Keywords Amyand's - appendicitis - inguinal hernia - neonate
Introduction
Amyand's hernia is defined as an inguinal herniation of a normal, perforated, or acutely
inflamed appendix. It was first described by Claudius Amyand in 1735, a French born
surgeon working at St George's Hospital in London. On December 6, 1735, he performed
the first recorded successful appendicectomy in an 11-year-old boy who had an inguinal
hernia combined with an acutely inflamed appendix.[1 ] Amyand's hernia is a relatively rare presentation of appendicitis, with an incidence
of less than 1% of all inguinal hernias according to the literature.[2 ] Although many presentations are described in the literature, the most common is
one of an incarcerated or partially reducible inguinal hernia. We report a case of
Amyand's hernia with appendicitis presenting only with inguinoscrotal edema and erythema
in an almost 3-week-old baby.
Case Report
Our patient is a 19-day-old, full-term baby, born in good general condition who presented
to our emergency department with 1-day history of inguinoscrotal erythema and persistent
irritability. The patient was struggling with achieving full feeds, although not vomiting
and managing to open bowels normally. Examination revealed a distended, soft, nontender
abdomen and bilateral scrotal edema along with moderate inguinoscrotal erythema and
tenderness ([Fig. 1 ]). Examination of the inguinal region revealed no evidence of irreducible hernia
or inguinal thickening on either side. Laboratory investigations were normal apart
from increased C-reactive protein levels. Due to the unclear signs, we resorted to
imaging for answers. An abdominal X-ray showed some dilated bowel loops but no evidence
of intestinal obstruction or aeration in the scrotal compartments ([Fig. 2 ]). An inguinoscrotal ultrasound (US) with Doppler did not show any evidence of incarcerated
bowel loops or testicular torsion, although pointing to increased vascularity and
echogenicity of the right testis. The patient was, therefore, admitted on the working
diagnosis of epididymo-orchitis and started on intravenous antibiotics.
Fig. 1 Neonate presenting with inguinoscrotal erythema and edema.
Fig. 2 Preoperative abdominal X-ray.
Twelve hours later, our patient exhibited bilious vomiting and worsening inguinoscrotal
edema. Abdominal examination showed signs of generalized rigidity and guarding. With
no clinical evidence of an incarcerated hernia, the exclusion of testicular torsion
by Doppler, and the development of an acute abdomen, we opted for an exploratory laparotomy
through a right upper quadrant incision. This allowed us to be able to exclude any
source of intra-abdominal sepsis while at the same time providing adequate surgical
access to deal with the possibility of a missed irreducible hernia. The bowel was
inspected and the appendix was found to be incarcerated in the right internal ring
with the evidence of inflammation and suppuration ([Fig. 3 ]). The appendix was gently reduced. Appendectomy and closure of the internal ring
with a purse-string suture were done. Postoperatively, feeding was started through
the nasogastric tube after 8 hours that was tolerated well and built up gradually.
Antibiotics were discontinued in 5 days after improvement in inflammatory markers
and resolution of the inguinoscrotal erythema. No other clinical evidence of associated
comorbidities common with neonatal appendicitis was illustrated. The patient was discharged
home and had an unremarkable 3-month follow-up.
Fig. 3 Intraoperative finding showing inflamed appendix.
Discussion
Neonatal appendicitis is a rare condition associated with comorbidities such as prematurity
or other diagnoses like Hirschsprung's disease, meconium plug syndrome, and cystic
fibrosis.[3 ] Inflamed appendix within the hernial sac, on the other hand, accounts for one-third
of the neonatal appendicitis described in the literature. Variable presentations ranging
from irritability with signs of irreducible hernia to systemic signs of sepsis and
shock.[4 ]
Although the exact pathophysiology of this entity is unknown, one of the theories
is the presence of a congenital band extending from the appendix to the right testis
going through the inguinal canal.[5 ] A link between Amyand's hernia and appendicitis has also been debated. Some authors
suggest that it is an incidental finding, while others arguing that the appendix becomes
entrapped due to the contraction of abdominal muscles, which leads to compromised
blood flow with consequent inflammation and bacterial overgrowth.[6 ]
Most authors consider diagnosis of this condition to be intricate. This is because
of the nonspecific manifestations, as in this case, where no obvious signs of irreducible
hernia or intestinal obstruction were found. Cankorkmaz et al described a series of
12 cases, where only one was diagnosed preoperatively by US. All patients presented
with an inguinal nonreducible mass and only a few exhibited fever, vomiting, or intestinal
obstruction symptoms. These patients were taken to theater with a working diagnosis
of incarcerated hernia. Intraoperatively they found six cases of Amyand's hernia with
inflamed appendices: two normal appendices and four of equivocal nature.[7 ] Another larger case series described 46 patients diagnosed with Amyand's hernia,
most of which after the incidental finding of the appendix in the hernial sac.[8 ] The differential diagnosis for a painful mass in the inguinal area may include incarcerated
hernia, inguinal lymphadenitis, epididymo-orchitis, testicular torsion, or soft-tissue
infection. Making the correct diagnosis preoperatively requires awareness of this
rare entity by the clinician, as well as physical examination findings consistent
with a hernia mass without obstructive signs.[6 ] Utilizing imaging like inguinoscrotal US was helpful in achieving a preoperative
diagnosis in few reported cases.[9 ] In one series, US scan was successful in diagnosing 9 out of 12 patients by revealing
a blind ending intestinal loop.[10 ] At the same time, Color Doppler US can be very useful in excluding testicular torsion
from the differential diagnosis with an 80 to 98% sensitivity and an accuracy of 97%.[11 ] Moreover, computed tomography scan was able to visualize an inflamed appendix extending
into the inguinal canal of a 77-year-old female.[2 ] Whether or not it is worthwhile exposing a neonate to such radiation to arrive at
a diagnosis will be subject to controversy. Although no mortality is reported in the
literature, delayed diagnosis and surgical intervention meant that on surgical exploration,
many appendices were found to be perforated. Scrotal abscess, peritonitis, and a case
progressing to septic shock are other reported complications.[4 ]
Due to the majority of cases being incidentally found on surgical exploration, treatment
is mainly by primary hernia repair and appendectomy through an inguinal incision.
In cases with unclear diagnosis, reports of laparotomy as a primary incision, combined
abdominal and inguinal incisions, and even scrotal explorations were described.[12 ] Some authors advocate against removing the appendix when found incidentally without
any obvious signs of inflammation.[8 ] Laparoscopic approach is now gaining more popularity in such cases, as it allows
increased visualization of the abdominal cavity and can be diagnostic and therapeutic.[12 ] However, utilizing this approach would be more likely if the patient's clinical
condition is stable.
Diagnosing this rare entity requires a high index of suspicion by clinicians and when
imaging is inconclusive, resorting to diagnostic laparoscopy should be considered
to avoid laparotomy or multiple surgical incisions. While a tender, irreducible inguinal
mass is the most common presentation, we report a case of Amyand's hernia with concurrent
appendicitis presenting only with inguinoscrotal erythema and edema.
