Congenital Esophageal Stenosis:
Therapeutic Strategy Based on Etiology
By Shigeru Takamizawa, Chikara Tsugawa, Naruaki Mouri, Shiiki Satoh, Kimio Kanegawa,
Eiji Nishijima, and Toshihiro Muraji
Kobe, Japan
Background/Purpose:
The diagnosis and treatment of con-
genital esophageal stenosis (CES) can be a vexing clinical
problem. This study aims at determining the ideal therapeu-
ticstrategy for the management of CES.
Methods:
Medical records of patients with CES were re-
viewed retrospectively with regard to diagnostic method,
therapy, and outcome.
Results:
During the last 29 years, 36 patients, aged 1 month
to 9 years, were found to have CES. Diagnosis was made by
fluoroscopy, esophagoscopy, endoscopic ultrasonography
(EUS), and pathologicexamination. Of the 36 patients, 15
had tracheobronchial remnants (TBR), 13 had fibromuscular
stenosis (FMS), 5 had membranous stenosis (MS), and 3 had
multiple stenoses (MPS). EUS was diagnosticin distinguish-
ing TBR from FMS. All patients with TBR underwent opera-
tive repair in which 13 patients had resection and anastomo-
sis with or without preoperative dilation. The 14th patient
had enucleation and the 15th underwent myotomy. Postop-
erative dilation was needed 2.2 times for 2.1 months on
average in this group of patients. Ten patients with FMS
were treated by dilation only (a mean of 4.7 dilations was
required for a duration of 2.6 years), whereas 3 patients
underwent open surgical intervention. Most patients with
MS or MPS were treated by dilation (with or without endo-
scopic resection of the membranes), whereas 2 patients had
surgical intervention. All patients in this study are doing well
after a mean follow-up period of 5.3 years (range, 0.5 to 25
years) without any further operative interventions.
Conclusions:
If the etiologicdiagnosis of CES is unclear, EUS
is useful in distinguishing TBR from FMS. This distinction is
critical, because patients with TBR should undergo surgical
resection, whereas most cases of FMS, MS, and MPS can be
treated with esophageal dilation alone.
J Pediatr Surg 37:197-201. Copyright
©
2002 by W.B.
Saunders Company.
INDEX WORDS: Esophageal stenosis, congenital, therapeu-
ticstrategy.
C
ONGENITAL ESOPHAGEAL STENOSIS (CES)
is a rare clinical condition of childhood. The de-
finitive preoperative diagnosis often is difficult to make,
and the ideal therapeutic strategy remains controversial.
We reviewed retrospectively the medical charts of pa-
tients with congenital esophageal stenosis, with the in-
tention of defining the ideal therapeutic strategy for the
management of CES.
MATERIALS AND METHODS
Patient Characteristics
From 1972 to 2001, 36 patients had CES diagnosed at Kobe
Children’s Hospital. The patients ranged in age from 1 month to 9
years, and the average age at diagnosis was 1.8 years. Symptoms
started at 7.4 months of age on average. There were 21 boys and 15
girls in this series, and associated anomalies were found in 17 patients
(47%). These were esophageal atresia with distal tracheoesophageal
fistula (EA/TEF; n ϭ 13), tracheomalacia with or without subglottic
stenosis (n ϭ 2), esophageal hiatal hernia (n ϭ 2), imperforate anus
(rectourethral fistula; n ϭ 1), ventricular septal defect (n ϭ 1), vesi-
coureteral reflux (n ϭ 1), Goldenhar syndrome (n ϭ 1), and Down’s
syndrome (n ϭ 2). Of the 36 patients with CES, the type of stenosis
was classified: (1) tracheobronchial remnants (TBR, n ϭ 15); (2)
fibromuscular stenosis (FMS, n ϭ 13); (3) membranous stenosis (MS,
n ϭ 5); and (4) multiple stenoses (MPS, n ϭ 3). Twenty-nine patients
(81%) presented with dysphagia or vomiting, and 3 patients (8%)
presented with respiratory distress. Four patients (11%) had CES
diagnosed on follow-up esophagram after repair of esophageal atresia
before clinical symptoms developed. In all patients with CES and
EA/TEF repair, the narrowing segment caused by CES is separate from
the site of esophageal anastomosis.
Diagnostic Methods
All patients suspected of having CES underwent barium esophagram
to confirm the diagnosis of CES (Fig 1). In this study, all but one
patient received a correct diagnosis for the type of stenosis by preop-
From the Departments of Surgery and Radiology, Kobe Children’s
Hospital, Kobe, Japan and Yamanashi Medical University, Yamanashi,
Japan.
Presented at the 48th Annual International Congress of the British
Association of Paediatric Surgeons, London, England, July 18-21,
2001.
Address reprint requests to Shigeru Takamizawa, MD, Department
of Surgery, Kobe Children’s Hospital, 1-1-1, Takakuradai, Suma-ku,
Kobe, 654-0081, Japan.
Copyright © 2002 by W.B. Saunders Company
0022-3468/02/3702-0011$35.00/0
doi:10.1053/jpsu.2002.30254
197
Journal of PediatricSurgery,
Vol 37, No 2 (February), 2002: pp 197-201