Heineke-Mikulicz plasty as a Treatment for Proximal Type I Jejunal Atresia: Presentation of 4 Cases

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Heineke-Mikulicz plasty as a Treatment for Proximal Type I Jejunal Atresia: Presentation of 4 Cases

https://doi.org/10.21203/rs.3.rs-5075406/v1

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Purpose: Proximal jejunal atresia is a congenital obstruction of the jejunum, typically located within the first 15 cm from the ligament of Treitz. This condition prevents the passage of intestinal contents, leading to symptoms such as bilious vomiting, abdominal distension, and a lack of meconium passage in newborns. The standard treatment for proximal jejunal atresia is primary anastomosis, which involves resecting the atretic segment and directly joining the proximal and distal ends of the intestine. However, this procedure carries risks due to its proximity to the angle of Treitz, leading to the development of alternative techniques to manage this pathology.

Methods: We present four patients with proximal type I jejunal atresia successfully treated with Heineke–Mikulicz plasty from August 2023 to May 2024.

Results: The patients experienced a favorable postoperative course,with an average time to full oral intake ranging from 9--25 days, with a median of 13 days.

Conclusion: This technique avoids complications such as high-output stomas, anastomotic leaks, dehiscence, and repeated surgical reinterventions. We propose this technique as an alternative treatment for this condition. To our knowledge, this is the first reported case series in Mexico that uses this approach.

Pediatrics

Gastrointestinal Surgery

General Surgery

jejunal atresia

proximal jejunal atresia

Heineke-Mikulicz

Mikulicz

congenital obstruction.

Neonatal intestinal obstructions are a common cause of neonatal surgical pathology and are characterized by distension, bilious vomiting, and the absence of bowel movements within the first 24 hours. These obstructions can be functional or mechanical, with mechanical causes being either intraluminal or extraluminal, the latter including intestinal atresias.
Intestinal atresia is a congenital anomaly characterized by complete occlusion of the intestinal lumen, resulting in neonatal intestinal obstruction. This term encompasses various forms of atresia that can occur in different parts of the intestine, including the duodenum, jejunum, ileum, and, rarely, the colon. (1)
Jejunoileal atresia is defined as complete occlusion of the intestinal lumen, which can be simple or multiple, and may occur anywhere from the ligament of Treitz to the jejunoileal junction. This type of atresia is one of the most common causes of neonatal intestinal obstruction and requires immediate surgical intervention to prevent mortality and morbidity. (2)
Jejunal atresia (JA) affects approximately 1 in every 12,000 live births, with a prevalence of 0.7 per 10,000 live births. It is associated with a variety of congenital malformations, the most common being gastrointestinal, cystic fibrosis, and genitourinary and neurological malformations. The identification and management of associated malformations are crucial for optimizing the clinical outcomes of these patients. (3-7)

Etiology:
During intestinal formation, the lumen becomes occluded and recanalizes to form the digestive tube. If one of these mechanisms fails, luminal obstructions, such as diaphragms, can occur, leading to atresia. These atresias can also arise due to vascular insults, resulting in a loss of continuity in the digestive tract, either in one or multiple portions, depending on the type of atresia. (1)

(Figure 1)

Clinical presentation:
Patients with JA typically present with characteristic signs of neonatal obstruction, including abdominal distension (which may be absent in more proximal portions), bilious vomiting, and the absence of bowel movements within the first 24 hours of life. Initially, there may be passage of meconium corresponding to the more distal portions of the intestine, followed by an absence of bowel movements. Indirect hyperbilirubinemia may also be present. Patients may also experience growth restrictions. (1, 8, 9)
A prenatal diagnosis can be made via obstetric ultrasound, although its accuracy varies, with detection rates ranging from 10% to 100% and an overall predictive rate of 50.6%. Typical findings include dilated intestinal loops and polyhydramnios. (10) Postnatal diagnosis can be supported by a simple abdominal X-ray showing dilated intestinal loops with air‒fluid levels corresponding to the site proximal to the obstruction. (11, 12) Postnatal ultrasound helps to better define the anatomy and confirm the presence of dilated proximal intestines and microcolon. (13, 14) A contrast enema is used to identify a microcolon, which is characteristic of small bowel atresia, including jejunal atresia. (13) Additional studies, such as sweat tests, may be conducted to rule out cystic fibrosis, which can be associated with jejunoileal atresia, or cardiac and renal ultrasounds to detect associated anomalies, especially in cases of multiple congenital anomalies. (11)

Classification:
The classification of JA, which is based on the proposal by Grosfeld and widely accepted in the medical literature, includes four main subtypes, each with distinctive morphological and clinical features:

Type I: The intestinal lumen is obstructed by a mucosal membrane, but the continuity of the intestinal wall is intact. This type is the least severe and has a good postoperative prognosis, which is the focus of this study.
Type II: There is a complete interruption of the intestinal lumen, with a fibrous cord connecting the proximal and distal segments. Vascularization through the fibrous cord is present, facilitating surgical anastomosis.
Type IIIa: This type is similar to Type II but without a fibrous connection between the proximal and distal segments. This type involves a complete disruption of intestinal continuity and vascularization, which can complicate surgery and patient prognosis.
Type IIIB (apple peel or Christmas tree): This type is characterized by proximal atresia with a distal segment spiraling around its vascular pedicle, terminating in the cecum. This type is associated with a greater risk of short bowel syndrome and intestinal failure due to significant loss of intestinal length.
Type IV: Multiple areas of atresia along the intestine, resulting in a "string of sausages" appearance. This type is the most complex and is associated with high morbidity and mortality due to the extent of the disease. (15)

Treatment:
The treatment of high JA, which is subjectively defined as occurring near the angle of Treitz, is based on surgical correction to restore continuity and intestinal function in the newborn. Various strategies are available to optimize outcomes and minimize complications from failed anastomosis or proximal jejunostomy. (16, 17)
Primary anastomosis is the preferred approach for most cases of high jejunal atresia, involving resection of the atretic segment and direct anastomosis of the proximal and distal intestinal ends. (18) Another method used for this pathology is intestinal plication of the dilated proximal intestine before primary anastomosis. Compared with primary anastomosis alone, bilateral plication during jejunojejunal anastomosis helps control the intestinal caliber and prevent kinking at the anastomosis site, thus reducing postoperative complications and resulting in faster recovery of intestinal function and a shorter duration of total parenteral nutrition (TPN). (19, 20) Serial transverse enteroplasty (STEP) is used to lengthen the intestine and improve function in neonates with proximal jejunal atresia, especially in patients with short residual bowels. (21) Duodenojejunostomy with lateral duodenectomy may be useful for atresias near the ligament of Treitz, involving resection of the dilated proximal jejunum and anastomosis of the residual duodenum to a spatulated distal jejunum. Although this is a more radical procedure, it has shown good results in difficult-to-treat cases. (22)
Several strategies have been developed to treat proximal JA, as these present a high mortality risk, especially for those with atresias located 15 cm or more proximal to the angle of Treitz. We chose to define high JA values in this series as those located up to 30 cm from the angle of Treitz, as this is not clearly defined in the literature. (23)

Heineke‒Mikulicz procedure for jejunal atresia:
In 2005, Yamataka et al. described the case of a 4-day-old patient with clinical and imaging findings suggestive of JA. During surgery, they encountered proximal type I jejunal atresia 5 cm from the angle of Treitz, which was treated with longitudinal jejunotomy over the site of stenosis. They found a mucosal membrane, which was resected, and the jejunotomy was closed transversely, following the Heineke‒Mikulicz principle. Owing to the significant difference in intestinal caliber, proximal plication of the affected intestine was performed. (19)
The Heineke‒Mikulicz principle is a surgical technique used to treat stenosis or narrowing of tubular structures, such as the intestine or urethra. This principle involves making a longitudinal incision over the stenosis and then closing the incision transversely. This method allows for enlargement of the lumen diameter without significantly shortening its length. (24)
In this paper, we describe the results obtained with this technique in a series of four patients treated from August 2023 to May 2024.
We propose Heineke‒Mikulicz plasty with membrane resection as an effective option for treating proximal type I jejunal atresia, which offers good outcomes in terms of reducing morbidity and achieving early bowel movement recovery.

We describe the clinical case of four full-term newborn patients with normo-evolving pregnancies and no significant medical history. They were referred for pediatric surgery for evaluation due to high neonatal intestinal obstruction. Exploratory laparotomy was performed, revealing the proximal JA.

Clinical presentation:

The patients presented with abdominal distension, oral feeding intolerance, bilious vomiting, and an absence of bowel movements since birth, with no improvement despite rectal stimulation, gastric decompression via an orogastric tube, and intestinal rest with fasting and nutritional support through parenteral nutrition (PN). Surgical intervention was decided between the first and fifth days of life, with a median surgery on the second day.

A complete blood test revealed no abnormalities, normal coagulation times for age, and a normal biochemical profile.

Imaging:

Anteroposterior abdominal X-ray was performed, which revealed stomach distension and dilation of the proximal bowel loops, with clear signs of high intestinal obstruction. No further studies were conducted because of the unavailability of fluoroscopy in the hospital.

Treatment:

All four patients were initially managed with close monitoring in the neonatal intensive care unit, respiratory support with supplemental oxygen if needed due to restrictive patterns from abdominal distension, antibiotic prophylaxis due to the risk of bacterial translocation, gastric decompression via an orogastric tube, and intestinal rest with parenteral nutrition for nutritional support. Once the patients were stable and in suitable condition for surgery, an exploratory laparotomy was performed through a supraumbilical midline incision. The site of obstruction was identified as a proximal type I JA.

A longitudinal jejunotomy was performed at the site of stenosis, located between the dilated proximal jejunum and the hypotrophic distal segment. A transverse mucosal membrane was found and completely resected, followed by transverse closure of the jejunotomy with simple absorbable sutures (5 − 0), following the Heineke‒Mikulicz principle. In one of the patients, proximal remodeling with plication was performed using simple absorbable sutures (5 − 0). Adequate passage of air and intestinal contents was confirmed at the site of stenosis, ensuring proper permeability and sealing of the plasty. The incision was closed in layers.

Follow-up:

Postoperatively, patients were closely monitored with gastric decompression via an orogastric tube, intestinal rest, and parenteral nutrition. One patient developed intestinal obstruction requiring surgical exploration and adhesiolysis. The patients were monitored until the clearance of the orogastric drainage tube, which was removed once intestinal function was restored, with the presence of peristalsis and bowel movements. In two patients, a contrast study was performed to confirm proper intestinal transit through the plasty site and the rest of the bowel. Oral feeding was gradually reintroduced via a conservative approach because of the significant difference in caliber at the site of the operation. All patients tolerated oral feeding, achieving 100% intake between 17 and 53 days of extrauterine life. The patients were discharged with outpatient follow-up.

All the information and calculations of the means were analyzed with Microsoft Office Excel 365.

The ethics committee of the Hospital Regional de Alta Especialidad Materno Infantil approved this study. Informed consent to publish the case report was not obtained. This research does not contain any personal information that could lead to the identification of patients.

Compared with other types of jejunoileal atresias, proximal JA is a complex condition that requires individualized treatment. Although not considered a complex intestinal atresia, it requires special management owing to the risks associated with this anatomical level. (5,16,25)

We treated four patients with an average gestational age of 34 weeks and an average weight of 2100 g; these patients were younger and smaller than those reported by other authors (26). These patients had type I intestinal atresia, with an average distance of 13.75 cm from the Treitz ligament, classifying them as proximal jejunal atresia cases. We performed a Heineke‒Mikulicz procedure to avoid previously described complications. In one patient, a Penrose drain was placed and removed when oral feeding began. In addition, proximal plication was performed to reduce the caliber of the proximal limb, as described by other authors, to improve oral tolerance postoperatively in patients with significant caliber discrepancies. (19,20)

One of our patients developed superior vena cava thrombosis during hospitalization, complicating their recovery, and another presented with intestinal obstruction treated with adhesiolysis. All four patients had favorable outcomes, were referred to a tertiary hospital with the necessary means for treatment, and had appropriate prenatal diagnoses. Three patients were preterm, all required mechanical ventilation, and central venous lines were placed, which could increase intra- and postoperative complications and prolong hospitalization due to the invasive nature of these measures.

All patients were discharged home, tolerating 100% of the recommended intake for their age, at an average of 37.5 days of life, which is comparable to other series reporting an average of 32.6 days of extrauterine life, with no postoperative complications noted during outpatient follow-up. (26)

(Table 3)

Proximal JA type I is a rare condition that requires a different approach than more common types of jejunoileal atresia. Various techniques have been described to avoid associated complications, such as high-output jejunostomies, anastomotic dehiscence, and surgical reinterventions.

We present a case series of four patients who were successfully treated with Heineke-Mikulicz plasty without requiring primary resection and end-to-end anastomosis or diversion. All four patients were successfully discharged but could have benefited from techniques described in the literature to address the significant caliber discrepancy between the dilated proximal intestine and the hypotrophic distal intestine, as we only performed this procedure on one patient. Further comparative studies with larger samples are necessary to objectively confirm the efficacy of this technique. On the basis of our experience, we propose this technique as an alternative treatment for proximal JA type I in Mexico because of its replicability and accessibility to the required resources. To our knowledge, this is the first case series reported in Mexico that uses this technique.

Jejunal atresia (JA)

Acknowledgments

The authors would like to express their gratitude to Eduardo de la Rosa-Avila, an artist and graphic designer, for his assistance in original image design.

Authorship contribution statement

All the authors attest that they meet the current ICMJE criteria.

Eduardo de la Rosa-Bustamante: Investigation (lead), Conceptualization (supporting), Methodology (equal), Data curation(lead), Formal analysis (lead), Methodology (equal), Validation (lead), Visualization (lead), Writing – original draft (lead), Writing – review & editing(equal)

Gerardo Luna-Lopez: Conceptualization (lead), Methodology (equal), Project administration (lead), Writing – review & editing (equal) Supervision (lead)

Hugo Gonzalez-García: Methodology (equal), Writing – review & editing (equal), Project administration (supporting), Resources (supporting), supervision (supporting)

Jorge Colin-Garnica: Formal analysis (supporting), Investigation (supporting), Methodology (equal), Visualization Writing – review & editing (equal), Validation (supporting), Visualization (supporting)

Arturo Guillen-Cardenas: Formal analysis (supporting), Visualization (supporting), Writing – review & editing (supporting), Validation (supporting), Visualization (supporting)

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Financial support statement:

No financial support or funding was granted for this manuscript.

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Table 1

Patients characteristics
Type of Defect	Proximal Jejunal Atresia (n=4)
Prenatal Diagnosis	4 (100%)
Sex (M: F)	2:2
Gestational Age in Weeks, Mean (Range)	34 (32-37)
Birth Weight in Grams, Mean (Range)	2100 (1600-2630)
Premature Infants	3 (75%)
Mechanical Ventilation	4 (100%)
Central Venous Line	4 (100%)

Table 2

Surgical Characteristics
Defect Type	Proximal Jejunal Atresia (n=4)
Age at Surgery (days), Mean (Range)	2.2 (1-5)
Distance from Atresia to Treitz Angle (cm), Mean (Range)	13.75 (5-20)
Drainage	1 (25%)
Proximal Plication	1 (25%)

Table 3

Postoperative Period
Defect Type	Proximal Jejunal Atresia (n=4)
Complications	1 (25%)
Intestinal Obstruction	1 (25%)
Surgical Reintervention	1 (25%)
Adhesiolysis	1 (25%)
Contrast administration Before Oral Intake	2 (50%)
Time to Resume Oral Intake (days), Mean (Range)	18.75 (8-35)
Hospital Stay Duration (days), Mean (Range)	37.5 (17-53)

The authors declare no competing interests.

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Heineke-Mikulicz plasty as a Treatment for Proximal Type I Jejunal Atresia: Presentation of 4 Cases

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