and Kimura 1996; Clark and Oldham duodenum and the proximal small bowel, the
2002), but an incidence up to 1 % has been location of the caecum and the orientation of the
reported (Adams and Stanton 2014). Improved mesenteric vessels using intravenous, per oral
radiological facilities, including multi-detector as well as intrarectal contrast (triple-contrast).
CT-scans, provide new possibilities to identify Twisting of the mesentery of the small bowel,
anatomical aberrations. the “whirlpool-sign”, typical for a volvulus was
noted. This evaluation was also re-scrutinized
During a 12-year period, we have treated 39
and confirmed independently by one dedicated
consecutive cases of adult malrotation at the
radiologist. Surgery
Karolinska University Hospital, Huddinge. The
aim of this study was to increase knowledge Symptomatic malrotation was treated by
concerning this diagnosis by describing corrective surgery according to the technique
symptoms, treatment and clinical outcome in originally described by Ladd. If a volvulus was
our cohort of adolescent and adult patients with present, the intestines were de-rotated in a
intestinal malrotation. counter clockwise manner and all Ladd’s bands
were carefully removed and dissected. If
Methods needed, the mesentery was broadened and the
Patients adhesions surrounding the mesenteric vessels
Thirty-nine patients, 22 females and 17 males, dissected in order to avoid future recurrence of
aged between 15 and 67 years, were diagnosed volvulus. When the dissection was done, the
with congenital intestinal malrotation. The small bowel was placed to the right and the
patients were prospectively investigated at the colon to the left side of the abdominal cavity in
Karolinska University Hospital from 2002 to a “non-rotational” position. Two different
2013. After identification of the first patient, it surgeons registered data from medical charts on
was decided to prospectively monitor patients these surgical details independently. Follow up
treated for malrotation in order to analyze and
publish data when a reasonable number of The patients were routinely assessed 6 weeks, 6
patients had been treated. months and 12 months after surgery. After that,
occasional contact occurred if further

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complaints presented. During 2012–2013 a obstruction (Table 1). Another predominant


research nurse performed telephone interviews symptom was sensations of extreme fullness
with a semi-structured concept concerning the and discomfort after meals, sometimes followed
patients’ past and present situation and possible by nausea and vomiting, described by 29
remaining symptoms after surgery. The patients (Table 2). Thirteen of these patients
questions focused on remaining intense or were previously assessed and diagnosed with
chronic pain, postprandial nausea, vomiting and gastro-oesophageal reflux. In six cases the
constipation. Patients were also asked whether diagnosis was achieved during surgical
they regarded their general physical condition treatment focused on other conditions.
as improved to a high degree, improved with
Concomitant malformations were observed in
some reservation or without any notable
15 patients (38 %), including seven patients
improvement.
with CNS disturbances and mental retardation.
Ethical considerations Other malformations noticed were bicorn
The Regional Ethical Review Board approved uterus, vaginal atresia, double ureters, Tuberose
this study 12-06-20. Dnr 2012/957-31/3. sclerosis, Mb Hirschprung, pelvic kidney,
Cornelia de Lange syndrome and scoliosis.
Results Eight patients had a history of disease within the
Clinical data hepatobiliary and pancreatic system with a
Twelve patients presented as emergency cases, history of pancreas divisum and in four cases
whereas the remaining 27 were admitted on an pancreatitis. Six further patients had
elective basis. The most common symptom was gastrointestinal motility disturbances, verified
abdominal pain, followed by signs of intestinal by small bowel manometry and/or full thickness
specimens.
Table 1 Clinical data
Total Age <21 years Age 21–50 years Age >50
years
Sex ratio (m:f) 17:22 5:5 5:13 6:4
Number patients 39 10 18 11
Secondary malrotationa 3 1 2 0
Symptoms at diagnosis
Abdominal pain 31 (79 %) 7 (70 %) 16 (89 %) 8 (73 %)
Intestinal obstruction 5 (13 %) 3 (30 %) 1 (6 %) 1 (9 %)
Incidental diagnosis 3 (8 %) 0 (0 %) 2 (11 %) 1 (9 %)
Duration of symptoms
Hours/days 3 (8 %) 1 (10 %) 1 (6 %) 1 (9 %)
Months 7 (18 %) 1 (10 %) 1 (6 %) 5 (45 %)
Years 26 (67 %) 8 (80 %) 13 (72 %) 5 (45 %)
During childhood 19 (49 %) 6 (60 %) 10 (56 %) 3 (27 %)
Imagingb
4 2 0 2

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UGI
CT 32 7 16 9
MRI 1 0 1 0
“Whirlpool sign”c 7/33 (21 %) 1/5 (20 %) 3/13 (23 %) 3/7 (43
%)
Treatment
Conservative treatment 8 (21 %) 0 (0 %) 4 (22 %) 4 (36 %)
Ladd’s surg. procedure 31 (79 %) 10 (100 %) 14 (78 %) 7 (64 %)
Midgut volvulus without impaired 7 1 5 1
bloodflow
Midgut volvulus with impaired 8 5 1 2
blood flow
Resection small intestine 4 3 0 1
Recurrencies 5 (16 %) 2 (20 %) 2 (14 %) 2 (29 %)
a
CDH n = 1, gastroschisis n = 1, omphalocele n = 1 b “Imaging” denotes the radiologic procedure

that lead to diagnosis. Two patients had no imaging due to emergency surgery (Age ≤20 years n =

1, age 21–50 years n = 1)


c
Out of 33 patients where CT-studies were available for reviewing

Table 2 Preoperative symptoms from medical charts (n = 31) and postoperative symptoms
from a telephone interview (n = 26)
Total Age <21 years Age 21–50 years Age >50
years
Symptoms preop (one or more symptoms from medical charts, n
= 31)
Number 31 10 14 7
Fullness after meals 25 (81 %) 7 (70 %) 14 (100 %) 4 (57 %)
Pain 29 (94 %) 9 (90 %) 13 (93 %) 7 (100
%)
Constipation 13 (42 %) 4 (40 %) 7 (50 %) 2 (29 %)
Symptoms postoperative e interview, n =
(from a telephon 26)
Number 26 5 14 7
Free of symptoms 10 (38 %) 2 (40 %) 4 (29 %) 4 (57 %)

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Fullness after meals 8 (31 %) 1 (20 %) 5 (36 %) 2 (29 %)


Pain (chronic) 12 (46 %) 3 (60 %) 6 (43 %) 3 (43 %)
Pain (“malrotation-like”) 1 (4 %) 1 (20 %) 0 (0 %) 0 (0 %)
Constipation 8 (31 %) 0 (0 %) 8 (57 %) 0 (0 %)
Symptoms postoperative 16 (62 %) 3 (60 %) 10 (71 %) 3 (43 %)
Improved QoL 25 (96 %) 4 (80 %) 14 (100 %) 7 (100
%)
Radiological findings
Investigation with multi-detector computer
tomography was used in 32 cases and MRI in
one. The three cardinal radiological diagnostic
criteria were identified in 19 of the cases. In all
but one of the patients, the small bowel was
located to the right with a pathological vertical
course of the duodenum that failed to traverse the
vertebral spine. In the remaining patient, the
duodenal course initially crossed the midline to
the left, but turned back again forming a loop. In
Fig. 2 CT scan in axial position showing the inverted vessels
addition, the ascending colon had a short
attachment to the parietal left side. In 22 cases
the caecum had the expected abnormal position according to radiology, whereas the caecum in the
remaining 11 patients was located on the right
side. It was later revealed during surgery that in
all these cases the ascending colon was mobile
and not fixed to the parietal abdominal wall,
except in one case where the right flexure of
colon was shortly attached. Malposition of the
superior mesenteric artery and vein was noted

Fig. 3 Whirl pool sign where when the mesenteric of the small
intestine has been twisted

39
Fig. 1 CT scan showing inverted vessels, front view
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radiologically in 26 patients, of whom 25 had inverted vessels and one presented vessels in a
vertical position (Figs. 1, 2). A “whirlpool-sign” signifying a presence of rotation of the bowel
could be detected in seven cases (Fig. 3).

Surgery
Thirty-one patients were operated (Fig. 4). Sixteen patients had undergone previous abdominal
surgery before the Ladd procedure, with chart notifications of intestinal malrotation in 11 of the
cases. Emergency surgery was performed in 9 of 31 cases. In three patients the operation was
performed semi-urgently because of progression of abdominal complaints. One patient had a
complete mid-gut volvulus causing ischemia and necrosis of the bowel, necessitating resection of
the entire small bowel. Another seven patients exhibited signs of acute volvulus, compromising
circulation in a segment of the small bowel (2 of them >50 years). Three of these required minor
resections. There was a tendency towards an increased risk for volvulus in the younger patients
(Table 1).

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Seventeen symptomatic patients were operated on electively after a radiological diagnosis.


Appendectomy performed in all cases where the appendix still remained, in order to avoid future
diagnostic problems caused by the new position of the intestines in the abdomen. In 27 patients twisting
of the mesentery between 1 and 3 turns was described at the initiation of surgery (Fig. 5).
Eight patients chose a conservative attitude awaiting eventual more disabling symptoms, an attitude
that was more common in the older patients.

Fig. 5 Twisting of the small bowel

Postoperative clinical outcome


Twenty-seven patients had an uneventful postoperative course, leaving the hospital within a week.
Three patients had a prolonged hospital stay due to transient postoperative intestinal failure and one died
shortly postoperatively in the aftermath of midgut volvulus with total bowel necrosis. An early routine
follow-up after 6 weeks confirmed that all patients except one were relieved from episodes of intense
abdominal pain. Caretakers of the mentally disabled patients stated that their patients exhibited less
signs of distress from episodes of pain. Clinical and/or radiological signs of late recurrence appeared in
six patients requiring surgery once or twice. Surgical procedures are described in a flowchart (Fig. 6).

Mortality
Altogether five patients operated for congenital malrotation have died through the course of this study.
Four of them died due to co-morbidity not related to the malrotation syndrome or surgery. These
patients had all undergone a follow-up CT without signs of recurrence.

Telephone interview
Twenty-six patients were available for a telephone interview and were asked about their situation after
surgery. Details are shown in Table 2.

Discussion
Intestinal congenital malrotation should be recognized as a reason for abdominal pain also in adults
which has also been emphasized in a recent population based study by Coe et al. (2015). We describe a
substantial number of symptomatic patients being diagnosed in mature age often after several years of
suffering. Malrotation may present with alarming symptoms, causing life-threatening conditions which
in one case resulted in death due to short bowel syndrome. We also show that young adults have a
tendency towards more severe symptoms requiring emergency treatment. No statistical comparison has
been made with patients suffering adhesive bowel obstruction from other reasons, and thus age and
concurrent developmental disorders are the only markers identified necessitating increased awareness
when considering malrotation as cause for obstruction with severe symptoms.

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In this 12-year clinical study, the majority of the patients experienced a considerable improvement in
their general status after surgical intervention. Nehra presents an excellent retrospective study, which
includes 130 patients of all ages treated at a single institution (Nehra and Goldstein 2011). Only 30 % of
the patients were below 1 year of age, and as many as 48 % were above 18 years of age at the time of
diagnosis. They described a decreased risk for volvulus with age, which also was confirmed among
adult cases in the present study. Consequently, a conservative attitude towards surgery is more
reasonable in the older age group.
The increased recognition of intestinal malrotation in adults may be explained by the more frequent
use of abdominal CT-scan and refinements of methods that more correctly visualize variations in the
abdominal anatomy (Pickhardt and Bhalla 2002; Emanuva et al. 2011). A multi-detector CT-scan
provides the possibility of following the exact course of the duodenum as well as the position of the
small bowel and the caecum. Importantly, the orientation of the superior mesenteric vessels also
becomes assessable, sometimes with an additional depicted rotation of the mesentery of the bowel
forming a “whirlpool-sign”. This may indicate a precarious circulation of the bowel, possibly requiring
rapid surgical intervention. In the present study there were 11 patients with the ascending colon located
at an allured right abdominal quadrant. This confirms that a “normal-looking” anatomical finding of the
colon should not rule out the malrotation diagnosis as earlier reported by El-Gohary who describes
reciprocal findings in 20 % of the cases (El-Gohary et al. 2010). The entire set of radiological criteria
was demonstrated in only 18 of the investigated patients. However, all patients exhibited at least one of
the radiological criteria for malrotation, implying that a radiological investigation focusing on
appropriate radiological signs provides at least a suspected diagnosis, while waiting further assessment.
In children a contrast enema of the stomach and small intestine is usually enough to diagnose
malrotation where the displacement of duodenum is clearly shown. In adults, where other reasons for
intestinal obstruction are more frequent, a more detailed imaging including exact criteria prior to surgery
is valuable.

Fig. 6 Flowchart over the procedure for the patients

One third of the patients were operated as emergencies, compared to the higher incidence of 75 %
reported in pediatric series (El-Gohary et al. 2010). Many patients had ongoing abdominal discomforts
since childhood, while others encountered a relatively sudden onset of symptoms leading to chronic

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episodes of abdominal pain. A considerable proportion of the patients in this series had reached a high
age before being informed of their abnormality. Gastroenterologists and surgeons treating adults
probably put less emphasis on the possibility of a congenital malformation causing the abdominal
symptoms (Nehra and Goldstein 2011; Nagdeve et al. 2012).
Intestinal malrotation may have a “syndromal” appearance and is often accompanied by other
anomalies (30– 80 %), including developmental disorders of the CNS (Penco et al. 2007; Nagdeve et al.
2012). It is important to have a vigilant strategy for malrotation when investigating abdominal
complaints in mentally disabled patients who lack the possibility to describe their symptoms. The
comorbidity caused by these concurrent disorders may be one reason for the high mortality during
follow up (5/39), since only one patient died from complications after surgery in terms of short bowel
after resection of ischemic intestine.
In pediatric reports, the recurrence rate after Ladd’s procedure is considered low with a reported
incidence between 2 and 7 % (El-Gohary et al. 2010; Freitz and Vos 1997). The higher recurrence rate
reported here is partly explained by a learning curve among the involved surgeons, but more long-
lasting preoperative symptoms may also add to the complexity of surgical problems. Interestingly, it has
been shown that also children operated later during childhood has a higher incidence of reoperation
(Durkin et al. 2008). Chronic inflammatory changes in the intestinal wall may have affected the outcome
of the Ladd’s procedure and may influence on postoperative pain.
Seven patients with a radiological malrotation diagnosis have not yet undergone surgery, claiming that
they currently experience only mild symptoms and wish for a conservative approach. Today, many
authors advocate surgical correction of malrotation due to the difficulty in predicting who will be striked
by torsion of the midgut, bringing an urgent, life-threatening condition in the future. Furthermore, we
cannot be certain that patients without complaints are truly free from symptoms (Raitio et al. 2015;
Moldrem et al. 2008).

Conclusion
Intestinal malrotation shall be regarded as a malformation affecting all age groups since it is obviously
more common in the adult population than earlier anticipated. A properly performed contrast enhanced
computer tomography reveals the malformation and enables surgical treatment and relieve of symptom
also in adults with a history of long-periods of abdominal complaints. In addition, and most importantly,
acute obstruction with volvulus occurs in all ages and needs emergency surgery.
Authors’ contributions
BH contributed with idea, surgery, data processing, manuscript and manuscript revision. KS contributed
with data processing and manuscript. TP contributed with radiology, data processing and manuscript.
UG contributed with idea, surgery, data processing, manuscript and manuscript revision. MM
contributed with telephone interviews and data processing. AN contributed with data processing,
manuscript and manuscript revision. KS contributed with idea, surgery, data processing, manuscript and
manuscript revision. All authors read and approved the final manuscript.
Author details
1
Department of Clinical Sciences, Danderyd Hospital, Stockholm, Sweden. 2 Department of Surgery,
Ersta Hospital, Stockholm, Sweden. 3 Department of Clinical Intervention and Technology, CLINTEC,
Karolinska Institutet, Stockholm, Sweden. 4 Department of Gastrointestinal Surgery, Karolinska
University Hospital, Stockholm, Sweden. 5 Department of Women’s and Children’s Health, and Center
for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden. 6 Unit of Paediatric Surgery, Astrid
Lindgren Children Hospital, Stockholm, Sweden. 7 Department of Radiology, Karolinska University
Hospital, Stockholm, Sweden. 8 Department of Surgical and Perioperative Sciences, Umeå University
Hospital, 901 87 Umeå, Sweden.

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Acknowledgements
The Swedish Research Council, the Foundation Frimurare Barnhuset Stockholm, the Stockholm City
Council, the Swedish Society for Medical Research and Karolinska Institutet supported this work.
Competing interests
The authors declare that they have no competing interests.
Received: 10 November 2015 Accepted: 15 February 2016

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APPENDIX

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